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Total Anomalous Pulmonary Venous Connection: Clinical and Physiologic Observations of 75 Pediatric Patients

Experience with 75 cases of proven total anomalous pulmonary venous connection without other significant cardiac malformations treated at the Children's Hospital Medical Center in Boston from January 1950 to June 1968 forms the basis of this report. Clinical observations of patients with this defect presenting in infancy are reviewed in depth, and the few patients presenting after 1 year of age are included to give a spectrum of this disease in the pediatric age group.Chest x-rays and complete electrocardiograms were available in each case. Sixty-one patients had partial or complete cardiac catheterizations. The anatomic and physiologic data from these studies are presented, and the results of medical management are evaluated.On the basis of the physiologic data, an approach to management is proposed which includes early operative intervention in those patients with pulmonary vascular obstruction. If pulmonary hypertension is present without vascular obstruction, medical management may be tried. If pulmonary hypertension is not present and particularly if there is a significant gradient across the right ventricular outflow tract, medical management appears to be the treatment of choice until operative intervention can be undertaken with less operative mortality.