[Obstruction of total anomalous pulmonary venous drainage. Factors influencing improvement in surgical results].

Of 107 patients operated for total anomalous pulmonary venous drainage (TAPVD) at Marie-Lannelongue Hospital between January 1980 and November 1989, 57 had severe pulmonary venous obstruction. The average age of the patients at operation was 13.6 days and their average body weight was 3.2 kg. Twenty eight patients had an infracardiac, 23 patients a supracardiac, 2 patients an intracardiac and 4 patients a mixed type of TAPVD. The diagnosis of pulmonary venous obstruction was made on the findings of low cardiac output and pulmonary hypertension exceeding the systemic blood pressure, and on the results of angiography and Doppler echocardiography. Seventy five per cent of patients were admitted in Class IV of the NYHA Classification. Since 1985, treatment has been directed towards prevention of pulmonary hypertension. Echocardiographic diagnosis is considered sufficient for posing the surgical indication. Surgery should be performed before degradation of the patient's clinical condition or after a short period of stabilisation. The prevention of hypertensive crises in the postoperative period depends on continuous monitoring of pulmonary pressures. This approach has considerably improved our surgical results as the mortality rate has fallen from 59 p. 100 (1980-1984) to 13.3 p. 100 (1985-1989) (p less than 0.005). In conclusion, obstruction of the pulmonary veins in TAPVD is no longer considered to be a poor prognostic factor, providing measures are taken to prevent pulmonary hypertension before, during and after surgery.