J Korean Pediatr Soc.  2002 Feb;45(2):199-207.

Conversion of Total Atrio-pulmonary Connection to Total Cavo-pulmonary Connection-Review of Indications and Hemodynamic Characteristics-

• Seo JH
• Lee JK
• Choi JY
• Sul JH
• Lee SK
• Park YW
• Cho BK

Abstract

PURPOSE: Since the successful application of total atrio-pulmonary connection(TAPC) to patients with various types of physiologic single ventricles in 1971, post-operative survival rates have reached more than 90%. However some patients have been shown to present with late complications such as right atrial thrombosis, atrial fibrillation and protein losing enteropathy eventually leading to re-operation to control the long-term complications. The aim of this study is to review the results of total cavo-pulmonary connection(TCPC) in cases with late complications after TAPC.
METHODS
Between Jan. 1995 and Dec. 2000, 6 patients(5 males and 1 female) underwent cardiac catheterization 11+/-3 months after conversion of previous TAPC to TCPC. We compared the hemodynamic and morphologic parameters before and after TCPC and also assessed the clinical outcomes. The indications for TAPC were tricuspid atresia in 4 cases and complex double-outlet right ventricle with single ventricle physiology in 2 cases.
RESULTS
There was no peri-operative mortality and all patients were clinically and hemodynamically improved at a mean follow-up of 11 months(range: 4 to 13). However, protein losing enteropathy recurred in 2 patients; this was were successfully treated with subcutaneous administration of heparin. Right atrial pressure before TCPC was 18.0+/-3.6 mmHg, but baffle pressure, corresponding to right atrial pressure decreased to 14.8+/-3.6 mmHg after TCPC. The size of the pulmonary arteries did not regress after TCPC.
CONCLUSION
The conversion of TAPC to TCPC improves clinical and hemodynamic status by decreasing the right atrial pressure and by providing a laminar cavo-pulmonary flow which enhances the effective pulmonary circulation in the so-called Fontan circulation.

Keyword

Total atrio-pulmonary connection; Total cavo-pulmonary connection; Protein losing enteropathy; Single ventricle

MeSH Terms

Atrial Fibrillation
Atrial Pressure
Cardiac Catheterization
Cardiac Catheters
Double Outlet Right Ventricle
Follow-Up Studies
Hemodynamics*
Heparin
Humans
Male
Mortality
Physiology
Protein-Losing Enteropathies
Pulmonary Artery
Pulmonary Circulation
Survival Rate
Thrombosis
Tricuspid Atresia
Heparin