Total Anomalous Pulmonary Venous Connection Beyond the First Decade of Life

We report our experience with surgery for total anomalous pulmonary venous connection (TAPVC) beyond first decade of life.

Between January 1987 and July 2017, 98 patients ≥ten years underwent TAPVC repair. Their detailed case-records were analyzed.

Mean age was 19.05 (± 12.8; median 18) years. Anatomic subtypes were supracardiac (n = 62), cardiac (n = 20), and mixed (n = 16). An atrial septal defect (ASD) was present in all. Severe tricuspid valve regurgitation was present in four patients and severe rheumatic mitral regurgitation was present in one. On preoperative cardiac catheterization, mean pulmonary artery pressure was 67 ± 15.6 mm Hg (median 58; range 37-96). Mean pulmonary vascular resistance was 5.6 ± 3.9 Woods units·m² (median 4.7, range 2.9-11.8). Twenty-five patients had moderate and eight had severe pulmonary arterial hypertension. Right ventricular dysfunction was present in eight patients. At repair, a small ASD was left open in 87 patients while in 11 patients, the ASD was closed using a unidirectional valved patch. Follow-up was available for 90 (92%) patients. There were no late deaths over a mean follow-up of 163 ± 103.98 months (median 163, range 1-362). Eighty-two patients were in NYHA class I and eight were in class II. Right ventricular function normalized in 82 patients while 80 patients had reduction in pulmonary artery pressure. Event-free survival was 94.2% at 5 years, 92.3% at 10 years, and 90% at 20 and 30 years.

Outcomes of surgical repair for TAPVC beyond first decade of life are satisfactory. Close follow-up, however, is necessary for possible persistence of pulmonary arterial hypertension.