Abstract
Introduction: The aim of our prospective study was to evaluate cardiovascular function and the quality of life of patients with a functionally single ventricle following total cavopulmonary connection (TCPC). Patients and method: 148 patients were examined at age 4 to 33 years (median 10 years) and at interval 1 to 9 years (median 5 years) after TCPC.
Their age at surgery was 1 to 28 years (median 5 years). An intraatrial lateral tunnel was used in 131 patients and extracardiac conduit in 17 patients, a fenestration was performed in a total of 56 (38%) patients.
Primary TCPC was undertaken in 58 (39%) patients. TCPC was preceded by pulmonary artery banding in 32 patients, arterial anastomosis in 36, bidirectional cavopulmonary anastomosis in 43, and modified Fontan operation in 2 patients.
Results: Normal or limited exercise tolerance (NYHA Class I-II) was reported by a total of 96% patients. 121 patients were on antiaggregation dose of aspirin and 27 patients were on warfarin. Postoperative thromboembolic events were reported in (4%) patients.
Echocardiography revealed good cardiac function in 135 (91%) patients. In a subgroup of 62 patients with tricuspid atresia or double inlet left ventricle, the mean left ventricular fractional shortening was 0.31 +- 0.06.
Compared with preoperative values there was a significant decrease in end-diastolic dimension (from 136 +- 20% to 113 +- 18%, p < 0.001) and in posterior wall thickness (from 132 +- 27% to 111 +- 16%, p < 0.001). There was no change in the wall thickness/ventricular dimension ratio.
Good atrioventricular (AV) valve function was shown in 43 (81%) out of 53 patients with a single mitral valve, in 47 (64%) out of 73 patients with two AV valves; in 4 (40%) out of 10 patients with a single tricuspid valve; and in 2 (17%) out of 12 patients with a common AV valve (P2 = 40.6; p < 0.001). In 8 (67%) out of 14 patients undergoing AV valve surgery for regurgitation, the valve function improved by at least one grade.
On 24 hours ECG monitoring, 73% patients had regular sinus rhythm without documented dysrhythmia and 16% patients had insignificant sinoatrial (SA) automaticity dysfunction. Permanent cardiac pacing required 5% of patients and 4% of patients used antiarrhythmics for significant supraventricular tachydysrhythmias.
Sinus rhythm was present in 82% of patients prior to TCPC. Actuarial probability of sinus rhythm at 2 and 5 years postoperatively was 97% and 82%, respectively.
A fenestration was closed at catheterization in 21 patients, surgically in 4 patients and spontaneous closure occurred in 8 patients. Mean maximal oxygen consumption (VO2max.) during exercise test was 34.1 +- 7.4 ml/kg/min and standardized maximal oxygen consumption was -2.2 +- 1.7 SD of the normal population.
VO2max. was significantly reduced (p < 0.001) in patients with open fenestration or residual intraarterial shunt. There was a significant decrease in oxygen saturation at maximal exercise in patients with right-to-left intraarterial shunt as well as in patients without a shunt.
Conclusion: Total cavopulmonary connection allows an effective physiological correction of complex congenital heart defects with a functionally single ventricle. All patients require lifelong follow-up by a cardiologist.