Long-term results after repair of total anomalous pulmonary venous connection.

BACKGROUND: Operative strategies and early results concerning repair of Total Anomalous Pulmonary Venous Connection (TAPVC) are relatively well known. Less well defined data are available to evaluate the long-term outcome. We would therefore like to contribute our long-term data in this presentation.

PATIENTS AND METHODS: Between 1958 and 1992 52 consecutive patients aged two days to 42 years (15 neonates, 16 infants, 9 children and 12 adults) with TAPVC were operated on. The data were collected retrospectively from the records. In 24 patients, a current follow-up study was performed, including clinical evaluation, echocardiography, and a twenty-four-hour ambulatory ECG.

RESULTS: Early mortality was 34.6% (n = 18). The postoperative follow-up period ranged from 4 months to 28 years (mean 10.7 years). There were 4 late deaths, yielding an overall long-term mortality of 7.7% (4/52). Causes of death were severe hypoplasia of central pulmonary veins in 1, ventricular fibrillation (2) and non-cardiac in one case. 80% of the operative survivors were available for assessment. Preoperatively, 11 of these patients were in NYHA functional class II, six in class III and seven in class IV. After treatment, 22 patients were in class I and two in class II. Ventricular function was evaluated by echocardiography and invasive catheterization. Only two of 24 patients (8%) showed an abnormal IVS-motion and enlargement of the right ventricle. Cardiac catheterization revealed a mean PA pressure of 26 mmHg, the peak systolic pressure in the RV was 34 mmHg. All 24 long-term survivors underwent assessment of cardiac rhythm by 24 h electrocardiogramm (ECG) monitoring. Significant arrhythmias were recorded in 11 of 24 cases (46%), including sinus node dysfunction in 3 patients. Multiform ventricular ectopic beats were evaluated in 9 cases. According to the Lown classification, 7 patients were class I while 2 cases were considered to be class IV.

CONCLUSIONS: A normal hemodynamic state can be achieved in most cases. Significant arrhythmias may exist in asymptomatic patients late after surgical correction of TAPVC, and therefore, long-term follow-up of these patients, including 24 h ECG monitoring, is recommended, even if they are asymptomatic.