Aortico-left ventricular tunnel is a rare congenital cardiac anomaly. A 45-year-old man was referred to our clinic with unstable angina pectoris. The patient had an aortico-left ventricular tunnel that had been operated on 15 years before and that now showed a recurrence. We performed a new surgical technique, including closure of orifices of the tunnel by resection of the aorta at the left coronary ostium, reconstruction of the aorta with patch plasty, and formation of a neo-left main branch by applying a saphenous magna vein patch at the noncoronary cusp. In this technique, the possibility of aortic regurgitation caused by stretching and distortion of the aortic ring and leaflets by primary suture closure of tunnel is eliminated. The postoperative 2-D colored Doppler echocardiography and cardiac MRI showed an excellent result of the procedure. Coronary flow could be restored, and thus anginal symptoms disappeared.
Cardiac surgical procedures can be safely performed after heart transplantation. To improve graft and patient survival, such procedures must be carefully performed after heart transplantation to avoid retransplantation. The shortage of donor organs will and must lead to an increase in the number of conventional procedures performed to improve allograft function in transplanted hearts.
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.
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.
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