Jason F. Goldberg

Pulmonary Artery Band Reduces Left Atrial Pressure in Dilated Cardiomyopathy.

We describe a case of pulmonary artery band placement in a 2-month-old infant with dilated cardiomyopathy and moderate mitral regurgitation in impending need of ventricular assist device. Despite minimal change in echocardiographic appearance, pulmonary artery band placement resulted in significantly reduced left atrial pressure and unchanged right atrial pressure. Improved cardiac output has enabled weaning from ventilator support and favorable somatic growth with enteral feeding. The patient has been stable on milrinone for 3 months, awaiting cardiac transplantation on a regular ward. This therapy represents a potential alternative in small children, who are deemed high-risk candidates for mechanical circulatory support.

Extracorporeal Membrane Oxygenation Support in Severe Hypertrophic Obstructive Cardiomyopathy Associated With Persistent Pulmonary Hypertension in an Infant of a Diabetic Mother

A male infant was delivered at 37 weeks of gestation via cesarean delivery because of minimal fetal heart tone variability and late fetal decelerations to a 31-year-old mother whose pregnancy was complicated by maternal type 2 diabetes mellitus and chronic hypertension. Fetal echocardiography had been performed at 20 weeks of gestation, which showed a structurally normal heart without ventricular hypertrophy (Figure 1 and Movie IA and IB in the online-only Data Supplement). The mother had been treated previously with insulin, and during pregnancy, had hemoglobin A1c of 7.9% (diabetic patients with adequate glycemic control have values <7% to 8%).1 The birth weight was 4.8 kilograms. The infant developed respiratory failure within the first 15 minutes of life, requiring intubation and mechanical ventilation. The umbilical cord venous blood gas demonstrated a pH of 7.16 and partial pressure of oxygen of 31 mm Hg. A transthoracic echocardiogram (TTE) was performed following the patient’s admission to the neonatal intensive-care unit because of auscultation of a grade-IV/VI harsh systolic ejection murmur heard throughout the precordium. TTE showed severe biventricular hypertrophy, mild right ventricular outflow tract obstruction, severe left ventricular outflow tract obstruction with a …

Fibrolamellar Hepatocellular Carcinoma With Cardiac Spread Causing Severe Inferior Vena Cava Obstruction in a 9-Year-Old Child

Fibrolamellar carcinoma (FLC), a subset of hepatocellular carcinoma (HCC), is a very uncommon form of pediatric liver cancer, with fewer than 200 cases reported in the literature [3]. It is found primarily in older children and young adults, clustering between the ages of 10 and 35 years [4]. Fibrolamellar carcinoma is not associated with a history of cirrhosis or underlying liver disease, distinguishing it from HCC and hepatoblastoma (a much more common tumor in children) [4]. The clinical syndrome of FLC is characterized by malaise, abdominal pain, hepatomegaly/ abdominal mass, and elevation of transaminases and alphafetoprotein [4]. The prognosis is variable because the tumor is quite chemotherapy resistant [3]. Lymph node and peritoneal metastases are common, and nearly half of FLC patients experience development of distant metastases to sites such as the mediastinum, abdominal organs, skeletal muscle, and pericardium [3]. However, to our knowledge, no cases of inferior vena cava (IVC) or right atrial spread in children have been reported to date. We report a case of FLC involving a 9-year-old child, with direct extension into the IVC and right atrium, resulting in severe IVC obstruction. Case Report

Postoperative complications associated with perioperative sirolimus prior to pediatric cardiac retransplantation.

OBJECTIVES Sirolimus has been used in pediatric cardiac transplantation for the past decade for chronic renal dysfunction, recurrent rejection, and/or coronary allograft vasculopathy. There has been concern regarding the effect of sirolimus on wound healing and other postoperative complications. To date, the pediatric literature on its use is limited and has not specifically addressed its use in the perioperative period following repeat cardiac transplantation. METHODS We compared the patients in our institution who received sirolimus before repeat cardiac transplantation to those in the same era who did not receive sirolimus. RESULTS Of the 5 patients in the study group, 5 (100%) developed pleural effusions vs 1 (17%) in the control group (p=0.013). There was no increase in mortality in the sirolimus group, and there were no significant differences in renal dysfunction, serious bacterial infection, rejection, or postoperative length of stay. CONCLUSIONS In this small data set, there was a statistically significant increase in pleural effusions in patients on sirolimus. Further study is needed to develop an appropriate strategy to avoid postoperative complications in this patient population.

Pharmacologic Approach to Heart Failure in Children

Heart failure may result from a wide variety of causes and present with varying degrees of severity. In large part, patients present with varying degrees of congestion and compromised cardiac output. In this review a pathophysiologic construct is provided to guide the pharmacologic management of acute heart failure that leads to decreased congestion and improved cardiac output. Pharmacologic therapies that are used to treat chronic heart failure are also highlighted and provide the framework for transitioning from acute to preventative pharmacologic strategies.

Pulmonary vein stenosis with collateralization via esophageal varices: Long-term follow-up after successful treatment with drug-eluting stent

OBJECTIVE We describe the long-term follow-up of a child with recurrent hemoptysis due to severe pulmonary vein stenosis decompressing via collaterals to esophageal varices. DESIGN Case report SETTING: Tertiary childrens hospital PATIENT: Single child through ages 2- to 11-year old INTERVENTIONS: The child underwent cutting balloon angioplasty, bare metal stenting, and implantation of a PTFE-covered stent, all of which failed rapidly. Only after placement of a paclitaxel drug eluting stent did he have prolonged relief from hemoptysis and long-term patency of the treated vein. The stents were serially dilated to keep up with somatic growth of the child, eventually culminating in the need to induce intentional stent fracture. CONCLUSIONS We highlight novel transcatheter techniques to treat this vexing condition, discuss mechanisms of disease treatment and progression, and present the only patient with this rare combination of lesions to have achieved both longstanding pulmonary vein patency and resolution of esophageal varices.