Transthoracic Echocardiographic Assessment of Coronary Flow in the Diagnosis of Right Ventricular-Dependent Coronary Circulation in Pulmonary Atresia with Intact Ventricular Septum.

Right ventricular-dependent coronary circulation (RVDCC) is associated with pulmonary atresia with intact ventricular septum and is defined by two or more epicardial coronary arteries with atresia or severe stenosis resulting in the dependency of coronary supply by retrograde flow. The hypothesis of this study is that coronary Doppler flow patterns on echocardiography can be used to distinguish patients with RVDCC. Between 2007 and 2016, we reviewed 16 patients with pulmonary atresia or critical pulmonary stenosis.

Access to the native atria following conduit total cavopulmonary anastomosis.

Objectives: We describe the use of trans-thoracic and trans-conduit puncture to access the atria and perform interventional procedures in patients who have undergone conduit total cavopulmonary anastomosis.

Background: Catheter access to the atria following intra or extra-cardiac Fontan is desirable when there is a need for trans-atrial interventions.

Methods: Between 2009 and 2014, 5 patients ages 7 to 28 years underwent this approach; three trans-thoracic and 2 trans-conduit punctures.

Arrhythmias in a long-term adult survivor with uncorrected tetralogy of Fallot: Case report and review of the literature.

We present a case of a middle-aged adult with uncorrected Tetralogy of Fallot (TOF) with pulmonary atresia who developed symptomatic supraventricular and ventricular arrhythmias. The lack of data regarding management of electrical and other complications in adults with uncorrected TOF is highlighted and emphasizes the need for a registry to better understand the medical management of long-standing adult survivors.

Bacterial pancarditis with myocardial abscess: successful surgical intervention in a 14-month-old boy.

A 14-month-old boy with a structurally normal heart presented with signs of cardiac tamponade caused by purulent pericarditis. During his hospital stay, mitral and tricuspid valve endocarditis developed, and a ventricular septal abscess expanded despite appropriate, prolonged antibiotic therapy for methicillin-resistant Staphylococcus aureus. The day before scheduled surgical correction, the abscess ruptured, creating a septal aneurysm.

Hybrid versus Norwood procedure for hypoplastic left heart syndrome: contemporary series from a single center.

Objective: Two different strategies have emerged in the initial palliation for hypoplastic left heart syndrome, the conventional Norwood operation and the so-called hybrid procedure. We have used each of these at our center. The purpose of the present study was to compare the outcomes of both procedures.

Partial anomalous pulmonary venous return: transcatheter repair.

This report describes a case of partial anomalous pulmonary venous return in a young white girl for whom transcatheter intervention successfully replaced surgical correction. This intervention was feasible because the left lung had dual pulmonary venous drainage.

A comparison of the modified Blalock-Taussig shunt with the right ventricle-to-pulmonary artery conduit.

Background: This study compared the modified Blalock-Taussig (MBT) shunt with the right ventricle-to-pulmonary artery (RVPA) conduit with respect to outcome and PA growth.

Methods: PA growth was assessed in 19 MBT patients and in 15 RVPA patients before stage 2 palliation for hypoplastic left heart syndrome. The RVPA was done with a ringed Gore-Tex tube (W.

Hybrid procedure for obstructive neonatal left ventricular tumors.

We report the use of the hybrid procedure (bilateral pulmonary artery banding, ductal stenting followed by balloon atrial septostomy) in the first week of life for the treatment of two neonates with large left ventricular fibromas resulting in univentricular physiology. At 6 months of age, second-stage palliation was accomplished and the 2 patients are growing well with normal developmental milestones and no atrial or ventricular ectopy at 30 months. Hybrid strategy offers a less invasive, initial intervention and the options of typical second-stage univentricular palliation, conversion to biventricular physiology in cases of tumor regression, planned surgical resection, or transplantation.

The fantastic journey of a bullet: out with a snare.

Bullet embolus is a rare complication of penetrating missile trauma. Removal of the bullet previously required surgery. We report the case of a 14-year-old with an hepatic vein bullet embolus following a gunshot wound to the left buttock.

Stents in the successful management of protein-losing enteropathy after fontan.

A 5-year-old female presented with anasarca secondary to protein-losing enteropathy after fenestrated extracardiac Fontan. There was no response to digoxin, furosemide, spironolactone and captopril. She had coarctation of the aorta and left pulmonary artery stenosis resistant to multiple surgical and balloon interventions.

An alternative method to fenestrate the extracardiac fontan conduit in a patient with atrial situs inversus and levocardia.

This report describes the technique to fenestrate the extracardiac Fontan conduit without cardiopulmonary bypass in a patient with levocardia and atrial situs inversus.

Comparison of angioplasty and surgery for neonatal aortic coarctation.

Background: The efficacy of balloon dilatation as primary treatment for neonatal aortic coarctation remains controversial.

Methods: A retrospective comparison between balloon angioplasty and surgery for the treatment of neonatal aortic coarctation was undertaken on 57 neonates younger than 40 days of age (angioplasty, 23 patients; surgery, 34 patients) treated between 1994 and 2004.

Results: Cohorts were similar with respect to the preinterventional variables of age, weight, upper extremity systolic blood pressure, coarctation gradient, degree of aortic arch hypoplasia, associated conditions, and mean follow-up (angioplasty, 36 months; surgery, 38 months).

Cardiac surgery in the newborn: improved results in the current era.

Pediatric cardiovascular surgeons can now safely operate on newborns to palliate or completely correct congenital heart defects in babies as small as 1.5-2.0 kilograms.

Aberrant coronary arteries: a reliable echocardiographic screening method.

Background: Aberrant origin (ABO) of a coronary artery (CA) from the contralateral aortic sinus with a subsequent interarterial course is a life-threatening condition. It carries a 28% to 55% risk for a sudden coronary event or death, but there are no reliable screening methods.

Objective: We sought to determine whether imaging a cross section of a coronary segment in the anterior aortic wall on the long-axis view may be used as an echocardiographic screening sign for ABO CA.

Severe aortic coarctation in infants less than 3 months: successful palliation by balloon angioplasty.

The optimal management strategy of the neonate and young infant with native aortic coarctation (AC) is controversial. We reviewed our experience with balloon angioplasty (BA) in neonates and infants 3 months to test our thesis that BA provides successful palliation, defined as avoidance of surgery for 4 weeks along with control of presenting symptoms. We also compared the results of the transumbilical arterial (UA), transfemoral arterial (FA) and transfemoral venous anterograde (FVA) approaches we have used to accomplish BA.

Transcatheter occlusion of ruptured sinus of valsalva aneurysm: innovative use of available technology.

A 9-year-old boy was found to have ruptured sinus of Valsalva aneurysm (RSVA) and aortic coarctation. Following relief of aortic coarctation by balloon angioplasty, transcatheter coil occlusion of the RSVA was performed successfully under transesophageal echocardiographic and fluoroscopic monitoring; bioptome-assisted delivery of 0.052" Gianturco coil was undertaken via a 7 Fr sheath stabilized by an 0.

Congenital Coronary Artery Abnormalities in Children.

After a significant coronary artery abnormality is recognized in a pediatric patient, surgery or appropriate transcatheter intervention should be performed. The risk of fatality from a congenital coronary abnormality far outweighs the small risks of surgical or transcatheter intervention. Angiography, although considered the state-of-the-art method of diagnosis, has significant spatial limitations and is not always diagnostic of aberrant coronary origins from the contralateral aortic sinus.