MedWorm: Hole in the Heart

Dextrocardia, atrial septal defect, severe developmental delay, facial anomalies, and supernumerary ribs in a child with a complex unbalanced 8;22 translocation including partial 8p duplication

American Journal of Medical Genetics Part A — Thu, 02 Feb 2012 05:00:00 +0100

We report on a child with dextrocardia, atrial septal defect (ASD), severe developmental delay, hypotonia, 13 pairs of ribs, left preauricular choristoma, hirsutism, and craniofacial abnormalities. Prenatal cytogenetic evaluation showed karyotype 46,XY,?dup(8p)ish del(8)pter. Postnatal array CGH demonstrated a 6.8 Mb terminal deletion at 8p23.3–p23, an interstitial 31.1 Mb duplication within 8p23.1–p11, and a terminal duplication of 0.24 Mb at 22q13.33, refining the karyotype to 46,XY,der(8)dup(8)(p23.1p11.1)t(8;22)(p23.1;q13.1).ish der(8)dup(8)(p23.1p11.1)t(8;22)(p23.1;q13.1) (D8S504‐,MS607 + ,ARSA + ,D8Z1 + , RP115713 + +). Previous reports of distal 8p deletion, 8p duplication, and distal 22q duplication have shown similar manifestations, including congenital...

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Chromosome 4q deletion syndrome: Narrowing the cardiovascular critical region to 4q32.2–q34.3

American Journal of Medical Genetics Part A — Thu, 02 Feb 2012 05:00:00 +0100

Traumatic bronchial rupture and platypnea-orthodeoxia.

The Annals of Thoracic Surgery — Tue, 31 Jan 2012 04:17:36 +0100

Authors: Odell JA, Keller CA, Erasmus DB, Stritt MT Abstract A patient with blunt trauma and traumatic bronchial rupture and lung collapse had prominent symptoms of platypnea-orthodeoxia syndrome. These symptoms were relieved by bronchial repair. The syndrome is rarely seen and is usually associated with a patent foramen ovale or atrial septal defect. The syndrome has not been described previously in association with traumatic bronchial rupture. PMID: 22269739 [PubMed - in process] (Source: The Annals of Thoracic Surgery)

Life-threatening isometric-exertion related cardiac perforation 5 years after amplatzer atrial septal defect closure: should isometric activity be limited in septal occluder holders?

The Annals of Thoracic Surgery — Tue, 31 Jan 2012 04:16:56 +0100

Authors: Santini F, Morjan M, Onorati F, Morando G, Faggian G, Mazzucco A PMID: 22269743 [PubMed - in process] (Source: The Annals of Thoracic Surgery)

Iatrogenic atrial septal defect and aortoatrial fistula in a patient with endovascular prosthesis in the inferior vena cava.

The Annals of Thoracic Surgery — Tue, 31 Jan 2012 04:13:34 +0100

We report the case of a liver transplant patient with a stenosis in the anastomosis of the suprahepatic veins to inferior vena cava, treated by self-expanding prosthesis, who developed an aorto-right atrial fistula and an atrial septal defect. Open heart surgery was performed to correct the defects. Transthoracic echocardiogram 1 year later revealed no evidence of residual shunt. PMID: 22269763 [PubMed - in process] (Source: The Annals of Thoracic Surgery)

Voluminous atrial septal aneurysm may mask a large double atrial septal defect.

The Annals of Thoracic Surgery — Tue, 31 Jan 2012 04:12:43 +0100

Authors: Scaffa R, Spaziani C, Leporace M, Leonetti S, Di Roma M, Gaspardone A, De Paulis R PMID: 22269768 [PubMed - in process] (Source: The Annals of Thoracic Surgery)

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

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

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

A case report of an accidental vertebral arterial puncture videotaped during central venous catheterization in a child undergoing a ventricular septal defect repair

Pediatric Anesthesia — Thu, 26 Jan 2012 12:13:26 +0100

(Source: Pediatric Anesthesia)

[Stent implantation in recoarctation of aorta in infant.]

Kardiologia Polska — Wed, 25 Jan 2012 06:54:02 +0100

We present the case of a 12 month-old infant with critical coarctaion of aorta, arch hypoplasia, atrial and ventricular septal defects who underwent interventional treatment with stent implantation due to recoarctation of aorta. In neonatal period the patient went through complete surgical correction. At the age of four months balloon angioplasty of recurrent coractation was conducted. Eight months later the patient was hospitalised with heart failure (HF) symptoms. Clinical and echographic examination confirmed critical stenosis of aortic isthmus. We performed heart catheterisation with stent implantation to transverse and descending arch with immediate reduction of gradient from 45 mm Hg to 0 mm Hg, widening of the isthmus from 2 to 8 mm and gradual regression of HF symptoms. At 3 years ...

[Massive intravascular haemolysis after percutaneous atrial septal defect closure.]

Kardiologia Polska — Wed, 25 Jan 2012 06:54:02 +0100

We present a case of a 55 year-old female, who survived a complication of percutaneous closure of atrial septal defect never described before. Within the first day after treatment the device has dislodged and got stuck in the mitral valve apparatus. This has caused mitral insufficiency and massive haemolysis which resolved after interventional removal of the device. Kardiol Pol 2012; 70, 1: 58-59. PMID: 22267428 [PubMed - as supplied by publisher] (Source: Kardiologia Polska)

Severe hypoxaemia with a left ventricular assist device in a minipig model with an undiagnosed congenital cardiac disease

Laboratory Animals — Wed, 25 Jan 2012 05:00:00 +0100

In conclusion, if hypoxaemia presents after implementation of an LVAD, the presence of a right–left shunt must be ruled out. The first step must be a judicious reduction in assist device flow to minimize intracardiac shunting. Subsequently, atrial septal closure of the defect should be considered. We report an experimental model of severe hypoxaemia after placement of an LVAD as part of a larger research project. (Source: Laboratory Animals)

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Periventricular Closure of a Perimembranous VSD: Treatment Option in Selected Patients

The Thoracic and Cardiovascular Surgeon — Wed, 25 Jan 2012 05:00:00 +0100

Thorac cardiovasc SurgDOI: 10.1055/s-0032-1301750Ventricular septal defects (VSDs) are a common congenital heart disease. Usually, surgical repair with cardiopulmonary bypass (CPB) is the treatment of choice, whereas percutaneous techniques have technical limitations, predominantly a mismatch of catheter size and body weight. A 7-year-old girl underwent periventricular closure of a perimembranous VSD on the beating heart. Echocardiography guided implantation through a minimally invasive sternotomy was uneventful. The described approach adds favorably to the current practice avoiding the use of CPB. Cosmetic aspect and rapid early postoperative recovery are convincing.[...]Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.Article in Thieme eJournals:Table of contents ...

Iatrogenic Atrial Septal Defect and Aortoatrial Fistula in a Patient With Endovascular Prosthesis in the Inferior Vena Cava [CASE REPORTS]

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

We report the case of a liver transplant patient with a stenosis in the anastomosis of the suprahepatic veins to inferior vena cava, treated by self-expanding prosthesis, who developed an aorto–right atrial fistula and an atrial septal defect. Open heart surgery was performed to correct the defects. Transthoracic echocardiogram 1 year later revealed no evidence of residual shunt. (Source: The Annals of Thoracic Surgery)

Voluminous Atrial Septal Aneurysm May Mask a Large Double Atrial Septal Defect [IMAGES IN CARDIOTHORACIC SURGERY]

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

(Source: The Annals of Thoracic Surgery)

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

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

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

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

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

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

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Traumatic Bronchial Rupture and Platypnea-Orthodeoxia [CASE REPORTS]

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

A patient with blunt trauma and traumatic bronchial rupture and lung collapse had prominent symptoms of platypnea-orthodeoxia syndrome. These symptoms were relieved by bronchial repair. The syndrome is rarely seen and is usually associated with a patent foramen ovale or atrial septal defect. The syndrome has not been described previously in association with traumatic bronchial rupture. (Source: The Annals of Thoracic Surgery)

Life-Threatening Isometric-Exertion Related Cardiac Perforation 5 Years After Amplatzer Atrial Septal Defect Closure: Should Isometric Activity Be Limited in Septal Occluder Holders? [IMAGES IN CARDIOTHORACIC SURGERY]

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

(Source: The Annals of Thoracic Surgery)

Noninvasive evaluation of 3D hemodynamics in a complex case of single ventricle physiology

Journal of Magnetic Resonance Imaging — Mon, 23 Jan 2012 05:00:00 +0100

We report the comprehensive evaluation of the complex hemodynamics in a rare case of a pediatric patient after repair of congenital heart disease with multiple abnormalities including hypoplastic left heart, double outlet right ventricle, transposition of great arteries, ventricular septal defect, aortic coarctation, and total cavopulmonary connection. Based on a single measurement, whole‐heart flow‐sensitive 4D magnetic resonance imaging (MRI) was able to demonstrate a number of regional flow alterations such as poststenotic helix formation and asymmetric flow distributions for the double arterial outlet and to the left and right lungs. Our findings illustrate the potential role of flow‐sensitive 4D MRI as a noninvasive and radiation‐free technique for the frequent postinterventio...

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

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

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

Unusual Endocarditis: "Rare Bug, Rare Site"

The American Journal of the Medical Sciences — Fri, 20 Jan 2012 05:00:00 +0100

Unusual Endocarditis: "Rare Bug, Rare Site" Am J Med Sci. 2012 Jan 20; Authors: Lodha A, Enakpene E, Haran M, Sadiq A Abstract A 27-year-old man was diagnosed with infective endocarditis due to Streptococcus agalactiae. Large vegetations were seen on the anterior mitral valve leaflet and also on the right ventricular side of a membranous ventricular septal defect. Streptococcus agalactiae is a rare cause of endocarditis, and it is very rare to find large vegetations around ventricular septal defect. The authors present this interesting case of unusual endocarditis with vegetations in both the right and left heart. This case is the first reported case of infective endocarditis involving the left and the right sides of the heart at the same time in a nonintravenous drug user....

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Bicuspid pulmonary valve in transposition of the great arteries: impact on outcome [CONGENITAL]

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

CONCLUSIONS ASO is a safe option for TGA associated with a well-functioning bicuspid pulmonary valve with low morbidity and mortality. Prevalence of AR was not particularly high. Even though ARD was frequent, neoaortic bicuspid valve did not represent a high risk for aortic reoperation. Long-term individual follow-up is mandatory to observe the potential risk of root dilatation and AR. (Source: European Journal of Cardio-Thoracic Surgery)

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

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

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

Intra-operative device closure of multiple atrial septal defects facilitated by a unique atrial septum remodeling technique: initial results and experience in 11 patients [CONGENITAL]

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

CONCLUSIONS Atrial septum remodeling technique seems to be a safe and effective method that could largely facilitate the successful IODC of multiple ASDs. (Source: European Journal of Cardio-Thoracic Surgery)

Aortopulmonary Window Associated with Interrupted Aortic Arch: Report of Surgical Repair of Eight Cases and Review of Literature

The Thoracic and Cardiovascular Surgeon — Tue, 17 Jan 2012 05:00:00 +0100

Conclusion Neonatal repair with thorough mobilization of the aortic arch and extended end-to-side anastomosis or use of patch augmentation carries potential for the best early and late outcome for combined APW/IAA malformation.[...]Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.Article in Thieme eJournals:Table of contents | Abstract | Full text (Source: The Thoracic and Cardiovascular Surgeon)

Aortopulmonary Window Associated with Interrupted Aortic Arch: Report of Surgical Repair of Eight Cases and Review of Literature.

The Thoracic and Cardiovascular Surgeon — Tue, 17 Jan 2012 05:00:00 +0100

Conclusion Neonatal repair with thorough mobilization of the aortic arch and extended end-to-side anastomosis or use of patch augmentation carries potential for the best early and late outcome for combined APW/IAA malformation. PMID: 22252330 [PubMed - as supplied by publisher] (Source: The Thoracic and Cardiovascular Surgeon)

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Effect of remote ischaemic preconditioning on ischaemic-reperfusion injury in pulmonary hypertensive infants receiving ventricular septal defect repair

British Journal of Anaesthesia — Mon, 16 Jan 2012 05:00:00 +0100

Conclusions RIPC does not reduce the postoperative TnI release after VSD repair in infants with pulmonary hypertension. Additionally, it is difficult to find significant clinical benefits of RIPC in this population. The effect of RIPC varies according to clinical situation and patient condition. Clinical trial registration: ClinicalTrials.gov, NCT01313832. (Source: British Journal of Anaesthesia)

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

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

(Source: Journal of Surgical Research)

What other anomalies? Failure to wean post ventricular septal defect repair secondary to anomalous origin of the left coronary artery from the pulmonary artery

Pediatric Anesthesia — Fri, 13 Jan 2012 12:08:52 +0100

SummaryA six week old infant underwent ventricular septal defect and atrial septal defect closure. Preoperative echocardiography showed evidence of pulmonary hypertension. The post operative course was complicated failure to wean from ventilatory and inotropic support. Echocardiography showed severe left ventricular (LV) dysfunction and suggested some fistulous drainage of the left coronary artery into the right pulmonary artery; this anomalous drainage of the left coronary artery into the right pulmonary artery (ALCAPA) was confirmed with coronary angiogram. Re‐implantation of the left coronary artery into the aorta was performed. Extra‐corporeal membrane oxygenation (ECMO) was required to allow time for ventricular recovery. Supports were weaned gradually, with concurrent evidence of...

Does the amplatzer septal occluder device alter ventricular contraction pattern? A ventricular motion analysis by MR tagging

Journal of Magnetic Resonance Imaging — Fri, 13 Jan 2012 05:00:00 +0100

Conclusion:The Amplatzer occluder itself does not change the ventricular contraction pattern. All volume and myocardial deformation changes were caused by ventricular loading shifts. J. Magn. Reson. Imaging 2012;. © 2012 Wiley Periodicals, Inc. (Source: Journal of Magnetic Resonance Imaging)

Subclavian and pulmonary artery steal phenomenon in a patient with isolated left subclavian artery and right aortic arch

Journal of Clinical Ultrasound — Thu, 12 Jan 2012 05:00:00 +0100

We describe a patient with an isolated left subclavian artery associated with right aortic arch, patent ductus arteriosus, and ventricular septal defect. As the isolated left subclavian artery is supplied by the left vertebral artery in which blood flows in the retrograde direction, this anomaly is usually responsible for a congenital subclavian steal phenomenon. Atrophy of the left cerebral hemisphere and inverted left vertebral arterial flow were clearly depicted by echoencephalography in this patient, whose subclavian artery was connected to the main pulmonary artery by a patent ductus arteriosus. © 2012 Wiley Periodicals, Inc. J Clin Ultrasound, 2012. (Source: Journal of Clinical Ultrasound)

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CD44 expression in plexiform lesions of idiopathic pulmonary arterial hypertension

Pathology International — Tue, 10 Jan 2012 05:00:00 +0100

Plexiform lesions in pulmonary arteries are a characteristic histological feature for idiopathic pulmonary arterial hypertension (IPAH). The pathogenesis of the plexiform lesion is not fully understood, although it may be related to endothelial cell dysfunction and local inflammation. CD44 is a cell adhesion molecule and it is also involved in angiogenesis, endothelial cell proliferation and migration. The expression of CD44 was examined in lung plexiform lesions obtained from patients with IPAH (IPAH group, n= 7) and pulmonary arterial hypertension associated with atrial septal defect (ASD‐PAH group, n= 4). Expression of CD44 was detected in 49 out of 52 plexiform lesions (93%) from all patients in the IPAH group, whereas 31 plexiform lesions obtained from the ASD‐PAH group lacked CD4...

Transcatheter closure of atrial septal defect in a geriatric population

Catheterization and Cardiovascular Interventions — Tue, 10 Jan 2012 05:00:00 +0100

Conclusion: Transcatheter closure of ASD in geriatric patients can be performed safely. This procedure contributes to significant improvement of symptoms and positive cardiac remodeling. Long‐term follow‐up is mandatory, especially for patients with mitral regurgitation. © 2012 Wiley Periodicals, Inc. (Source: Catheterization and Cardiovascular Interventions)

Accessory mitral valve tissue: an unusual cause of congenital mitral stenosis

Interactive CardioVascular and Thoracic Surgery — Mon, 09 Jan 2012 05:00:00 +0100

We report here the first and a rarest presentation of the AMVT in a 19-year old female patient diagnosed to have double outlet right ventricle, ventricular septal defect (VSD), infundibular stenosis and congenital mitral stenosis (MS). She presented with a history of shortness of breath and chest pain over 3 years. Diagnosis was made by chest X-ray, transthoracic and transoesophageal echocardiography, which was confirmed by cardiac catheterization, and angiography. A successful closure of the VSD with excision of the right ventricular bundle and excision of the AMVT was done. Post-operative course was uneventful and an echocardiogram before the discharge showed no residual shunt, no right ventricular outflow gradient or mitral regurgitation, and the gradient across the mitral valve was 5/3...

No escape from a VSD device? Complete heart block and cardiac arrest associated with a ventricular septal defect occluder device

Pediatric Anesthesia — Wed, 04 Jan 2012 16:36:55 +0100

SummaryA 15 month old boy with a ventricular septal defect (VSD) underwent percutaneous device closure of the VSD. Five days later he collapsed; on arrival to hospital he was asystolic and received prolonged cardio‐pulmonary resuscitation (CPR) with intermittent return of spontaneous circulation (ROSC). He had recurrent episodic complete heart block with no ventricular escape rhythm, associated with loss of cardiac output, unresponsive to transcutaneous pacing. He was transferred to theatre, while receiving CPR, for urgent removal of the VSD device. Estimated total ‘down time’ was 70 min. The device was removed and patch closure of the VSD was performed. He made a full neurological recovery. Device closure of septal defects has become widespread. We discuss the incidence and type of ...

[Clinical and hemodynamic features of Eisenmenger syndrome patients at the time of first admission: a tertiary referral-center experience.]

Anadolu Kardiyol Der... — Wed, 04 Jan 2012 05:00:00 +0100

CONCLUSION: The most common underlying heart disease of ES patients is VSD. In this cases exercise capacity is restricted and this restriction is reflected in laboratory parameters. PMID: 22214737 [PubMed - as supplied by publisher] (Source: Anadolu Kardiyol Der...)

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Classic and non-classic forms of mitral valve prolapse.

Anadolu Kardiyol Der... — Wed, 04 Jan 2012 05:00:00 +0100

CONCLUSION: The classic form of mitral valve prolapse is more tightly associated with morbid complications, and a more frequent follow-up control in this group of patients may be useful. PMID: 22214735 [PubMed - as supplied by publisher] (Source: Anadolu Kardiyol Der...)

Aortic Leaflet Replacement with Fresh Autologous Pericardium: At 17-Year Follow-Up.

The Thoracic and Cardiovascular Surgeon — Tue, 03 Jan 2012 05:00:00 +0100

We presented a case of a 2-year-old boy who underwent right coronary leaflet replacement with fresh autologous pericardium and ventricular septal defect correction. At 17-year follow-up, the valve was in good condition and the patient remained asymptomatic without any medication. PMID: 22215499 [PubMed - as supplied by publisher] (Source: The Thoracic and Cardiovascular Surgeon)

Aortic Leaflet Replacement with Fresh Autologous Pericardium: At 17-Year Follow-Up

The Thoracic and Cardiovascular Surgeon — Tue, 03 Jan 2012 05:00:00 +0100

We presented a case of a 2-year-old boy who underwent right coronary leaflet replacement with fresh autologous pericardium and ventricular septal defect correction. At 17-year follow-up, the valve was in good condition and the patient remained asymptomatic without any medication.[...]Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.Article in Thieme eJournals:Table of contents | Abstract | Full text (Source: The Thoracic and Cardiovascular Surgeon)

Successful maternal-foetal outcome using nitric oxide and sildenafil in pulmonary hypertension with atrial septal defect and HIV infection.

Singapore Medical Journal — Sun, 01 Jan 2012 05:00:00 +0100

We describe successful maternal-foetal outcome for a 30-year-old woman who was found to have severe pulmonary hypertension, human immunodeficiency virus (HIV) and an atrial septal defect. Prior to delivery, she was managed with subcutaneous enoxaparine, sildenafil, nitric oxide, careful maintenance of a euvolemic status and antiretroviral therapy. She was planned for an elective Caesarean section to reduce the risk of maternal-foetal HIV transmission, but went into labour in the coronary care unit. During delivery, antibiotic prophylaxis was given, although there was insufficient time for intravenous zidovudine. Peripartum, the patient was continued on nitric oxide and subcutaneous enoxaparine. She was eventually weaned off the nitric oxide and recovered well. PMID: 22252195 [PubMed - ...

Graded balloon dilatation as a prerequisite to Amplatzer device closure of perforated interatrial septal aneurysm.

Singapore Medical Journal — Sun, 01 Jan 2012 05:00:00 +0100

Authors: Goel PK, Kapoor A Abstract This case report describes a modification in the technique of atrial septal device closure in a patient with atrial septal defect (ASD) with associated atrial septum aneurysm and significant left-to-right shunt through multiple perforations. Graded balloon dilatation of the aneurysm was performed in this patient, as a preamble to successful deployment of a single large Amplatzer atrial septal occluder, closing the ASD completely and entrapping the aneurysm as a whole. PMID: 22252193 [PubMed - in process] (Source: Singapore Medical Journal)

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Intraoperative device closure of perimembranous ventricular septal defects in the young children under transthoracic echocardiographic guidance; initial experience

Journal of Cardiothoracic Surgery — Wed, 28 Dec 2011 05:00:00 +0100

Conclusions: Minimally invasive transthoracic device closure of perimembranous VSDs is safe and feasible, using a domestically made device under transthoracic echocardiographic guidance, without the need for cardiopulmonary bypass. This technique should be considered an acceptable alternative to surgery or device closure guided by transesophageal echocardiography in selected young children. However, a long-term evaluation of outcomes is necessary. (Source: Journal of Cardiothoracic Surgery)

Cardiopulmonary bypass

Surgery (Medicine Publishing) — Fri, 23 Dec 2011 00:08:11 +0100

This article gives an insight into the acute and long-term complications of CPB; it also discusses the support devices allied with cardiac surgery, from intra-aortic balloon counterpulsation to the components of long-term extracorporeal membrane oxygenation. (Source: Surgery (Medicine Publishing))

Clarifying the Natural History of the Doubly Committed Ventricular Septal Defect

Cardiology — Thu, 22 Dec 2011 23:00:00 +0100

Cardiology 2011;120:146–148 (DOI:10.1159/000334801) (Source: Cardiology)

Doubly Committed Ventricular Septal Defect: Single-Centre Experience and Midterm Follow-Up

Cardiology — Thu, 22 Dec 2011 23:00:00 +0100

Cardiology 2011;120:149–156 (DOI:10.1159/000334427) (Source: Cardiology)

Novel NKX2-5 mutations responsible for congenital heart disease.

Genetics and Molecular Research — Thu, 22 Dec 2011 03:54:02 +0100

Novel NKX2-5 mutations responsible for congenital heart disease. Genet Mol Res. 2011;10(4):2905-15 Authors: Wang J, Liu XY, Yang YQ Abstract Congenital heart disease (CHD) is the most common birth defect and is the leading cause of infant morbidity and mortality resulting from birth defects. Increasing evidence demonstrates that genetic variation in the NKX2-5 gene, which encodes a homeobox-containing transcription factor crucial to cardiogenesis, is an important molecular determinant for CHD. Nevertheless, the genetic components underlying CHD remain largely unknown. We screened NKX2-5 for potential molecular defects in patients with CHD. The entire coding region of NKX2-5 was initially sequenced in a cohort of 268 unrelated patients with CHD. The relatives of the patients...

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Pulmonary Atresia With Aortopulmonary and Coronary Artery Collaterals: Precise Depiction by Low-Dose Computed Tomography

Journal of the American College of Cardiology — Thu, 22 Dec 2011 02:25:47 +0100

A 6-year-old female with tetralogy of Fallot whose echocardiogram and cardiac catheterization showed pulmonary atresia, ventricular septal defect, aortopulmonary collaterals, and collaterals from the coronary arteries to the main pulmonary artery. Computed tomography (2 × 64-slice, 0.75-mm reconstruction; Online Video) with prospective electrocardiographic gating showed a large collateral from the right coronary artery ostium and another collateral arising from the left coronary artery ostium supplying a small main pulmonary artery (A and B). There was severe hypoplasia and isolation of the right pulmonary artery before bifurcation into upper and lower lobe branches, which were supplied by multiple aortopulmonary collaterals (A and C). This rare variant of tetralogy of Fallot has been pre...

The prevalence of chromosome 22q11.2 deletions in 2,478 children with cardiovascular malformations. A population‐based study

American Journal of Medical Genetics Part A — Wed, 21 Dec 2011 05:00:00 +0100

AbstractDeletion of chromosome 22q11.2 is considered one of the most frequent genetic causes of cardiovascular malformations. It is frequently associated with conotruncal malformations, but may also be present among patients with nonconotruncal malformations. The aim of the present study was to establish the prevalence of the 22q11.2 deletion in an unselected population‐based cohort of children with various cardiovascular malformations. The study population was defined as children born in 2000–2008 who were registered in the Danish National Patient Registry with a diagnosis of cardiovascular malformation from one of the two national departments of pediatric cardiology. Sensitive multiplex ligation‐dependent probe amplification was performed on dried blood spot samples from each indiv...

Detecting 22q11.2 deletion in Chinese children with conotruncal heart defects and single nucleotide polymorphisms in the haploid TBX1 locus

BMC Medical Genetics - Latest articles — Wed, 21 Dec 2011 05:00:00 +0100

Conclusions: CTDs, especially pulmonary atresia with ventricular septal defect and tetralogy of Fallot, are the most common disorders associated with the 22q11.2 deletion syndrome. Those patients with both CTDs and 22q11.2 deletion generally have a typical or atypical deletion region within the TBX1 gene. Our results indicate that TBX1 genetic variants may be associated with CTDs. (Source: BMC Medical Genetics - Latest articles)

Determinants of arterial gas embolism after scuba diving

Journal of Applied Physiology — Wed, 21 Dec 2011 05:00:00 +0100

Scuba diving is associated with breathing gas at increased pressure, which often leads to tissue gas supersaturation during ascent and the formation of venous gas emboli (VGE). VGE crossover to systemic arteries (arterialization), mostly through the patent foramen ovale, has been implicated in various diving-related pathologies. Since recent research has shown that arterializations frequently occur in the absence of cardiac septal defects, our aim was to investigate the mechanisms responsible for these events. Divers who tested negative for patent foramen ovale were subjected to laboratory testing where agitated saline contrast bubbles were injected in the cubital vein at rest and exercise. The individual propensity for transpulmonary bubble passage was evaluated echocardiographically. The...

Coronary Artery Disease in Adult Congenital Heart Disease: Outcome After Coronary Artery Bypass Grafting [ORIGINAL ARTICLES: ADULT CARDIAC]

The Annals of Thoracic Surgery — Wed, 21 Dec 2011 05:00:00 +0100

Conclusions Concomitant CABG may be required at the time of correction of ACHD. Survival is higher when a LIMA graft is used, and late functional outcome is good, with a low incidence of late angina and need for reintervention. (Source: The Annals of Thoracic Surgery)

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Pulmonary Atresia, Ventricular Septal Defect, and Major Aortopulmonary Collaterals: Neonatal Pulmonary Artery Rehabilitation Without Unifocalization [ORIGINAL ARTICLES: PEDIATRIC CARDIAC]

The Annals of Thoracic Surgery — Wed, 21 Dec 2011 05:00:00 +0100

Conclusions Rehabilitation of hypoplastic native pulmonary arteries by a neonatal shunting regimen, without MAPCA translocation, for pulmonary atresia, VSD, and MAPCAs, provides encouraging results with excellent early survival. (Source: The Annals of Thoracic Surgery)

Transcaval Correction of Partial Anomalous Pulmonary Venous Drainage Into the Superior Vena Cava [ORIGINAL ARTICLES: PEDIATRIC CARDIAC]

The Annals of Thoracic Surgery — Wed, 21 Dec 2011 05:00:00 +0100

Conclusions The vertical transcaval approach is a simple, highly reproducible technique for correction of partial anomalous pulmonary venous drainage into the SVC. It yields excellent results, with unobstructed pulmonary and systemic venous flow and without arrhythmia development. It can also be performed through a cosmetic right posterior thoracotomy. (Source: The Annals of Thoracic Surgery)

Outcomes of Surgical Repair of Double-Chambered Right Ventricle [ORIGINAL ARTICLES: PEDIATRIC CARDIAC]

The Annals of Thoracic Surgery — Wed, 21 Dec 2011 05:00:00 +0100

Conclusions Surgical correction of double-chambered right ventricle results in excellent functional and hemodynamic long-term results, with complete relief of the right ventricular obstruction. The presence of a double-chambered right ventricle should be considered in anomalies with high or persistent right ventricular outflow tract obstruction. (Source: The Annals of Thoracic Surgery)

Digital Subtraction Phonocardiography (DSP) applied to the detection and characterization of heart murmurs

BioMedical Engineering OnLine — Tue, 20 Dec 2011 05:00:00 +0100

Conclusions: Cardiac murmurs can be separated from underlying deterministic heart sounds using DSP. DSP has the potential to become a reliable and economical new diagnostic approach to screening for structural heart disease. However, DSP must be further evaluated in a large series of patients with well-characterized pathology to determine its clinical potential. (Source: BioMedical Engineering OnLine)

Transfusion-Free Complex Cardiac Surgery with Use of Deep Hypothermic Circulatory Arrest in a Preterm 2.96-kg Jehovah's Witness Neonate.

Texas Heart Institute Journal — Mon, 19 Dec 2011 09:30:03 +0100

Authors: Huebler M, Habazettl H, Boettcher W, Kuppe H, Hetzer R, Redlin M Abstract In neonates, the major obstacle to transfusion-free complex cardiac surgery is the severe hemodilution that can result from the mismatch between the priming volume of the circuit and the patients' blood volume. Herein, we report the case of a 13-day-old, 2.96-kg preterm neonate who had a hypoplastic aortic arch and atrial and ventricular septal defects. At the insistence of her Jehovah's Witness parents, we performed corrective surgery without transfusing homologous blood products-using deep hypothermic circulatory arrest in the process. A specially designed cardiopulmonary bypass circuit with a priming volume of only 95 mL was the key component of an interdisciplinary effort to avoid transfusion whi...

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Perimembranous ventricular septal defect with aneurysm: two options for transcatheter closure.

Texas Heart Institute Journal — Mon, 19 Dec 2011 09:30:03 +0100

We present our experience with 2 options for device closure of perimembranous ventricular septal defect with aneurysm. Thirty-four patients with perimembranous ventricular septal defect with aneurysm, aged from 14 to 42 years, underwent transcatheter closure with modified double-disk occluders. A sheath was used to deliver the occluder after establishment of a stable "arteriovenous loop" under fluoroscopy. Electrocardiography and transthoracic echocardiography were used for follow-up. All but 1 patient experienced successful transcatheter closure of perimembranous ventricular septal defect with aneurysm, when occluders were used in 2 different positions. There were 19 patients whose perimembranous ventricular septal defects were closed at the inlet of the aneurysm and 15 patients whose def...

Long-term follow-up of iatrogenic atrial septal defect after percutaneous mitral balloon valvuloplasty.

Texas Heart Institute Journal — Mon, 19 Dec 2011 09:30:03 +0100

In this study, we evaluated the factors affecting the development of iatrogenic atrial septal defect and searched for the possible influence of this defect on long-term outcomes.We reviewed the medical records of 267 patients who had undergone successful percutaneous mitral balloon valvuloplasty for symptomatic moderate or severe mitral stenosis from January 2000 through March 2004. Sixty-three of the 267 patients were enrolled in a face-to-face follow-up study. We noted their clinical and demographic characteristics. All included patients were asked for the endpoints of repeat percutaneous mitral balloon valvuloplasty or mitral valve surgery, cerebrovascular accident or transient ischemic attack, and the need of intervention for the iatrogenic atrial septal defect. They underwent standard...

First trimester diagnosis of parapagus diprosopus dibrachius dipus twins with cranirachischisis totalis by three‐dimensional ultrasound

Journal of Obstetrics and Gynaecology Research — Mon, 19 Dec 2011 05:00:00 +0100

AbstractParapagus (laterally fused), diprosopus (two faces), dibrachius (two upper extremities), dipus (two lower extremities) conjoined twinning is extremely rare. The coexistence of anencephaly with a contiguous spinal defect (craniorachischisis totalis) makes the present case one of the rarest of the published cases. In our case, it was difficult to make the final diagnosis by two‐dimensional abdominal and vaginal ultrasound. Three‐dimensional ultrasound was helpful for final diagnosis and post‐abortal examination confirmed the prenatal ultrasound diagnosis. The heart, diaphragm, liver and perineum were all united. Fine dissection of the heart showed four vessels arising from the ventricles and a membranous type ventricular septal defect. (Source: Journal of Obstetrics and Gynaeco...

Direct Percutaneous Left Ventricular Access and Port Closure: Pre-Clinical Feasibility

Journal of the American College of Cardiology: Cardiovascular Interventions — Mon, 19 Dec 2011 05:00:00 +0100

Conclusions Direct percutaneous LV access and closure is feasible using real-time MRI. A commercial occluder achieved hemostasis without evident deleterious effects on the LV. Having established the concept, further clinical development of this approach appears realistic. (Source: Journal of the American College of Cardiology: Cardiovascular Interventions)

Closed-Chest Transthoracic Magnetic Resonance Imaging-Guided Ventricular Septal Defect Closure in Swine

Journal of the American College of Cardiology: Cardiovascular Interventions — Mon, 19 Dec 2011 05:00:00 +0100

Conclusions Real-time MRI guidance allowed closed-chest transthoracic perventricular muscular VSD closure in a clinically meaningful animal model. Once applied to patients, this approach may avoid traditional surgical, percutaneous, or open-chest transcatheter ("hybrid") risks. (Source: Journal of the American College of Cardiology: Cardiovascular Interventions)

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Unrepaired Tetralogy of Fallot with Major Aortopulmonary Collateral Arteries in an Adult Patient

Congenital Heart Disease — Sun, 18 Dec 2011 05:00:00 +0100

We describe a 36‐year‐old man with unrepaired tetralogy of Fallot with distinctive aortopulmonary collaterals, who underwent complete surgical repair with good outcome. Two‐dimensional echocardiogram, cardiac magnetic resonance imaging, and cardiac catheterization each provided vital details allowing a stepwise approach to defining his unique anatomy for surgical correction. (Source: Congenital Heart Disease)

Pulmonary Atresia with Ventricular Septal Defect and Multifocal Pulmonary Blood Supply: Does an Intensive Interventional Approach Improve the Outcome?

Congenital Heart Disease — Fri, 16 Dec 2011 05:00:00 +0100

Conclusions. Combining surgical unifocalization procedures with subsequent early and intensive catheter‐based pulmonary artery rehabilitation may improve vascular growth, ultimately rendering many patients suitable for fenestrated VSD closure. Risk stratification, including intraoperative exit angiography, is essential to determine the need for early transcatheter interventions. (Source: Congenital Heart Disease)

Preferential Ductus Venosus Streaming Toward the Right Heart Is Associated with Left Heart Underdevelopment and Aortic Arch Hypoplasia in Human Fetuses.

Ultraschall in der Medizin — Fri, 16 Dec 2011 05:00:00 +0100

Conclusion: As in human fetuses with left diaphragmatic hernia, preferential ductus venosus streaming toward the right side of the heart can be found in a subgroup of fetuses with hypoplasia of left-sided cardiovascular structures. PMID: 22179800 [PubMed - as supplied by publisher] (Source: Ultraschall in der Medizin)

Absent pulmonary valve syndrome: prenatal cardiac ultrasound diagnosis with autopsy correlation

European Journal of Echocardiography — Wed, 14 Dec 2011 05:00:00 +0100

Absent pulmonary valve syndrome (APVS) is a rare conotruncal anomaly consisting of a severely hypoplastic pulmonary valve with annular stenosis, aneurysmal dilatation of main pulmonary artery with dilatation of one or both pulmonary artery branches, and a ventricular septal defect. Here, we report a prenatal echo diagnosis of APVS in a 27-year-old primi gravida at 20 weeks of gestation confirmed on fetal autopsy. A ‘bow tie’-like hypoechoic shadow in fetal cardiac ultrasound observed by us in a modified four-chamber view was suggestive of aneurysmal dilatation of branch pulmonary arteries. The consequences of continuation of pregnancy including immediate neonatal complications and possible medical and multistaged surgical interventions were well explained. Parents opted fo...

Echocardiography based estimation of pulmonary vascular resistance in patients with pulmonary hypertension: a simultaneous Doppler echocardiography and cardiac catheterization study

European Journal of Echocardiography — Wed, 14 Dec 2011 05:00:00 +0100

Conclusion The proposed Doppler-derived formula for estimating PVR based on the conventionally used invasive equation strongly correlates with invasive gold standard measures. (Source: European Journal of Echocardiography)

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Absent pulmonary valve syndrome: prenatal cardiac ultrasound diagnosis with autopsy correlation

European Journal of Echocardiography — Wed, 14 Dec 2011 05:00:00 +0100

Echocardiography based estimation of pulmonary vascular resistance in patients with pulmonary hypertension: a simultaneous Doppler echocardiography and cardiac catheterization study

European Journal of Echocardiography — Wed, 14 Dec 2011 05:00:00 +0100

Surgical Management of Left Ventricular Outflow Tract Obstruction

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

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

Transcatheter closure of the perimembranous ventricular septal defect—preclinical trial of a new amplatzer device

Catheterization and Cardiovascular Interventions — Mon, 12 Dec 2011 05:00:00 +0100

Conclusions: The success of this animal study confirms safety and feasibility of the Amplatzer pmVSO2 device. Human trials are planned. © 2011 Wiley Periodicals, Inc. (Source: Catheterization and Cardiovascular Interventions)

Orthodontic treatment of a transposed gigantic canine --a case report [ClinicalSection]

Journal of Orthodontics — Fri, 09 Dec 2011 05:00:00 +0100

Cuspid gigantism or radicomegaly is a rare occurrence often associatedwith a hereditary, X-linked condition, called oculo-cardio-facio-dental (OFCD)syndrome. This syndrome is also characterized by atrial septal defects and/orventricular septal defects, congenital cataracts and unusual facial characteristics.Confirmation of this syndrome is often provided by dentists or orthodontistswhen they diagnose an extremely large canine root length from the panoramicradiograph. The following case report shows a multidisciplinary approach tothe dental treatment of a patient with OFCD syndrome and a canine-first premolartransposition. The orthodontic approach required a mini-plate to achieve significantmesial movement of a gigantic maxillary canine that erupted in close contactwith the first molar. (So...

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Orthodontic treatment of a transposed gigantic canine --a case report [Clinical Section]

Journal of Orthodontics — Fri, 09 Dec 2011 05:00:00 +0100

Polycomb Repressive Complex 2 Regulates Normal Development of the Mouse Heart.

Circulation Research — Thu, 08 Dec 2011 05:00:00 +0100

Conclusions:Our study reveals a previously undescribed role of EZH2 in regulating heart formation and shows that perturbation of the epigenetic landscape early in cardiogenesis has sustained disruptive effects at later developmental stages. PMID: 22158708 [PubMed - as supplied by publisher] (Source: Circulation Research)

Totally endoscopic atrial septal repair using no robotic techniques [ORIGINAL ARTICLE]

Asian Cardiovascular and Thoracic Annals — Wed, 07 Dec 2011 05:00:00 +0100

We describe a technique of totally endoscopic closed-chest atrial septal defect closure without the aid of a robotic device. Twenty patients underwent totally endoscopic atrial septal defect repair using no robotic techniques between May 2009 and December 2009. No major intraoperative or postoperative complications were observed. One operation was converted to a conventional sternotomy because bleeding from the aortic root could not be controlled. Closure of an atrial septal defect can be performed safely and effectively via an endoscopic approach using no robotic techniques. (Source: Asian Cardiovascular and Thoracic Annals)

Fenestration closure in a calcified ventricular septal defect patch [CASE STUDIES]

Asian Cardiovascular and Thoracic Annals — Wed, 07 Dec 2011 05:00:00 +0100

We describe the closure of such a defect in a 6-year-old boy, using a double Dacron patch sandwich. (Source: Asian Cardiovascular and Thoracic Annals)

Late Migration of Percutaneous Bio‐Absorbable Devices—A Word of Caution

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

Conclusions: The overall incidence of perforation of cardiac structures due to secondary dislocation is low. However this complication exists and should kept in mind in symptomatic patients with new onset of chest pain, after percutaneous procedures. The concept of biodegradation, with residual, non absorbable metal braiding, should be reviewed, analyzing in particular long term results and incidence of secondary dislocation. (J Card Surg 2011; **:1‐3) (Source: Journal of Cardiac Surgery)

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Surgical Correction for Sinus of Valsalva Aneurysm with Right Ventricular Outflow Tract Stenosis

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

Conclusions: Surgical correction of SVA with right ventricular outflow tract stenosis results in good mid‐term results. Longer follow‐up is needed to determine the efficacy of this procedure as this cohort of patients ages. (J Card Surg 2011;**:1‐4) (Source: Journal of Cardiac Surgery)

Early Fetal Echocardiography: Ready for Prime Time?

American Journal of Perinatology — Mon, 05 Dec 2011 05:00:00 +0100

In conclusion, early fetal echocardiography appears to be a reasonable screening tool for major cardiac defects.[...]Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.Article in Thieme eJournals:Table of contents | Abstract | Full text (Source: American Journal of Perinatology)

Cobalamin F Disease Detected by Newborn Screening and Follow-up on a 14-Year-Old Patient

PEDIATRICS — Thu, 01 Dec 2011 05:00:00 +0100

We present the clinical and biochemical characterization of a patient with newly diagnosed cblF disease and a follow-up on a 14-year-old patient. The new patient presented with elevation of propionyl carnitine found on a newborn screen. The patient was small for gestational age, exhibited dysmorphic features and mild developmental delay, and had trigonocephaly and ventricular septal defect. There was biochemical normalization and clinical improvement within 3 weeks of parenteral cobalamin treatment. The other patient presented at 4 weeks of life with failure to thrive and feeding difficulties. She was treated only with monthly cyanocobalamin shots. The patient has never experienced metabolic decompensation. She had short stature and was an average student with no behavioral concerns. Her m...

Anesthesia management of totally endoscopic atrial septal defect repair with a robotic surgical system

Journal of Clinical Anesthesia — Thu, 01 Dec 2011 05:00:00 +0100

Conclusions: The key issue for anesthetic management of robot-assisted totally endoscopic ASD repair is maintaining stable hemodynamics and oxygenation, especially during one-lung ventilation and capnothorax. (Source: Journal of Clinical Anesthesia)

Transcatheter aortic valve implantation and simultaneous closure of ostium secundum atrial septal defect.

The Heart Surgery Forum — Thu, 01 Dec 2011 05:00:00 +0100

Authors: Pasic M, Berger F, D'Ancona G, Dreysse S, Buz S, Drews T, Hetzer R, Unbehaun A, Kukucka M Abstract We were faced with a difficult question: how to treat a high-risk patient with severe aortic valve stenosis and a secundum atrial septal defect (ASD II). An 85-year-old woman with progressive dyspnea and pedal edema and in New York Heart Association class IV was treated with concomitant transapical aortic valve implantation and transcatheter closure of the ASD II. The combined procedure and postoperative course were completely uneventful. At 2 years after the clinical follow-up, the patient is doing well. This case report demonstrates, for the first time, the feasibility, safety, and effectiveness of simultaneous application of 2 transcatheter methods-aortic valve implantatio...

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Cognitive P300‐evoked potentials in school‐age children after surgical or transcatheter intervention for ventricular septal defect

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

Conclusion: VSD patients, especially those undergoing surgery, showed poor cognitive function, which may be associated with duration of cardiopulmonary bypass or aortic‐clamping. (Source: Pediatrics International)

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

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

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

Transvenous Extraction of Pacemaker Leads in Infective Endocarditis With Vegetations ≥20 mm: Our Experience

Clinical Cardiology — Thu, 01 Dec 2011 05:00:00 +0100

Conclusions:Transvenous extraction of pacing leads with larger vegetations is a feasible technique. There was a tendency toward symptomatic pulmonary embolism in patients with vegetations larger than 20 mm; however, morbidity and mortality were not influenced. We agree with the consensus that this procedure is highly useful and that the selection of the removal techniques will depend not only on the size of vegetation but also on prior cardiopulmonary conditions, concomitant cardiac surgery, atrial septal defect with risk of paradoxical embolism, center experience, and the possibility of complete removal of the device. © 2011 Wiley Periodicals, Inc.The authors have no funding, financial relationships, or conflicts of interest to disclose. (Source: Clinical Cardiology)

Postinfarction Ventricular Septal Defects: Surgical or Percutaneous Closure—Between a Rock and a Hard Place

Journal of Cardiothoracic and Vascular Anesthesia — Thu, 01 Dec 2011 00:53:10 +0100

To comment on this case and the clinical decisions made, please go to: (Source: Journal of Cardiothoracic and Vascular Anesthesia)

Unusual Combination of Holt‐Oram Syndrome and Persistent Left Superior Vena Cava

Congenital Heart Disease — Wed, 30 Nov 2011 05:00:00 +0100

Conclusion. We report an uncommon association of HO and PLSVC. This association was only reported twice in the past and this is the first one that constitutes a casual finding during the attempt of CRT device implant. This is a combination that may complicate a device implant and recognition of it in advance may avoid performing potentially unsuccessful procedures. (Source: Congenital Heart Disease)

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A deflectable guiding catheter for real‐time MRI‐guided interventions

Journal of Magnetic Resonance Imaging — Tue, 29 Nov 2011 05:00:00 +0100

Conclusion:Kevlar and short nitinol component substitutions preserved mechanical properties. The active design offered the best visibility and usability but reintroduced metal conductors. We describe versatile deflectable guiding catheters with a 0.057” lumen for interventional MRI catheterization. Implementations are feasible using active, inductive, and passive visualization strategies to suit application requirements. J. Magn. Reson. Imaging 2011. © 2011 Wiley Periodicals, Inc. (Source: Journal of Magnetic Resonance Imaging)

Ischemic Stroke Caused by Paradoxical Embolism After an Unsuccessful Transcatheter Atrial Septal Defect Closure Procedure: A Word of Caution

Pediatric Cardiology — Sat, 26 Nov 2011 16:46:31 +0100

Abstract Transcatheter device closure of atrial septal defect (ASD) has become a well-accepted alternative to surgical repair. Serious complications of transcatheter ASD closure are rare, but when they occur, devastating consequences may result. Herein, we present the case of a 4-year-old girl who had an ischemic stroke caused by a presumptive paradoxical embolism after an unsuccessful transcatheter ASD procedure and in whom subsequent venous color Doppler showed deep venous thrombosis (DVT) of the right lower extremity. The risk factors that predisposed to paradoxical cerebral embolism and DVT in this patient are discussed, and the literature is reviewed. Content Type Journal ArticleCategory Case ReportPages 1-4DOI 10.1007/s00246-011-0150-2Authors Zhen-fei Fang, Dep...

[Non-compacted cardiomyopathy: clinical characteristics, evolution and prognostic data in childhood. results of a multicentre study.]

Anales de Pediatria — Fri, 25 Nov 2011 05:00:00 +0100

CONCLUSIONS: Early onset of symptoms is associated with a poor prognosis. Clinical and prognostic heterogeneity is described. PMID: 22119727 [PubMed - as supplied by publisher] (Source: Anales de Pediatria)

A Simple Technique to Facilitate the Right Ventricular Outflow Tract Reconstruction During the Arterial Switch Operation

Heart, Lung and Circulation — Fri, 25 Nov 2011 02:38:01 +0100

The arterial switch operation has now become a standard procedure in the management of patients with d-transposition of the great arteries with or without a ventricular septal defect. Following the coronary anastomosis and aortic reconstruction, the right ventricular outflow tract (RVOT) is reconstructed. The proximal neo-pulmonary artery is often reconstructed on cardioplegic arrest whereas the distal anastomosis to the transected main pulmonary artery is often performed on a beating perfused heart, although the techniques may vary according to the surgeon preference. We have simplified this technique to accomplish this procedure in a bloodless field. (Source: Heart, Lung and Circulation)

Pulmonary arteriovenous fistula in a patient with cryptogenic stroke

Heart — Thu, 24 Nov 2011 05:00:00 +0100

A 43-year-old female patient presented with transient right-sided facial numbness and dysarthria. MRI of the brain demonstrated an ischaemic infarct of the right medial occipital lobe. Embolic cerebral infarction was suspected, and transoesophageal echocardiography with injection of agitated saline was undertaken. This revealed microbubbles entering the left atrium (figure 1A,B) from the left upper pulmonary vein. There was no evidence of atrial septal defect or patent foramen ovale. Computed tomographic arteriography confirmed a pulmonary arteriovenous fistula (PAVF) originating from the left superior lingular segmental pulmonary artery and draining into the left upper pulmonary vein (figure 1C). There was no other clinical sign of hereditary haemorrhagic telangiectasia (HHT) and no mutat...

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Surgical removal of fractured ATRIASEPT occluder 5 months after successful percutaneous closure of atrial septal defect [Images in cardio-thoracic surgery]

European Journal of Cardio-Thoracic Surgery — Wed, 23 Nov 2011 05:00:00 +0100

(Source: European Journal of Cardio-Thoracic Surgery)

Early Thrombosis of a Tricuspid Annuloplasty Ring and Mild Hyperhomocysteinemia [CASE REPORTS]

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

We present the case of a 40-year-old female with isolated mild hyperhomocysteinemia and early ring thrombus formation after surgical primary closure of an atrial septal defect and ring tricuspid annuloplasty. Clinical management, antithrombotic treatment, and hyperhomocysteinemia implications are discussed. (Source: The Annals of Thoracic Surgery)

Variation in Outcomes for Benchmark Operations: An Analysis of The Society of Thoracic Surgeons Congenital Heart Surgery Database [RICHARD E. CLARK AWARD]

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

Conclusions This analysis documents contemporary benchmarks for common pediatric cardiac surgical operations and the range of outcomes among centers. Variation was most prominent for the more complex operations. These data may aid in quality assessment and quality improvement initiatives. (Source: The Annals of Thoracic Surgery)

A Growing Problem: Maternal Death and Peripartum Complications Are Higher in Women With Grown-Up Congenital Heart Disease [ORIGINAL ARTICLES: PEDIATRIC CARDIAC]

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

Conclusions The GUCH parturients, especially those with VSD, have increased risk of mortality and peripartum complications compared with other age-matched women. Despite these risks, nearly 50% of GUCH patients deliver at nonteaching hospitals. Current national practice patterns for GUCH women are inadequate, and outcomes could be improved by education and proper triage of even relatively "simple" GUCH lesions such as atrial septal defect and VSD. Further studies that investigate risk-adjusted outcomes in a variety of care settings are necessary to resolve this complex issue. (Source: The Annals of Thoracic Surgery)

Mechanisms of Pulmonary Hypertension Related to Ventricular Septal Defect in Congenital Heart Disease [ORIGINAL ARTICLES: PEDIATRIC CARDIAC]

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

Conclusions In patients with TGA + VSD, high oxygenation in the pulmonary circulation decreases expression of MMP-2 and eNOS, which may affect the progress and reversibility of pulmonary hypertension. (Source: The Annals of Thoracic Surgery)

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

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

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

Quality of Life in Patients Undergoing Totally Thoracoscopic Closure for Atrial Septal Defect [ORIGINAL ARTICLES: PEDIATRIC CARDIAC]

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

Conclusions Compared with conventional sternotomy, totally thoracoscopic atrial septal defect closure was associated with a faster recovery of physical function and a better quality of life. (Source: The Annals of Thoracic Surgery)

Dgcr8 controls neural crest cells survival in cardiovascular development.

Developmental Biology — Wed, 23 Nov 2011 05:00:00 +0100

Authors: Chapnik E, Sasson V, Blelloch R, Hornstein E Abstract DiGeorge syndrome (DGS), characterized genetically by a deletion within chromosome 22q11.2, is associated with a constellation of congenital heart defects. DiGeorge critical region 8 (Dgcr8), a gene that maps to the common deletion region for DGS, encodes a double stranded RNA-binding protein that is essential for miRNA biogenesis. To address the potential contribution of Dgcr8 insufficiency to cardiovascular development, we have inactivated Dgcr8 in cardiac neural crest cells (cNCCs). Dgcr8 mutants displayed a wide spectrum of malformations, including persistent truncus arteriosus (PTA) and ventricular septal defect (VSD). Interestingly, Dgcr8-null cNCCs that properly migrated into the cardiac outflow tract (OFT) proli...

Transcatheter closure of perimembranous and intracristal ventricular septal defects with the SHSMA occluder

Catheterization and Cardiovascular Interventions — Tue, 22 Nov 2011 05:00:00 +0100

Conclusions:Transcatheter closure of pmVSD in selected patients using the SHSMA occluder is effective and safe. Transcatheter closure of icVSD in experienced hands using this device is also feasible and safe. © 2011 Wiley‐Liss, Inc. (Source: Catheterization and Cardiovascular Interventions)

Closure of multiple fenestrations in an aneurismal atrial septum using overlapping GORE® HELEX septal occluders

Catheterization and Cardiovascular Interventions — Tue, 22 Nov 2011 05:00:00 +0100

We present the successful use of overlapping GORE® HELEX atrial septal defect occluders in an adult patient with 5 distinct fenestrations in an aneurismal septum and how 3D echo can aid device positioning. © 2011 Wiley‐Liss, Inc. (Source: Catheterization and Cardiovascular Interventions)

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Wire fractures in solysafe® septal occluders: a single center experience

Catheterization and Cardiovascular Interventions — Tue, 22 Nov 2011 05:00:00 +0100

Conclusions:The incidence of DF after iSSO unacceptably high. sF is imperative for accurate diagnosis of DF. Further follow‐up is needed to determine the risk of clinical complications and to optimize management. © 2011 Wiley‐Liss, Inc. (Source: Catheterization and Cardiovascular Interventions)

Anterior Ethmoidal Artery Septal Flap for the Management of Septal Perforation [Original Article]

Archives of Facial Plastic Surgery — Mon, 21 Nov 2011 05:00:00 +0100

Conclusion Closure of a perforated nasal septum through an endonasal technique can be achieved with a unilateral mucosal flap based on the anterior ethmoidal artery. (Source: Archives of Facial Plastic Surgery)

Trisomy 18 mosaicism: report of two cases.

World Journal of Pediatrics : WJP — Mon, 21 Nov 2011 05:00:00 +0100

CONCLUSION: There is the possibility of good outcome for patients with mosaic trisomy 18, even in the presence of multiple congenital anomalies. PMID: 22105572 [PubMed - as supplied by publisher] (Source: World Journal of Pediatrics : WJP)

A Novel GATA4 Loss-of-Function Mutation Associated With Congenital Ventricular Septal Defect

Pediatric Cardiology — Sat, 19 Nov 2011 16:54:34 +0100

In this study, the entire coding region of GATA4, a gene encoding a zinc finger transcription factor essential for normal cardiac morphogenesis, was sequenced in 160 unrelated patients with VSD. The available relatives of the index patient harboring the identified mutation and 200 unrelated control individuals were subsequently genotyped. The disease-causing potential of a sequence alteration was evaluated by MutationTaster, and the functional effect of the mutation was characterized using a luciferase reporter assay system. As a result, a novel heterozygous GATA4 variation, p.R43W, was identified in a proband with VSD, that was absent in control subjects. Genetic analysis of the family members of the variation carrier showed that the substitution co-segregated with VSD. The p.R43W va...

Azygous Continuation of the Inferior Vena Cava With Anomalous Hepatic Vein Drainage.

The American Journal of the Medical Sciences — Thu, 17 Nov 2011 05:00:00 +0100

Authors: Guardado FJ, Byrd TM, Petersen WG Abstract A 62-year-old woman was referred for evaluation of hypoxemia. Despite a resting SaO2 of 89%, she was otherwise asymptomatic. The arterial blood gas on room air demonstrated a pO2 of 58 mm Hg. Two shunt studies confirmed a shunt fraction of 25%. Echocardiogram with agitated saline demonstrated bubbles in the left atrium. Nuclear perfusion study revealed no uptake over the kidneys or brain. Computed tomography of the chest and cardiac magnetic resonance imaging confirmed the presence of a large shunt (azygous continuation of the inferior vena cava [ACIVC] with anomalous hepatic vein drainage). A large shunt fraction dictates either significant cardiac septal defect or an extracardiac shunt. The finding of ACIVC should prompt conside...

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[Follow-up of our patients with transposition of the great arteries and arterial switch operation; comparison of simple and complex transposition cases.]

Anadolu Kardiyol Der... — Wed, 16 Nov 2011 05:00:00 +0100

CONCLUSION: Arterial switch operation provides anatomical correction in TGA. Appropriate timing and good perioperative planning facilitates low morbidity and mortality in patients with VSD as in patients with simple TGA. PMID: 22088861 [PubMed - as supplied by publisher] (Source: Anadolu Kardiyol Der...)

Outcomes of Surgical Repair of Double-Chambered Right Ventricle.

The Annals of Thoracic Surgery — Tue, 15 Nov 2011 05:00:00 +0100

CONCLUSIONS: Surgical correction of double-chambered right ventricle results in excellent functional and hemodynamic long-term results, with complete relief of the right ventricular obstruction. The presence of a double-chambered right ventricle should be considered in anomalies with high or persistent right ventricular outflow tract obstruction. PMID: 22093693 [PubMed - as supplied by publisher] (Source: The Annals of Thoracic Surgery)

Reoperation for prosthetic ventricular septal defect patch endocarditis: long-term results with an autologous atrial septal patch

General Thoracic and Cardiovascular Surgery — Mon, 14 Nov 2011 17:03:44 +0100

We report a successful case of reclosure of a ventricular septal defect using an autologous atrial septal patch taken from the fossa ovalis. There were no complications, and the long-term results were satisfactory. The autologous septal patch might be a promising material for prosthetic patch endocarditis. Content Type Journal ArticleCategory Case ReportPages 753-755DOI 10.1007/s11748-010-0768-7Authors Takako Miyazaki, Department of Pediatric Cardiovascular Surgery, Children’s Research Hospital, Kyoto Prefectural University of Medicine, 456 Kajii-cho, Hirokoji, Kawaramachi, Kamigyo-ku, Kyoto, 602-8566 JapanMasaaki Yamagishi, Department of Pediatric Cardiovascular Surgery, Children’s Research Hospital, Kyoto Prefectural University of Medicine, 456 Kajii-cho, Hirokoji, Kawaramac...

Surgical management of muscular trabecular ventricular septal defects

General Thoracic and Cardiovascular Surgery — Mon, 14 Nov 2011 17:03:35 +0100

This article describes the anatomical details and classification of muscular trabecular VSDs. It also reviews several techniques currently utilized and their outcomes. Content Type Journal ArticleCategory Current Topics Review ArticlePages 723-729DOI 10.1007/s11748-011-0826-9Authors Toshihide Asou, Department of Cardiovascular Surgery, Kanagawa Children’s Medical Center, 2-138-4 Mutsukawa, Minami-ku, Yokohama, Kanagawa, 232-8555 Japan Journal General Thoracic and Cardiovascular SurgeryOnline ISSN 1863-6713Print ISSN 1863-6705 Journal Volume Volume 59 Journal Issue Volume 59, Number 11 (Source: General Thoracic and Cardiovascular Surgery)

Ventricular septal necrosis after blunt chest trauma. - Sabzi F, Sahebjamee F, Zokaei AH, Niazi M, Bazarghan-Hejazi S, Ahmadi A.

SafetyLit: All (Unduplicated) — Sun, 13 Nov 2011 23:51:22 +0100

We report here a case of blunt chest injury-related VSD and pseudoaneurysm. A 30-year old male truck driver was referred from a trauma center to our hospital seven... (Source: SafetyLit: All (Unduplicated))

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Renal cell carcinoma with right atrial extension with associated atrial septal defect: a case report

Indian Journal of Thoracic and Cardiovascular Surgery — Fri, 11 Nov 2011 12:48:23 +0100

We present a case of locally advanced renal cell carcinoma with tumor thrombus in the inferior vena-cava extending into the right atrium with associated atrial septal defect. Tumor removal posed a unique challenge in this patient as there was a risk of paradoxical embolism during the surgery. Content Type Journal ArticleCategory Case ReportPages 1-3DOI 10.1007/s12055-011-0120-6Authors Prashant Sevta, Department of Cardiothoracic & Vascular Surgery, G. B. Pant Hospital, New Delhi, IndiaSaket Agarwal, Department of Cardiothoracic & Vascular Surgery, G. B. Pant Hospital, New Delhi, IndiaSaket Singh, Department of Cardiothoracic & Vascular Surgery, G. B. Pant Hospital, New Delhi, IndiaSubodh Satyarthi, Department of Cardiothoracic & Vascular Surgery, G. B. Pant Hospital, New Delhi, In...

Surgical Treatment of Infective Valve Endocarditis in Children with Congenital Heart Disease

Journal of Cardiac Surgery — Fri, 11 Nov 2011 05:00:00 +0100

Conclusions: Mitral and tricuspid valve repairs can be performed with low morbidity/mortality rates and satisfactory intermediate‐term results in children with infective valve endocarditis. (J Card Surg 2011;**:1‐6) (Source: Journal of Cardiac Surgery)

Tips and tricks to prevent prolapse of the amplatzer septal occluder through Large atrial septal defects

Catheterization and Cardiovascular Interventions — Wed, 09 Nov 2011 05:00:00 +0100

We report on a new technique “using a partially inflated balloon through the atrial septal defect” to prevent prolapse of the left atrial disk of the Amplatzer septal occulder in large atrial septal defects (ASDs). Two patients underwent successful closure of their ASDs using this new technique. © 2011 Wiley‐Liss, Inc. (Source: Catheterization and Cardiovascular Interventions)

Critical Congenital Heart Disease—Utility of Routine Screening for Chromosomal and Other Extracardiac Malformations

Congenital Heart Disease — Wed, 09 Nov 2011 05:00:00 +0100

Conclusion. Infants with critical CHD and particularly premature infants have high incidence of genetic and other extracardiac anomalies. Universal screening for these abnormalities with ultrasonographic and genetic testing maybe warranted because early detection could impact short and long‐term outcomes. (Source: Congenital Heart Disease)

Evaluation of Right Ventricular Function in Early Period Following Transcatheter Closure of Atrial Septal Defect

Echocardiography — Tue, 08 Nov 2011 05:00:00 +0100

Conclusion: Closure of ASD by using Amplatzer devices led to decrease in right heart chamber size, tissue Doppler–derived tricuspid annular velocities and TAPSE in early period. (Echocardiography ****;**:1‐5) (Source: Echocardiography)

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Identification and characterization of a complex pure mosaic of small supernumerary marker chromosomes involving 11p11.12 → q12.1 and 19p12 → q12 regions in a child featuring multiple congenital anomalies

American Journal of Medical Genetics Part A — Tue, 08 Nov 2011 05:00:00 +0100

AbstractUnstable, gene‐rich pericentric regions have been associated with various structural aberrations including small supernumerary marker chromosomes (sSMCs). We hereby report on a complex pure mosaic sSMCs derived from chromosomes 11 and 19 in a child featuring multiple congenital anomalies. As indicated by microarray analysis, the sSMCs have involved materials from 11p11.12 → q12.1 and 19p12 → q12 in complex forms (with four cell lines harboring from 1 to 4 sSMCs) in all peripheral blood lymphocytes. The patient featured facial dysmorphism, generalized hypotonia, cryptorchidism, transverse palmar creases, cerebral hemorrhage, atrial septal defect secundum, strabismus, epilepsy, immunodeficiency, and severe cognitive and motor impairment. Literature review indicated this...

Neurohormonal and cytokine fluctuations following transcatheter closure for an atrial septal defect.

Cytokine — Tue, 08 Nov 2011 05:00:00 +0100

CONCLUSIONS: Transcatheter closure could improve, on a mid- term basis, the inflammatory process but natriuretic peptides' secretion continues in parallel with left atrial volume increase. Further follow up is required to determine the long-term progress of the inflammatory and neurohormonal response to the procedure. PMID: 22075401 [PubMed - as supplied by publisher] (Source: Cytokine)

Longest survivor of pulmonary atresia with ventricular septal defect: well-developed major aortopulmonary collateral arteries demonstrated by multidetector computed tomography.

Circulation — Tue, 08 Nov 2011 05:00:00 +0100

Authors: Fukui D, Kai H, Takeuchi T, Gondo T, Oba T, Mawatari K, Tonai T, Matsuo Y, Ueda S, Niiyama H, Ueno T, Imaizumi T PMID: 22064959 [PubMed - in process] (Source: Circulation)

Pulmonary artery sling: Current results with cardiopulmonary bypass

The Journal of Thoracic and Cardiovascular Surgery — Fri, 04 Nov 2011 04:00:00 +0100

Conclusions: Pulmonary artery sling is best repaired with median sternotomy, cardiopulmonary bypass, and left pulmonary artery reimplantation. This resulted in uniformly patent left pulmonary arteries in all patients. Preoperative computed tomography imaging, echocardiography, and bronchoscopy are essential for precise operative planning. The frequently associated tracheal stenosis is best repaired with slide tracheoplasty. (Source: The Journal of Thoracic and Cardiovascular Surgery)

Over-Expression of DSCAM and COL6A2 Cooperatively Generates Congenital Heart Defects

PLoS Genetics — Thu, 03 Nov 2011 04:00:00 +0100

by Tamar R. Grossman, Amir Gamliel, Robert J. Wessells, Ouarda Taghli-Lamallem, Kristen Jepsen, Karen Ocorr, Julie R. Korenberg, Kirk L. Peterson, Michael G. Rosenfeld, Rolf Bodmer, Ethan Bier A significant current challenge in human genetics is the identification of interacting genetic loci mediating complex polygenic disorders. One of the best characterized polygenic diseases is Down syndrome (DS), which results from an extra copy of part or all of chromosome 21. A short interval near the distal tip of chromosome 21 contributes to congenital heart defects (CHD), and a variety of indirect genetic evidence suggests that multiple candidate genes in this region may contribute to this phenotype. We devised a tiered genetic approach to identify interacting CHD candidate genes. We first us...

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Cor Triatriatum Dextro Iatrogenica: An Unusual Complication of Atrial Septal Defect Closure Device

Echocardiography — Wed, 02 Nov 2011 04:00:00 +0100

We present a case of a rare complication of atrial septal defect (ASD) device closure causing cor triatriatum dextro iatrogenica. A 29‐year‐old female presented with sudden onset dysarthria and ataxia and was found to have basilar and thalamic infarcts. Further evaluation using transthoracic echocardiography revealed an ASD which was repaired using the Gore HELEX septal occluder. Transesophageal echocardiography done after 2 months of ASD closure revealed an interesting finding termed cor triatriatum dextro iatrogenica. We briefly describe the case and discuss the relevant literature. (Echocardiography ****;**:E1‐E3) (Source: Echocardiography)

Deterioration of Mitral Valve Competence After the Repair of Atrial Septal Defect in Adults [ORIGINAL ARTICLES: ADULT CARDIAC]

The Annals of Thoracic Surgery — Tue, 01 Nov 2011 04:00:00 +0100

Conclusions Mitral valve competence may deteriorate after ASD closure in older patients with large ASD. (Source: The Annals of Thoracic Surgery)

Long-Term Functional Analysis of the Atrioventricular Valve in Patients Undergoing Single Ventricle Palliation [ORIGINAL ARTICLES: PEDIATRIC CARDIAC]

The Annals of Thoracic Surgery — Tue, 01 Nov 2011 04:00:00 +0100

Conclusions If performed properly, atrioventricular valve repair may neutralize the risk of regurgitation regardless of the valve morphology. The effort to incorporate the mitral valve into the systemic circulation may be important to reduce tricuspid regurgitation. The effort to decompress a nonsystemic left ventricle, if present, may be important to avoid unfavorable ventricular interactions on the mitral valve. (Source: The Annals of Thoracic Surgery)

Oral presentation

Pacing and Clinical Electrophysiology : PACE — Tue, 01 Nov 2011 04:00:00 +0100

Conclusion: i.v. niferidile in doses up to 30 mkg/kg seems to be very effective (up to 88%) and safe for sinus rhythm restoration in patients with persistent AF and AFL.O002ELECTROPHYSIOLOGIC AND ANTIARRHYTHMIC EFFECTS OF NEW CLASS III AGENT NIFERIDILE IN PATIENTS WITH PAROXYSMAL SUPRAVENTRICULAR TACHYCARDIASMironov NYu; Golitsyn SP; Sokolov SF; Yuricheva YuA; Maikov EB; Shlevkov NB; Rosetstraukh LV; Chazov EI  Russian cardiology research complexBackground: According to preclinical studies Niferidile (Nf) is a novel potassium channel blocker that inhibits transient outward and delayed rectifier currents and increases effective refractory periods (ERP) more in atria, less in ventricles. High affinity of Nf to atrial myocardium predispose to high efficacy in patients with supraventricular ...

Echocardiographic Diagnosis and Surgical Closure of Coronary Sinus Type of Atrial Septal Defect

Journal of Cardiac Surgery — Tue, 01 Nov 2011 04:00:00 +0100

Abstract Coronary sinus type of atrial septal defect (ASD CS) is a rare cardiac anomaly and is rarely diagnosed by only transthoracic echocardiography prior to surgical operation. We now describe a technique to treat this defect with the aid of two‐dimensional echocardiography, which prevents trauma to the conduction system and maintains drainage from the coronary sinus into the right atrium (RA). (J Card Surg 2011;26:653‐656) (Source: Journal of Cardiac Surgery)

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[Surgical treatment of DeBakey type II aortic dissection combined with true aneurysm in the distal aortic arch: a case report].

Journal of Southern Medical University — Tue, 01 Nov 2011 04:00:00 +0100

We report a case with this pathology in a 23-year-old man, who received a previous open heart surgery for ventricular septal defect at the age of 6 years. Aortic valve, ascending aorta and total aortic arch replacements were performed. Cardiopulmonary bypass (CPB) including deep hypothermic circulatory arrest with selective antegrade cerebral perfusion was used. No postoperative complications occurred, and the follow-up examination at 6 months after the operation showed good therapeutic results. PMID: 22126790 [PubMed - in process] (Source: Journal of Southern Medical University)

Transcatheter Closure of a Large Atrial Septal Defect under Microprobe Transesophageal Echocardiographic Guidance

Echocardiography — Tue, 01 Nov 2011 04:00:00 +0100

We present a case of an atrial septal defect (ASD) in a 59‐year‐old man with an indication for ASD closure who also had a history of chronic obstructive pulmonary disease. Because of his decreased respiratory function with multiple bullae in his lungs, the procedure was performed without general anesthesia under the guidance of fluoroscopy and two‐dimensional (2D) transesophageal echocardiography (TEE) using a transesophageal echocardiographic microprobe (micro‐TEE) (S8‐3t; Philips Medical Systems, Andover, MA, USA). The micro‐TEE probe was inserted into the esophagus smoothly and easily in the supine position without sedation. It revealed a deficient superior‐anterior rim and adequate rims elsewhere, and the maximal diameter of ASD was measured to be 25 mm. Balloon sizing re...

Efficacy and Safety of Bosentan in Adults with Simple and Complex Eisenmenger's Syndrome

Congenital Heart Disease — Tue, 01 Nov 2011 04:00:00 +0100

Conclusions. Treatment with bosentan is both safe and effective in patients with both simple and complex forms of ES. (Source: Congenital Heart Disease)

Incidence of Severe Congenital Heart Disease at the Province of Al‐Qassim, Saudi Arabia

Congenital Heart Disease — Tue, 01 Nov 2011 04:00:00 +0100

Conclusion. Severe CHD is a major health problem at the Kingdom of Saudi Arabia and worldwide. The incidence of severe CHD likely to require intervention in infancy, in the province of Al‐Qassim, Saudi Arabia, is 5.4 per 1000 live births. (Source: Congenital Heart Disease)

Multimodality Evaluation and Transcatheter Closure of a Muscular Ventricular Septal Defect

Journal of the American College of Cardiology — Mon, 31 Oct 2011 00:58:29 +0100

A 34-year-old man with a history of aortic coarctation repair presented with shortness of breath on exertion and was found to have a muscular ventricular septal defect (VSD) on echocardiography and mild pulmonary hypertension (estimated pulmonary artery pressure 41 mm Hg). A cardiac MRI study revealed a Qp:Qs of 1.6:1 by phase contrast. Given his worsening symptoms, increased shunt, and pulmonary hypertension, closure of the VSD was indicated. (Source: Journal of the American College of Cardiology)

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A Unique Case of Tessier 30 and Bilateral Cleft Lip and Palate: A New Clinical Syndrome?

The Cleft Palate-Craniofacial Journal — Sun, 30 Oct 2011 04:00:00 +0100

Conclusion: We present a unique case of a child with a Tessier 30 cleft associated with bilateral cleft lip and palate, in the absence of intraoral masses or limb anomalies. Previous reports of median facial clefts have occurred either in the presence of intraoral hamartomas suggesting the palatal defects are a result of sequence abnormalities, or in association with extremity findings consistent with the spectrum of orofaciodigital syndrome. The case we present is distinct and may represent a new clinical syndrome. Keywords: Tessier 30; Bilateral cleft lip; Bilateral cleft palate; Bilateral cleft lip and palate; Mandible/mandibular cleft; Bifid tongue; Craniofacial cleft. PMID: 22034959 [PubMed - as supplied by publisher] (Source: The Cleft Palate-Craniofacial Journal)

The Modified Ablation Guided by Ibutilide Use in Chronic Atrial Fibrillation (MAGIC‐AF) Study:

Journal of Cardiovascular Electrophysiology — Fri, 28 Oct 2011 04:00:00 +0100

Conclusion:The MAGIC‐AF study will assess the utility of a combined pharmaco‐ablative strategy in patients with persistent AF undergoing a CFAE based ablation strategy.(J Cardiovasc Electrophysiol, Vol. pp. 1‐7) (Source: Journal of Cardiovascular Electrophysiology)

Measurement of nuchal translucency for prenatal screening of congenital heart defects: a population‐based evaluation

Prenatal Diagnosis — Wed, 26 Oct 2011 04:00:00 +0100

ConclusionPrevalence of CHD was about fourfold higher for fetuses with NT ≥ 3.5 mm (3.2%) than in the general population. This higher risk is comparable to that of other risk factors commonly used for early referral to specialized echocardiography. Nevertheless, our results, suggest that NT is not a very effective or efficient tool for the prenatal screening of CHD. Copyright © 2011 John Wiley & Sons, Ltd. (Source: Prenatal Diagnosis)

The Role of Cardiovascular Magnetic Resonance in Adults with Congenital Heart Disease

Progress in Cardiovascular Diseases — Sat, 22 Oct 2011 01:47:04 +0100

Abstract: The comprehensive coverage and versatility of cardiovascular magnetic resonance (CMR), providing functional as well as anatomical information, make it an important facility in a center specializing in the care of adults with congenital heart disease. Imaging specialists using CMR to investigate acquired heart disease should also be able to recognize and evaluate previously unsuspected congenital malformations. Conditions that may present or be picked up during imaging in adulthood include atrial septal defect, anomalously connected pulmonary veins, double-chambered right ventricle, congenitally corrected transposition of the great arteries, aortic coarctation, and patent arterial duct. To realize its full potential and to avoid pitfalls, CMR of adults with congenital heart diseas...

Left main coronary artery compression by a giant pulmonary artery aneurysm associated with large atrial septal defect and severe pulmonary hypertension.

Anadolu Kardiyol Der... — Thu, 20 Oct 2011 14:20:19 +0100

Authors: Oz F, Emet S, Baykız D, Oflaz H PMID: 21967796 [PubMed - in process] (Source: Anadolu Kardiyol Der...)

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Double-chambered right ventricle associated with ventricular septal defect and subaortic stenosis in an adult.

Anadolu Kardiyol Der... — Thu, 20 Oct 2011 14:20:19 +0100

Authors: Minardi G, Pulignano G, Pino PG, Pergolini A, Musumeci F PMID: 21967795 [PubMed - in process] (Source: Anadolu Kardiyol Der...)

Cor triatriatum sinister with secundum atrial septal defect in a patient with recurrent pulmonary infections.

Anadolu Kardiyol Der... — Thu, 20 Oct 2011 14:20:19 +0100

Authors: Işılak Z, Uzun M, Cay S PMID: 21967793 [PubMed - in process] (Source: Anadolu Kardiyol Der...)

Hybrid procedure with cardiopulmonary bypass for muscular ventricular septal defects in children [Original articles]

European Journal of Cardio-Thoracic Surgery — Thu, 20 Oct 2011 04:00:00 +0100

Conclusion: The hybrid procedure is safe and effective for the closure of congenital heart defects in children. (Source: European Journal of Cardio-Thoracic Surgery)

Atrial septal defect with drainage of the inferior vena cava into the left atrium [Case reports]

European Journal of Cardio-Thoracic Surgery — Thu, 20 Oct 2011 04:00:00 +0100

We present a 17-year-old female with an ASD and an anomalous drainage of the IVC into the LA leading to cyanosis since early childhood. Diagnosis was documented by computed tomography (CT) angiography and confirmed intra-operatively. The patient underwent successful surgical correction with an uneventful postoperative course. (Source: European Journal of Cardio-Thoracic Surgery)

Five‐year Follow‐up of Intracardiac Echocardiography‐assisted Transcatheter Closure of Complex Ostium Secundum Atrial Septal Defect

Congenital Heart Disease — Thu, 20 Oct 2011 04:00:00 +0100

Conclusions. Intracardiac echocardiography‐guided complex secundum ASD transcatheter closure is safe and effective and appears to have excellent long‐term results, thus minimizing potential complications resulting from the complex anatomy. (Source: Congenital Heart Disease)

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How Do We Define Congenital Heart Defects for Scientific Studies?

Congenital Heart Disease — Thu, 20 Oct 2011 04:00:00 +0100

ABSTRACTEstimates of the prevalence of congenital heart defects (CHD) have been published over many years and from many regions. As they are based on different definitions of which cases to include in the CHD prevalence, published prevalence estimates vary substantially. With the increasing use of echocardiography in neonatal intensive care, a patent ductus arteriosus (PDA) or flow over the atrial septum will often be visible. These findings may be coded as CHD at discharge and in this way falsely increase the CHD prevalence in the population. There are several purposes for which population‐based data on CHD may be used: etiology, planning of treatment, or obtain information on outcome, including mortality. For etiology studies, it is important to include terminations of pregnancy as wel...

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

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

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

Expression of Id2 in the second heart field and cardiac defects in Id2 knock‐out mice

Developmental Dynamics — Wed, 19 Oct 2011 20:41:02 +0100

AbstractThe inhibitor of differentiation Id2 is expressed in mesoderm of the second heart field, which contributes myocardial and mesenchymal cells to the primary heart tube. The role of Id2 in cardiac development is insufficiently known. Heart development was studied in sequential developmental stages in Id2 wildtype and knockout mouse embryos. Expression patterns of Id2, MLC‐2a, Nkx2.5, HCN4, and WT‐1 were analyzed. Id2 is expressed in myocardial progenitor cells at the inflow and outflow tract, in the endocardial and epicardial lineage, and in neural crest cells. Id2 knockout embryos show severe cardiac defects including abnormal orientation of systemic and pulmonary drainage, abnormal myocardialization of systemic and pulmonary veins, hypoplasia of the sinoatrial node, large intera...

Horseshoe lung associated with left lung hypoplasia and single left pulmonary vein; a rare combination

Clinical Radiology — Wed, 19 Oct 2011 04:00:00 +0100

Horseshoe lung (HL) is a rare bronchovascular anomaly in which the posterobasal segments of the right and left lung are fused with a narrow isthmus of pulmonary parenchyma in a common pleural space behind the heart and anterior to the aorta and oesophagus. HL is mostly associated with unilateral lung hypoplasia (most frequently the right lung) in conjunction with the Scimitar syndrome and is also associated with cardiovascular anomalies, such as atrial septal defects, ventricular septal defects, tetralogy of Fallot, and hypoplastic left ventricule. HL with left lung hypoplasia is extremely rare, with only a few cases reported in the literature. To the authors’ knowledge, this is the first case of HL associated with left lung hypoplasia and a single left pulmonary vein as depicted using m...

Two novel HAND1 mutations in Chinese patients with ventricular septal defect.

International Journal of Clinical Chemistry — Tue, 18 Oct 2011 04:00:00 +0100

CONCLUSIONS: This is the first report of mutations in the HAND1 gene in Chinese patients with VSD and provides new insight into the etiology of VSD. PMID: 22032825 [PubMed - as supplied by publisher] (Source: International Journal of Clinical Chemistry)

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Socioeconomic status and prevalence of congenital heart defects: Does universal access to health care system eliminate the gap?

Birth Defects Research Part A: Clinical and Molecular Teratology — Mon, 17 Oct 2011 04:00:00 +0100

DISCUSSION: It is assumed that increased ascertainment is responsible for observed increase in the prevalence of CHD, especially minor defects. While the trend and pattern over time changed for severe and nonsevere CHDs, the SES gap remained consistent during the study period. Our results indicate that even free and universal access to a health care system does not eliminate the SES gap observed in the prevalence of CHD. Birth Defects Research (Part A) 2011. © 2011 Wiley‐Liss, Inc. (Source: Birth Defects Research Part A: Clinical and Molecular Teratology)

Selective Cerebro‐Myocardial Perfusion in Complex Congenital Aortic Arch Pathology: A Novel Technique

Artificial Organs — Fri, 14 Oct 2011 04:00:00 +0100

AbstractSimultaneous cerebro‐myocardial perfusion has been described in neonatal and infant arch surgery, suggesting a reduction in cardiac morbidity. Here reported is a novel technique for selective cerebral perfusion combined with controlled and independent myocardial perfusion during surgery for complex or recurrent aortic arch lesions. From April 2008 to April 2011, 10 patients with arch pathology underwent surgery (two hypoplastic left heart syndrome [HLHS], four recurrent arch obstruction, two aortic arch hypoplasia + ventricular septal defect [VSD], one single ventricle + transposition of the great arteries + arch hypoplasia, one interrupted aortic arch type B + VSD). Median age was 63 days (6 days–36 years) and median weight 4.0 kg (1.6–52). Via midline ster...

New Syndrome of Congenital Circumferential Skin Folds Associated with Multiple Congenital Anomalies

Pediatric Dermatology — Thu, 13 Oct 2011 04:00:00 +0100

We describe a 7‐month‐old girl with a novel combination of symmetrical congenital circumferential skin folds, dysmorphic features, and multiple congenital abnormalities. Examination of the patient revealed symmetrical congenital circumferential skin folds and dysmorphic features, as well as multiple congenital anomalies including nasal pyriform aperture stenosis, ventricular septal defect, absent spleen, camptodactyly, and severe psychomotor retardation. Skin biopsy demonstrated subcutaneous fat extending into the superficial and deep reticular dermis. Sequencing of the CDON, SHH, ZIC2, SIX3, and TGIF genes (associated with holoprosencephaly) did not disclose pathogenic alterations. Extensive review of previously described cases of syndromic congenital circumferential skin folds did no...

The impact of cardiac surgery in patients with trisomy 18 and trisomy 13 in Japan

American Journal of Medical Genetics Part A — Tue, 11 Oct 2011 04:00:00 +0100

In this study, approximately a quarter of patients underwent surgery for CHD in both trisomies. Cardiac surgery may improve survival in selected patients with T18. © 2011 Wiley Periodicals, Inc. (Source: American Journal of Medical Genetics Part A)