Journal of Pediatric Cardiology and Cardiac Surgery

Stenosis of a Fontan conduit is a well-known complication which could occur even many years after the Fontan surgery. Reoperation methods to solve the problem vary from institution to institution, and views how to proceed are not unified. Here, we report a case of reoperation 34 years after the previous surgery because of calcification in the conduit and the inferior vena cava (IVC). This was a complex case, a 43-year-old woman with multiple congenital heart abnormalities, including right isomerism, dextrocardia, and univentricular heart. The patient had undergone the Fontan type operation using an intra-atrial conduit at 8 years of age and her condition was stable. Her condition unexpectedly deteriorated expressing a gradual onset of fatigue on effort, leading to the discovery of calcification-induced stenosis in the intra-atrial conduit and the IVC. It became imperative to arrange surgical intervention because of increasing calcification and developing symptoms. The challenging surgery through the second sternotomy revealed significant adhesion. There was a calcified tissue within the IVC, necessitating meticulous removal. The intra-atrial conduit previously placed could only be extracted partially due to firm adhesion, prompting us to decide to perform an extracardiac Fontan method while leaving the majority of the intra-atrial conduit. Postoperative assessments, including contrast-enhanced computed tomography, confirmed the patient’s uneventful recovery without conduit stenosis. This case highlights the importance of frequent follow-up and early surgical intervention in case calcification-induced stenosis would progress after the initial Fontan type procedure. Our decision to revise the Fontan pathway using an extracardiac conduit, while retaining the intra-atrial conduit, provided valuable insights for such a challenging circumstance with severe adhesion.