Pulmonary vascular resistance in repaired congenital diaphragmatic hernia vs. age-matched controls.

INTRODUCTION: Infants and children with repaired congenital diaphragmatic hernia (CDH) often continue to show delayed growth and development that may be, in part, secondary to unrecognized persistence of increased pulmonary vascular resistance (PVR). METHODS: Data were reviewed from all patients ages 6-36 mo with repaired CDH who underwent cardiac catheterization from 2007 to 2010 and were compared to data from a control population of patients undergoing percutaneous closure of a patent ductus arteriosus (PDA). Indexed pulmonary blood flow (Qp), mean pulmonary artery pressure (mPAP), pulmonary capillary wedge pressure (PCWP), and PVR were examined. RESULTS: Data from 8 CDH patients and 10 control patients were examined. The mPAP (22.5 ± 3.33 vs. 18.2 ± 4.13 mm Hg) and PVR (3.66 ± 0.79 vs. 1.22 ± 0.4 iwU (indexed Wood's units)) were both significantly elevated in the CDH population, whereas the Qp (4.08 ± 1.43 vs. 6.82 ± 1.46 l/min/m(2)) was significantly lower in this population. There was no significant difference in pulmonary capillary wedge pressure (PCWP). Less than half of the CDH patients had signs of pulmonary hypertension (PH) on echocardiogram. DISCUSSION: Our data suggest that children who are ages 6-36 mo with repaired CDH have significantly increased PVR compared with controls and early consideration of cardiac catheterization may be warranted.

Fenestrated ASD closure in a child with idiopathic pulmonary hypertension and exercise desaturation.

Pulmonary arterial hypertension is a progressive disorder that may result in right heart failure and death. Atrial level shunts in the presence of pulmonary hypertension may allow right-to-left mixing with maintenance of cardiac output and improved survival. However, excessive mixing at the atrial level can cause undue systemic desaturation, increased fatigue and decreased exercise tolerance even in the presence of adequate cardiac output. A 5½-year-old was diagnosed with pulmonary hypertension, a large atrial septal defect and right-to-left shunting. Medical therapy over an 18-month period was successful in decreasing pulmonary artery pressure and pulmonary vascular resistance. However, because of the size and position of the intracardiac defect, symptoms of fatigue, and severe systemic desaturation with only minor activities persisted. Fenestrated surgical closure of the defect was thus undertaken to decrease the degree of atrial mixing, but still allow atrial decompression if necessary. Subsequent hemodynamic evaluation has demonstrated continued improvement, and all previous symptoms have resolved. Repeated echocardiography has confirmed patency of the atrial fenestration with left-to-right atrial flow.