日本小児循環器学会雑誌 Pediatric Cardiology and Cardiac Surgery

Background: This study aimed to ascertain midterm prognosis by comparing adult and child patients while accounting for the risk factors of complications and the effectiveness of the total cavopulmonary connection (TCPC) procedure.

Method: We analyzed data for 25 patients aged 18 or older who underwent TCPC procedure. The data for 75 patients aged 5 years or lower were analyzed for comparing the results.

Results: We observed perioperative complications in 13 patients including one perioperative death. The overall 5-year survival ratio after performing the TCPC procedure in adults was 96.0％, which was not different from that in child patients. On the contrary, cardiac events after discharge occurred more frequently in adult patients. Three patients died after performing the Fontan procedure in the observation period, all of whom had high end-diastolic pressure of the main ventricle and high pulmonary artery pressure. Cardiac events after discharge occurred more frequently among the patients with heterotaxy. Supraventricular tachyarrhythmia occurred more frequently after conducting the TCPC procedure in adult patients than in child patients both in the hospital and after discharge. Percutaneous oxygen saturation and cardiothoracic ratio improved significantly after the Fontan procedure; however, the mean cardiac index remained low at 1.9 L/min/m²．

Conclusion: The overall survival after performing the Fontan procedure with TCPC was satisfactory in adults. TCPC improved cyanosis and reduced volume overload in adult patients. However, certain patients may develop supraventricular tachyarrhythmia, and some may have high central venous pressure and low cardiac output after the surgery.