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

Surgical intervention after the Fontan procedure includes Fontan conversion, arrhythmia surgery, and atrioventricular valve repair/replacement. One possible complication is conduit stenosis, but few reports discuss this procedure. In this study, we discuss two patients who underwent conduit replacement after extracardiac total cavopulmonary connection to treat conduit stenosis. Patient 1 was a 20-year-old man diagnosed with tricuspid atresia who underwent the Fontan procedure at 3 years of age. As an adult, he developed complete atrioventricular block, and pacemaker implantation was planned. Further examination showed moderate neoaortic valve regurgitation and extracardiac conduit stenosis. He successfully underwent conduit replacement, neoaortic valve replacement, and pacemaker implantation. Patient 2 was a 20-year-old man with pulmonary atresia. At 5 years of age, he underwent the Fontan procedure, and at 19 years of age, he presented with malaise, melena, and hemoptysis. Cardiac catheterization demonstrated severe conduit stenosis with a 14-mm Hg pressure gradient. Conduit replacement was performed successfully. Conduit stenosis after Fontan completion is a rare but serious complication, and awareness of this complication is important during long-term follow-up, especially when cardiac imaging shows a curved conduit.