Search

Introduction: Total cavo-pulmonary connection (TCPC) represents the current surgery standard for congenital univentricular heart disease (CUHD) patients. We studied the health related quality of life (HRQOL) expressed by TCPC operated patients., Patients and Methods: This transversal study is based on 24 children and 10 adult TCPC patients aged from 5 to 41 years old. The HRQOL evaluation relies on generic self report instruments such as the PedsQL 4.0 for the children and their parents, and the Duke Health profile for the adult patients. Additional questions were specifically designed for this study as well., Results and Discussion: The estimated HRQOL is 68.15 for the children and 68.71 for their parents. In these young cases, the global HRQOL or some specific score are altered when any comorbidity or scholar difficulties are present, or when TCPC was performed more than 10 years ago. The estimated HRQOL is 74.67 in adulthood. In these patients, the young age, the lifestyle, the onset of more than 2 early complications ans the necessity of anti-arrhythmia therapy decrease various HRQOL scores. The physical handicap is considered as the major limitation for children and adult patients. Thanks to adaptative and cognitive compensatory mechanisms, such as resilience, both groups express a satisfying HRQOL assessment., Conclusion: Our study indicates that TCPC provides a good HRQOL in CUHD patients despite the complex management and follow-up of these diseases, the risk of complication onset and the physical handicap usually observed.

The aim of the study was to assess the short and medium term results of total cavo-pulmonary connection based on analysis of the functional status, the cavo-pulmonary circulation and the surgical techniques, and the hepatic consequences. Fifteen patients with congenital defects beyond repair were treated by total cavo-pulmonary connection at Tours between March 1st 1992 and July 30th 1996. There were 12 children (mean age: 6.3 years) and 3 adults aged 25 to 28. Results were assessed by clinical examination, hepatic function tests and cardiovascular investigations including right heart catheterisation with angiography in 14 patients. There were no fatalities. Seven patients were in functional Class I and 8 in Class II at medium term (average follow-up of 33 months). Hepatic function was mildly abnormal in all patients with an increase in serum bilirubin and gamma GT, and a decrease in the coagulation factors. The mean pressures in the atrial channel were 12 mmHg (9-16 mmHg), in the superior vena 13.2 mmHg (10-18 mmHg), in the right pulmonary artery 9.5 mmHg (7-15 mmHg) and 11.6 mmHg (8-16 mmHg) in the left pulmonary artery. Significant residual stenosis of a pulmonary branch was observed in 2 cases. The cavo-pulmonary anastomoses were out of line, one from the other, in all cases. The atrial channel was tubular in 9 cases and dilated with slight stagnation of the contrast medium in its inferior region in 5 cases. Total cavo-pulmonary connection transformed the clinical status of these patients but was associated with minor abnormalities of liver function. The quality of the cavo-pulmonary circulation and the surgical anastomoses was estimated to be satisfactory in the majority of cases.