MedWorm: Atrioventricular Septal Defect

Aberrant right subclavian artery: incidence and correlation with other markers of Down syndrome in second‐trimester fetuses

Ultrasound in Obstetrics and Gynecology — Fri, 27 Jan 2012 08:35:16 +0100

ConclusionsThis represents the largest Down syndrome population assessed for ARSA. In this series, the incidence of ARSA was 25%, lower than previously reported in much smaller series. Its presence did not correlate with the presence of any other marker or major anomaly, including heart defects. Copyright © 2012 ISUOG. Published by John Wiley & Sons, Ltd. (Source: Ultrasound in Obstetrics and Gynecology)

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Risk Factor Analysis for Second-Stage Palliation of Single Ventricle Anatomy [ORIGINAL ARTICLES: PEDIATRIC CARDIAC]

The Annals of Thoracic Surgery — Tue, 24 Jan 2012 05:00:00 +0100

Conclusions Second-stage palliation can be performed at low risk for patients with left ventricular dominance, but significant risk remains for patients with left ventricular hypoplasia and unbalanced atrioventricular septal defect. Atrioventricular valve insufficiency is a persistent problem that has not been neutralized by repair strategies. (Source: The Annals of Thoracic Surgery)

Evolution of Mitral Valve Replacement in Children: A 40-Year Experience [ORIGINAL ARTICLES: PEDIATRIC CARDIAC]

The Annals of Thoracic Surgery — Tue, 24 Jan 2012 05:00:00 +0100

Conclusions Pediatric MVR can be performed with low initial mortality but should be reserved for medical and reconstruction failure because reoperation, valve-related complications, and late mortality are high. Bileaflet prostheses larger than 23 mm have the lowest reoperation risk. Ross MVR may offer select patients a durable tissue valve without lifelong anticoagulation and its associated complications. (Source: The Annals of Thoracic Surgery)

Long-Term Results of Reoperation for Left Atrioventricular Valve Regurgitation After Correction of Atrioventricular Septal Defects.

The Annals of Thoracic Surgery — Fri, 20 Jan 2012 05:00:00 +0100

CONCLUSIONS: After previous correction of AVSD, LAVVR can usually be corrected by valve repair. A very dysplastic valve may necessitate replacement. Overall survival is higher after repair than after replacement. In general, overall survival of patients reoperated on for LAVVR is favorable. The overall mortality rate after primary repair of AVSD is explained only for a small part by mortality after reoperation for LAVVR. PMID: 22265201 [PubMed - as supplied by publisher] (Source: The Annals of Thoracic Surgery)

Passive peritoneal drainage improves fluid balance after surgery for congenital heart disease [CONGENITAL]

European Journal of Cardio-Thoracic Surgery — Wed, 18 Jan 2012 05:00:00 +0100

CONCLUSIONS Passive PD is safe and promotes negative fluid balance after repair of complete AVSD without adversely affecting intravascular volume. (Source: European Journal of Cardio-Thoracic Surgery)

Spontaneous Closure of the Ventricular Septal Defect in Fetuses and Neonates With Complete Atrioventricular Septal Defects (AVSD)

Journal of Surgical Research — Sat, 14 Jan 2012 22:28:53 +0100

(Source: Journal of Surgical Research)

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Surgical Management of Left Ventricular Outflow Tract Obstruction

Journal of Cardiac Surgery — Mon, 12 Dec 2011 05:00:00 +0100

Conclusions: Utilizing a multitude of operative strategies for surgery of the LVOT results in favorable early and midterm outcomes. Residual LVOTO and original cardiac diagnosis are associated with increased reoperation risk. Release of the fibrous trigones decreases reoperation risk in patients with complex LVOTO. (J Card Surg 2011;**:1‐9) (Source: Journal of Cardiac Surgery)

Persistent left superior vena cava: Experience of a tertiary health‐care center

Pediatrics International — Thu, 01 Dec 2011 05:00:00 +0100

Conclusion: Transthoracic echocardiography usually visualizes dilated coronary sinus in association with PLSVC. However, SVC injection should be performed in patients undergoing angiography so that morbidity and mortality related with persistent left superior vena cava can be avoided during cardiovascular surgery. (Source: Pediatrics International)

Supra-Annular Mitral Valve Replacement in Children [ORIGINAL ARTICLES: PEDIATRIC CARDIAC]

The Annals of Thoracic Surgery — Wed, 23 Nov 2011 05:00:00 +0100

Conclusions Supra-annular MVR is useful for children with a small annulus. Operative survival is good with infrequent heart block. Complications are common, including redo MVR and need for left ventricular outflow tract obstruction relief. Pulmonary vein stenosis is a marker for poor outcome; all patients without pulmonary vein stenosis survive long term. (Source: The Annals of Thoracic Surgery)

BMPR1A is a candidate gene for congenital heart defects associated with the recurrent 10q22q23 deletion syndrome.

European Journal of Medical Genetics — Thu, 20 Oct 2011 04:00:00 +0100

We report on a de novo intragenic deletion of the BMPR1A gene in a normally developing adolescent boy with short stature, delayed puberty, facial dysmorphism and an atrioventricular septal defect. Based on this finding, complemented with computational prioritization data and molecular evidence in literature, the critical region for CHD on 10q23 can be downsized to a single gene, BMPR1A. Although loss-of-function mutations in BMPR1A typically result in juvenile polyposis syndrome, none of the patients with the typical 10q22q23 microdeletion syndrome, comprising this gene, were reported to have juvenile polyposis thus far. We reason that, even in the absence of juvenile polyposis syndrome, sequencing and copy number analysis of BMPR1A should be considered in patients with (atrioventricular) ...

Twenty‐Five–Year survival of children with birth defects in New York State: A population‐based study

Birth Defects Research Part A: Clinical and Molecular Teratology — Fri, 30 Sep 2011 04:00:00 +0100

CONCLUSION: Using the statewide, population‐based birth defects surveillance data in New York State, the survival experience of the study cohort was examined across all survival time periods by individual birth defect of interest. Several risk factors that affect survival were identified. Birth Defects Research (Part A), 2011. © 2011 Wiley‐Liss, Inc. (Source: Birth Defects Research Part A: Clinical and Molecular Teratology)

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Surgical Interventions for Atrioventricular Septal Defect Subtypes: The Pediatric Heart Network Experience [ORIGINAL ARTICLES: PEDIATRIC CARDIAC]

The Annals of Thoracic Surgery — Wed, 28 Sep 2011 04:00:00 +0100

Conclusions AVSD subtype was significantly associated with patient characteristics and clinical status before repair and influenced age at repair. Significant postoperative LAVVR is the most common sequela, with a similar prevalence across centers 6 months after the intervention. Annuloplasty failed to decrease the postoperative prevalence of moderate or severe LAVVR at 6 months. After accounting for age at repair, AVSD subtype was not associated with postoperative LAVVR severity or growth failure at 6 months. Further investigation is needed to determine if interventional strategies specific to AVSD subtype improve surgical outcomes. (Source: The Annals of Thoracic Surgery)

Should we always plan a Fontan completion after a Kawashima procedure? [Original articles]

European Journal of Cardio-Thoracic Surgery — Mon, 26 Sep 2011 04:00:00 +0100

Conclusion: Unless some hepatic blood flow is directed to both lungs, most, if not all patients with a Kawashima procedure will ultimately develop pulmonary arteriovenous malformations. Elective non-fenestrated Fontan completion in the years following Kawashima procedure should be recommended. (Source: European Journal of Cardio-Thoracic Surgery)

Neurodevelopmental outcomes in children with Down syndrome and congenital heart defects

American Journal of Medical Genetics Part A — Mon, 19 Sep 2011 04:00:00 +0100

AbstractTrisomy 21, the chromosomal condition responsible for Down syndrome (DS, OMIM #190685), is the most common identifiable genetic cause of intellectual disability. Approximately half of all children with DS are born with a significant congenital heart defect (CHD), the most common of which is an atrioventricular septal defect (AVSD). As children with comorbid DS and CHD increasingly survive cardiac surgery, characterization of their early developmental trajectories is critical for designing early interventions to maximize individual potential. Herein, the developmental domains (cognitive, language, and motor) of children with DS and AVSD (DS + AVSD, n = 12) were compared to children with DS and a structurally normal heart (DS − CHD, n = 17) using the Bayley Scales o...

Partial anomalous pulmonary venous connection and partial atrioventricular septal defect in an adult: Report of a case

Surgery Today — Thu, 15 Sep 2011 15:53:02 +0100

Abstract A partial anomalous pulmonary venous connection (PAPVC) associated with partial atrioventricular septal defect (AVSD) is a rare combination. A 51-year-old man who experienced dyspnea on exertion was found to have a PAPVC with the middle portion of the superior vena cava and a comorbid partial AVSD. A patch diversion was successfully performed using an expanded polytetrafluoroethylene patch to create a pulmonary venous drainage route while preserving the cavoatrial junction. The postoperative course was uneventful. The patient was in good health, without arrhythmia and thromboembolism, 3 years after the operation. Content Type Journal ArticleCategory Case ReportPages 1421-1423DOI 10.1007/s00595-010-4438-2Authors Yoshimasa Seike, Department of Cardiovascular Su...

First report of right sided interrupted aortic arch type A in combination with atrioventricular septal defect and microdeletion 22q11

International Journal of Cardiology — Mon, 12 Sep 2011 04:00:00 +0100

We present the case of a newborn with the hitherto undescribed combination of right sided interrupted aortic arch (IAA) type A (Celoria and Patton, 1959 ) with complete atrioventricular septal defect (AVSD) and microdeletion 22q11. () The patient was referred to our tertiary care center in severely compromised hemodynamic state. Despite complex and untypical anatomy, diagnosis was achieved by non-invasive imaging as echocardiography and cardiac magnetic resonance (CMR) only. After stabilization using continuous dobutamine and prostaglandine E1infusion, surgical repair with direct termino-lateral anastomosis between ascending and descending aorta, as well as palliation with central pulmonary artery banding was performed at the 9th day of life without complications and an excellent result. (...

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Early Outcomes and Prognostic Factors for Left Atrioventricular Valve Reoperation After Primary Atrioventricular Septal Defect Repair

Pediatric Cardiology — Sat, 10 Sep 2011 15:41:33 +0100

This study aimed to identify early outcomes of and prognostic factors for left atrioventricular valve (LAVV) reoperation after primary atrioventricular septal defect (AVSD) repair. A retrospective cohort study was performed using the Pediatric Cardiac Care Consortium database, which included 370 patients with previously repaired AVSDs who subsequently underwent LAVV reoperation between 1982 and 2007. Of these patients, 243 underwent LAVV repair, and 127 underwent LAVV replacement. Median time to first reoperation after primary repair was 0.67 years in the repair subgroup and 0.18 years in the replacement subgroup. Thirty-day survival was significantly poorer in the replacement subgroup compared with the repair subgroup (P = 0.0002). In multivariable analysis, indep...

Challenges in Echocardiographic Assessment of Mitral Regurgitation in Children After Repair of Atrioventricular Septal Defect

Pediatric Cardiology — Sat, 10 Sep 2011 06:02:16 +0100

In conclusion, echocardiographic assessment of MR severity after AVSD remains challenging. Among the quantitative methods used in this trial, i-VCW and i-VCA performed the best but offered little advantage compared with qualitative MR grade. The utility of regurgitant volume and fraction was severely limited by poor interobserver agreement and frequently negative values. Content Type Journal ArticleCategory Original ArticlePages 1-10DOI 10.1007/s00246-011-0107-5Authors Ashwin Prakash, Department of Cardiology, Children’s Hospital Boston, 300 Longwood Avenue, Boston, MA 02115, USARonald V. Lacro, Department of Cardiology, Children’s Hospital Boston, 300 Longwood Avenue, Boston, MA 02115, USALynn A. Sleeper, New England Research Institutes, Watertown, MA, USAL. LuAnn Minich, Uni...

Impact of Maternal Obesity and Maternal Overweight on the Detection Rate of Fetal Heart Defects and the Image Quality of Prenatal Echocardiography.

Ultraschall in der Medizin — Sun, 28 Aug 2011 23:00:00 +0100

CONCLUSION: The prenatal detection of fetal AVSD, DORV, TOF and D-TGA was also satisfactory in overweight and obese patients, but image quality substantially decreases with an increasing maternal BMI. If there is a BMI-associated difference in the detection rate, it probably will not exceed 20 %. PMID: 21877317 [PubMed - as supplied by publisher] (Source: Ultraschall in der Medizin)

Surgical Interventions for Atrioventricular Septal Defect Subtypes: The Pediatric Heart Network Experience.

The Annals of Thoracic Surgery — Wed, 24 Aug 2011 23:00:00 +0100

CONCLUSIONS: AVSD subtype was significantly associated with patient characteristics and clinical status before repair and influenced age at repair. Significant postoperative LAVVR is the most common sequela, with a similar prevalence across centers 6 months after the intervention. Annuloplasty failed to decrease the postoperative prevalence of moderate or severe LAVVR at 6 months. After accounting for age at repair, AVSD subtype was not associated with postoperative LAVVR severity or growth failure at 6 months. Further investigation is needed to determine if interventional strategies specific to AVSD subtype improve surgical outcomes. PMID: 21872212 [PubMed - as supplied by publisher] (Source: The Annals of Thoracic Surgery)

Lack of maternal folic acid supplementation is associated with heart defects in Down syndrome: A report from the National Down Syndrome Project

Birth Defects Research Part A: Clinical and Molecular Teratology — Tue, 23 Aug 2011 23:00:00 +0100

CONCLUSIONS: Our results suggest that lack of maternal folic acid supplementation is associated with septal defects in infants with Down syndrome. Birth Defects Research (Part A), 2011. © 2011 Wiley‐Liss, Inc. (Source: Birth Defects Research Part A: Clinical and Molecular Teratology)

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Outcome after repair of atrioventricular septal defect with tetralogy of Fallot

The Journal of Thoracic and Cardiovascular Surgery — Mon, 22 Aug 2011 04:00:00 +0100

Conclusions: Excellent long-term survival was achieved after repair of tetralogy of Fallot associated with complete atrioventricular septal defect. Palliation and primary repair resulted in comparable outcomes; as such, primary repair is favored. The choice of right ventricular outflow tract reconstruction affects the need for reoperation. (Source: The Journal of Thoracic and Cardiovascular Surgery)

Persistent left superior vena cava: experience of a tertiary health care center

Pediatrics International — Mon, 01 Aug 2011 23:00:00 +0100

Conclusion: TTE usually visualizes dilated coronary sinus in association with PLSVC. However, SVC injection should be performed in patients undergoing angiography so that morbidity and mortality related with persistent left superior vena cava can be avoided during cardiovascular surgery. (Source: Pediatrics International)

Balancing Stenosis and Regurgitation During Mitral Valve Surgery in Pediatric Patients [ORIGINAL ARTICLES: PEDIATRIC CARDIAC]

The Annals of Thoracic Surgery — Wed, 27 Jul 2011 23:00:00 +0100

Conclusions Within the limits of our analysis, residual mild mitral valve stenosis was associated with less hazard than mild to moderate regurgitation. Taken together, our data suggest that an intraoperative strategy to minimize mitral valve regurgitation is rational, even at the expense of mild mitral stenosis. (Source: The Annals of Thoracic Surgery)

Congenital Subclavian Steal Syndrome Associated With Heterotaxy Syndrome and Atrioventricular Septal Defect

Pediatric Cardiology — Thu, 21 Jul 2011 18:10:47 +0100

Abstract An infant under follow-up evaluation since the fetal stage was detected to have subclavian steal syndrome (SSS) associated with heterotaxy syndrome and atrioventricular septal defect. Such a complex association has not been reported previously. Congenital SSS occurs as a result of a subclavian artery originating from the main pulmonary artery through the ductus instead of the aortic arch. In rare cases, as in the reported case, it may occur due to proximal segment atresia of the left subclavian artery. Content Type Journal ArticlePages 1-2DOI 10.1007/s00246-011-0048-zAuthors Murat Ciftel, Department of Pediatric Cardiology, Faculty of Medicine, Akdeniz University, Antalya, TurkeyFirat Kardelen, Department of Pediatric Cardiology, Faculty of Medicine, Akdeniz ...

Maternal injuries during the periconceptional period and the risk of birth defects, National Birth Defects Prevention Study, 1997–2005

Paediatric and Perinatal Epidemiology — Wed, 20 Jul 2011 23:00:00 +0100

SummaryTinker SC, Reefhuis J, Dellinger AM, Jamieson DJ, the National Birth Defects Prevention Study. Maternal injuries during the periconceptional period and the risk of birth defects, National Birth Defects Prevention Study, 1997–2005. Paediatric and Perinatal Epidemiology 2011; ••:••–••.Maternal injuries during pregnancy are common (∼7% prevalence). However, few studies have examined the association between maternal injuries and birth defects. The National Birth Defects Prevention Study is a population‐based case–control study of birth defects in 10 US states. Cases were ascertained through surveillance; controls were randomly selected from infants delivered without major birth defects in the study regions. Mothers completed a telephone interview on exposures befor...

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Development and clinical application of minimally invasive cardiac surgery using percutaneous cardiopulmonary support

General Thoracic and Cardiovascular Surgery — Fri, 15 Jul 2011 16:04:10 +0100

Conclusions: Minimally invasive cardiac surgery using percutaneous cardiopulmonary support is safe and an excellent option for selected patients affected by single valve lesion, simple cardiac anomalies, and coronary artery bypass grafting. Content Type Journal ArticlePages 562-567DOI 10.1007/BF03218201Authors Hiroshi Ohuchi, Department of Surgery, Saitama Medical School, 38 Morohongo, Moroyama, 350-0495 Saitama, JapanShunei Kyo, Department of Surgery, Saitama Medical School, 38 Morohongo, Moroyama, 350-0495 Saitama, JapanHaruhiko Asano, Department of Surgery, Saitama Medical School, 38 Morohongo, Moroyama, 350-0495 Saitama, JapanHiroaki Tanabe, Department of Surgery, Saitama Medical School, 38 Morohongo, Moroyama, 350-0495 Saitama, JapanYuji Yokote, Department of Surgery, Saitama ...

A genetic contribution to risk for postoperative junctional ectopic tachycardia in children undergoing surgery for congenital heart disease

Heart Rhythm — Thu, 07 Jul 2011 04:00:00 +0100

Background: Junctional ectopic tachycardia (JET) is a common arrhythmia complicating pediatric cardiac surgery, with many identifiable clinical risk factors but no genetic risk factors to date.Objective: To test the hypothesis that the angiotensin-converting enzyme insertion/deletion (ACE I/D) polymorphism associates with postoperative JET.Methods: DNA samples were collected from children undergoing the Norwood procedure; arterial switch operation; and repairs of Tetralogy of Fallot, balanced atrioventricular septal defect, and ventricular septal defect at a single center. The incidence of postoperative JET was associated with previously identified clinical risk factors and ACE I/D genotype.Results: Of the 174 children who underwent the above-mentioned surgeries, 21% developed JET. Postope...

The Contribution of Chromosomal Abnormalities to Congenital Heart Defects: A Population-Based Study

Pediatric Cardiology — Tue, 05 Jul 2011 05:34:18 +0100

In conclusion, in our study, approximately 1 in 8 infants with a CHD had a chromosomal abnormality. Clinicians should have a low threshold at which to obtain testing for chromosomal abnormalities in infants with CHDs, especially those with certain types of CHDs. Use of new technologies that have become recently available (e.g., chromosomal microarray) may increase the identified contribution of chromosomal abnormalities even further. Content Type Journal ArticlePages 1-11DOI 10.1007/s00246-011-0034-5Authors Robert J. Hartman, National Center on Birth Defects and Developmental Disabilities, Centers for Disease Control and Prevention, 1600 Clifton Road, MS E-86, Atlanta, GA, USASonja A. Rasmussen, National Center on Birth Defects and Developmental Disabilities, Centers for Disease ...

Risk Factors and Early Outcomes of Multiple Reoperations in Adults With Congenital Heart Disease [ORIGINAL ARTICLES: ADULT CARDIAC]

The Annals of Thoracic Surgery — Sun, 26 Jun 2011 23:00:00 +0100

Conclusions Subsequent sternotomy showed increased early mortality, yet neither sternotomy number nor cardiac injury was an independent predictor of early death. Two variables were protective: early mortality was reduced with increased ejection fraction and cardiac injury was less likely with increased interval from the previous sternotomy. (Source: The Annals of Thoracic Surgery)

In adult patients undergoing redo surgery for left atrioventricular valve regurgitation after atrioventricular septal defect correction, is replacement superior to repair? [Best evidence topic - Congenital]

Interactive CardioVascular and Thoracic Surgery — Tue, 07 Jun 2011 23:00:00 +0100

A best evidence topic in cardiac surgery was written according to a structured protocol. The question addressed was ‘In adult patients undergoing redo surgery for left atrioventricular valve regurgitation after atrioventricular septal defect correction, is replacement superior to repair?’ Altogether more than 109 papers were found using the reported search, of which eight represented the best evidence to answer the clinical question. The authors, journal, date and country of publication, patient group studied, study type, relevant outcomes, and results of these papers are tabulated. We conclude that left atrioventricular valve (LAVV) repair should be the first line approach and the use of transesophageal echocardiography (TEE) in operating room is mandatory. When complex anatom...

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Origin and development of the atrioventricular myocardial lineage: Insight into the development of accessory pathways

Birth Defects Research Part A: Clinical and Molecular Teratology — Mon, 30 May 2011 23:00:00 +0100

AbstractDefects originating from the atrioventricular canal region are part of a wide spectrum of congenital cardiovascular malformations that frequently affect newborns. These defects include partial or complete atrioventricular septal defects, atrioventricular valve defects, and arrhythmias, such as atrioventricular re‐entry tachycardia, atrioventricular nodal block, and ventricular preexcitation. Insight into the cellular origin of the atrioventricular canal myocardium and the molecular mechanisms that control its development will aid in the understanding of the etiology of the atrioventricular defects. This review discusses current knowledge concerning the origin and fate of the atrioventricular canal myocardium, the molecular mechanisms that determine its specification and different...

Periconceptional febrile illnesses and congenital heart defects

The Journal of Pediatrics — Thu, 19 May 2011 14:59:15 +0100

Through a large population-based case control study, Oster et al add precision to the evidence of effects of maternal illness on the likelihood of congenital heart defects (CHD) in offspring. The specificity of the events considered (ie, “fever of 101°F or higher” or “influenza [flu]” in the periconceptional period from 3 months prior to conception until 3 months of gestation) and outcomes found (ie, statistically significantly increased odds ratio for right-sided obstructive lesions, as well as atrioventricular septal defects in cases with Down syndrome, but no association with CHD in aggregate) are impressive. Investigators also found that use of antipyretics tended to decrease these associations. An important limitation of the study is that the specific antipyretic agents used ...

Subaortic Stenosis After Atrioventricular Septal Defect Repair.

Anesthesia and Analgesia — Wed, 18 May 2011 23:00:00 +0100

Authors: De Mey N, Couture P, Denault AY, Poirier NC, Rochon AG PMID: 21596872 [PubMed - as supplied by publisher] (Source: Anesthesia and Analgesia)

Outcomes of Hospitalization in Adults in the United States With Atrial Septal Defect, Ventricular Septal Defect, and Atrioventricular Septal Defect

The American Journal of Cardiology — Wed, 04 May 2011 23:00:00 +0100

In conclusion, this study of hospitalizations in adults with septal defects found that admissions are common and associated with significant co-morbidities. Overall mortality is low but is increased in patients with VSD. Cardiac and noncardiac co-morbidities are commonly encountered. Many noncardiac conditions, including trisomy 21 and the youngest and oldest groups, are associated with an increased risk of death. (Source: The American Journal of Cardiology)

The Adult With Fontan Physiology: Systematic Approach to Perioperative Management for Noncardiac Surgery

Journal of Cardiothoracic and Vascular Anesthesia — Thu, 31 Mar 2011 23:00:00 +0100

SINCE 1971, Fontan palliation has been the primary surgical approach for complex congenital heart lesions not suitable for biventricular repair. Such lesions include hypoplastic left-heart syndrome, tricuspid atresia, unbalanced atrioventricular septal defects, double-inlet left ventricle, double-outlet right ventricle, and some forms of heterotaxy syndrome (). The advancement of surgical techniques and medical management have resulted in greater than 90% survival rate 10 years after Fontan palliation, allowing patients with complex congenital heart lesions to survive into adulthood. As a result, patients with Fontan palliation increasingly are presenting to the adult perioperative setting for laparoscopic, obstetric, orthopedic, and other noncardiac procedures. (Source: Journal of Cardiot...

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Congenital mitral valve regurgitation in adult patients.

The Heart Surgery Forum — Thu, 31 Mar 2011 23:00:00 +0100

Conclusion: Congenital MVR is rare and often misdiagnosed in adults. Mitral valve repair is feasible in the majority of patients, with excellent immediate and medium-term results. PMID: 21521674 [PubMed - in process] (Source: The Heart Surgery Forum)

Role of four‐dimensional ultrasound (spatiotemporal image correlation and Sonography‐based Automated Volume Count) in prenatal assessment of atrial morphology in cardiosplenic syndromes

Ultrasound in Obstetrics and Gynecology — Mon, 14 Mar 2011 00:00:00 +0100

ConclusionFour‐dimensional ultrasound with SonoAVC rendering allows correct identification of the morphology of atrial appendages in all cases of cardiosplenic syndromes in which an adequate cardiac volume dataset can be obtained for analysis. Copyright © 2011 ISUOG. Published by John Wiley & Sons, Ltd. (Source: Ultrasound in Obstetrics and Gynecology)

Cardiac-specific transcription factor genes Smad4 and Gata4 cooperatively regulate cardiac valve development [Developmental Biology]

Proceedings of the National Academy of Sciences — Tue, 08 Mar 2011 00:00:00 +0100

We report that the dominant human missense mutations G303E and G296S in GATA4, a cardiac-specific transcription factor gene, cause atrioventricular septal defects and valve abnormalities by disrupting a signaling cascade involved in endocardial cushion development. These GATA4 missense mutations, but not a mutation causing secundum atrial septal defects (S52F), demonstrated impaired protein interactions with SMAD4, a transcription factor required for canonical bone morphogenetic protein/transforming growth factor-β (BMP/TGF-β) signaling. Gata4 and Smad4 genetically interact in vivo: atrioventricular septal defects result from endothelial-specific Gata4 and Smad4 compound haploinsufficiency. Endothelial-specific knockout of Smad4 caused an absence of valve-forming activity: Smad4-deficien...

Maternal Smoking and Congenital Heart Defects in the Baltimore-Washington Infant Study

PEDIATRICS — Tue, 01 Mar 2011 00:00:00 +0100

CONCLUSIONS: These findings add to the existing body of evidence that implicates first-trimester maternal cigarette smoking as a modest risk factor for select CHD phenotypes. (Source: PEDIATRICS)

Echocardiographic diagnosis of atrioventricular septal defect without primum atrial septal defect: a relatively "common" congenital heart defect in Down's syndrome.

Anadolu Kardiyol Der... — Tue, 01 Mar 2011 00:00:00 +0100

Echocardiographic diagnosis of atrioventricular septal defect without primum atrial septal defect: a relatively "common" congenital heart defect in Down's syndrome. Anadolu Kardiyol Derg. 2011 Mar 1; Authors: Ali S PMID: 21362598 [PubMed - as supplied by publisher] (Source: Anadolu Kardiyol Der...)

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Prenatally detectable congenital heart defects in Down syndrome fetuses

Ultrasound in Obstetrics and Gynecology — Mon, 28 Feb 2011 00:00:00 +0100

ConclusionsMost fetuses (66‐76%) with trisomy 21 have a structurally normal heart on echocardiography. CHD did not additionally increase the measurement of nuchal translucency. The increased incidence of AVSD in females was confirmed in our study, although an ethnic difference could not be confirmed. CHD does not appear to increase the chance of spontaneous intrauterine loss in ongoing pregnancies. Copyright © 2011 ISUOG. Published by John Wiley & Sons, Ltd. (Source: Ultrasound in Obstetrics and Gynecology)

Prenatally detectable congenital heart defects in fetuses with Down syndrome

Ultrasound in Obstetrics and Gynecology — Mon, 28 Feb 2011 00:00:00 +0100

ConclusionMost fetuses (66–76%) with trisomy 21 have a structurally normal heart on echocardiography. The presence of structural CHD was not associated with increased NT. The increased incidence of AVSD in females was confirmed in our study, although an ethnic difference could not be confirmed. CHD does not appear to increase the chance of spontaneous intrauterine loss in ongoing pregnancies. Copyright © 2011 ISUOG. Published by John Wiley & Sons, Ltd. (Source: Ultrasound in Obstetrics and Gynecology)

Maternal treatment with opioid analgesics and risk for birth defects

American Journal of Obstetrics and Gynecology — Thu, 24 Feb 2011 00:00:00 +0100

Conclusion: Consistent with some previous investigations, our study shows an association between early pregnancy maternal opioid analgesic treatment and certain birth defects. This information should be considered by women and their physicians who are making treatment decisions during pregnancy. (Source: American Journal of Obstetrics and Gynecology)

The hypoplastic left heart syndrome is not a risk factor for Fontan operation

Revista Brasileira de Cirurgia Cardiovascular — Sat, 19 Feb 2011 02:07:23 +0100

OBJETIVO: Demonstrar a mortalidade hospitalar de crianças submetidas à operação de Fontan e determinar se a síndrome do coração esquerdo hipoplásico (SCEH) constitui fator de risco para mortalidade. MÉTODOS: De agosto 2001 a junho 2008, 76 pacientes foram submetidos à operação de Fontan, sendo divididos em dois grupos: grupo A com 54 pacientes, sendo 31 (40,7%) portadores de atresia tricúspide e variantes, seis (7,8%) de dupla via de entrada ventrículo esquerdo, quatro (5,3%) de defeito do septo atrioventricular total e 13 (17,1%) de outras cardiopatias congênitas complexas; e grupo B constituído por portadores de SCEH, num total de 22 (28,9%) pacientes. RESULTADOS: Os pacientes do grupo A tiveram média de idade de 6,47 anos ± 4,83 e do grupo B de 2,08 anos ± 0,24 P

Descriptive study of nonsyndromic atrioventricular septal defects in the National Birth Defects Prevention Study, 1997–2005

American Journal of Medical Genetics Part A — Fri, 18 Feb 2011 00:00:00 +0100

AbstractNonsyndromic atrioventricular septal defects (AVSDs) are serious congenital heart defects for which information on prevalence and descriptive characteristics based on large, geographically, and ethnically diverse populations has been limited. To describe the birth prevalence and phenotype of nonsyndromic AVSDs, we used data from the National Birth Defects Prevention Study (NBDPS), a multisite, population‐based case–control study aimed at identifying genetic and environmental risk factors for birth defects. For this analysis, infants born during the period 1997–2005 and meeting the NBDPS case definition for AVSDs were included. Infants with an AVSD associated with recognized or strongly suspected chromosomal abnormalities or single‐gene disorders (syndromic case infants) wer...

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Congenital Heart Defects and Major Structural Noncardiac Anomalies, Atlanta, Georgia, 1968 to 2005

The Journal of Pediatrics — Fri, 18 Feb 2011 00:00:00 +0100

Conclusions: Including those with syndromes and laterality defects, 28.7% of case infants with CHDs had associated major noncardiac malformations. Thus, infants with CHDs warrant careful examination for the presence of noncardiac anomalies. (Source: The Journal of Pediatrics)

Prevalence and distribution of structural heart diseases in high and low risk pregnancies.

Anadolu Kardiyol Der... — Tue, 08 Feb 2011 00:00:00 +0100

CONCLUSION: Congenital heart diseases rate, as expected, was found to be higher in high-risk group. However, regarding intrauterine fetal death in previous pregnancy, abnormal first or second trimester screening tests and multiple gestation, no statistically significant difference was observed between low-risk and high-risk groups. Therefore, we suggest the routine use of fetal echocardiography in cases we have mentioned above if the staff and equipment of the pediatric cardiology clinic are eligible. PMID: 21303759 [PubMed - as supplied by publisher] (Source: Anadolu Kardiyol Der...)

Lessons learned from a pediatric clinical trial: The Pediatric Heart Network Angiotensin-Converting Enzyme Inhibition in Mitral Regurgitation Study

American Heart Journal — Tue, 01 Feb 2011 00:00:00 +0100

Background: Mitral regurgitation is the most common indication for reoperation in children following repair of atrioventricular septal defect (AVSD). We hypothesized that angiotensin-converting enzyme inhibitor therapy would decrease the severity of mitral regurgitation and limit left ventricular volume overload in children following AVSD repair.Methods: The Pediatric Heart Network designed a placebo-controlled randomized trial of enalapril in this population. The primary aim was to test the effect of enalapril on the change in left ventricular end-diastolic dimension body surface area–adjusted z score. Before the launch of the trial, a feasibility study was performed to estimate the number of patients with at least moderate mitral regurgitation following AVSD repair.Trial experience: Se...

Associations Between Maternal Fever and Influenza and Congenital Heart Defects

The Journal of Pediatrics — Mon, 24 Jan 2011 00:00:00 +0100

Conclusions: Prenatal maternal fever or influenza may be associated with right-sided obstructive lesions in all infants and with atrioventricular septal defects in infants with Down syndrome. The use of antipyretics might attenuate such associations. (Source: The Journal of Pediatrics)

Surgical management of complete atrioventricular septal defect: Associations with surgical technique, age, and trisomy 21

The Journal of Thoracic and Cardiovascular Surgery — Thu, 16 Dec 2010 00:00:00 +0100

Objectives: We sought to evaluate the contemporary results after repair of a complete atrioventricular septal defect and to determine the factors associated with suboptimal outcomes.Methods: The demographic, procedural, and outcome data were obtained within 1 and 6 months after repair of a complete atrioventricular septal defect in 120 children in a multicenter observational study from June 2004 to 2006.Results: The median age at surgery was 3.7 months (range, 9 days to 1.1 years). The type of surgical repair was a single patch (18%), double patch (72%), and a single atrial septal defect patch with primary ventricular septal defect closure (10%). The incidence of residual septal defects and the degree of left atrioventricular valve regurgitation (LAVVR) did not differ by repair type. The m...

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More than 30 years' experience with surgical correction of atrioventricular septal defects.

The Annals of Thoracic Surgery — Mon, 01 Nov 2010 00:00:00 +0100

CONCLUSIONS: AVSD repair can be accomplished with good long-term results. Early surgical era, associated cardiovascular anomalies, left AV valve dysplasia, and absence of cleft closure negatively influence survival and risk of reoperation. PMID: 20971263 [PubMed - indexed for MEDLINE] (Source: The Annals of Thoracic Surgery)

Novel CRELD1 gene mutations in patients with atrioventricular septal defect.

World Journal of Pediatrics : WJP — Mon, 01 Nov 2010 00:00:00 +0100

CONCLUSIONS: Two novel CRELD1 mutations were identified in the calcium-binding EGF domain in patients with AVSD. CRELD1 is likely to be an AVSD-susceptibility gene and CRELD1 mutations may increase the risk of developing a heart defect rather than being a direct causative mutation. PMID: 21080147 [PubMed - in process] (Source: World Journal of Pediatrics : WJP)

More Than 30 Years' Experience With Surgical Correction of Atrioventricular Septal Defects [ORIGINAL ARTICLES: PEDIATRIC CARDIAC]

The Annals of Thoracic Surgery — Thu, 21 Oct 2010 23:00:00 +0100

Conclusions AVSD repair can be accomplished with good long-term results. Early surgical era, associated cardiovascular anomalies, left AV valve dysplasia, and absence of cleft closure negatively influence survival and risk of reoperation. (Source: The Annals of Thoracic Surgery)

Down's syndrome-like cardiac developmental defects in embryos of the transchromosomic Tc1 mouse

Cardiovascular Research — Mon, 11 Oct 2010 16:07:21 +0100

Conclusion The Tc1 mouse line provides a suitable model for studying the underlying genetic causes of the DS AVSD cardiac phenotype. (Source: Cardiovascular Research)

Impact of prenatal diagnosis and anatomical subtype on outcome in double outlet right ventricle

American Heart Journal — Thu, 30 Sep 2010 23:00:00 +0100

Background: We sought to investigate the influence of prenatal diagnosis and risk factors for adverse outcomes in double outlet right ventricle (DORV) not associated with heterotaxy.Methods: Patients with a pre or postnatal diagnosis of DORV from 2000 to 2007 were identified and classified into 3 subgroups: subaortic ventricular septal defect (VSD) and normal great artery (GA) arrangement (=VSD type), tetralogy of Fallot type, and transposition of the GA type (=TGA type). Patients with heterotaxy, atrioventricular septal defect, valve atresia, and ventricular hypoplasia were excluded. Complex postnatal care was defined as prematurity, need for prostaglandins, surgical repair (Source: American Heart Journal)

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Investigating 22q11.2 Deletion and Other Chromosomal Aberrations in Fetuses With Heart Defects Detected by Prenatal Echocardiography

Pediatric Cardiology — Thu, 16 Sep 2010 16:46:26 +0100

This study aimed to investigate the incidence of the 22q11.2 deletion and other chromosomal alterations in a Brazilian sample of fetuses with structural cardiac anomalies detected by fetal echocardiography. In a prospective study, 68 fetuses with a heart defect were evaluated. Prenatal detection of cardiac abnormalities led to identification of aneuploidy or structural chromosomal anomaly in 35.3% of these cases. None of the fetuses with apparently normal karyotypes had a 22q11.2 deletion. The heart defects most frequently associated with chromosomal abnormalities were atrioventricular septal defect (AVSD), ventricular septal defect (VSD), and tetralogy of Fallot. Autosomal trisomies 18 and 21 were the most common chromosomal abnormalities. The study results support the strong associ...

Echocardiographic definition and surgical decision-making in unbalanced atrioventricular septal defect: a Congenital Heart Surgeons' Society multiinstitutional study.

Circulation — Mon, 13 Sep 2010 23:00:00 +0100

CONCLUSIONS: AVVI effectively characterizes the transition between balanced and unbalanced AVSD with important correlation to anatomic substrate and selected surgical strategy. PMID: 20837915 [PubMed - in process] (Source: Circulation)

Interruption of the Inferior Vena Cava and Azygos Continuation in a Child with Partial Atrioventricular Septal Defect

The Thoracic and Cardiovascular Surgeon — Mon, 06 Sep 2010 23:00:00 +0100

Thorac cardiovasc Surg 2010; 58: 369-372DOI: 10.1055/s-0029-1240834AbstractCongenital pathologies of the inferior vena cava are uncommon. Isolated congenital interruption of the inferior vena cava with azygos vein continuation could be considered part of the group of venous return anomalies. The major significance of this anomaly is its association with complex cardiac defects and the associated technical difficulties at the time of cardiac catheterization and abdominal surgery or interventional procedures. In our case, we describe a common atrium and partial atrioventricular septal defect in an 8-year-old girl with infrahepatic interruption of the inferior vena cava and azygos continuation without polysplenia. Magnetic resonance angiography and computed tomography, together with a clinica...

Interruption of the inferior vena cava and azygos continuation in a child with partial atrioventricular septal defect.

The Thoracic and Cardiovascular Surgeon — Tue, 31 Aug 2010 23:00:00 +0100

Authors: Erkut B, Ates A, Arslan S, Kaygin MA, Dag O, Alper F Congenital pathologies of the inferior vena cava are uncommon. Isolated congenital interruption of the inferior vena cava with azygos vein continuation could be considered part of the group of venous return anomalies. The major significance of this anomaly is its association with complex cardiac defects and the associated technical difficulties at the time of cardiac catheterization and abdominal surgery or interventional procedures. In our case, we describe a common atrium and partial atrioventricular septal defect in an 8-year-old girl with infrahepatic interruption of the inferior vena cava and azygos continuation without polysplenia. Magnetic resonance angiography and computed tomography, together with a clinical awarene...

Primary Biventricular Repair of Atrioventricular Septal Defects: An Analysis of Reoperations [ORIGINAL ARTICLES: PEDIATRIC CARDIAC]

The Annals of Thoracic Surgery — Tue, 24 Aug 2010 20:35:44 +0100

Conclusions Down syndrome and right ventricular dominance are independent predictors of reoperation after complete AVSD repair. Biventricular repair of isolated AVSD with a small left ventricle can be successfully accomplished with no mortality. (Source: The Annals of Thoracic Surgery)

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Congenital Heart Surgery Outcomes in Down Syndrome: Analysis of a National Clinical Database

PEDIATRICS — Mon, 02 Aug 2010 15:02:40 +0100

CONCLUSION: In this large, contemporary cohort, Down syndrome did not confer a significant mortality risk for the most common operations; however, postoperative morbidity remained common. (Source: PEDIATRICS)

Variations of Atrioventricular Septal Defects Predisposing to Regurgitation and Stenosis.

The Annals of Thoracic Surgery — Sat, 31 Jul 2010 21:46:21 +0100

CONCLUSIONS: An individualized technique is required to maintain coaptation of the atrioventricular valve, but in many cases, they are not completely correctable. Deferring complete repair by placing a pulmonary arterial band did not reduce left valvular problems. PMID: 20667360 [PubMed - as supplied by publisher] (Source: The Annals of Thoracic Surgery)

Variations of Atrioventricular Septal Defects Predisposing to Regurgitation and Stenosis [ORIGINAL ARTICLES: PEDIATRIC CARDIAC]

The Annals of Thoracic Surgery — Tue, 27 Jul 2010 18:48:06 +0100

Conclusions An individualized technique is required to maintain coaptation of the atrioventricular valve, but in many cases, they are not completely correctable. Deferring complete repair by placing a pulmonary arterial band did not reduce left valvular problems. (Source: The Annals of Thoracic Surgery)

A novel mutation of GATA4 in a familial atrial septal defect.

International Journal of Clinical Chemistry — Fri, 23 Jul 2010 23:00:00 +0100

CONCLUSION: T280M mutation of GATA4 is suggested to be associated with ASD in this Chinese family. PMID: 20659440 [PubMed - as supplied by publisher] (Source: International Journal of Clinical Chemistry)

Tracheostomy after pediatric cardiac surgery: Frequency, indications, and outcomes

The Journal of Thoracic and Cardiovascular Surgery — Tue, 20 Jul 2010 00:00:00 +0100

Conclusions: Requirement for tracheostomy in pediatric patients after cardiac surgery was associated with significant mortality. Patients with single ventricle have the highest late death rate and those with chronic ventilator dependency were unlikely to undergo successful Fontan completion. (Source: The Journal of Thoracic and Cardiovascular Surgery)

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Correction

The Journal of Pediatrics — Fri, 16 Jul 2010 06:28:11 +0100

In the article, “Long-Term Survival of Infants with Atrioventricular Septal Defects,” by Miller et al, J Pediatr 2010;156:882-8, the authors request that the legend for Figure 2 be changed to: “Survival probability of infants with AVSD. A, Infants without Down syndrome (some children have heterotaxy) and B, Infants with Down syndrome (none had heterotaxy), by complexity of heart defects, Atlanta, Georgia, 1979 to 2003.” (Source: The Journal of Pediatrics)

Anatomical repair of a persistent left superior vena cava into the left atrium [Case report - Congenital]

Interactive CardioVascular and Thoracic Surgery — Tue, 13 Jul 2010 16:14:05 +0100

The anatomy of a persistent left superior vena cava (SVC) to the left atrium (LA) without the innominate vein can make it challenging to complete intracardiac repair. We reviewed our five cases of the direct end-to-side anastomosis of SVCs to facilitate anatomical repair of SVC-right atrial connection for biventricular repair. Diagnoses were two partial atrioventricular septal defect with left isomerism, one complete atrioventricular septal defect (CAVSD) with left isomerism, one CAVSD without isomerism and one atrioventricular discordance and double outlet right ventricle with right isomerism. Mean age at the operation was 20±23 months (4–58 months) and body weight was 7.8±3.4 kg (4.8–12.7 kg). After completion of intracardiac repair, the SVC to LA was divided an...

Introduction to congenital heart disease articles in Cardiovascular Pathology

Cardiovascular Pathology — Sun, 04 Jul 2010 23:00:00 +0100

Congenital heart disease is common and causes substantial consequences for patients, their families, their communities and the health care system. Congenital heart defects occur in nearly 1% of live births and comprise at least 18 distinct types (most commonly ventricular septal defect, tetralogy of Fallot, transposition of the great arteries, and atrioventricular septal defect). They range in severity from minute holes between chambers, which may resolve spontaneously, to major malformations that can require multiple surgical procedures (frequently in the first years of life) and may result in death in infancy, childhood, or as a young adult. (Source: Cardiovascular Pathology)

Complete atrioventricular septal defect: Outcome of pulmonary artery banding improved by adjustable device

The Journal of Thoracic and Cardiovascular Surgery — Fri, 02 Jul 2010 00:00:00 +0100

Objective: We sought to evaluate pulmonary artery banding in infants with complete atrioventricular septal defects.Methods: From 2000 to 2009, 20 infants with complete atrioventricular septal defects underwent pulmonary artery banding because of unsuitable anatomy (unbalanced ventricles, associated lesions, or both) or clinical condition (infection, chronic lung disease, or noncardiac malformation). Patients were divided into 2 groups: the conventional PAB group (n = 13 [65%]; mean age, 74 ± 56 days [range, 6–187 days]; mean weight, 3.3 ± 1.1 kg [range, 2.1–5.8 kg]) and the FloWatch-PAB group (n = 7 [35%]; mean age, 111 ± 40 days [range, 81–187 days]; mean weight, 4.3 ± 1.2 kg [range, 3.2–6.1 kg]). There was no statistical difference in age or weight. Preoperative mecha...

Oral sildenafil for persistent pulmonary hypertension early after congenital cardiac surgery in children [Original articles]

European Journal of Cardio-Thoracic Surgery — Mon, 28 Jun 2010 17:38:03 +0100

Conclusions: Our results suggest that oral sildenafil is a safe and effective alternate for persistent pulmonary hypertension following congenital heart surgery in children. (Source: European Journal of Cardio-Thoracic Surgery)

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Left atrial isomerism: biventricular repair [Original articles]

European Journal of Cardio-Thoracic Surgery — Tue, 01 Jun 2010 15:22:06 +0100

Conclusions: Survival and functional status of left isomerism patients after biventricular repair is good. Complex repairs with atrial or ventricular baffles are frequent. Arrhythmias were common and pose a concern late after repair. (Source: European Journal of Cardio-Thoracic Surgery)

The genetics of heart defects

NHS News Feed — Thu, 27 May 2010 10:32:00 +0100

Conclusion This study has furthered the research understanding of molecular events in the development of heart defects, and it can focus further research efforts on the function of this gene. There are several strong points to this study, including the replication of findings in a separate set of cases and controls, and the separate analysis of different ethnic groups. It is still possible that some of the differences found could be due to the ethnic mix of those with heart defects compared to those without, although the separate analyses performed do reduce this risk. It is also possible that the selection of patients (cases) for the study may have led to bias in the results. As the ISL1 gene is known to be involved with heart development, the finding of a moderate link with all types of ...

Comprehensive genotype-phenotype analysis in 230 patients with tetralogy of Fallot

Journal of Medical Genetics — Fri, 07 May 2010 08:07:36 +0100

Conclusion This study shows that 22q11.2 deletion represents the most common known cause of ToF, and that the associated cardiac phenotype is distinct for obstruction of the proximal pulmonary artery, hypoplastic central pulmonary arteries and subclavian artery anomalies. Atrioventricular septal defect associated with ToF is very suggestive of trisomy 21 and almost excludes 22q11.2 deletion. We report a further patient with a recurrent polyalanine stretch elongation within TBX1 and for the first time link TBX1 cytoplasmatic protein aggregation to congenital heart defects. (Source: Journal of Medical Genetics)

[Clinical outcome in Down syndrome patients with congenital heart disease.]

Cirugia y Cirujanos — Fri, 30 Apr 2010 23:00:00 +0100

Conclusions: Left atrioventricular valve insufficiency and left ventricle outflow tract obstruction were the most frequent long-term complications requiring surgical reintervention in patients with atrioventricular septal defect. PMID: 20642908 [PubMed - in process] (Source: Cirugia y Cirujanos)

Reoperations After Repair of Partial Atrioventricular Septal Defect: A 45-Year Single-Center Experience [ORIGINAL ARTICLES: PEDIATRIC CARDIAC]

The Annals of Thoracic Surgery — Tue, 27 Apr 2010 19:47:04 +0100

Conclusions The most common indication for reoperation after initial repair of partial AVSD is LAVV pathology, followed by recurrent left ventricular outflow tract obstruction. The need for further reoperation and late survival is similar between patients undergoing valve repair or replacement. When reoperation is required overall late survival is significantly reduced. (Source: The Annals of Thoracic Surgery)

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Prevalence of congenital heart defects and persistent pulmonary hypertension of the neonate with Down syndrome

European Journal of Pediatrics — Thu, 22 Apr 2010 09:21:28 +0100

Abstract The aim of this study was to assess the prevalence of congenital heart defects (CHDs) and persistent pulmonary hypertension of the neonate (PPHN) in children with Down syndrome (DS) and to assess its impact on neonatal factors. It was a prospective study of a birth cohort of children with DS born between 2003 and 2006 registered by the Dutch Paediatric Surveillance Unit (DPSU). A CHD occurred in 43% of 482 children with trisomy 21. Atrioventricular septal defect was found in 54%, ventricular septal defect in 33.3% and patent ductus arteriosus in 5.8%. The incidence of PPHN in DS was 5.2%, which is significantly higher than the general population (p < 0.001). The reported mortality in newborns with DS was overall 3.3% and was still significant higher in child...

Reply to the Editor

The Journal of Thoracic and Cardiovascular Surgery — Wed, 21 Apr 2010 15:59:25 +0100

We thank Kestelli and associates for their comments. Two mechanisms are involved in the etiology of the association of tetralogy of Fallot and complete atrioventricular septal defect: the anterior deviation of conal septum and the lack of conal septum contribution to the development of the atrioventricular valves. These mechanisms are responsible for the right ventricular outflow tract obstruction and the creation of a wide ventricular septal defect (VSD) with subaortic extension, respectively. The embryologic basis explains that the closure of the VSD is not responsible for pulmonary stenosis and that resection of the septomarginalis trabecula could not be sufficient to relieve the right ventricular outflow tract. (Source: The Journal of Thoracic and Cardiovascular Surgery)

Birth Weight and Prematurity in Infants with Single Ventricle Physiology: Pediatric Heart Network Infant Single Ventricle Trial Screened Population

Congenital Heart Disease — Mon, 29 Mar 2010 23:00:00 +0100

We describe the birth outcomes (i.e., gestational age and birth weight) in neonates with single ventricle physiology screened for enrollment in the Pediatric Heart Network's Infant Single Ventricle Trial, compare these outcomes with US norms, and examine the association of birth outcomes with anatomic diagnosis and race.Patients and Methods. All neonates with single ventricle physiology presenting to Infant Single Ventricle Trial centers were screened for enrollment. Demographic data and anatomic diagnoses were obtained from medical records.Results. A total of 1245 neonates with single ventricle physiology were screened at 10 centers (63 to 266 per center). Diagnoses included hypoplastic left heart syndrome in 49%, unbalanced atrioventricular septal defect in 12%, and tricuspid atresia in ...

Long-Term Results After Repair of Complete Atrioventricular Septal Defect With Two-patch Technique.

The Annals of Thoracic Surgery — Mon, 29 Mar 2010 06:10:04 +0100

CONCLUSIONS: Definitive early repair for complete AVSD can be performed with excellent results. The two-patch technique with complete cleft closure is a safe and reproducible surgical method that can achieve very low mortality and stable long-term outcomes, even in neonates. PMID: 20338343 [PubMed - as supplied by publisher] (Source: The Annals of Thoracic Surgery)

Left ventricular outflow tract obstruction after the modified single patch repair of atrioventricular septal defects: teasing fact from fiction.

The Annals of Thoracic Surgery — Mon, 29 Mar 2010 06:06:36 +0100

Authors: Kanani M, Cook A, Kostolny M PMID: 20338382 [PubMed - in process] (Source: The Annals of Thoracic Surgery)

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Long-Term Results After Repair of Complete Atrioventricular Septal Defect With Two-patch Technique [ORIGINAL ARTICLES: PEDIATRIC CARDIAC]

The Annals of Thoracic Surgery — Fri, 26 Mar 2010 15:49:11 +0100

Conclusions Definitive early repair for complete AVSD can be performed with excellent results. The two-patch technique with complete cleft closure is a safe and reproducible surgical method that can achieve very low mortality and stable long-term outcomes, even in neonates. (Source: The Annals of Thoracic Surgery)

Left Ventricular Outflow Tract Obstruction After the Modified Single Patch Repair of Atrioventricular Septal Defects: Teasing Fact From Fiction [CORRESPONDENCE]

The Annals of Thoracic Surgery — Fri, 26 Mar 2010 15:49:11 +0100

(Source: The Annals of Thoracic Surgery)

Long-Term Survival of Infants with Atrioventricular Septal Defects

The Journal of Pediatrics — Mon, 15 Mar 2010 00:00:00 +0100

Conclusions: Down syndrome was not a prognostic factor. Our findings might be helpful in assessing the long-term prognosis of infants with AVSD. (Source: The Journal of Pediatrics)

Heterogeneity of Genetic Modifiers Ensures Normal Cardiac Development.

Circulation — Mon, 08 Mar 2010 00:00:00 +0100

Conclusions-Alleles of modifier genes can either buffer perturbations on cardiac development or direct the manifestation of a defect. In a genetically heterogeneous population, the predominant effect of modifier genes is health. PMID: 20212279 [PubMed - as supplied by publisher] (Source: Circulation)

Intraatrial Baffle Repair of Anomalous Systemic Venous Return Without Hepatic Venous Drainage in Heterotaxy Syndrome

Pediatric Cardiology — Wed, 24 Feb 2010 06:47:08 +0100

This report describes an intraatrial baffle repair of anomalous systemic venous return without hepatic venous drainage. This resulted in good oxygenation postoperatively, with oxygen saturation ranging from 93% to 98%. Content Type Journal ArticleCategory Case ReportDOI 10.1007/s00246-010-9686-9Authors Rıza Turkoz, İstanbul Teaching and Medical Research Center, Baskent University Department of Cardiovascular Surgery Istanbul TurkeyCanan Ayabakan, İstanbul Teaching and Medical Research Center, Baskent University Department of Pediatric Cardiology Istanbul TurkeyCan Vuran, İstanbul Teaching and Medical Research Center, Baskent University Department of Cardiovascular Surgery Istanbul TurkeyOğuz Omay, İstanbul Teaching and Medical Research Center, Baskent University Department o...

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Symptomatic Neonatal Tetralogy of Fallot: Repair or Shunt? [ORIGINAL ARTICLES: PEDIATRIC CARDIAC]

The Annals of Thoracic Surgery — Tue, 23 Feb 2010 22:46:26 +0100

Conclusions Shunting or primary repair of neonates with symptomatic TOF provides equivalent mortality and results. Shunted patients had fewer transannular patch repairs despite having more emergent initial operations. Compared with the primary repair group, shunted patients had decreased intensive care unit and hospital stays for the first hospitalization, which were neutralized when the second operation (repair) values were added. (Source: The Annals of Thoracic Surgery)

Symptomatic Neonatal Tetralogy of Fallot: Repair or Shunt?

The Annals of Thoracic Surgery — Tue, 23 Feb 2010 13:10:33 +0100

CONCLUSIONS: Shunting or primary repair of neonates with symptomatic TOF provides equivalent mortality and results. Shunted patients had fewer transannular patch repairs despite having more emergent initial operations. Compared with the primary repair group, shunted patients had decreased intensive care unit and hospital stays for the first hospitalization, which were neutralized when the second operation (repair) values were added. PMID: 20172143 [PubMed - as supplied by publisher] (Source: The Annals of Thoracic Surgery)

Biventricular repair of atrioventricular septal defect with common atrioventricular valve and double-outlet right ventricle.

The Annals of Thoracic Surgery — Mon, 01 Feb 2010 00:00:00 +0100

CONCLUSIONS: Although technically challenging, the surgical repair can be accomplished with acceptable early results. Heterotaxy syndrome, with concurrent anomalous connections of the pulmonary veins, represented the strongest identified risk factor for death or significant complication. PMID: 20103338 [PubMed - indexed for MEDLINE] (Source: The Annals of Thoracic Surgery)

Partial and transitional atrioventricular septal defect outcomes.

The Annals of Thoracic Surgery — Mon, 01 Feb 2010 00:00:00 +0100

CONCLUSIONS: Surgical repair for partial/transitional AVSD is associated with low morbidity and mortality, short hospital stays, and catch-up growth, particularly in underweight children repaired between 3 and 18 months of age. Left atrioventricular valve regurgitation remains the most common residual defect, occurring more frequently in children repaired after 4 years of age. PMID: 20103337 [PubMed - indexed for MEDLINE] (Source: The Annals of Thoracic Surgery)

Supravalvar Mitral Ring with Complete Atrioventricular Septal Defect: A Case Report and Three-Dimensional Echocardiography Evaluation

Journal of the American Society of Echocardiography — Mon, 01 Feb 2010 00:00:00 +0100

Supravalvar mitral ring (SVMR) is a rare cause of congenital mitral stenosis. It can occur in isolation but often coexists with other cardiac anomalies, such as a ventricular septal defect or left-sided obstructive lesions. Conversely, a complete atrioventricular septal defect (AVSD) is a much more common anomaly. An AVSD may be associated with other major cardiac defects, such as tetralogy of Fallot, transposition of the great arteries, or double-outlet right ventricle. The authors describe what they believe is the first case of SVMR and complete AVSD occurring together; the SVMR was diagnosed by two-dimensional echocardiography, and its morphology could be more accurately delineate using three-dimensional echocardiography. (Source: Journal of the American Society of Echocardiography)

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Long-term results after mitral valve repair in children [Original articles]

European Journal of Cardio-Thoracic Surgery — Thu, 28 Jan 2010 19:40:36 +0100

Conclusions: MV repair in children showed excellent survival, acceptable re-operation rate and satisfactory valve function at long-term follow-up. Residual MV dysfunction was a significant risk factor for re-operation, but re-repair was successful in more than half of the patients who underwent re-operation. (Source: European Journal of Cardio-Thoracic Surgery)

Re-repair of the left atrioventricular valve in atrioventricular septal defects: the morphologic approach to the role of Gore-tex band reduction annuloplasty [Original articles]

European Journal of Cardio-Thoracic Surgery — Thu, 28 Jan 2010 19:40:36 +0100

Conclusions: Left atrioventricular valve incompetence following previous repair usually involves repair of the zone of apposition between the left bridging leaflets. Many of these valves also require annuloplasty to attain competence. Given the shape, growth potential and morphologic composition of the annulus in these cases, we have performed some of these repairs with a partial flexible ring from a thin-walled 3.5-mm Gore-Tex graft as a reduction annuloplasty that can be shaped to fit the exact contours of this annulus. (Source: European Journal of Cardio-Thoracic Surgery)

Partial and Transitional Atrioventricular Septal Defect Outcomes [ORIGINAL ARTICLES: PEDIATRIC CARDIAC]

The Annals of Thoracic Surgery — Wed, 27 Jan 2010 19:27:19 +0100

Conclusions Surgical repair for partial/transitional AVSD is associated with low morbidity and mortality, short hospital stays, and catch-up growth, particularly in underweight children repaired between 3 and 18 months of age. Left atrioventricular valve regurgitation remains the most common residual defect, occurring more frequently in children repaired after 4 years of age. (Source: The Annals of Thoracic Surgery)

Biventricular Repair of Atrioventricular Septal Defect With Common Atrioventricular Valve and Double-Outlet Right Ventricle [ORIGINAL ARTICLES: PEDIATRIC CARDIAC]

The Annals of Thoracic Surgery — Wed, 27 Jan 2010 19:27:19 +0100

Conclusions Although technically challenging, the surgical repair can be accomplished with acceptable early results. Heterotaxy syndrome, with concurrent anomalous connections of the pulmonary veins, represented the strongest identified risk factor for death or significant complication. (Source: The Annals of Thoracic Surgery)

Cryothermal Catheter Ablation of Atrioventricular Nodal Reentrant Tachycardia in a Pediatric Patient after Atrioventricular Canal Repair

Congenital Heart Disease — Wed, 20 Jan 2010 00:00:00 +0100

We describe successful cryothermal modification of the slow atrioventricular nodal pathway in a 12-year-old patient with a previously repaired partial atrioventricular septal defect and inducible atrioventricular nodal reentrant tachycardia. The use of a steerable catheter to locate the displaced His signal combined with the use of cryothermal energy allowed for the safe and effective treatment of this patient's tachycardia. (Source: Congenital Heart Disease)

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Oral Manifestations in Ellis-van Creveld Syndrome: Report of a Case and Review of the Literature

Journal of Oral and Maxillofacial Surgery — Tue, 19 Jan 2010 14:19:34 +0100

Ellis-van Creveld syndrome (EVC), initially called chondroectodermal dysplasia, was first described in 1940 by the pediatricians Richard Ellis and Simon van Creveld. It is a rare recessive autosomal disorder characterized by chondrodysplasia, postaxial polydactyly, ectodermal dysplasia, and congenital malformations, most frequently an atrioventricular septal defect, which is observed in 60% of patients. Even though these 4 features classically define the syndrome, a variable spectrum of clinical manifestations is often present. (Source: Journal of Oral and Maxillofacial Surgery)

Umbilical vein anomaly in fetuses with Down syndrome

Ultrasound in Obstetrics and Gynecology — Tue, 05 Jan 2010 00:00:00 +0100

To describe the prevalence of abnormal umbilical vein (UV) anatomy in fetuses with Down syndrome.This was a retrospective survey covering a 24-month period of fetuses with a genetic diagnosis of Down syndrome following a routine early second-trimester (12-16-week) detailed fetal anomaly scan at a single academic tertiary referral center. In our unit this exam includes fetal umbilicoportal venous system evaluation.During the study period, 37 fetuses were diagnosed with Down syndrome and had a detailed early anatomy scan. In four (11%) the detailed early anomaly scan revealed that the UV was connected to the hepatic portion of the inferior vena cava (IVC) at a position lower than its usual site. Their average gestational age at diagnosis was 13 + 6 (range, 11 + 6 to 15 + 2) weeks. Three of t...

Electroanatomic Relationships in Patients With Primum Atrioventricular Septal Defect

Journal of the American College of Cardiology: Cardiovascular Imaging — Mon, 14 Dec 2009 22:01:29 +0100

Conclusions Abnormality in the position of the papillary muscles changes continuously with the abnormality of the QRS axis. Understanding the electroanatomic relationships provides important insight into developmental relationships between the conduction system and the trabecular structures in primum AVSD patients. These results may provide insights in understanding the continuity of primum AVSD abnormality, in estimating the best surgical approach, and predicting the prognosis of primum AVSD patients. (Source: Journal of the American College of Cardiology: Cardiovascular Imaging)

Atrioventricular septal defect with tetralogy of Fallot in patient with Down's syndrome

Revista Brasileira de Cirurgia Cardiovascular — Sat, 12 Dec 2009 16:02:07 +0100

We present a case of a 44-year-old man with prosthetic aortic endocarditis complicated by a perivalvular abscess. He evolved with improvement of the infectious process only under clinical treatment. The patient presented a prior history of rheumatic fever and had previously been undergone three valve replacements due to prosthesis dysfunction and previous endocarditis. In this case report we discuss the main features of perivalvular abscess complicating infective endocarditis (Source: Revista Brasileira de Cirurgia Cardiovascular)

Electrophysiologic and anatomical relationships studied in primum atrioventricular septal defect

Journal of Electrocardiology — Thu, 03 Dec 2009 00:00:00 +0100

This article presents a review of the anatomical and electrophysiologic characteristics of patients with primum AVSD and provides recent knowledge of electroanatomical relationships of the heart. (Source: Journal of Electrocardiology)

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Prenatal diagnosis of left atrial isomerism

Heart — Fri, 27 Nov 2009 18:01:44 +0100

Conclusion: Left atrial isomerism presents a varied spectrum of cardiac malformations when it is detected prenatally. Complete heart block, complex cardiac abnormalities and fetal hydrops are poor prognostic features. Those with only minor cardiac malformations are at risk postnatally for biliary atresia and for bowel obstruction due to malrotation. (Source: Heart)

Late Pulmonary Valve Replacement in Congenital Heart Disease Patients Without Original Congenital Pulmonary Valve Pathology

Pediatric Cardiology — Sat, 14 Nov 2009 06:58:50 +0100

This report describes five cases of patients with a history of congenital heart surgery but no congenital pulmonary valve pathology who required pulmonary valve replacement due to the consequences of prolonged pulmonary regurgitation. Although pulmonary regurgitation may be well tolerated for many years, it is further evidence for the importance of close follow-up assessment and monitoring of young adults with congenital heart disease. Content Type Journal ArticleCategory Original ArticleDOI 10.1007/s00246-009-9574-3Authors Brian E. Kogon, Emory University School of Medicine, Children’s Healthcare of Atlanta Division of Cardiothoracic Surgery Atlanta GA USAManisha Patel, Children’s Healthcare of Atlanta Sibley Cardiology Atlanta GA USAMaria Pernetz, Emory University School of ...

Noninvasive Assessment of Pulmonary Artery Flow and Resistance by Cardiac Magnetic Resonance in Congenital Heart Diseases With Unrestricted Left-to-Right Shunt

Journal of the American College of Cardiology: Cardiovascular Imaging — Mon, 09 Nov 2009 22:01:22 +0100

Conclusions Measurement of Qp or left-to-right shunt noninvasively by CMR has potential to predict the PVR in patients with an unrestricted left-to-right shunt and could potentially determine operability without having to undertake invasive testing. (Source: Journal of the American College of Cardiology: Cardiovascular Imaging)

Variation in the Prevalence of Congenital Heart Defects by Maternal Race/Ethnicity and Infant Sex

The Journal of Pediatrics — Mon, 12 Oct 2009 00:00:00 +0100

Conclusions: We found differences in ethnic susceptibilities to CHD by sex, but the cause remains unclear. (Source: The Journal of Pediatrics)

Two-patch repair for atrioventricular septal defect with mitral aneurysm.

The Annals of Thoracic Surgery — Thu, 24 Sep 2009 02:17:01 +0100

Authors: Imura H, Sakamoto S, Maruyama Y, Ochi M, Shimizu K We experienced an unusual case of partial atrioventricular septal defect in an elderly patient. A preoperative ultrasonic cardiogram revealed the mitral leaflet pouching toward the right atrium and suggested the presence of a ventricular septal defect underneath the atrioventricular valve. The mitral aneurysm was diagnosed as a septal aneurysm on preoperative ultrasonic cardiogram. A crescent-shaped Dacron patch (InterVascular S. A., La Ciotat Cedex, France) was placed beneath the atrioventricular valve to prevent rupture of the mitral aneurysm and support the anterior mitral leaflet by creating a new annulus. We believe that this is the first report describing this type of mitral aneurysm and its surgical repair. PMID: 19...

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Two-Patch Repair for Atrioventricular Septal Defect With Mitral Aneurysm [CASE REPORTS]

The Annals of Thoracic Surgery — Mon, 21 Sep 2009 23:00:00 +0100

We experienced an unusual case of partial atrioventricular septal defect in an elderly patient. A preoperative ultrasonic cardiogram revealed the mitral leaflet pouching toward the right atrium and suggested the presence of a ventricular septal defect underneath the atrioventricular valve. The mitral aneurysm was diagnosed as a septal aneurysm on preoperative ultrasonic cardiogram. A crescent-shaped Dacron patch (InterVascular S. A., La Ciotat Cedex, France) was placed beneath the atrioventricular valve to prevent rupture of the mitral aneurysm and support the anterior mitral leaflet by creating a new annulus. We believe that this is the first report describing this type of mitral aneurysm and its surgical repair. (Source: The Annals of Thoracic Surgery)

Journal Scan: Left Ventricular Morphology Influences Mortality After the Norwood Operation (Heart 2009;95:1238-1244.)

Cardiosource — Thu, 17 Sep 2009 12:15:10 +0100

A retrospective review was performed at a single center. Patients with HLHS undergoing stage I Norwood procedures were identified. Exclusion criteria included heterotaxy syndrome, unbalanced atrioventricular septal defect, ventricular septal defect greater than 2 mm, and critical aortic stenosis wit. . . (Source: Cardiosource)

Spectrum of congenital heart defects in Croatia

European Journal of Pediatrics — Wed, 16 Sep 2009 19:02:58 +0100

Abstract The aim of our study was to investigate the incidence of congenital defects in children born in Croatia during a period of 5 years, its association with extracardiac malformations, its treatment, and outcome. Medical information about the patients was obtained from 14 paediatric cardiology centres that cover the whole country. Diagnosis was made by clinical findings, electrocardiography, chest X-ray, echocardiography, catheterisation, or autopsy. Between October 1, 2002 and October 1, 2007, there were 205,051 live births in Croatia, 1,480 of which were patients diagnosed with congenital heart disease, accounting for 0.72% of the live-born children. The distribution was made up of 34.6% children with ventricular septal defect, 15.9% with atrial septal defect, ...

Pulmonary stenosis development and reduction of pulmonary arterial hypertension in atrioventricular septal defect: a case report

Journal of Cardiothoracic Surgery — Tue, 15 Sep 2009 23:00:00 +0100

A 24-year-old patient was admitted for dyspnoea and syncope. He had a previous history of complete atrio-ventricular septal defect and trisomy 21.At the age of 6 months, in 1984, cardiac catheterization revealed a quasi-systemic pulmonary arterial hypertension with a bidirectional shunt corresponding to an Eisenmenger syndrome. Corrective cardiac surgery was not performed at this time because surgical risk was considered too high. Until the age of 20 years old, he showed few symptoms while under medical treatment. But since 2006, his functional status became worse with an increased dyspnoea , syncopes, and severe cyanosis. In these conditions, haemodynamic parameters have been re-evaluated in 2006 and 2008 .They highlighted a late and progressive development of a valvular and infundibular ...

Microarray based analysis of 3p25-p26 deletions (3p- syndrome)

American Journal of Medical Genetics Part A — Tue, 15 Sep 2009 23:00:00 +0100

Distal deletion of chromosome 3p25-pter (3p- syndrome) produces a distinct clinical syndrome characterized by low birth weight, mental retardation, telecanthus, ptosis, and micrognathia. Congenital heart disease (CHD), typically atrioventricular septal defect (AVSD) occurs in about a third of patients. Previously we reported on an association between the presence of CHD and the proximal extent of the deletion such that a CHD susceptibility gene was mapped between D3S1263 and D3S3594. In addition, we and others have suggested several candidate genes for the psychomotor retardation usually seen with constitutional 3p25 deletions. In order to further investigate genotype-phenotype correlations in 3p- syndrome we analyzed 14 patients with cytogenetically detectable deletions of 3p25 (including...

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Isolated Cleft of the Mitral Valve: Distinctive Features and Surgical Management [ORIGINAL ARTICLES: PEDIATRIC CARDIAC]

The Annals of Thoracic Surgery — Sun, 30 Aug 2009 23:00:00 +0100

Conclusions Echocardiographic description of isolated cleft of the mitral valve is not always as accurate as intraoperative analysis. This is a distinct morphologic entity from the cleft of the left-sided valve of atrioventricular septal defect, and seems associated with a strong female predominance, with various cardiac and extracardiac features. Surgical repair is successful with excellent midterm results. (Source: The Annals of Thoracic Surgery)

Isolated cleft of the mitral valve: distinctive features and surgical management.

The Annals of Thoracic Surgery — Tue, 25 Aug 2009 16:24:29 +0100

CONCLUSIONS: Echocardiographic description of isolated cleft of the mitral valve is not always as accurate as intraoperative analysis. This is a distinct morphologic entity from the cleft of the left-sided valve of atrioventricular septal defect, and seems associated with a strong female predominance, with various cardiac and extracardiac features. Surgical repair is successful with excellent midterm results. PMID: 19699908 [PubMed - in process] (Source: The Annals of Thoracic Surgery)

Partial atrioventricular septal defect in the fetus: diagnostic features and associations in a multicenter series of 30 cases.

The Ultrasound Review of Obstetrics and Gynecology — Sun, 23 Aug 2009 23:00:00 +0100

CONCLUSIONS: We describe the key echocardiographic features of pAVSD in the fetus. In addition, we have confirmed that the association with trisomy 21 holds also for pAVSD, though to a lesser extent, with a 12.5% association rate in this series. In the fetus, pAVSD seems to be associated with a high rate of chromosomal/non-chromosomal syndromic conditions, including skeletal dysplasias. Inutero, aortic coarctation represents the most frequently associated cardiac lesion (13.3%). Copyright (c) 2009 ISUOG. Published by John Wiley & Sons, Ltd. PMID: 19705406 [PubMed - as supplied by publisher] (Source: The Ultrasound Review of Obstetrics and Gynecology)

Prevalence of congenital heart defects in carriers of Down syndrome in the municipality of Pelotas, Brazil.

Jornal de Pediatria — Wed, 05 Aug 2009 23:00:00 +0100

CONCLUSIONS: The prevalence of Down syndrome and congenital heart defect in our region is similar to the rates found by other authors; therefore, we highlight the importance of diagnostic suspicion and early referral by pediatricians to cardiac evaluation. Another relevant aspect is the small number of patients who underwent karyotype testing. In addition, the number of associated malformations was lower than that found by other authors. PMID: 19662319 [PubMed - as supplied by publisher] (Source: Jornal de Pediatria)

Med Sci Monit 2009; 15(8):CR409-412 "Down’s syndrome and the pattern of congenital heart disease in a community with high parental consanguinity"

Medical Science Monitor — Fri, 31 Jul 2009 11:40:24 +0100

Conclusions: A slightly higher frequency of CHD was documented in DS children from this population with a high consanguineous marriage rate. The frequencies of specific lesions were similar to those reported locally and internationally; VSD was the most frequently detected in this study. Interesting was the predominance of left-right shunt lesions and the relative rarity of cyanotic and complex CHD in this DS population. (Source: Medical Science Monitor)

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Modified single-patch technique: Repairing complete atrioventricular septal defect

Annals of Pediatric Cardiology — Thu, 30 Jul 2009 10:45:48 +0100

Carl Lewis Backer, Sunjay Kaushal, Constantine MavroudisAnnals of Pediatric Cardiology 2009 2(1):51-54 (Source: Annals of Pediatric Cardiology)

Surgery for complete atrioventricular septal defect: Is a uniform strategy applicable?

Annals of Pediatric Cardiology — Thu, 30 Jul 2009 10:45:48 +0100

Sachin Talwar, Shiv Kumar Choudhary, Balram AiranAnnals of Pediatric Cardiology 2009 2(1):58-60 (Source: Annals of Pediatric Cardiology)

Reply

The Journal of Pediatrics — Wed, 22 Jul 2009 11:28:22 +0100

Discussion section, there were no infants with an atrioventricular septal defect in our study, and thus the potential “added value” of ECG with regard to this diagnosis could not be demonstrated. There were also no infants in our study with tricuspid atresia or Wolff-Parkinson-White syndrome. Our study population (n = 201) was an unselected group of consecutively enrolled neonates seen in an outpatient setting for a murmur assessment, and we believe this is representative of neonates referred to pediatric cardiology programs. (Source: The Journal of Pediatrics)

Recurrence of Congenital Heart Defects in Families.

Circulation — Sun, 12 Jul 2009 23:00:00 +0100

Conclusions-Specific CHDs showed highly variable but strong familial clustering in first-degree relatives, ranging from 3-fold to 80-fold compared with the population prevalence, whereas the crossover risks between dissimilar cases of CHD were weaker. Family history of any CHD among first-degree relatives accounted for a small proportion of CHD cases in the population. PMID: 19597048 [PubMed - as supplied by publisher] (Source: Circulation)

Results of surgical repair of atrioventricular septal defect with double-orifice left atrioventricular valve

The Journal of Thoracic and Cardiovascular Surgery — Sun, 28 Jun 2009 23:00:00 +0100

Conclusion: Atrioventricular septal defect with double-orifice left atrioventricular valve can be repaired with low mortality. However, double-orifice left atrioventricular valve is a predictor for reoperation. The accessory orifice is often competent and should then be left untouched. If regurgitation of the accessory orifice is present, this is best managed with suture or patch closure. (Source: The Journal of Thoracic and Cardiovascular Surgery)

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Biventricular repair in unbalanced atrioventricular septal defect

Arquivos Brasileiros de Cardiologia — Sun, 28 Jun 2009 15:02:51 +0100

We present the case of a 2.5 year-old child with unbalanced atrioventricular septal defect due to a small left ventricle (LV) (mitral annulus of 10mm and a 0.4 ratio in relation to the tricuspid annulus, LVDD: 17 mm, LV Vd2: 15 ml/m² and LV/RV long-axis ratio of 0.71); he had a favorable outcome after biventricular surgical repair. Normal LV development was observed three months after the operation (mitral annulus of 22 mm, with a 0.84 ratio in relation to the tricuspid annulus, and LVDD of 30 mm). Current parameters for utilization of the hypoplastic ventricle are discussed.Presenta evolución favorable después de corrección operatoria biventricular, de niño con 2,5 años de edad, con Defecto del Septo Atrioventricular Desbalanceado con ventrículo izquierdo (VI) pequeño (anillo mitr...

Acquired left atrial-to-right ventricular shunt with mitral valve incompetence: a rare sequela after repair of atrioventricular septal defect.

Texas Heart Institute Journal — Sat, 27 Jun 2009 15:04:03 +0100

Authors: Mohapatra S, Minhas HS, Virmani S, Mishra BB, Mukherjee K, Banerjee A Acquired left ventricular-to-right atrial communication is encountered periodically. This condition is chiefly attributable to surgical mishaps, trauma, endocarditis, or endomyocardial biopsy. In a few instances, a Gerbode-like defect develops after the repair of an atrioventricular septal defect. Our search of the worldwide medical literature revealed just 1 report of a "mirror" occurrence of a Gerbode-like defect: a shunt between the left atrium and the right ventricle. Herein, we present the case of a 22-year-old woman who had severe mitral valve incompetence accompanying an acquired shunt between the left atrium and the right ventricle-a late sequela of the earlier repair of an atrioventricular septal de...

Modified single-patch technique: Repairing complete atrioventricular septal defect

Annals of Pediatric Cardiology — Thu, 25 Jun 2009 07:26:53 +0100

Backer Carl Lewis, Kaushal Sunjay, Mavroudis ConstantineAnnals of Pediatric Cardiology 2009 2(1):51-54 (Source: Annals of Pediatric Cardiology)

Surgery for complete atrioventricular septal defect: Is a uniform strategy applicable?

Annals of Pediatric Cardiology — Thu, 25 Jun 2009 07:26:53 +0100

Talwar Sachin, Choudhary Shiv Kumar, Airan BalramAnnals of Pediatric Cardiology 2009 2(1):58-60 (Source: Annals of Pediatric Cardiology)

[Institutional report - Congenital] Outcome after reoperation for atrioventricular septal defect repair

Interactive CardioVascular and Thoracic Surgery — Sun, 21 Jun 2009 23:00:00 +0100

This report describes our experience in 59 patients who underwent reoperation after AVSD repair, between 1977 and 2008. Thirty-one patients had a PAVSD, 28 had a CAVSD. Mean interval between initial repair and reoperation was 10±11 years (PAVSD vs. CAVSD: 13±12 vs. 6±9 years, P=0.063). Reoperations were required for left atrioventricular valve regurgitation (LAVVR) in 53 patients (combined with right atrioventricular valve regurgitation in 10, atrial septal defect (ASD) in 11, ventricular septal defect (VSD) in 7, left ventricular outflow tract (LVOT) obstruction in 1, and aortic valve stenosis in 1), ASD in 3, and LVOT obstruction in 3. Valve repair was performed in 45 patients and replacement in 8. Repair techniques of the left-sided atrioventricular valve ...

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[eComment] eComment: Discrete subaortic stenosis following repair of atrioventricular septal defects

Interactive CardioVascular and Thoracic Surgery — Sun, 21 Jun 2009 23:00:00 +0100

(Source: Interactive CardioVascular and Thoracic Surgery)

Outcome after reoperation for atrioventricular septal defect repair [Institutional report - Congenital]

Interactive CardioVascular and Thoracic Surgery — Sun, 21 Jun 2009 23:00:00 +0100

eComment: Discrete subaortic stenosis following repair of atrioventricular septal defects [eComment]

Interactive CardioVascular and Thoracic Surgery — Sun, 21 Jun 2009 23:00:00 +0100

(Source: Interactive CardioVascular and Thoracic Surgery)

Results of Left Atrioventricular Valve Reoperations Following Previous Repair of Atrioventricular Septal Defects

Journal of Cardiac Surgery — Sun, 14 Jun 2009 23:00:00 +0100

Conclusions: LAVVR results in significant clinical improvement and lasting recovery in ventricular chamber function and size. Valve repair offers survival advantage and should be aggressively attempted; however, it is only achievable in 50% of cases. Valve replacement is necessary in cases associated with complex LAVV morphology or following repair failure. At intermediate follow-up, patients continue to be at risk of major valve-related morbidity, requirement for re-intervention, and cardiac death. (J Card Surg ****;**:**-**) (Source: Journal of Cardiac Surgery)

RTN3 inducing apoptosis is modulated by an adhesion protein CRELD1.

Molecular and Cellular Biochemistry — Thu, 11 Jun 2009 23:00:00 +0100

Authors: Xiang R, Zhao S Reticulon3 (RTN3), as a member of the reticulon family, is generally regarded as a novel human apoptosis-inducing protein. But the extensional role of RTN3 remains virtually unknown. Herein, we showed that cysteine rich with EGF like domains 1(CRELD1), a cell adhesion molecule played a critical role in atrioventricular septal defects and it had mutual effect with RTN3 in vitro. Furthermore, we discovered that ectopic CRELD1 could interact with ectopic or endogenous RTN3. CRELD1 bound with RTN3 so as to increase the localization of RTN3 on the plasma membrane and decreased the apoptotic activity of RTN3 moderately. Moreover, the tunicamycin-inducing cell apoptosis was partly suppressed by this kind of interaction mentioned above. These results suggested that CRE...

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Left cardiac isomerism in the Sonic hedgehog null mouse.

Journal of Anatomy — Sun, 31 May 2009 23:00:00 +0100

In this study, we describe the morphology of the atrial chambers and atrioventricular junctions of the Shh null mouse heart. We demonstrate that the essential phenotypic feature is isomerism of the left atrial appendages, in combination with an atrioventricular septal defect and a common atrioventricular junction. These malformations are known to be frequent in humans with left isomerism. To confirm the presence of left isomerism, we show that Pitx2c, a recognized determinant of morphological leftness, is expressed in the Shh null mutants on both the right and left sides of the inflow region, and on both sides of the solitary arterial trunk exiting from the heart. It has been established that derivatives of the second heart field expressing Isl1 are asymmetrically distributed in the develo...

[Pulmonary artery banding operation and results of terminal biventricular and univentricular repair]

Anadolu Kardiyol Der... — Sun, 31 May 2009 23:00:00 +0100

CONCLUSION: Pulmonary artery banding operation has still a significant role in the palliation of certain congenital cardiac anomalies. Outcome of patients who are candidates for biventricular repair is better than the univentricular repairs. PMID: 19520656 [PubMed - in process] (Source: Anadolu Kardiyol Der...)

[ORIGINAL ARTICLES: PEDIATRIC CARDIAC] Reoperations After Initial Repair of Complete Atrioventricular Septal Defect

The Annals of Thoracic Surgery — Thu, 21 May 2009 04:00:00 +0100

Conclusions The most common indication for reoperation after CAVSD repair is LAVV regurgitation. LAVV re-repair offers good durability, and LAVV replacement does not preclude additional reoperations. Long-term survival is very good despite need for multiple reoperations in some. (Source: The Annals of Thoracic Surgery)

Reoperations After Initial Repair of Complete Atrioventricular Septal Defect [ORIGINAL ARTICLES: PEDIATRIC CARDIAC]

The Annals of Thoracic Surgery — Wed, 20 May 2009 23:00:00 +0100

[Original articles] Mid-term results of right axillary incision for the repair of a wide range of congenital cardiac defects

European Journal of Cardio-Thoracic Surgery — Thu, 30 Apr 2009 04:00:00 +0100

Conclusions: The right axillary incision provides a quality of repair for various congenital defects similar to that obtained by using standard surgical approaches. Because of its deceitful location, and the camouflaging effect of being hidden by the resting arm, it has superior cosmetic appeal compared to conventional incisions. The incision does not interfere with subsequent development of the thorax or the breast (in case of females). (Source: European Journal of Cardio-Thoracic Surgery)

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[Review] Current options and outcomes for the management of atrioventricular septal defect

European Journal of Cardio-Thoracic Surgery — Thu, 30 Apr 2009 04:00:00 +0100

This article reviews our current understanding of the morphology of this defect, aspects of diagnosis and surgical treatment options. (Source: European Journal of Cardio-Thoracic Surgery)

Oral contraceptives/prednisone: Fatal thromboembolism in a patient with repaired atrioventricular septal defect: case report.

Reactions Weekly — Wed, 29 Apr 2009 00:48:54 +0100

Page: 33 (Source: Reactions Weekly)

Oral contraceptives/prednisone: Fatal thromboembolism in a patient with repaired atrioventricular septal defect: case report

Reactions — Sun, 26 Apr 2009 04:55:40 +0100

(Source: Reactions)

Torsades de Pointes in atrioventricular septal defect

International Journal of Cardiology — Fri, 24 Apr 2009 09:02:24 +0100

Abstract: Complete heart block is a known association of atrioventricular septal defect. Bradycardia secondary to heart block can lead to abnormal QT prolongation and precipitate Torsades de Pointes and arrest. Here, a case report of these events is described, together with a brief literature review. (Source: International Journal of Cardiology)

[ORIGINAL ARTICLES: PEDIATRIC CARDIAC] Common Arterial Trunk With Atrioventricular Septal Defect: New Observations Pertinent to Repair

The Annals of Thoracic Surgery — Tue, 21 Apr 2009 04:00:00 +0100

Conclusions Owing to the unique ventriculoarterial connection, the surgeon, considering anatomical repair, needs to pay attention to the anterosuperior margin of the ventricular scoop, which determines the adequacy of left ventricular outflow size. (Source: The Annals of Thoracic Surgery)

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Common Arterial Trunk With Atrioventricular Septal Defect: New Observations Pertinent to Repair [ORIGINAL ARTICLES: PEDIATRIC CARDIAC]

The Annals of Thoracic Surgery — Mon, 20 Apr 2009 23:00:00 +0100

Cardiac Surgery in Patients with Trisomy 18

Pediatric Cardiology — Thu, 02 Apr 2009 05:55:14 +0100

Abstract Cardiac surgery is infrequently but increasingly being used to repair congenital heart defects associated with trisomy 18. The clinical details of trisomy 18 patients undergoing cardiac surgery have rarely been reported. Seventeen patients with trisomy 18 and serious cardiac symptoms underwent cardiac surgery in our institution. Age at surgery ranged from 7 to 258 days (median, 66 days). One patient had an atrioventricular septal defect and coarctation of the aorta. The remaining patients had ventricular septal defects, including four patients with coarctation of the aorta. Fourteen patients had associated patent ductus arteriosus. Fourteen patients underwent palliative surgery without cardiopulmonary bypass, and four of these underwent a second-stage in...

Combination of aortopulmonary window and complete atrioventricular septal defect in a patient with heterotaxy syndrome.

The Annals of Thoracic Surgery — Wed, 01 Apr 2009 04:00:00 +0100

Authors: Mirzaaghayan MR, Shabanian R, Kiani A A rare combination of aortopulmonary window and complete atrioventricular septal defect diagnosed in a 2-month-old infant with heterotaxy syndrome is presented. Being aware of this combination of cardiac anomalies before surgical intervention is crucial for perioperative anesthetic technique and preservation of the myocardium. PMID: 19324181 [PubMed - in process] (Source: The Annals of Thoracic Surgery)

Transannular patching is a valid alternative for tetralogy of Fallot and complete atrioventricular septal defect repair.

The Journal of Thoracic and Cardiovascular Surgery — Wed, 01 Apr 2009 04:00:00 +0100

CONCLUSIONS: Tetralogy of Fallot associated with complete atrioventricular septal defect can be corrected at low risk with favorable intermediate survival. Use of right ventricle-to-pulmonary artery conduit can be avoided in two thirds of patients with no impact on survival, possibly improving overall freedom from reintervention. PMID: 19327518 [PubMed - in process] (Source: The Journal of Thoracic and Cardiovascular Surgery)

[Original articles] Outcomes following surgical repair of aortic arch obstructions with associated cardiac anomalies

European Journal of Cardio-Thoracic Surgery — Mon, 30 Mar 2009 04:00:00 +0100

Conclusions: The type of associated intracardiac anomalies with arch obstruction influenced postoperative outcomes. Although both approaches could achieve good results, our data suggest that single-stage repair for arch obstruction with VSD may be associated with better survival, and is therefore recommended. (Source: European Journal of Cardio-Thoracic Surgery)

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[CASE REPORTS] Combination of Aortopulmonary Window and Complete Atrioventricular Septal Defect in a Patient With Heterotaxy Syndrome

The Annals of Thoracic Surgery — Wed, 25 Mar 2009 04:00:00 +0100

A rare combination of aortopulmonary window and complete atrioventricular septal defect diagnosed in a 2-month-old infant with heterotaxy syndrome is presented. Being aware of this combination of cardiac anomalies before surgical intervention is crucial for perioperative anesthetic technique and preservation of the myocardium. (Source: The Annals of Thoracic Surgery)

[Institutional report - Congenital] Surgical outcomes of the modified single-patch technique in complete atrioventricular septal defect

Interactive CardioVascular and Thoracic Surgery — Fri, 20 Mar 2009 04:00:00 +0100

We examined the usefulness of the modified single-patch technique for the surgical management of complete atrioventricular septal defect (AVSD). Sixty-one patients undergoing total correction for complete AVSD from January 1997 to December 2006 were classified to the modified single-patch technique group (18 patients) and the classical one-/two-patch technique group (43 patients). The surgical outcomes of the modified single-patch technique were compared with those of the classical-patch technique. Aortic cross-clamp time was shorter in the modified single-patch technique group (110.8±27.5 min vs. 134.4±42.5 min, P=0.03). During the follow-up period, two patients required reoperation for atrioventricular valve dysfunction in the modified single-patch technique group...

National time trends in congenital heart defects, Denmark, 1977-2005.

American Heart Journal — Sun, 01 Mar 2009 05:00:00 +0100

CONCLUSIONS: CHD birth prevalence increased from the beginning of the 1980s but stabilized in the late 1990s. PMID: 19249416 [PubMed - as supplied by publisher] (Source: American Heart Journal)

Specific issues after surgical repair of partial atrioventricular septal defect: Actuarial survival, freedom from reoperation, fate of the left atrioventricular valve, prevalence of left ventricular outflow tract obstruction, and other events.

The Journal of Thoracic and Cardiovascular Surgery — Sun, 01 Mar 2009 05:00:00 +0100

CONCLUSIONS: Detailed assessment of the valve morphology and individualized valvuloplasty techniques improves the long-term survival after repair of partial atrioventricular septal defects. The presence of grossly malformed left valvular apparatus, pulmonary artery hypertension, and moderate-to-severe left atrioventricular valve regurgitation are independent predictors of death and defect-related morbidity after surgical repair. PMID: 19258063 [PubMed - in process] (Source: The Journal of Thoracic and Cardiovascular Surgery)