MedWorm: Atrioventricular Septal Defect

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)

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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...

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)

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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)

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 - in process] (Source: American Heart Journal)

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Pericardial patch repair of the left atrioventricular valve in atrioventricular septal defect: long-term changes in the patch

Cardiovascular Pathology — Sun, 01 Mar 2009 05:00:00 +0100

Abstract: A 31-year-old woman with partial atrioventricular septal defect underwent left atrioventricular valve (LAVV) replacement. Her initial repair was at 8 years of age. At 23 years of age, she underwent reoperation due to a combination of severe left ventricular outlet obstruction and moderate LAVV regurgitation. At that reoperation, she had a Dacron patch enlargement of the infundibular septum and repair of her LAVV with a xenograft (bovine) pericardial patch sutured into the superior bridging leaflet. LAVV replacement was required 8 years later because of valve insufficiency. There was a perforation in the patch with fibrosis, thickening due to pannus, and calcification of the pericardial tissue and the leaflet tissue, leading to stiffening of the tissue. (Source: Cardiovascular Pat...

Cardiac abnormalities of Sudanese patients with Down's syndrome and their short-term outcome.

Cardiovascular Journal of Africa — Sun, 01 Mar 2009 00:00:00 +0100

CONCLUSION: The pattern of CHD in Sudanese patients with DS was comparable with that in the literature, including the rare occurrence of AVSD with intact atrial septum. In addition, we described an unreported association with right atrioventricular valve malformation. Although there was a significant delay in diagnosis and surgery, surgical results and short-term follow up were good. PMID: 19421645 [PubMed - in process] (Source: Cardiovascular Journal of Africa)

International Mosaic Down Syndrome Association Student Research Scholarship

ScanGrants feed — Tue, 24 Feb 2009 05:00:00 +0100

The International Mosaic Down Syndrome Association is a non-profit organization designed to providesupport, information, and research to those touched by mosaic Down syndrome. To increase the numberof studies on mosaic Down syndrome (mDs) and Down syndrome (Ds), WE WILL BE OFFERINGTWO $500 SCHOLARSHIPS TO MASTER’S LEVEL STUDENTS STUDYING MOSAICDOWN SYNDROME OR RELATED TOPICS.Applications will be accepted twice a year (September 15th and March 15th) and awardees will beselected within one month of the application deadline (October 15th and April 15th). Two scholarshipswill be awarded in each 12 month period.Topics for research are not limited to mDs, but can include studies on individual clinical features of mDs(ie. atrioventricular septal defects, autism, thyroid disorders). Chosen s...

Conotruncal defects associated with anomalous pulmonary venous connections.

Archives of Cardiovascular Diseases — Sun, 01 Feb 2009 05:00:00 +0100

CONCLUSION: Our findings suggest that congenital heart defects that associate anomalies of the arterial and venous poles may have a common embryology, which results from a myocardial defect. PMID: 19303577 [PubMed - as supplied by publisher] (Source: Archives of Cardiovascular Diseases)

Outcomes for Patients with Unbalanced Atrioventricular Septal Defects

Pediatric Cardiology — Fri, 30 Jan 2009 09:03:47 +0100

In conclusion, infants with an unbalanced AVSD are a high-risk population with diminished midterm survival compared with palliated patients who have more classic forms of hypoplastic heart syndromes. This may be due to the higher incidence of both severe atrioventricular valve regurgitation and important associated congenital anomalies. Content Type Journal ArticleCategory Original ArticleDOI 10.1007/s00246-008-9376-zAuthors Gabe E. Owens, University of Michigan Michigan Congenital Heart Center Ann Arbor MI USACarlen Gomez-Fifer, University of Michigan Michigan Congenital Heart Center Ann Arbor MI USASarah Gelehrter, University of Michigan Michigan Congenital Heart Center Ann Arbor MI USASonal T. Owens, University of Michigan Michigan Congenital Heart Center Ann Arbor MI USA ...

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Is the Prevalence of Specific Types of Congenital Heart Defects Different for Non-Hispanic White, Non-Hispanic Black and Hispanic Infants?

Maternal and Child Health Journal — Sat, 24 Jan 2009 12:16:08 +0100

Conclusions Although few racial/ethnic differences in prevalence are present among infants with major CHD, observed differences are clinically meaningful. However, the underlying etiologies for the observed differences remain unknown. Content Type Journal ArticleDOI 10.1007/s10995-009-0442-9Authors Wendy N. Nembhard, University of South Florida Department of Epidemiology & Biostatistics, College of Public Health 13201 Bruce B. Downs Blvd, MDC 56 Tampa FL 33612-3805 USAJason L. Salemi, University of South Florida Department of Epidemiology & Biostatistics, College of Public Health 13201 Bruce B. Downs Blvd, MDC 56 Tampa FL 33612-3805 USATao Wang, University of South Florida Department of Epidemiology & Biostatistics, College of Public Health 13201 Bruce B. Downs Blvd, MDC 56 Tampa FL ...

[Case report - Congenital] Emergency redo mitral valve replacement and caesarean section in a patient with previous atrioventricular septal defect repair in childhood

Interactive CardioVascular and Thoracic Surgery — Fri, 02 Jan 2009 05:00:00 +0100

A 19-year-old primigravida, with a history of mechanical mitral prosthesis at the age of six years following previous repair of an atrioventricular septal defect, presented at 26 weeks' gestation with cardiogenic shock secondary to obstruction of the mitral prosthesis. She underwent successful emergency redo mitral valve replacement and caesarean section. At operation the prosthesis was found to be virtually completely obstructed with pannus. Both mother and baby had an excellent outcome following surgery. The case report is presented with a brief review of the literature. (Source: Interactive CardioVascular and Thoracic Surgery)

Morphologic features of atrioventricular septal defect with only ventricular component: further observations pertinent to surgical repair.

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

CONCLUSIONS: Our observations suggest this entity might represent the mildest end of the whole spectrum of hearts with atrioventricular septal defect. Since "upwardly" displaced leaflets are not modifiable and could be aggravated further after surgery, they might play a role in late valve dysfunction. PMID: 19154915 [PubMed - in process] (Source: The Journal of Thoracic and Cardiovascular Surgery)

[Original articles] Late risk of outcomes for adults with repaired tetralogy of Fallot from an inception cohort spanning four decades

European Journal of Cardio-Thoracic Surgery — Wed, 31 Dec 2008 05:00:00 +0100

Conclusions: Surgical progress has not influenced late risks for death, re-operation or PVR in adults with repaired tetralogy of Fallot. Instead, reduction of early surgical mortality to <2% is responsible for excellent late survival >90% overall. The constant risk of PVR is low and independent of repair type. Baseline morphologic features are important determinants of late outcome. (Source: European Journal of Cardio-Thoracic Surgery)

Ventricular scoop in atrioventricular septal defect: relevance to simplified single-patch method.

The Annals of Thoracic Surgery — Wed, 24 Dec 2008 10:24:10 +0100

CONCLUSIONS: The antero-superiorly extended and skewed scoop could lead to asymmetric configuration of the valvar leaflets and outflow tract obstruction if the simplified technique is applied. Therefore, not only scoop depth but also the antero-superior extension should be recognized when repairing this lesion. PMID: 19101296 [PubMed - in process] (Source: The Annals of Thoracic Surgery)

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[ORIGINAL ARTICLES: PEDIATRIC CARDIAC] Ventricular Scoop in Atrioventricular Septal Defect: Relevance to Simplified Single-Patch Method

The Annals of Thoracic Surgery — Tue, 23 Dec 2008 05:00:00 +0100

Conclusions The antero-superiorly extended and skewed scoop could lead to asymmetric configuration of the valvar leaflets and outflow tract obstruction if the simplified technique is applied. Therefore, not only scoop depth but also the antero-superior extension should be recognized when repairing this lesion. (Source: The Annals of Thoracic Surgery)

Effectiveness of prenatal diagnosis of congenital heart defects in South Australia: A population analysis 1999–2003

The Australian and New Zealand Journal of Obstetrics and Gynaecology — Mon, 22 Dec 2008 05:00:00 +0100

Conclusion: Routine obstetric ultrasound detects CHD infrequently. Outflow tract lesions are most commonly missed. Fetal echocardiography is an accurate tool for prenatal diagnosis of CHD. Pregnancy outcomes after prenatal diagnosis are comparable to other Australasian and western countries, suggesting uniformity in parental counselling and population behaviour. Strategies that improve routine obstetric ultrasound detection of CHD are likely to yield the greatest impact. (Source: The Australian and New Zealand Journal of Obstetrics and Gynaecology)

Atrioventricular septal defect. In: ACC/AHA 2008 guidelines for the management of adults with congenital heart disease. A report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines on the Management of Adults With Congenital Heart Disease).

National Guideline Clearinghouse (NGC) — Tue, 02 Dec 2008 05:00:00 +0100

(Source: National Guideline Clearinghouse (NGC))

Borderline hypoplasia of the left ventricle in neonates: Insights for decision-making from functional assessment with magnetic resonance imaging.

The Journal of Thoracic and Cardiovascular Surgery — Mon, 01 Dec 2008 05:00:00 +0100

CONCLUSIONS: Magnetic resonance imaging is feasible in neonates with borderline left ventricular hypoplasia. Echocardiography does not accurately measure left ventricular hypoplasia in these patients and may unfairly preclude some patients from a biventricular repair in whom magnetic resonance is reassuring. PMID: 19114185 [PubMed - in process] (Source: The Journal of Thoracic and Cardiovascular Surgery)

Implantation of drug-eluting stents for relief of obstructed infra-cardiac totally anomalous pulmonary venous connection in isomerism of the right atrial appendages

Cardiology in the Young — Wed, 19 Nov 2008 05:51:25 +0100

We describe an infant with severe obstruction of infra-cardiac totally anomalous pulmonary venous connection associated with right isomerism, atrioventricular septal defect, pulmonary atresia, and multiple aortopulmonary collateral arteries. Implantation of a stent into the obstructed descending vertical vein provided effective palliation, with a dramatic increase in saturations of oxygen obviating the need for urgent high-risk surgery. (Source: Cardiology in the Young)

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[REVIEW PAPER] Surgical Anatomy of Atrioventricular Septal Defect

Asian Cardiovascular and Thoracic Annals — Tue, 04 Nov 2008 05:00:00 +0100

This review aims to describe the anatomic spectrum of hearts classified with the collective term atrioventricular septal defect. Despite their anatomical variety, hearts with the stigmata of atrioventricular septal defect share the characteristic feature of a common atrioventricular junction guarded by a 5-leaflet valve. The lack of normal atrioventricular septation makes the aorta un-wedged, resulting in an elongated outlet length on the left ventricular surface (known as inlet-outlet disproportion). The major determinant of anatomic variations is the relationship of the bridging leaflets to the septal structures. This important relationship determines not only the level of intracardiac shunting (interatrial only, interventricular only, or both) but also the propensity for left ventricula...

Surgical Anatomy of Atrioventricular Septal Defect [REVIEW PAPER]

Asian Cardiovascular and Thoracic Annals — Tue, 04 Nov 2008 00:00:00 +0100

Right atrial isomerism: preponderance in Asian fetuses. Using the stomachdistance ratio as a possible diagnostic tool for prediction of right atrial isomerism.

Annals of the Academy of Medicine, Singapore — Sat, 01 Nov 2008 04:00:00 +0100

Conclusion: Our study suggests an Asian predilection towards right isomerism compared to Western populations. We postulate that there may be racial differences in the expression of these 2 forms of isomerism. The ultrasound findings of complex heart disease and abnormal arrangement of great vessels in abdominal cavity, though important, are varied and non-specific evidence for either form of fetal atrial isomerism. There is a possibility of using the SDR <0.34 (representing stomach proximity to the fetal spine) as a possible diagnostic tool to predict right-sided atrial isomerism. PMID: 19082194 [PubMed - in process] (Source: Annals of the Academy of Medicine, Singapore)

Right atrial isomerism: preponderance in Asian fetuses. Using the stomach-distance ratio as a possible diagnostic tool for prediction of right atrial isomerism.

Ann Acad Med Singapo... — Sat, 01 Nov 2008 04:00:00 +0100

CONCLUSION: Our study suggests an Asian predilection towards right isomerism compared to Western populations. We postulate that there may be racial differences in the expression of these 2 forms of isomerism. The ultrasound findings of complex heart disease and abnormal arrangement of great vessels in abdominal cavity, though important, are varied and non-specific evidence for either form of fetal atrial isomerism. There is a possibility of using the SDR <0.34 (representing stomach proximity to the fetal spine) as a possible diagnostic tool to predict right-sided atrial isomerism. PMID: 19082194 [PubMed - in process] (Source: Ann Acad Med Singapo...)

[Surgical management with mitral valve replacement in young infants with congenital mitral valve diseases and complete atrioventricular septal defect.]

Orvosi Hetilap — Fri, 26 Sep 2008 16:06:08 +0100

Conclusions: The surgical repair of congenital mitral valve diseases and CAVSD can be performed successfully in very young infants. If the anatomic characteristics of the mitral valve is not suitable for repair, only mechanical mitral valve replacement can be performed successfully even in sometimes hopeless situation (one of our pts of 3.5 kg weight, is the smallest baby mentioned in the literature). Our early and mid-time results are good, but the re-replacement will be an unavoidable problem in the future. PMID: 18815108 [PubMed - in process] (Source: Orvosi Hetilap)

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[Case report - Cardiac general] Titanium plate osteosynthesis for the correction of severe sternal deformity in a 13-year-old boy

Interactive CardioVascular and Thoracic Surgery — Thu, 18 Sep 2008 04:00:00 +0100

In a 13-year-old boy a correction of an atrioventricular septal defect (AVSD) was performed at the age of one year and a mitral valve reconstruction at the age of two years. The patient developed, after two median sternotomies, a massive sternal deformity. At the age of 12 years another mitral valve reconstruction had to be performed. At this time titanium plates were used for sternal closure and correction of the deformity. Six months later, and after a CT-follow-up, the plates could be removed with a very satisfying result. Sternal plate osteosynthesis represents an excellent alternative for the correction of difficult sternal closures and deformities in adults as well as in children. (Source: Interactive CardioVascular and Thoracic Surgery)

Plasma endothelin-1 and nitrate levels in Down’s syndrome with complete atrioventricular septal defect-associated pulmonary hypertension: a comparison with non-Down’s syndrome children

European Journal of Pediatrics — Wed, 06 Aug 2008 05:49:24 +0100

Abstract Children with Down’s syndrome (DS)-associated complete atrioventricular septal defect (AVSD) have rapid and aggressive development of pulmonary vascular disease when compared with non-Down’s syndrome (ND) children. We aimed to evaluate the role of plasma endothelin-1 (ET-1) and nitrate levels in DS children with complete AVSD-associated pulmonary hypertension (PH) and compare this to ND patients. The study included 20 patients (11 males, nine females) who had complete AVSD associated with PH. Comparisons were made between DS patients (n = 12) aged 4 to 8 months (median 5 months) and ND patients (n = 8) aged 4 to 12 months (median 7 months). Blood samples were drawn from the inferior vena cava, pulmonary artery, pulmonary vein, and ao...

[Three-dimensional echocardiography: preliminary experience in congenital cardiac disease.]

Anales de Pediatria — Fri, 01 Aug 2008 04:00:00 +0100

CONCLUSIONS: 3D real time echocardiography is a feasible, easy and rapid technique. It provides anatomical and functional details needed for an accurate comprehension of congenital cardiac diseases. In foetal screening, it provides an easier segmentary analysis of the entire foetal heart. PMID: 18755119 [PubMed - in process] (Source: Anales de Pediatria)

Single umbilical artery in fetopathological investigations.

Pathology, Research and Practice — Wed, 30 Jul 2008 04:00:00 +0100

In this study, we processed the details of 204 cases in which SUA was confirmed fetopathologically after miscarriage or induced abortion between 1990 and 2007. In our sample, SUA occurred in 7.38% of the cases. The history was positive in almost 30% of the cases. The majority of the cases had a positive obstetric and the minority of them a positive genetic history. The highest association of SUA with other malformations was found for craniospinal ones, but an association with cardiovascular malformations should also be mentioned. Regarding the individual types of malformation, SUA was most commonly associated with hydrocephalus, but Potter's sequence, trisomy 21, and atrioventricular septal defect also reached a higher rate in associated SUA. Previously published articles dealing with asso...

[ORIGINAL ARTICLES: PEDIATRIC CARDIAC] Results of Definitive Repair of Complete Atrioventricular Septal Defect in Neonates and Infants

The Annals of Thoracic Surgery — Thu, 24 Jul 2008 04:00:00 +0100

Conclusions Definitive repair for complete AVSD can be performed in early infancy with excellent results. The two-patch technique is a safe and reproducible surgical method that can achieve low mortality and good midterm outcomes even in very young infants. (Source: The Annals of Thoracic Surgery)

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Results of definitive repair of complete atrioventricular septal defect in neonates and infants.

The Annals of Thoracic Surgery — Wed, 23 Jul 2008 10:00:41 +0100

CONCLUSIONS: Definitive repair for complete AVSD can be performed in early infancy with excellent results. The two-patch technique is a safe and reproducible surgical method that can achieve low mortality and good midterm outcomes even in very young infants. PMID: 18640339 [PubMed - in process] (Source: The Annals of Thoracic Surgery)

[Evaluation of surgical approaches and early and midterm results of treatment for atrioventricular septal defect.]

Turk Kardiyoloji Dernegi arsivi — Wed, 16 Jul 2008 16:24:21 +0100

CONCLUSION: Total repair of complete AVSD should be the procedure of choice in early infancy. Left AV valve insufficiency continues to be the most important cause of postoperative morbidity in these cases. PMID: 18626209 [PubMed - as supplied by publisher] (Source: Turk Kardiyoloji Dernegi arsivi)

Severe Left Ventricular Hypoplasia in Patients with Unbalanced Incomplete Atrioventricular Septal Defect and Pulmonary Hypertension: Feasibility of Biventricular Repair

Pediatric Cardiology — Wed, 02 Jul 2008 15:25:08 +0100

We report three patients with partial atrioventricular septal defect who presented in the neonatal period with excessive left to right shunting and progressive pulmonary hypertension. Successful biventricular repair was accomplished despite the fact that left ventricular area and left sided structures did not meet the criteria established previously for biventricular management in children with critical aortic stenosis or complete atrioventricular septal defect. Indication for biventricular management was based on the fact that none of our patients had morphological mitral or aortic stenosis and that the hypoplastic left ventricle proved capable to maintain the systemic circulation following closure of the arterial duct. Fenestrated closure of the atrial septum and an individualized ...

[Surgery of 56 patients having a partial atrioventricular septal defect.]

Annales de Cardiologie et d'Angeiologie — Tue, 01 Jul 2008 04:00:00 +0100

Authors: Jerbi S, Tarmiz A, Romdhani N, Kortas C, Chaouch N, Alimi F, Khelil N, Mlika S, Limayem F, Ennabli K Between January 1991 and December 2006, 56 patients having a partial atrioventricular septal defect (AVSD) were operated. The purpose of this retrospective study is to analyze the immediate and long-term results of the surgery by granting of the importance to two main problems which are the disturbances of the rhythm and the conduction and the residual mitral regurgitation (MR). The mean age of our patients is of 10 and a half years with a net feminine ascendancy. Ninety-three percent of the patients were in regular sinus rhythm. No case of complete atrioventricular block (AVB) was noted. The MR was of grade I in 28,5% of the cases, grade II in 60% of the cases and grade III an...

[ORIGINAL ARTICLES: PEDIATRIC CARDIAC] Reoperation for Left Atrioventricular Valve Regurgitation After Atrioventricular Septal Defect Repair

The Annals of Thoracic Surgery — Fri, 27 Jun 2008 04:00:00 +0100

Conclusions Surgical management of LAVVR after AVSD repair can be performed with excellent midterm outcomes. However, both repair and replacement are associated with a high incidence of reoperation. Nonetheless, an aggressive reparative approach should be pursued to avoid the morbidity of pediatric left atrioventricular valve replacement that includes anticoagulation, inevitable reoperation, and cardiomyopathy. (Source: The Annals of Thoracic Surgery)

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Reoperation for left atrioventricular valve regurgitation after atrioventricular septal defect repair.

The Annals of Thoracic Surgery — Wed, 25 Jun 2008 21:37:50 +0100

CONCLUSIONS: Surgical management of LAVVR after AVSD repair can be performed with excellent midterm outcomes. However, both repair and replacement are associated with a high incidence of reoperation. Nonetheless, an aggressive reparative approach should be pursued to avoid the morbidity of pediatric left atrioventricular valve replacement that includes anticoagulation, inevitable reoperation, and cardiomyopathy. PMID: 18573414 [PubMed - in process] (Source: The Annals of Thoracic Surgery)

Understanding atrioventricular septal defect. Anatomoechocardiographic correlation

Cardiovascular Ultrasound — Tue, 24 Jun 2008 04:00:00 +0100

Conclusions: Anatomo-echocardiographic correlation demonstrated a high degree of diagnostic precision with echocardiography. (Source: Cardiovascular Ultrasound)

Understanding atrioventricular septal defect: Anatomoechocardiographic correlation

Cardiovascular Ultrasound — Tue, 24 Jun 2008 04:00:00 +0100

Conclusion: Anatomo-echocardiographic correlation demonstrated a high degree of diagnostic precision with echocardiography. (Source: Cardiovascular Ultrasound)

Incomplete atrioventricular septal defect with hypoplastic left ventricle and left atrioventricular valve stenosis

General Thoracic and Cardiovascular Surgery — Sun, 08 Jun 2008 05:53:19 +0100

Abstract A 2-month-old male infant with incomplete atrioventricular septal defect associated with a hypoplastic left ventricle and left atrioventricular valve stenosis successfully underwent biventricular repair. Echocardiography showed marked dilatation in the right ventricle and a diminutive left ventricle. However, the left ventricular chamber occupied the apex. Left atrioventricular valve stenosis was due to a solitary papillary muscle. Cardiac catheterization showed pulmonary/systemic flow ratio of 3.61, left ventricular end-diastolic volume of 63% of normal, and right ventricular end-diastolic volume of 324% of normal. During surgical repair, the solitary papillary muscle was divided longitudinally and the ostium primum was closed with a bovine pericardium. Postopera...

Atrioventricular Septal Defect in the Fetus - Associated Conditions and Outcome in 246 Cases.

Ultraschall in der Medizin — Mon, 19 May 2008 04:00:00 +0100

CONCLUSION: Among fetuses with AVSD, those with trisomy 21 are detected earlier in pregnancy, have less distorted cardiac anatomy, higher rates of biventricular repair and better survival rates. Due to the limited sample size in euploid fetuses, it remains unclear whether this apparent protection afforded to Down syndrome accounts also for cohorts with isolated and balanced AVSD. PMID: 18491259 [PubMed - as supplied by publisher] (Source: Ultraschall in der Medizin)

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Maternal urinary tract infections and selected cardiovascular malformations

Birth Defects Research Part A: Clinical and Molecular Teratology — Thu, 01 May 2008 04:00:00 +0100

CONCLUSIONS: In the National Birth Defects Prevention Study there was little evidence to support an association between CVMs and UTIs during the first trimester of pregnancy. Associations between left ventricular outflow tract obstructive defects and maternal UTI as well as between atrioventricular septal defects and maternal UTI were found. Our findings, while not conclusive, suggest that the possible association between maternal UTI and CVMs should be investigated further. Birth Defects Research (Part A), 2008. © 2008 Wiley-Liss, Inc. (Source: Birth Defects Research Part A: Clinical and Molecular Teratology)

[ORIGINAL ARTICLES: PEDIATRIC CARDIAC] 28 Years' Experience With Transatrial-Transpulmonary Repair of Atrioventricular Septal Defect With Tetralogy of Fallot

The Annals of Thoracic Surgery — Thu, 01 May 2008 04:00:00 +0100

Conclusions Atrioventricular septal defect and tetralogy of Fallot can be repaired with low mortality by the transatrial-transpulmonary approach without the use of a conduit. (Source: The Annals of Thoracic Surgery)

Double orifice mitral valve by real-time three-dimensional echocardiography.

European Journal of Echocardiography — Thu, 01 May 2008 04:00:00 +0100

Authors: Anwar AM, McGhie JS, Meijboom FJ, Ten Cate FJ Double orifice mitral valve (DOMV) is a rare congenital malformation described as division of mitral orifice into two anatomically distinct orifices separated by an accessory bridge of fibrous tissue. In 85% of cases, both orifices are unequal in size. It is usually associated with other congenital defects such as atrioventricular septal defect and complex congenital heart disease. Most of cases could be diagnosed by two-dimensional echocardiography (2DE). The real-time three-dimensional echocardiography (RT3DE) helped in more detailed structure and function. Presented here RT3DE used for orientation of DOMV that allowed detailed and comprehensive assessment incremental to that obtained by 2DE. PMID: 18490284 [PubMed - as suppl...

28 years' experience with transatrial-transpulmonary repair of atrioventricular septal defect with tetralogy of fallot.

The Annals of Thoracic Surgery — Wed, 30 Apr 2008 20:38:14 +0100

CONCLUSIONS: Atrioventricular septal defect and tetralogy of Fallot can be repaired with low mortality by the transatrial-transpulmonary approach without the use of a conduit. PMID: 18442566 [PubMed - in process] (Source: The Annals of Thoracic Surgery)

Maternal Smoking and Congenital Heart Defects

PEDIATRICS — Tue, 01 Apr 2008 04:00:00 +0100

CONCLUSIONS. Maternal smoking during pregnancy was associated with septal and right-sided obstructive defects. Additional investigation into the timing of tobacco exposure and genetic susceptibilities that could modify this risk will provide a more precise evidence base on which to build clinical and public health primary prevention strategies. (Source: PEDIATRICS)

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[Case report - Congenital] Total cavo-pulmonary connection without foreign material for asplenic heart associated with partial anomalous pulmonary venous connection

Interactive CardioVascular and Thoracic Surgery — Tue, 01 Apr 2008 04:00:00 +0100

The presented case was a 3-year-old boy diagnosed with asplenia (SLL), double outlet right ventricle, pulmonary stenosis, atrioventricular septal defect, hypoplastic left ventricle and partial anomalous pulmonary venous connection to the superior vena cava. Partial anomalous pulmonary venous connection was repaired by translocation of pulmonary artery to avoid pulmonary venous obstruction when Glenn anastomosis was performed. Total cavo-pulmonary connection was established by re-routing the inferior vena cava to pulmonary artery using the atrial septal remnant and the left atrium free wall flap. (Source: Interactive CardioVascular and Thoracic Surgery)

Torsades de Pointes in atrioventricular septal defect.

International Journal of Cardiology — Tue, 18 Mar 2008 04:00:00 +0100

Authors: Nijjer S, White S, Gatzoulis MA 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. PMID: 18353464 [PubMed - as supplied by publisher] (Source: International Journal of Cardiology)

Single ventricle repair in children with Down’s syndrome

General Thoracic and Cardiovascular Surgery — Fri, 14 Mar 2008 07:32:49 +0100

Conclusion Down’s syndrome is a risk factor in patients with functionally single ventricle due to persistent pulmonary hypertension and airway obstruction. These results show that single ventricle repair in patients with Down’s syndrome is accompanied with difficulties, and patient selection for the Fontan procedure should be done carefully. Content Type Journal ArticleCategory Original ArticleDOI 10.1007/s11748-007-0201-zAuthors Naoki Wada, Japan Promotion Society for Cardiovascular Diseases Department of Cardiovascular Surgery, Sakakibara Heart Institute 3-16-1 Asahi-cho, Fuchu Tokyo 183-0003 JapanYukihiro Takahashi, Japan Promotion Society for Cardiovascular Diseases Department of Cardiovascular Surgery, Sakakibara Heart Institute 3-16-1 Asahi-cho, Fuchu Tokyo ...

Cardiopulmonary bypass for surgical correction of congenital heart disease in children with sickle cell disease: a case series

Anaesthesia — Fri, 29 Feb 2008 19:07:39 +0100

We present a series of three children with sickle cell disease aged 3 months, 3 weeks and 18 months, all presenting for cardiac surgery requiring cardiopulmonary bypass. The cardiac lesions were atrioventricular septal defect, transposition of the ... (Source: Anaesthesia)

[ORIGINAL ARTICLES: CARDIOVASCULAR] Minimally Invasive Congenital Cardiac Surgery Through Right Anterior Minithoracotomy Approach

The Annals of Thoracic Surgery — Fri, 29 Feb 2008 05:00:00 +0100

Conclusions The right anterior minithoracotomy incision is a safe and effective alternative to a median sternotomy for correction of congenital heart defects. Cosmetic results are highly satisfactory. (Source: The Annals of Thoracic Surgery)

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Minimally invasive congenital cardiac surgery through right anterior minithoracotomy approach.

The Annals of Thoracic Surgery — Sat, 23 Feb 2008 20:35:22 +0100

CONCLUSIONS: The right anterior minithoracotomy incision is a safe and effective alternative to a median sternotomy for correction of congenital heart defects. Cosmetic results are highly satisfactory. PMID: 18291151 [PubMed - in process] (Source: The Annals of Thoracic Surgery)

Mid-to-long term follow-up after surgical repair of atrioventricular septal defect with common atrioventricular junction and ventricular shunting associated with tetralogy of Fallot

Cardiology in the Young — Tue, 05 Feb 2008 08:22:56 +0100

Research ArticlesLeonardo S. Canale, Andrey J.O. Monteiro, Isabela Rangel, Divino F. Pinto, Paulo Soares, Rosa C. Barbosa, Milton A. Meier, Miguel L.B. Marcial, Cardiology in the Young, Volume 18 Issue 01 , pp 100-104AbstractObjectives Our aim is to describe our surgical approach in dealing with patients having atrioventricular septal defect with common atrioventricular junction and ventricular shunting associated with tetralogy of Fallot over the last 8 years, and to present our results in mid-to-long term follow-up. (Source: Cardiology in the Young)

[Anatomic spectrum between complete and partial atrioventricular septal defect. Two and three-dimensional echocardiography approach]

Archivos de Cardiologia de Mexico — Tue, 01 Jan 2008 05:00:00 +0100

CONCLUSIONS: The three-dimensional echocardiographic study of the spectrum of AVSD type A of Rastelli, defines accurately the valve components and septal structures, so we can understand the transition between complete and partial forms. This difference determines the clinical evolution of the patients. PMID: 18581712 [PubMed - in process] (Source: Archivos de Cardiologia de Mexico)

[Atrioventricular septal defect. Anatomopathologic study and embryological correlation]

Archivos de Cardiologia de Mexico — Tue, 01 Jan 2008 05:00:00 +0100

Authors: Nivon MK, Martínez EM, Castellanos LM, Zavaleta NE Atrioventricular septal defect is a complex congenital heart disease in which cardiac septation is deficient especially at atrioventricular and ventricular levels. The anatomopathologic experience at the National Institute of Cardiology "Ignacio Chávez" is described; an anatomo-embryological correlation was made to gain an insight of the pathogenesis. Seventy hearts were analyzed morphologically with the sequential segmentary system. Atrial situs, connections of cardiac segments, cardiac septa, septal defects, ventricular geometry, fibrous skeleton and associated anomalies were determined. Histologic sections of chick embryonic hearts were analyzed and compared with stages of human heart development, serving as basis...

Double-Outlet Left Ventricle with a Superiorly Positioned and Hypoplastic Left Ventricle

Pediatric Cardiology — Wed, 12 Dec 2007 15:43:16 +0100

We describe a case of DOLV with situs inversus, L-loop ventricles, d-malposition of the great arteries (I, L, D), superior–inferior ventricles, unbalanced complete atrioventricular septal defect, pulmonary stenosis, and total anomalous pulmonary venous return in which the left ventricle was also severely hypoplastic and superiorly positioned. Many morphologic variations of this malformation have been described, but to the best of our knowledge DOLV with superiorly positioned and hypoplastic left ventricle has not been reported before. Content Type Journal ArticleCategory Images in Pediatric CardiologyDOI 10.1007/s00246-007-9165-0Authors Suheyla Ozkutlu, Hacettepe University Faculty of Medicine, Department of Pediatric Cardiology Sihhiye 06100 Ankara TurkeyDursun Alehan, Hacett...

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Partial atrioventricular septal defect assessed by real-time three-dimensional echocardiography: a case report.

Journal of Cardiology — Sat, 01 Dec 2007 05:00:00 +0100

Authors: Toh N, Kanzaki H, Nakatani S, Kohyama K, Ohara T, Kim J, Hashimura K, Kitakaze M A 48-year-old woman was admitted with a heart murmur and increased difficulty in breathing. Two-dimensional echocardiography revealed a defect in the lower part of the atrial septum [(primum atrial septal defect (ASD)] and a pouch at the interventricular septum. Color Doppler echocardiography detected grade 3/4 mitral regurgitation. Real-time three-dimensional echocardiography (RT-3DE) revealed a cleft in the anterior leaflet of the mitral valve toward the tricuspid valve, and the ASD located near the atrioventricular valves with 14mm in minor axis. Color Doppler three-dimensional echocardiography disclosed left-to-right ASD shunt toward the atrial posterior wall. No shunt through the pouch at the...

[Institutional report - Congenital] Surgical treatment of complete atrioventricular septal defect with the two-patch technique: early-to-mid follow-up

Interactive CardioVascular and Thoracic Surgery — Mon, 26 Nov 2007 05:00:00 +0100

We report our results on surgical treatment of complete atrioventricular septal defects using the two-patch technique. Forty patients with complete atrioventricular septal defects were operated on in the period from November 1995 to January 2004 and retrospectively analyzed. The age at the time of surgery ranged from 4 months to 20 years (average=18.8±37 months). Their weights ranged from 3 to 39 kg (average=7.6±5.8 kg). Associated tetralogy of Fallot was present in 20% of the cases (8 patients). Monitoring was complete until January 2007, corresponding to a follow-up ranging from 36 to 135 months (average=74±33.7 months). The surgical mortality rate was 2.5% and the hospital mortality rate was 5%. A third patient died from a brain abscess...

Re: Atrioventricular septal defect with common atrioventricular junction, common arterial trunk and severe coartation of the aorta in a patient with Down’s syndrome

Cardiology in the Young — Thu, 22 Nov 2007 01:32:57 +0100

Letter PapersClaudia Saffirio, Anna Chiara Vittucci, Bruno Marino, Cardiology in the Young, Volume 17 Issue 06 , pp 694-694Abstract (Source: Cardiology in the Young)

Case report Inoperable sinus venosus atrial septal defect in a young woman with severe pulmonary hypertension – a case report

Articles of Polish Heart Journal - TERMEDIA publishing house — Wed, 21 Nov 2007 12:41:11 +0100

We describe a case of a 34-year-old woman with inoperable sinus venosus atrial septal defect with severe pulmonary hypertension and symptoms of the Eisenmenger syndrome. Electrocardiography, chest radiography, transthoracic and transoesophageal echocardiography with and without contrast were performed and provided valuable information about haemodynamic consequences of this malformation. (Source: Articles of Polish Heart Journal - TERMEDIA publishing house)

[Inoperable sinus venosus atrial septal defect in a young woman with severe pulmonary hypertension - a case report.]

Kardiologia Polska — Thu, 01 Nov 2007 04:00:00 +0100

We describe a case of a 34-year-old woman with inoperable sinus venosus atrial septal defect with severe pulmonary hypertension and symptoms of the Eisenmenger syndrome. Electrocardiography, chest radiography, transthoracic and transoesophageal echocardiography with and without contrast were performed and provided valuable information about haemodynamic consequences of this malformation. PMID: 18058586 [PubMed - in process] (Source: Kardiologia Polska)

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Near miss sudden cardiac death on a young patient with repaired atrioventricular septal defect.

International Journal of Cardiology — Wed, 03 Oct 2007 04:00:00 +0100

We describe the case of a 25-year-old man with repaired atrioventricular septal defect who was referred to our centre after a ventricular fibrillation arrest. Serial echocardiograms in previous years had shown progressive severe left ventricular outflow obstruction, but the patient had not been operated on as he was deemed asymptomatic and reluctant to consider surgery. Management and criteria for further intervention in ACHD patients often differ from those of patients with acquired heart disease and reliance on symptoms alone is not good practice and may prove catastrophic. PMID: 17919754 [PubMed - as supplied by publisher] (Source: International Journal of Cardiology)

Severe fatal course of axial mesodermal dysplasia spectrum associated with complex cardiac defect in an infant of a mother with insulin dependent diabetes

American Journal of Medical Genetics Part A — Fri, 03 Aug 2007 04:00:00 +0100

We describe a new patient, an infant born to a type 1 diabetic mother, with the phenotype of AMDS as well as severe congenital cardiac anomalies including transposition of the great arteries and an atrioventricular septal defect. Congenital heart defects had been reported with OAVS, sacral dysgenesis, and in an infant born to a diabetic mother and combinations of these findings have been reported in the same patient. To our knowledge, this is the first patient with AMDS with transposition of great arteries and an atrioventricular septal defect and the second patient with AMDS who had history of parental consanguinity. The mechanism through which maternal diabetes mellitus leads to malformations is not entirely clear, but the glycemic control is essential in the care of mothers. © 2007 Wil...

What is the optimal time to repair atrioventricular septal defect and common atrioventricular valvar orifice?

Cardiology in the Young — Mon, 30 Jul 2007 13:10:46 +0100

Research ArticlesBrian E. Kogon, Hunter Butler, Michael McConnell, Traci Leong, Paul M. Kirshbom, Kirk R. Kanter, Cardiology in the Young, Volume 17 Issue 04 , pp 356-359Abstract (Source: Cardiology in the Young)

[Surgery for Congenital Heart Disease] Pediatric cardiac surgery without homologous blood transfusion, using a miniaturized bypass system in infants with lower body weight

The Journal of Thoracic and Cardiovascular Surgery — Mon, 30 Jul 2007 04:00:00 +0100

Conclusions It is possible to do complex transfusion-free procedures safely for patients weighing more than 4 kg by using the low–priming volume circuit. The limiting factors of bloodless heart surgery are not preoperative hematocrit and complexity of procedure but the cardiopulmonary bypass time and the patient’s body weight. (Source: The Journal of Thoracic and Cardiovascular Surgery)

[Surgery for Congenital Heart Disease] The presence of Down syndrome is not a risk factor in complete atrioventricular septal defect repair

The Journal of Thoracic and Cardiovascular Surgery — Mon, 30 Jul 2007 04:00:00 +0100

Conclusions The presence of Down syndrome in patients with complete atrioventricular septal defect is not a risk factor for surgical repair. Because of the early development of pulmonary vascular disease, primary repair should be performed within the first 6 months of life. In the long term, reintervention on the left atrioventricular valve is more often required in children with a normal chromosomal pattern. (Source: The Journal of Thoracic and Cardiovascular Surgery)

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Anatomical correction of transposition of the great vessels, atrioventricular septal defect and subpulmonary obstruction in an adult

Indian Journal of Thoracic and Cardiovascular Surgery — Sun, 29 Jul 2007 07:09:50 +0100

Content TypeJournal Article JournalIndian Journal of Thoracic and Cardiovascular SurgeryOnline ISSN 0973-7723Print ISSN 0970-9134 Journal VolumeVolume 16 Journal IssueVolume 16, Number 1 / June, 2000 (Source: Indian Journal of Thoracic and Cardiovascular Surgery)

Population-based perspective of long-term outcomes after surgical repair of partial atrioventricular septal defect.

The Annals of Thoracic Surgery — Tue, 24 Jul 2007 13:21:11 +0100

CONCLUSIONS: The survival rate for this simple cardiac defect is lower than the general population. In addition, the reoperation rate is significant. Despite this, in general, patients without Down syndrome can expect normal functional health status. PMID: 17643646 [PubMed - in process] (Source: The Annals of Thoracic Surgery)

[ORIGINAL ARTICLES: CARDIOVASCULAR] Population-Based Perspective of Long-Term Outcomes After Surgical Repair of Partial Atrioventricular Septal Defect

The Annals of Thoracic Surgery — Tue, 24 Jul 2007 04:00:00 +0100

Conclusions The survival rate for this simple cardiac defect is lower than the general population. In addition, the reoperation rate is significant. Despite this, in general, patients without Down syndrome can expect normal functional health status. (Source: The Annals of Thoracic Surgery)

Clinical significance of persistent left superior vena cava diagnosed in fetal life

Ultrasound in Obstetrics and Gynecology — Sat, 07 Jul 2007 04:00:00 +0100

Conclusions PLSVC is associated with CHDs. The identification of PLSVC should prompt a thorough examination of the fetus to identify additional cardiac and extracardiac anomalies. The prognosis of affected fetuses largely depends on whether or not the PLSVC is associated with a CHD. Copyright © 2007 ISUOG. Published by John Wiley & Sons, Ltd. (Source: Ultrasound in Obstetrics and Gynecology)

Regurgitation of the atrioventricular valves after corrective surgery for complete atrioventricular septal defects - comparison of different surgical techniques.

The Thoracic and Cardiovascular Surgeon — Fri, 01 Jun 2007 04:00:00 +0100

CONCLUSION: The different surgical techniques used for the correction of AVSD do not have a major bearing on the degree of AV-valve regurgitation. PMID: 17546552 [PubMed - indexed for MEDLINE] (Source: The Thoracic and Cardiovascular Surgeon)

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Cardiovascular malformations in Omani Arab children with Down's syndrome

Cardiology in the Young — Wed, 25 Apr 2007 23:30:02 +0100

Conclusion: The prevalence of cardiovascular malformations is high, at 57 , in our studied population of Arab children. Overall, 92 of the malformations are defects which are characterized by a left-to-right shunt, and the potential for pulmonary hypertension. These findings underscore the need for early detection and prompt, appropriate care. To achieve this, if feasible, all babies born with Down s syndrome should have echocardiography in the first month of life. (Source: Cardiology in the Young)

Atrioventricular septal defect with common atrioventricular junction, common arterial trunk, and severe coarctation of the aorta in a patient with Down's syndrome

Cardiology in the Young — Wed, 25 Apr 2007 23:30:02 +0100

We report, as far as we are aware, the first case of Down s syndrome with atrioventricular septal defect with common atrioventricular junction, common arterial trunk, and severe coarctation of the aorta. (Source: Cardiology in the Young)

Prenatal diagnosis of complete atrioventricular septal defect with truncus arteriosus

Prenatal Diagnosis — Thu, 22 Mar 2007 04:00:00 +0100

Atrioventricular Septal Defect Recently Diagnosed by Fetal Echocardiography: Echocardiographic Features, Associated Anomalies, and Outcomes

Congenital Heart Disease — Wed, 14 Mar 2007 13:17:39 +0100

Congenital Heart Disease Volume 2, Issue 2, Page 110-114, Mar 2007. (Source: Congenital Heart Disease)

[ORIGINAL ARTICLES: CARDIOVASCULAR] Spontaneous Closure of Small Residual Ventricular Septal Defects After Surgical Repair

The Annals of Thoracic Surgery — Thu, 01 Mar 2007 05:00:00 +0100

Conclusions Postsurgical residual VSDs less than 2 mm closed spontaneously in the majority within a year. Defects greater than 2 mm are unlikely to close spontaneously. Residual shunts after atrioventricular septal defect repair almost always close, whereas one third will remain open after Fallot or isolated VSD repair. At midterm follow-up, residual shunts remained hemodynamically and clinically irrelevant. Revision of a residual defect greater than 2 mm on cardiopulmonary bypass at initial repair, guided by TEE, may spare late redo surgery and lifelong antibiotic prophylaxis. (Source: The Annals of Thoracic Surgery)

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Spontaneous Closure of Small Residual Ventricular Septal Defects After Surgical Repair.

The Annals of Thoracic Surgery — Tue, 20 Feb 2007 16:56:03 +0100

CONCLUSIONS: Postsurgical residual VSDs less than 2 mm closed spontaneously in the majority within a year. Defects greater than 2 mm are unlikely to close spontaneously. Residual shunts after atrioventricular septal defect repair almost always close, whereas one third will remain open after Fallot or isolated VSD repair. At midterm follow-up, residual shunts remained hemodynamically and clinically irrelevant. Revision of a residual defect greater than 2 mm on cardiopulmonary bypass at initial repair, guided by TEE, may spare late redo surgery and lifelong antibiotic prophylaxis. PMID: 17307430 [PubMed - as supplied by publisher] (Source: The Annals of Thoracic Surgery)

Cor Triatriatum and Partial Atrioventricular Septal Defect

Pediatric Cardiology — Sat, 17 Feb 2007 08:08:26 +0100

Abstract??This is a case report of an unusual association of cor triatriatum and partial atrioventricular septal defect in a child. Content TypeJournal Article JournalPediatric CardiologyOnline ISSN 1432-1971Print ISSN 0172-0643 (Source: Pediatric Cardiology)

[ORIGINAL ARTICLES] One-stage total repair of aortic arch anomaly using regional perfusion

European Journal of Cardio-Thoracic Surgery — Mon, 29 Jan 2007 07:00:00 +0100

Conclusions: One-stage total arch repair using our regional perfusion technique is an excellent method that may minimize neurologic and myocardial complications without mortality. Our surgical strategy for arch anomaly has a low rate of residual and recurrent coarctation when performed in neonates and infants. (Source: European Journal of Cardio-Thoracic Surgery)

[Institutional report - Congenital] Ideal timing of surgical repair of isolated complete atrioventricular septal defect

Interactive CardioVascular and Thoracic Surgery — Wed, 17 Jan 2007 07:00:00 +0100

The ideal timing and optimal management of surgical repair for isolated complete atrioventricular septal defect (CAVSD) still remains controversial. To determine outcomes after the surgical repair of CAVSD, we reviewed 100 consecutive patients who underwent complete repair at our institute between January 1992 and August 2003. Among these 100 patients, 52 were female and 73 had Down's syndrome. Twelve had received preceding pulmonary artery banding. A two-patch repair was employed in all cases. The patients' median age and weight were 4.5 (1.2–48) months and 4.7 (2.5–12.5) kg, respectively. The mean stays in the intensive care unit and in the hospital were 5.3±3.8 and 25.4±18.1 days, respectively. The median duration of mechanical ventilation was 11...

[Surgery for Congenital Heart Disease] Intermittent warm blood cardioplegia in the surgical treatment of congenital heart disease: Clinical experience with 1400 cases

The Journal of Thoracic and Cardiovascular Surgery — Fri, 05 Jan 2007 07:00:00 +0100

Conclusion In our experience, normothermic cardioplegia has not generated any particular inconvenience and its use was contemporary, with improved outcomes. (Source: The Journal of Thoracic and Cardiovascular Surgery)

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[ORIGINAL ARTICLES: CARDIOVASCULAR] Early Repair of Complete Atrioventricular Septal Defect is Safe and Effective

The Annals of Thoracic Surgery — Wed, 01 Nov 2006 07:00:00 +0100

Conclusions These results suggest that repair of CAVSD defects in children 3 months of age or younger had similar outcomes compared with those who underwent surgical repair after 3 months of age. (Source: The Annals of Thoracic Surgery)

CRELD1 mutations contribute to the occurrence of cardiac atrioventricular septal defects in Down syndrome

American Journal of Medical Genetics Part A — Fri, 13 Oct 2006 02:43:08 +0100

No Abstract. (Source: American Journal of Medical Genetics Part A)

Slow pathway ablation in a 5-year-old boy with atrioventricular septal defect:

Clinical Research in Cardiology — Mon, 25 Sep 2006 05:41:03 +0100

Content TypeJournal Article JournalClinical Research in CardiologyOnline ISSN 1861-0692Print ISSN 1861-0684 (Source: Clinical Research in Cardiology)

Repair of Complete Atrioventricular Septal Defects: Results with Maintenance of the Coronary Sinus on the Right Atrial Side

Journal of Cardiac Surgery — Fri, 15 Sep 2006 09:10:42 +0100

Journal of Cardiac Surgery Volume 0, Issue 0, Page ???-???. (Source: Journal of Cardiac Surgery)

[Surgery for Congenital Heart Disease] Late incompetence of the left atrioventricular valve after repair of atrioventricular septal defects: The morphologic perspective

The Journal of Thoracic and Cardiovascular Surgery — Tue, 29 Aug 2006 06:00:00 +0100

Conclusions Not only is the annular component in the left atrioventricular valve abnormal, but the subvalvar apparatus is characterized by deficiency and disarray. Furthermore, the axis of cordal insertion may potentiate to separation over the long term of the leaflets joined surgically. Valvar repair in this setting will never restore the arrangement of the normal mitral valve. (Source: The Journal of Thoracic and Cardiovascular Surgery)

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Usefulness of Live/Real Time Three-Dimensional Transthoracic Echocardiography in the Assessment of Atrioventricular Septal Defects

Echocardiography — Wed, 26 Jul 2006 15:34:53 +0100

Echocardiography Volume 23, Issue 7, Page 598-608, Aug 2006. (Source: Echocardiography)

Double-Orifice Repair for Left Atrioventricular Valve Regurgitation in Atrioventricular Septal Defect: Report of Two Cases

Journal of Cardiac Surgery — Fri, 07 Jul 2006 08:07:46 +0100

Journal of Cardiac Surgery Volume 0, Issue 0, Page ???-??? (Source: Journal of Cardiac Surgery)

[ORIGINAL ARTICLES: CARDIOVASCULAR] Myocardial Metabolism is Better Preserved After Blood Cardioplegia in Infants

The Annals of Thoracic Surgery — Fri, 30 Jun 2006 06:00:00 +0100

ConclusionsThere are differences in myocardial substrate metabolism between blood cardioplegia and crystalloid cardioplegia, which involve carbohydrates and amino acids. The differences may include lipids but our data in this respect are not conclusive. (Source: The Annals of Thoracic Surgery)

[Surgery for Congenital Heart Disease] Early postoperative arrhythmias after pediatric cardiac surgery

The Journal of Thoracic and Cardiovascular Surgery — Fri, 02 Jun 2006 06:00:00 +0100

ConclusionsHemodynamically significant postoperative arrhythmias are a frequent complication of pediatric cardiac surgery. Younger age and longer bypass and crossclamp times are risk factors for arrhythmia. In addition, the repair of atrioventricular septal defects carries an independent risk of arrhythmias. (Source: The Journal of Thoracic and Cardiovascular Surgery)