A Case of Neonatal Marfan Syndrome Requiring Double Atrioventricular Valve Replacement due to Rapid Exacerbation of the Repaired Valves / 日本心臓血管外科学会雑誌

Neonatal Marfan syndrome is the most severe form of Marfan syndrome usually showing critical cardio-respiratory symptoms from the neonatal period or early infancy. We report a boy with this syndrome who presented with heart failure at 3 months of age and was referred to our department at 6 months old after intense medical treatment. He had enophthalmos, funnel chest, arachnodactyly, and Steinberg's thumb sign, but had no family history of Marfan syndrome or other cardiac diseases. Left ventricular dilatation, severe mitral regurgitation and moderate tricuspid regurgitation were noted on echocardiography. Mitral valvuloplasty and tricuspid annuloplasty were performed, and the regurgitation improved to trivial and mild level, respectively. However, rapid exacerbation of mitral regurgitation occurred, and the patient fell into circulatory collapse which needed circulatory support with extracorporeal membrane oxygenator (ECMO) on 18th postoperative day. In the emergency operation, the previous surgical procedures on the mitral valve were intact and we thought that rapid progression of the mitral annular dilatation and valve expansion to be the cause of exacerbation. Mitral valve replacement (Regent® 21 mm aortic) was performed, and the cardiac function improved, but ECMO was still needed because of the depressed respiratory function. Furthermore, tricuspid regurgitation due to annular dilatation and valve expansion was aggravated rapidly which needed tricuspid valve replacement (ATS® 20 mm mitral) 9 days after the mitral valve replacement. ECMO was ceased on the 37th day and the patient was extubated on 71st day. He was discharged from the hospital 5 months after the first operation. One year has passed after discharge, and he is doing well with anticoagulation. In the treatment of neonatal Marfan syndrome, surgical procedure for valve repair is still controversial and it should be remembered that rapid exacerbation of the atrioventricular valve can occur even after satisfactory valve repair and there should be no hesitation regarding surgical intervention when needed.

A Rare Case of Intracardiac Angiomyolipoma / 日本心臓血管外科学会雑誌

A 34-year-old woman was admitted with a history of syncope and a mass was detected in the right atrium (RA) by transthoracic echocardiography (TTE). Preoperative chest computed tomography (CT) also demonstrated an RA tumor measuring 4×3 cm. We performed resection of the RA tumor under cardiopulmonary bypass. Histopathological findings showed that the tumor was an angiomyolipoma. It is well known that angiomyolipomas are most frequently found in the kidney and are associated with tuberous scleroses. There was no evidence of tuberous sclerosis in this case. Primary tumors of the heart are rare. However, there have been a few intracardiac angiomyolipomas reported previously.