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

Background: Operative strategies and early results concerning repair of Tot al 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) wi th TAPVC were operated on. The data were collected retrospectively from the records. In 24 patients, a current follow-up study was performed, includin g clinical evaluation, echocardiography, and a twenty-four-hour ambulatory EGG. 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). Cause s of death were severe hypoplasia of central pulmonary veins in 1, ventricu lar fibrillation (2) and non-cardiac in one case. 80% of the operative surv ivors were available for assessment. Preoperatively, 11 of these patients w ere in NYHA functional class Il, six in class III and seven in class IV. Af ter treatment, 22 patients were in class I and two in class II. Ventricular function was evaluated by echocardiography and invasive catheterization. O nly two of 24 patients (8%) showed an abnormal IVS-motion and enlargement o f 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-t erm survivors underwent assessment of cardiac rhythm by 24 h electrocardiog ramm (ECG) monitoring. Significant arrhythmias were recorded in 11 of 24 ca ses (46 %), including sinus node dysfunction in 3 patients. Multiform ventr icular ectopic beats were evaluated in 9 cases. According to the Lown class ification, 7 patients were class 1 while 2 cases were considered to be clas s IV. Conclusions: A normal hemodynamic state can be achieved in most cases . Significant arrhythmias may exist in asymptomatic patients late after sur gical correction of TAPVC, and therefore, long-term follow-up of these pati ents, including 24 h ECG monitoring, is recommended, even if they are asymp tomatic.