The initial surgical approach to the infant with pulmonary atresia and intact ventricular (PA-IVS) is to establish an adequate source of pulmonary blood flow and, when possible, relieve right ventricular (RV) outflow obstruction. The selection of patients for pulmonary valvotomy, alone or in combination with a systemic-pulmonary arterial shunt, depends on the presence of an RV outflow tract and...

To evaluate the effects of acute afterload reduction, hydralazine, 0.2 mg/kg, was administered at cardiac catheterization to seven infants who had a large ventricular septal defect (VSD). The infants were 2.5 - 11 months old (mean 5.1 months). Before and 5, 15, 25 and 35 minutes after hydralazine, aortic, pulmonary capillary wedge, pulmonary artery, right atrial and superior vena caval pressure...

AIMS To assess the occurrence and prognostic value of acute vasodilator response (AVR) in paediatric vs. adult pulmonary arterial hypertension, and idiopathic/hereditary pulmonary arterial hypertension (iPAH/HPAH) vs. pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) using three different response criteria. METHODS AND RESULTS Ninety-nine PAH patients underwen...

OBJECTIVES To identify the pulmonary hypertension (PH) patients who develop an exercise induced venous-to-systemic shunt (EIS) by performing the cardiopulmonary exercise test (CPET), analyse the changes of CPET measurements during exercise and compare the exercise physiology and resting pulmonary hemodynamics between shunt-PH and no-shunt-PH patients. METHODS Retrospectively, resting pulmonar...

A previously unreported association of tetralogy of Fallot and total anomalous pulmonary venous drainage is described in two infants. The initial clinical manifestation was tetralogy, and both patients had systemic-pulmonary shunt surgery to increase the pulmonary blood flow. Postoperatively, one infant died of massive pulmonary edema, and at postmortem examination total anomalous pulmonary ven...

This report presents a new, simple and reliable method of quantitating left-to-right shunts from arterial dilution curves. When indicator (e.g., indocyanine green) is injected centrally (right ventricle or pulmonary artery) and blood is withdrawn rapidly from either the aorta or other central systemic artery, in the presence of a left-to-right shunt the recorded indicator-dilution curve usually...

Partial anomalous pulmonary venous connection (PAPVC) causes a left-to-right shunt from the anomalous pulmonary vein (PV) to a systemic vein. We report an uncommon adult case of PAPVC, in which the left upper PV drained into both the innominate vein and the left atrium (LA), demonstrating retrograde shunting from the LA to the innominate vein. The anomaly was surgically repaired.

Eleven patients with a total of 17 palliative systemic-pulmonary artery shunts underwent evaluation by electrocardiogram-gated magnetic resonance imaging (GMRI). GMRI successfully imaged 1 1 of 17 shunts (65%), including five of nine Blalock-Taussig shunts, four of six Glenn shunts, and both aortopulmonary shunts. All shunts except for the Waterston were imaged on coronal sections during end-sy...

Partial anomalous pulmonary venous return (PAPVR) is a left-to-right shunt where one or more, but not all, pulmonary veins drain into a systemic vein or the right atrium. We report a case of a 45-year-old male with PAPVR to superior vena cava which was incidentally discovered during a right lower bilobectomy for lung cancer.