Total anomalous pulmonary venous connection in adults

Abstract

Between 1961 and 1989, 19 patients with total anomalous pulmonary venous connection underwent surgical correction. Ages ranged from 18 to 38 years (mean 26.2 +/- 6.5 years). The anatomic variants included 10 patients with total anomalous pulmonary venous connection to the vertical vein, 6 patients with total anomalous pulmonary venous connection to the coronary sinus, and 2 with total anomalous pulmonary venous connection directly to the right atrium. The last patient had mixed connection to the coronary sinus and left vertical vein. Two patients died, one because of left atrial rupture and another of low cardiac output. Long-term follow-up after surgical repair ranged from 2 to 24 years (mean 7 +/- 6.2 standard deviation). Preoperative disability, assessed according to New York Heart Association criteria, showed 12 patients in functional class II and seven in class III. After treatment, 16 patients were in class I and one in class II (p less than 0.001). Echocardiographic evaluation of six patients revealed in all a normal left ventricular ejection fraction; the diastolic function was also normal except in one patient. The postoperative evaluation of the pulmonary arterial systolic pressure performed by both Doppler echocardiography and right cardiac catheterization in 14 patients showed a significant reduction of the mean pulmonary arterial systolic pressure from 51.1 +/- 3.4 to 37.4 +/- 14.4 mm Hg (p less than 0.01). The remaining three patients evaluated in the follow-up period only by clinical examination are in New York Heart Association functional class I. The anatomic characteristics of our patients were responsible for the long-term outcome without correction. Surgical treatment of older patients can be performed with satisfactory results and excellent long-term survival.