Total Anomalous Pulmonary Venous Connection with Severe Pulmonary Venous Obstruction

Abstract

The clinical, physiologic, and anatomic features of a special group of cases of total anomalous pulmonary venous connection are reviewed. The occurrence of severe pulmonary venous obstruction in total anomalous pulmonary venous connection produces a characteristic syndrome: Very early onset of dyspnea and heart failure. Considerably more cyanosis than the usual case without obstruction. Typical X-ray showing diffuse hazy lung fields with reticulated appearance without cardiac enlargement. Electrocardio-graphic evidence of severe right ventricular hypertrophy with a tall R or qR pattern in lead V1 and reversal of the R/S ratio over the precordium. Physiologic findings of practically normal pulmonary flow, right ventricular and pulmonary arterial pressures greater than systemic, and a marked gradient between the pulmonary arterial "wedge" and right atrial pressures. Rapid deterioration and death in the first weeks or months of life.