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.

Usefulness of the Vein Cuff Technique for Recurrent Stenosis due to Intimal Hyperplasia in Prosthetic Graft and Autologous Vein Graft Anastomosis / 日本心臓血管外科学会雑誌

We sometimes encounter the case that we have to make an anastomosis between a prosthetic graft and an autologous vein graft in revascularization of a lower extremity. However, it is said that the intimal hyperplasia in the anastomosis site of a prosthetic graft and autologous vein graft has a tendency to become severe in the long term postoperatively. We herein report a case in which a vein cuff (St. Mary's boot) technique was very useful to prevent recurrent stenosis due to intimal hyperplasia. No recurrence of stenosis in repair lesion has been detected for 7.5 years after operation.

Multiple Reoperations during a 48-Year Postoperative Course of Repaired Tetralogy of Fallot / 日本心臓血管外科学会雑誌

Due to the increasing number of patients with repaired tetralogy of Fallot (TOF), the amount of subsequent sequelae has also increased along with the need for further surgical interventions. This case report described a patient who underwent multiple reoperations for late sequelae over a period of 48 years after repair of TOF. The patient was a 58-year-old man who underwent a reparative operation for TOF at the age of 9 years. At the age of 30, he underwent patch closure of residual VSD and reconstruction of right ventricular outflow tract (RVOT) with a mono-cusped trans-annular patch combined with aortic valve replacement. At the age of 47, he underwent aortic root replacement for aneurysmal dilatation of the Valsalva sinus, and he was implanted with a pacemaker for bradycardia atrial fibrillation at the age of 51. By the age of 58, he required pulmonary valve replacement and tricuspid valve annuloplasty for right ventricular dysfunction resulting from regurgitation of the pulmonary and tricuspid valves as 4th midline sternotomy surgery. His status improved to NYHA functional class III to II after the most recent procedures, although he still needed strict medical control. A proactive approach during long-term follow-up after TOF repair is important for timely and appropriate surgical intervention in the event of late sequelae.

A Case of Cardiac Leiomyosarcoma Diagnosed with Recurrence after Resection of the Left Atrial Intramural Hematoma / 日本心臓血管外科学会雑誌

Cardiac leiomyosarcoma and atrial intramural hematoma are extremely rare. Here, we present the case of a 52-year-old woman with dyspnea. Echocardiography showed a large mass in the left atrium. Tumor resection was performed. A pathological diagnosis of intramural hematoma was made. Ten months later, she presented to our hospital again with dyspnea. Echocardiography and computed tomography showed recurrence of the left atrial tumor. Another tumor resection operation was performed. The tumor originated from the inferior wall of the left atrium, and it was surrounded by atrial wall thickening. Complete resection was not possible. Although postoperative chemotherapy was prescribed, the patient died of heart failure nine months after the second operation.