Total anomalous pulmonary venous drainage beyond childhood

We studied the anatomic characteristics and results of surgery in 27 patients with total anomalous pulmonary venous drainage who were 15 years or older between January 1997 and July 2007. Mean age was 19.7+/-11.6 years (15-48 years). The anatomic subtypes were supracardiac (n=15), cardiac (n=7), and mixed (n=5). Fourteen patients were in NYHA class II and 13 were in NYHA class III. Eleven patients had severe and the rest had moderate pulmonary arterial hypertension; six patients had significant right ventricular dysfunction. All patients underwent complete repair. A small inter-atrial communication was left open in four patients and in two patients, a fenestrated unidirectional valved patch was used to close the atrial septal defect. There were no early or late deaths. Follow-up was 3-127 months (mean 61.2+/-36.1 months). Twenty patients were in NYHA class I and seven were in class II. Echocardiography showed normal right and left ventricular function in all patients with reduction of pulmonary arterial pressures in 26 patients. One patient underwent radiofrequency ablation for new onset atrial flutter. Surgery can be safely undertaken in a few naturally selected group of patients with total anomalous pulmonary venous drainage who survive beyond childhood.