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 Case of Placement of the Reversed Gore Exclucder Contralateral Leg for Isolated Left Iliac Mycotic Artery Aneurysm / 日本心臓血管外科学会雑誌

A 49-year-old female with ruptured left common iliac mycotic arterial aneurysm (Lt. CIAA) was brought to our hospital as an emergency case. In Japan, endovascular treatment is unsuitable for the treatment of mycotic aneurysms, but findings from a Swedish national database showed that there was no difference in the long-term prognosis lasting over 10 years. Therefore, we performed endovascular aortic repair (EVAR) and saved the patient's life. The diameter of the proxymal sealing zone was larger than that of the distal zone. We used the Gore Excluder leg, which was inverted and implanted to match the caliber. Percutaneous abscess drainage was also performed on postoperative day 7 when hemostasis was confirmed for early infection control. The patient was discharged at 8 weeks postoperatively. After discharge from the hospital, oral antibiotics were continued until 6 months after the surgery. Six months postoperatively, contrast-enhanced computed tomography showed that the abscesses have disappeared. Blood samples that were taken at one month after the completion of antibiotics showed no evidence of the recurrence of infection and a curative course was achieved.

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.

A Case of Residual Atrial Septal Defect with Left Atrial Isomerism Requiring Reoperation 37 Years after the First Operation / 日本心臓血管外科学会雑誌

A 47-year-old female was admitted to our hospital for management of dyspnea. She had undergone surgery for an atrial septal defect (ASD) at the age of 17. Computed tomography revealed left isomerism, inferior vena cava interruption with azygos continuation and a residual ASD. Intra-operative findings showed that the residual ASD was positioned across the orifice of the hepatic vein. The previous suture line could be identified in the partially-closed atrial septum above the residual defect. Re-closure was performed without difficulties, and the patient's condition was good at discharge. Closure of ASD is a simple and basic procedure in cardiac surgery but care must be taken not to leave a shunt at the lower part of the defect, especially in cases with ASD defects in the lower margin.

Supra-Annular Mitral Valve Replacement in Two Infants / 日本心臓血管外科学会雑誌

When mitral valve dysfunction occurs in infants and mitral valve repair is difficult, mitral valve replacement (MVR) is required. However, commercially available prosthetic heart valves can be too large to implant in infants with a small annulus. In these children, the technique of supra-annular MVR is useful. Here we report two cases of supra-annular MVR, which were performed using an expanded polytetrafluoroethylene (ePTFE) graft as a skirt for a prosthetic valve. This method has been previously reported by Sung et al. The first case was a 16-month-old, 6.7-kg male infant who suffered from Shone' syndrome, mitral stenosis (MS) with a parachute mitral valve, coarctation of the aorta (CoA), and ventricular septal defect (VSD). MS progressed after CoA repair and VSD closure and a supra-annular MVR was performed. The second case was a 5-month-old, 4.9-kg female infant who suffered from polysplenia, intermediate atrioventricular septal defect (AVSD), and severe left atrioventricular valve regurgitation. AVSD repair was performed at the age of 3 months. However, valve stenosis and regurgitation gradually progressed postoperatively and consequently, a supra-annular MVR was performed. Postoperative prosthetic valve function was good in both cases. We believe that this method of performing supra-annular MVR is useful for infants with a small annulus.

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.

Type A Aortic Dissection during the Treatment of Tuberculous Pericarditis / 日本心臓血管外科学会雑誌

A 69-year-old woman, who had undergone a right nephrectomy for renal tuberculosis in her teens, was admitted with a low grade fever, anorexia and progressive dyspnea. Transthoracic echocardiography showed cardiac tamponade and chest CT revealed an enlarged ascending aorta. She was treated with pericardiocentesis. Specimens of pericardial effusion failed to demonstrate any acid-fast bacilli, but they did reveal a high level of adnosine deaminase (72 IU/<i>l</i>). A diagnosis of tuberculous pericarditis was considered, and antituberculous chemotherapy was started. However, he presented with severe back pain 32 days later and CT revealed type A acute aortic dissection. We therefore replaced the ascending aorta and aortic root. A histopathological examination of the ascending aorta revealed evidence of a granulomatous inflammatory reaction with Langhans giant cells. She thereafter received antituberculous chemotherapy with 4 drugs for 2 months, with continued rifampicin and isoniazid treatment. There was no evidence of any graft infection after 70 days.

A Case of Partial Aortic Root Remodeling for the Right Sinus of Valsalva Aneurysm with an Anomalous Origin of the Coronary Artery / 日本心臓血管外科学会雑誌

A sinus of Valsalva aneurysm is a rare cardiac disorder, and reports of it with an anomalous origin of the coronary artery are scarce. A 35-year-old male was admitted to our department with fatigue and cough. Multi-detector-row computer tomography (MDCT) revealed an isolated extracardiac right sinus of Valsalva aneurysm with an anomalous origin of the left circumflex artery (LCX) and total occlusion of the right coronary artery (RCA). Its diameter was about 70 mm. We performed a partial aortic root remodeling procedure with a trimmed J-graft because he had neither aortic regurgitation (AR) nor annuloaortic ectasia (AAE). Concomitantly, coronary artery bypass grafting to the RCA (Seg. 3) using a saphenous vein, and reconstruction of the LCX by Piehler's technique using a saphenous vein were added. The patient's postoperative course was uneventful, and he was discharged on the 28th postoperative day. Postoperative MDCT revealed that the aneurysm of the right sinus of Valsalva was not enhanced, and the RCA and LCX were patent. This procedure preserved the patient's own normal aortic valve and sinus of Valsalva and enables him to have more physiologically normal hemodynamics than aortic root reconstruction using a composite graft, e.g. Bentall procedure, Cabrol procedure, although the potential progression of the AR requires careful follow-up.

A Case of Early Progressive Aortic Valve Regurgitation after Coronary Artery Bypass Grafting in Aortitis Patient with Negative Findings for C-Reactive Protein and the Erythrocyte Sedimentation Rate / 日本心臓血管外科学会雑誌

We report the case of a 55-year-old woman with aortitis syndrome. She was admitted to our hospital because of repeated chest pain and syncope. An electrocardiogram and the laboratory data suggested acute myocardial infarction, and coronary angiography showed severe bilateral coronary ostial stenosis. No valvular disease was observed. Aortitis syndrome was suspected because of the stenosis of the brachiocephalic artery in addition to the bilateral coronary ostial stenosis, while the patient did not have elevated C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR). Coronary artery bypass grafting was performed, and the patient's postoperative course was uneventful. However, she again experienced chest pain 9 months after surgery due to aortic regurgitation (AR) and diffuse narrowing change of the left internal thoracic artery graft. Aortic valve replacement and Re-CABG was performed, and the patient was treated with steroid therapy postoperatively. The postoperative course was uneventful, but the patient thereafter died due to bleeding of a malignant adrenal tumor at 21 months after the second surgery.