Prevalence and Complications of Aberrant Subclavian Artery in Patients With Heritable and Nonheritable Arteriopathies.

Background: An aberrant subclavian artery (ASA) (or lusoria) is the most common congenital anomaly of the aortic arch (0.5%-2.2%; female-to-male ratio 2:1 to 3:1).

Technical Modifications That Might Improve Long-term Outcomes of the Ross Procedure in Children.

Background: Failure of the pulmonary valve autograft (PVA) after the Ross procedure (RP) has discouraged its widespread use and has led to modifications or alternatives to the procedure. We sought to analyze whether certain technical modifications could improve results of the RP in children.

Methods: Sixty-nine patients (median age, 12 years; range, 0.

Genotype Predicts Outcomes in Fetuses and Neonates With Severe Congenital Long QT Syndrome.

Objectives: This study sought to determine the relationship between long QT syndrome (LQTS) subtype (LTQ1, LTQ2, LTQ3) and postnatal cardiac events (CEs).

Background: LQTS presenting with 2:1 atrioventricular block or torsades de pointes in the fetus and/or neonate has been associated with risk for major CEs, but overall outcomes and predictors remain unknown.

Methods: A retrospective study involving 25 international centers evaluated the course of fetuses/newborns diagnosed with congenital LQTS and either 2:1 atrioventricular block or torsades de pointes.

Surgical Correction of Aberrant Right Coronary Anomalies Stranding an Aortic Commissure with and Without Unroofing.

The technique for successful surgical correction of an anomalous origin of the right coronary artery from the opposite aortic cusp with an aberrant course between the aorta and pulmonary artery is illustrated in a symptomatic 62-year-old woman. The intramural course of the right coronary artery traversed the tip of the commissure between the anterior and posterior leaflets, and its repair entailed unroofing of the intramural segment from inside the aortic intima. This technique required resuspension of the overlying commissure to maintain optimal aortic valve leaflet coaptation and prevent aortic insufficiency.

Significance of low peak Doppler velocity in the proximal sano conduit in hypoplastic left heart syndrome.

Background: The Sano modification of the Norwood operation is a well-established first step palliation for hypoplastic left heart syndrome (HLHS). Theoretically, the first point of resistance to pulmonary flow should be in the proximal Sano, generating high Doppler flow velocity. Paradoxically, however, some patients have low gradients in the proximal Sano conduit.

Fate of ventricular and valve performance following early bidirectional Glenn procedure after Norwood operation controlled for hypoplastic left heart syndome anatomic subtype.

The Norwood operation (NO) with a right ventricle (RV)-to-pulmonary artery (PA) shunt (NRVPA) is reportedly associated with early hemodynamic advantage. Shunt strategy has been implicated in ventricular function. Outcomes after NRVPA compared with classic procedure as part of a strategy involving early bidirectional Glenn (BDG) procedure were analyzed with reference to RV, tricuspid, and neoaortic valve performance.

Fetal heart rate predictors of long QT syndrome.

Background: Fetal long QT syndrome (LQTS) is associated with complex arrhythmias including torsades de pointes and 2° atrioventricular block. Sinus bradycardia has also been associated with fetal LQTS, but little is known of this rhythm manifestation. Our purpose was to characterize the fetal heart rate (FHR)/gestational age (GA) profile of fetal LQTS.

Proximal sano anastomosis aneurysm due to degeneration of bovine pericardial patch.

We report a 5-year-old girl with hypoplastic left heart syndrome who originally underwent a Norwood procedure with a Sano shunt. Subsequent procedures included a bidirectional Glenn and extracardiac Fontan with a Gore-Tex graft (W.L Gore and Associates, Flagstaff, AZ).

Shunt reintervention and time-related events after Norwood operation: impact of shunt strategy.

Background: The use of right ventricle-to-pulmonary artery shunt (RV-PA) is thought to improve postoperative hemodynamics in hypoplastic left heart syndrome (HLHS). Proximal shunt obstruction often prompts early reintervention. Description of our technique and analysis of outcomes after Norwood operation using RV-PA shunt (NRVPA) was undertaken.

Pulmonary arterial capacitance in children with idiopathic pulmonary arterial hypertension and pulmonary arterial hypertension associated with congenital heart disease: relation to pulmonary vascular resistance, exercise capacity, and survival.

Background: Pediatric pulmonary arterial hypertension (PAH), whether idiopathic PAH (iPAH) or PAH associated with congenital heart disease (aPAH), carries high morbidity and mortality. Low pulmonary arterial capacitance (PAC), defined as right ventricular stroke volume/pulmonary artery pulse pressure, is a risk factor for mortality in adults with PAH. However, the relation of PAC to pulmonary vascular resistance (PVR), exercise endurance, and survival is poorly defined in children.

Coronary artery-to-pulmonary artery fistula in a case of pulmonary atresia with ventricular septal defect.

Pulmonary atresia with ventricular septal defect (PA-VSD) can be associated with varying pulmonary artery connections. The origin of pulmonary blood flow can vary greatly among patients, and some case reports have described the presence of left coronary artery-to-pulmonary artery fistula. Two cases of patients found to have coronary artery-to-pulmonary artery fistula in the settings of PA-VSD are reported.

Hypoplastic left heart syndrome and aortic atresia-mitral stenosis variant: role of myocardial protection strategy and impact of ventriculo-coronary connections after stage I palliation.

Aortic atresia-mitral stenosis (AA-MS) has been implicated as a determinant of outcome after Stage-1 palliation (S1P) in hypoplastic left heart syndrome (HLHS).Studies evaluating the association of AA-MS with ventriculo-coronary connections (VCC) and mortality report conflicting results. The significance of VCC, myocardial protection, and shunt strategy after S1P has yet to be determined.

Post-cardiotomy extracorporeal cardiopulmonary resuscitation in neonates with complex single ventricle: analysis of outcomes.

Objective: Extracorporeal cardiopulmonary resuscitation (ECPR) in children with cardiac arrest refractory to conventional cardiopulmonary resuscitation (CPR) has been reported with encouraging results. We sought to review outcomes of neonates with functional single ventricle (FSV) receiving post-cardiotomy ECPR.

Methods: Forty-eight patients who required post-cardiotomy extracorporeal membrane oxygenation (ECMO) since the introduction of our ECPR protocol (January 2007-December 2009) were identified.

Three-dimensional transesophageal echocardiography of atrial septal defect: a qualitative and quantitative anatomic study.

Background: Real-time three-dimensional (3D) transesophageal echocardiography (TEE) was used to analyze atrial septal defect (ASD) with 4 goals: (1) to determine feasibility, (2) to analyze the accuracy of qualitative and quantitative data, (3) to assess strengths and weaknesses of the available modes of 3D TEE, and (4) to provide 3D transesophageal echocardiographic reference images.

Methods: Sixty-five patients with ASDs (age, 5-64 years; weight, 20-114 kg; body surface area, 0.8-2.

Spontaneous rupture of atrioventricular valve tensor apparatus as late manifestation of anti-Ro/SSA antibody-mediated cardiac disease.

Atrioventricular (AV) block and endocardial fibroelastosis associated with dilated cardiomyopathy are the most common clinical manifestations of anti-Ro/SSA-mediated fetal cardiac disease. Valvar dysfunction has not been a prominent feature of this disease; however, recent anecdotal cases have suggested an association between rupture of the AV valve tensor apparatus and maternal anti-Ro/SSA antibodies. In the present study, we have described the clinical and laboratory findings and reviewed the published data for infants of anti-Ro/SSA-positive pregnancies with AV valve insufficiency due to chordal rupture from the papillary muscles.

Doppler echocardiography for managing fetal cardiac arrhythmia.

The successful management of fetal arrhythmias is contingent on an accurate diagnosis of arrhythmia and its effects on fetal well-being. Regardless of the rate (tachycardia or bradycardia) or rhythm (regular or irregular), the diagnosis of arrhythmia by Doppler echocardiography is based on the atrioventricular relationship and the atrial and ventricular rates. Fetal well-being at the time of diagnosis and during treatment can be assessed by Doppler flow patterns in the fetal systemic veins and atrioventricular valves.

Mitral stenosis and aortic atresia in hypoplastic left heart syndrome: survival analysis after stage I palliation.

Background: Mitral stenosis with aortic atresia (MS-AA) has been implicated as a risk factor for decreased survival after stage 1 palliation for hypoplastic left heart syndrome. Conflicting results were reported in the literature evaluating the association of anatomic subtypes and mortality. Our objective was to determine whether MS-AA is associated with increased mortality after stage 1 palliation.

Ventriculocoronary artery connections with the hypoplastic left heart: a 4-year prospective study: incidence, echocardiographic and clinical features.

Ventriculocoronary connections (VCCs), also called sinusoids, occur with hypoplastic left heart (HLH). Previous reports are limited to case reports, pathologic series, and surgical series with limited detail, which may underestimate the incidence and overestimate the severity of VCCs in HLH. A study was conducted to determine the incidence VCCs in HLH, their effect on survival, and their echocardiographic and clinical features.

Effects of remifentanil anesthesia on cardiac electrophysiologic properties in children undergoing catheter ablation of supraventricular tachycardia.

Remifentanil is commonly used during anesthesia in pediatric electrophysiologic studies (EPS). The purpose of this study is to determine the effects of remifentanil on the cardiac electrophysiologic properties of children undergoing ablation of supraventricular tachycardia (SVT). A prospective study was performed in patients undergoing EPS before ablation of SVT.

Selective tricuspidization and aortic cusp extension valvuloplasty: outcome analysis in infants and children.

Background: Encouraging early outcomes of emerging aortic cusp extension valvuloplasty techniques have redirected attention to nonreplacement strategies in the management of younger patients with aortic insufficiency or aortic stenosis. Outcome analysis after aortic cusp extension valvuloplasty in infants and children was undertaken.

Methods: From July 1987 to December 2008, 78 patients younger than 10 years of age underwent aortic cusp extension valvuloplasty in the form of pericardial cusp extension and selective use of tricuspidization.

Hypoplastic left heart syndrome: feasibility study for patients undergoing completion fontan at or prior to two years of age.

Background: There is limited data regarding the management of children with HLHS (hypoplastic left heart syndrome) and completion Fontan (CF) at or prior to 2 years of age. A study was undertaken to investigate intermediate outcomes.

Methods: From August 1999 to December 2008, 52 HLHS survivors underwent extracardiac-conduit CF (29 prior to [group A] versus 23 after 25 months of age [group B]).

A management strategy for fetal immune-mediated atrioventricular block.

Introduction: The purpose of this study is to describe an in utero management strategy for fetuses with immune-mediated 2° or 3° atrioventricular (AV) block.

Methods And Results: The management strategy as applied to 29 fetuses consisted of three parts. First, using fetal echocardiography and obstetrical ultrasound, we assessed fetal heart rate (FHR), heart failure, growth and a modified biophysical profile score (BPS) assessing fetal movement, breathing and tone.

Aortic cusp extension valvuloplasty with or without tricuspidization in children and adolescents: long-term results and freedom from aortic valve replacement.

Objective: Aortic cusp extension valvuloplasty is increasingly used in the management of children and adolescents with aortic stenosis or regurgitation. The durability of this approach and the freedom from valve replacement are not well defined. A study was undertaken to investigate outcomes.

Surgical repair of anomalous coronary arteries arising from the opposite sinus of Valsalva in infants and children.

Background: Unroofing of anomalous coronary artery originating from the opposite sinus of Valsalva has become the procedure of choice for this congenital lesion, with surgery performed in children as young as two years old. An increasing number of this anomaly is diagnosed in infancy with no clear indication whether surgical repair should be done in this age group. This paper reviews our experience with this anomaly, and focuses on its surgical management in infants.