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.

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.

Stent-Graft Re-expansion Following Axillo-Bifemoral Bypass : A Case of Stent Graft Collapse due to Acute Type B Aortic Dissection / 日本心臓血管外科学会雑誌

We describe a rare complication and treatment progression that occurred in a 64-year-old man with an aortic abdominal aneurysm (AAA) that had been treated by endovascular aneurysm repair (EVAR). He had undergone EVAR to treat an infra-renal type AAA 21 months previously and returned to the emergency department with back pain. Contrast-enhanced computed tomography (CT) revealed acute type B aortic dissection, so he was admitted and conservative medical management was started. Acute stomachache and limb pain appeared on hospital day 7, which prevented him from moving his lower limbs. The main body of the stent graft had collapsed, blocking blood flow, and contrast was not found in arteries from the collapsed stent graft portion to the knee level on emergency contrast CT images of the leg. His legs were revascularized by an extra-anatomical right axial-bilateral external iliac bypass. His symptoms disappeared and reperfusion injury was avoided. The collapsed stent graft had retained its original shape at 11 and 18 days after surgery. Furthermore, follow-up CT 4.5 years later showed that the stent graft retained its original form.

Björk-Shiley Prosthetic Valve Dysfunction Caused by Complete Disc Fracture / 日本心臓血管外科学会雑誌

<p>We present herein a case of disc fracture of a Björk-Shiley valve prosthesis in the mitral position. A 69-year-old woman was admitted to our hospital with a sudden onset of dyspnea followed by deep shock. An echocardiography showed a severe degree of mitral regurgitation and moderate degree of tricuspid regurgitation. Forty-three years previously she had undergone a mitral valve replacement (MVR) for stenosis with the original version of a Björk-Shiley valve prosthesis in another institute. Emergency redo MVR was performed with a bioprosthesis and tricuspid annuloplasty with a semirigid ring. The disc of the extracted Björk-Shiley valve was found to have escaped from the metal housing with two intact struts. Although Björk-Shiley valve dysfunction due to Delrin disk abration has been rarely reported, complete disk fracuture is extremely rare. The important role of regular echocardiographic follow-up should be emphasized to prevent fatal valve fracture.</p>

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.