No abstract

Background Although detachment of the implanted valve prosthesis was a well-known complication in patients with Behçet’s disease, complete detachment of an aortic bileaflet valve prosthesis has never been reported. Case presentation An 18-year-old boy with Behçet’s disease (HLA-A26 positive) who had previously undergone aortic valve replacement with an 18-mm ATS-Advanced Performance (ATS-AP) valve (ATS Medical, Inc., Minneapolis, MN) at the age of 12 years, presented sudden-onset general fatigue and was emergently transferred to the regional hospital. Chest X-ray showed displacement of the implanted mechanical valve. An echocardiogram revealed mobile valve prosthesis and severe aortic regurgitation. Just before leaving for our hospital for surgical treatment, a completely detached valve prosthesis was floating in the ascending aorta. On arrival, the valve prosthesis was stuck to the transverse arch. Emergent removal of the previous mechanical valve from the aortic arch and redo aortic valve replacement with a 24-mm ATS-AP valve were performed under total circulatory arrest. Infectious endocarditis was denied by histopathological examination. The patient was back to the intensive care unit with extracorporeal membrane oxygenation support, which was successfully decannulated 5 days later. Conclusions This was the first report of a patient with Behçet’s disease who encountered a complete detachment of implanted aortic valve prosthesis. The patient could be rescued by emergent surgery.

A high proportion of the sudden deaths among young individuals is attributable to anomalous aortic origin of a coronary artery. Among congenital heart diseases, the incidence of congenital coronary artery anomaly is approximately 1.3%. Herein, we report 2 surgical cases of anomalous aortic origin of a coronary artery.

Although the ventricular septal defect (VSD) that coexists with the severe form of Ebstein’s anomaly was thought to be beneficial for hemodynamics, it is unclear whether biventricular repair is always possible. Ebstein’s anomaly with VSD was diagnosed at 4 days of age based on a heart murmur and mild cyanosis. The cardiothoracic ratio was 78% and the Celermajer index was 1.6 (grade 4), but the hemodynamics were stable and tricuspid valve regurgitation was less than mild. Pulmonary overcirculation developed, and therefore, biventricular repair was attempted at 4 months of age, consisting of VSD patch closure, atrial septal defect partial closure, and the cone reconstruction of the tricuspid valve. Due to persistent cyanosis, however, nitric oxide inhalation and high flow nasal oxygen inhalation could not be discontinued. Catheter examination showed a cardiac index of 2.1 L/min/m2, moderate tricuspid regurgitation, and a pulmonary-to-systemic blood flow ratio of 0.64, and thus, takedown to one and one-half ventricular circulation using bidirectional superior cavopulmonary anastomosis, atrial communication closure, and right pulmonary artery banding was performed. The patient was discharged on postoperative day 25. Four months after discharge, the patient is doing well with home oxygen therapy.