Derek A. Williams

Pulmonary hypertension secondary to partial pulmonary venous obstruction in a child with Cantu syndrome.

We report on an African‐American male with Cantu syndrome who required a pericardial window for a significant pericardial effusion in infancy and was subsequently found to have partial pulmonary venous obstruction (PVO) leading to pulmonary hypertension. Measurement of bilateral pulmonary capillary wedge pressures is important to uncover partial PVO. Pediatr Pulmonol. 2010; 45:727–729.

Progressively worsening hypertrophic cardiomyopathy in a child with newly diagnosed Costello syndrome while receiving growth hormone therapy

We describe a 6-year-old boy with newly diagnosed Costello syndrome after the diagnosis of severe hypertrophic cardiomyopathy. His neonatal asymmetric septal cardiomyopathy resolved by 9 months of age but reappeared at 6 years of age. This report highlights two important concepts: the association of genetic syndromes with hypertrophic cardiomyopathy and the possibility of worsening severity of hypertrophic cardiomyopathy linked to growth hormone therapy.

Giant Aneurysm of the Left Anterior Descending Coronary Artery in a Pediatric Patient with Behcet’s Disease

Behcet’s disease is a rare autoimmune vasculitis characterized by oral aphthosis, genital ulcers, and ocular and cutaneous lesions. Vascular involvement usually affects the veins more commonly than the arteries, and coronary arterial involvement is extremely rare. We report an adolescent with Behcet’s disease who developed a large pseudoaneurysm of the left anterior descending coronary artery requiring a coronary arterial bypass graft.

Coronary Anomaly Preventing Transcatheter Closure of an Atrial Septal Defect

A 4-year-old asymptomatic girl presented for elective transcatheter closure of her ostium secundum atrial septal defect (ASD). Her examination revealed a fixed split second heart sound with a 2/6 systolic ejection murmur at the left upper sternal border and a right ventricular lift. Transthoracic echocardiogram demonstrated a 15-mm secundum ASD with a mildly dilated right ventricle. The left coronary artery (LCA) could not be readily identified on the study. The patient was referred for percutaneous device closure when a preprocedural transesophageal echocardiogram revealed a moderate-sized secundum ASD with left to right shunting, moderate right ventricle dilation, and an anomalous origin of the LCA from the right sinus of Valsalva was identified (Figure 1A). The origin and distribution of the right coronary artery was normal. Cardiac catheterization revealed a Qp:Qs …

Atypically located cardiac haemangioma of the mitral valve

We report an adolescent with a benign cardiac haemangioma with attachments exclusively to the anterior leaflet of the mitral valve. On the basis of our review of the literature, this study has not been reported previously.

An infant with Williams–Beuren syndrome and Ebstein anomaly

We report an infant with Ebstein anomaly of the tricuspid valve and severe branch pulmonary artery stenosis who was diagnosed with Williams-Beuren syndrome.

Cardiac Myxoma After Treatment for Childhood Neuroblastoma

An asymptomatic 13 year old with a history of neuroblastoma treated with chemotherapy, radiation therapy, and autologous bone marrow transplant was found to have a cardiac mass on screening echocardiogram. Surgical resection and histologic examination revealed the mass was a myxoma. After resection the patient made a full recovery. This report reviews the features of myxoma and explores the potential relationship between chemotherapy, radiation, bone marrow transplant and myxoma.

Medium-Term Outcomes After Implantation of Expanded Polytetrafluoroethylene Valved Conduit.

BACKGROUND Pulmonary valve replacement is becoming the most frequent congenital heart surgery performed on adolescents and young adults. Numerous surgical options are available, including autologous pericardium, mechanical valves, allografts, and bioprosthetic valves. Each option has limitations with durability and freedom from reintervention for stenosis or insufficiency, particularly in the pediatric population. The purpose of this study was to analyze our uniquely designed, expanded polytetrafluoroethylene (ePTFE) valved conduit (VC) for right ventricular outflow tract reconstruction. METHODS Beginning in 2012, ePTFE VC were implanted in 26 patients with a median age of 9.8 years (range, 1.0 to 15.9). Bicuspid VC were used in the first 3 patients, and tricuspid VC were used in 23 patients. The ePTFE VC is fashioned from commercially available ePTFE tube graft (16 mm in 1 patient, 20 mm in 7 patients, 24 mm in 18 patients) and 0.1 mm thick ePTFE membrane for the leaflet material. Valve function was assessed by echocardiogram after the implantation. RESULTS There were no postoperative hospital deaths. Mean follow-up was 2.6 years (range, 4 months to 4.7 years), and there have been no reoperations for the ePTFE VC. There was one reintervention for stenosis at the distal anastomosis. Pulmonary insufficiency was mild or less in 24 patients (92%). The average peak instantaneous pressure gradient between the right ventricle and the pulmonary artery was 22.4 ± 15.1 mm Hg at latest follow-up. CONCLUSIONS Compared with historical data for other pulmonary valve replacement options, our ePTFE VC shows superior medium-term performance, with less reintervention or significant valve dysfunction.

Acute and mid-term outcomes of stent implantation for recurrent coarctation of the aorta between the Norwood operation and fontan completion: A multi-center Pediatric Interventional Cardiology Early Career Society Investigation

We sought to evaluate outcomes of stent implantation (SI) for recurrent coarctation of the aorta (RC) following the Norwood operation.

Mixed-type total anomalous pulmonary venous connection.

The mixed-type total anomalous pulmonary venous connection (TAPVC) occurs rarely (9%) and is associated with an increased risk of reoperation [1]. Although echocardiography is regarded as an initial screening method in the diagnosis of TAPVC, evaluation of pulmonary veins in the presence of anomalous drainage may be difficult. Computed tomographic (CT) angiography has certain advantages in delineating the drainage site of the common pulmonary vein, stenosis of the vertical vein, and the course of the atypical vessel into the systemic vein [4]. We report a case of mixed-type TAPVC in which CT angiography further delineated the complex features of pulmonary venous drainage. A full-term male infant was born with a birth weight of 2.65 kg. The pregnancy was complicated by pregnancyinduced hypertension and hemolysis as well as elevated liver enzymes and low platelet count (HELLP) syndrome. At birth, the boy was apenic and bradycardic with an Apgar score of 1 at 1 min and 5 at 5 min. His respiratory depression was likely due to maternal treatment of magnesium sulfate. His saturation oxygen was 88% on room air. Cardiopulmonary examination was unremarkable with no heart murmur. The chest radiograph was unremarkable. Echocardiography showed that the left pulmonary veins returned to a posterior confluence behind the left atrium and drained through a vertical vein to the innominate vein. The right pulmonary veins appeared to return to a confluence but drained to the coronary sinus into the right atrium, suggesting mixed-type TAPVC with components of both ‘‘supracardiac’’ and ‘‘intracardiac.’’ There was a peak gradient of 5 mmHg (mean, 3 mmHg) into the vertical vein at the level of the right pulmonary artery. The innominate vein, superior vena cava, and coronary sinus all were dilated. There was mild right atrial enlargement, moderate right ventricular dilation, and moderate secundum type atrial septum defect with right-to-left shunting. To clarify the anatomy, CT angiography was performed on a 64-channel system using electrocardiographic (ECG) gating. There was a common confluence into which all four pulmonary veins drained, emptying into two separate drainage systems, one to a vertical vein, which then coursed superior to the innominate vein, and one to the coronary sinus (Fig. 1). There was moderate stenosis in the midportion of the common confluence, which likely resulted in the two separate draining pathways (Fig. 2). Catheterization was performed for trial balloon occlusion of the vertical vein, which re-demonstrated the mixedtype TAPVC with central confluence stenosis. The mean pressures of the confluence and vertical vein were 10 and 8 mmHg, respectively. Surgery was performed with unroofing of the coronary sinus, followed by baffle connection of the coronary sinus to the atrial septal defect and direct side-to-side anastomosis of the left pulmonary venous confluence to the left atrium. In the preoperative evaluation of infants with TAPVC, CT angiography with three-dimensional reconstruction is a useful diagnostic tool [2]. A recent report illustrated the difficulty with echocardiographic diagnosis and the usefulness of CT angiography in mapping the pulmonary veins [3]. In our case, CT angiography was useful for clarifying the complex features of pulmonary venous drainage in this mixed-type TAPVC. Even for a patient who has undergone a cardiac catheterization, CT angiography may delineate D. Kobayashi (&) D. A. Williams A. L. Cook Department of Pediatrics, Wake Forest University School of Medicine, Medical Center Boulevard, Winston-Salem, NC 27157, USA e-mail: dkobayas@wfubmc.edu