Heart transplantation after Fontan procedure in adults
Tolga S Coskun1, Oguz K Coskun, Mahmut El Arousy
1Department of Cardiac Surgery, Heart and Diabetes Center, North-Rhine Westphalia, Bad Oeynhausen, Germany.
Insights
Heart transplantation can be successful for adults with complex congenital heart disease after a Fontan procedure. Careful patient selection and surgical timing are crucial for improving long-term survival in these challenging cases.
Area of Science:
- Cardiology
- Adult Congenital Heart Disease
- Cardiac Surgery
Background:
- Complex congenital heart disease accounts for 1.5%-2.4% of adult heart transplantations.
- Indications include ventricular dysfunction, failed Fontan physiology, and cyanosis.
Observation:
- A 43-year-old man with a history of Fontan operation for a univentricular heart, tricuspid atresia, L-Transposition of the Great Arteries, and subpulmonary stenosis underwent heart transplantation.
- The patient experienced persistent bleeding, requiring transfusions and reoperation, followed by extracorporeal circulation due to low output syndrome.
Findings:
- Heart transplantation can be successful in end-stage congenital heart disease patients post-Fontan procedure.
- Improved surgical and medical management contribute to successful outcomes.
Implications:
- Challenges in heart transplantation for these patients include complex anatomy, bleeding risks, and hemodynamic assessment difficulties.
- Careful patient selection and surgical timing are essential for optimizing long-term survival.
Abstract:
Complex congenital heart disease is the cause for 1.5%-2.4% of heart transplantations in adults. Indications for heart transplantation are ventricular dysfunction, failed Fontan physiology, and cyanosis. We present a 43-year-old man, 20 years after a Fontan operation by an univentricular heart, with a tricuspid atresia, L-Transposition of the Great Arteries, and high-grade subpulmonary stenosis. Persistent bleeding after successful transplantation necessitated major blood transfusions and reoperation. We reconstructed superior vena cava and vena brachiocephalica using a pericardial patch, and because of postoperative low output syndrome, he was connected to extracorporeal circulation through cannulation of femoral artery and explanted after hemodynamic stability. Heart transplantation can be successful in patients with end-stage congenital heart diseases after Fontan procedure, because of improved surgical and medical management. Challenges include limited ability to assess hemodynamics, complex anatomy, increased risk of bleeding caused by multiple prior procedures, and unique underlying pathologic states. Careful patient selection and surgical timing are essential to increase long-term survival.
