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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 V1 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.

Total Anomalous Pulmonary Venous Connection with Severe Pulmonary Venous Obstruction