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(Circulation. 1962;25:916.)
© 1962 American Heart Association, Inc.

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Total Anomalous Pulmonary Venous Connection with Severe Pulmonary Venous Obstruction

A Clinical Entity

ALOIS R. HASTREITER M.D.¹; MILTON H. PAUL M.D.¹; MARIAN E. MOLTHAN M.D.¹; ROBERT A. MILLER M.D.¹

¹ From the Division of Cardiology, The Children's Memorial Hospital, and the Department of Pediatrics, Northwestern University Medical School, Chicago, Illinois.

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.

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