Heart transplantation after Fontan procedure in adults.

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.

Surgical repair of congenital supravalvular aortic stenosis in adult.

Supravalvular aortic stenosis is a rare congenital cardiac anomaly occurring mainly as a part of Williams-Beuren syndrome. Aortic narrowing above the level of the aortic valve causes obstruction of the left ventricular outflow tract, and a pressure gradient between the left ventricle and the aorta causes left ventricle hypertrophy. We report here a case of a 22-year-old man who underwent extended patch aortoplasty because of supravalvular aortic stenosis accompanying Williams-Beuren syndrome. He was in New York Heart Association functional class III with localized hourglass type supravalvular aortic stenosis. Related to arterial hypertension he was in a cardiac decompensation. Mean pressure gradient was 73 mm Hg and maximum gradient 104 mm Hg. Electrocardiography indicated left ventricle hypertrophy, which was also seen in x-ray, as heart enlargement. We successfully treated this patient with extended patch aortoplasty and immediate postoperative echocardiography showed reduction of gradient. Good surgical outcome of congenital supravalvular aortic stenosis in adults can be achieved with this treatment. This technique provides symmetric reconstruction of the aorta with good postoperative results and no gradient across aortic valve and aortic valve insufficiency remains, providing excellent long-term relief of localized supravalvular gradients and preservation of aortic valve competence.