Total Anomalous Pulmonary Venous Connection with Severe Pulmonary Venous Obstruction

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: 1. Very early onset of dyspnea and heart failure. 2. Considerably more cyanosis than the usual case without obstruction. 3. Typical x-ray showing diffuse hazy lung fields with reticulated appearance without cardiac enlargement. 4. Electrocardiographic evidence of severe right ventricular hypertrophy with a tall R or qR pattern in lead V 1 and reversal of the R/S ratio over the precordium. 5. 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. 6. Rapid deterioration and death in the first weeks or months of life.