Background : Left atrial reverse remodeling occurs when the left atrial load is reduced after mitral valve repair for mitral valve regurgitation. However, several reports demonstrate mitral valve stenosis after mitral valve repair with a mitral ring, which leads to impaired left atrial reverse remodeling. Objective : To examine the effect of ring size and body size on left atrial reverse remodeling after mitral valve repair. Patients and Methods : Sixty patients underwent transthoracic echocardiography before and after mitral valve repair for mitral valve regurgitation in our hospital. The left atrial volume (LAV) was compared pre- and postoperatively. Ring-size/BSA (mm/m²) was defined as values to express the mismatch between ring-size and body size after mitral valve repair with a mitral ring. In addition, we examined which factors correlated with the left atrial volume reduction rate including ring-size/BSA. The mean ring size was 28 mm. The study interval was 17±6 months. Result : LAV changed from 82±37 ml to 47±20 ml postoperatively. The left atrial volume reduction rate was 39.8±18.6%. The peak pressure gradient measured from the transmitral flow (TMF p-PG) was 7.5±3.0 mmHg. Ring size showed a stronger correlation with body height than BSA. The ring-size/BSA was 17.7±2.1 mm/m². Multivariate analysis shows that correlates of the LAV reduction rate were ring-size/BSA, pre LAV and age. Ring-size, TMF p-PG, ring annuloplasty only and postoperative TRPG did not show a strong correlation with the LAV reduction rate. Conclusion : A mismatch between body size and ring size is a negative factor for left atrial reverse remodeling after mitral valve repair with a mitral ring. The ring-size/BSA may be a useful factor to express grade of the mismatch.

Mucolipidosis is an autosomal recessive lysosomal storage disorder that demonstrates a clinical resemblance to mucopolysaccharidosis. Accumulation of glycoproteins throughout the body causes dysfunction of several organs, in particular, valvular heart diseases are an important cause of mortality, however, there is no consensus guideline regarding the indications and optimal timing of the surgical repair because of the unclear and short natural history. Here we present 12- and 15-year-old siblings diagnosed with mucolipidosis who underwent aortic valve replacement. The senior sibling received redo-aortic valve replacement for prosthetic valve dysfunction 11 years after the initial surgery. A few surgical valve replacements in patients with mucopolysaccharidosis have been reported, however, there is no published case of aortic valve replacements in two siblings with mucolipidosis.

Surgical cases for pulmonary artery aneurysm after palliative operation for congenital heart disease are rare. A man in his 40s underwent Waterston operation (side-side anastomosis of the ascending aorta and right pulmonary artery) for pulmonary atresia and ventricular septal defect at the age of one. Medical follow-up was continued at a local clinic without definitive repair. He had complained of exertional dyspnea and cough for one month. CT scan on admission showed a large right pulmonary artery aneurysm and atelectasis of the right lung. An urgent operation was planned for the huge pulmonary artery aneurysm. Under cardiac arrest, the proximal end-to-side anastomosis to the aorta was conducted using a Y-shaped vascular prosthesis at the previous Waterston anastomosis. The peripheral pulmonary arteries were reconstructed by inclusion technique. Percutaneous cardiopulmonary support was necessary for one day after operation due to unstable hemodynamics. He was extubated on postoperative day (POD) 4 and transferred to our general ward on POD 5. He was discharged home on POD 38. He is now free from heart failure symptoms and recurrence of aneurysm one year after surgery. Careful follow-up is necessary for the dilatation of the pulmonary artery and aortic root. A definitive operation—Rastelli and closure of the ventricular septal defect—may be considered in the future if the pulmonary artery resistance is suitable for the repair.

A 65-year-old woman was admitted to our hospital complaining of chest pain in 2012. Coronary computed tomography angiogram revealed left main coronary artery atresia (LMCAA) with congenital absence of the left main trunk, and LAD received several collaterals perfusing from the RCA. She was observed because her symptoms were transient. However, her dyspnea on effort gradually became worse in 2016. Treadmill exercise electrocardiography showed ST depression in II・III・aVF and V4-6. Also, echocardiography showed severe MR due to the prolapse of the anterior leaflet and moderate TR. We performed mitral valve plasty and tricuspid valve plasty, and coronary artery bypass grafting using the left internal thoracic artery to the left descending artery in March, 2017. The postoperative course was uneventful. She was discharged in good condition 14 days after operation. LMCAA is a rare coronary anomaly. There are only 53 published case reports, and a few surgical reports in adult cases. This is a rare adult case of surgical treatment of LMCAA.

Pulmonary hypertension persisted in a 57-year-old man after mitral and tricuspid valve replacement to treat mitral and tricuspid regurgitation. Heart failure gradually worsened after surgery. Pulmonary hypertension was initially considered as the major reason for the heart failure, and inhaled nitric oxide was administered. Thereafter, the heart failure improved and mechanical circulatory assist could have been avoided. We believe that inhaled nitric oxide is a less invasive and effective method for improving pulmonary hypertension and hemodynamics after mitral valve replacement.

A 72-year-old woman underwent thoracic endovascular aortic repair (TEVAR) for an aortic arch aneurysm at a previous hospital. During follow-up, although the aneurysm was found to have become bigger, no further treatments were given, except for conservative follow-up. The patient sought a second opinion and thus visited our hospital. Enhanced computed tomography (CT) revealed a type I endoleak that required repair. Total arch replacement with removal of the partial stent-graft system was performed under deep hypothermic circulatory arrest. The patient made a steady progress postoperatively and was discharged without any complications. Endovascular repair is minimally invasive and frequently used in various medical facilities but carries a considerably high risk of reintervention. Treatment strategies for aortic aneurysm, including open surgery, should be carefully chosen.

A 71-year-old man with Behçet's disease was admitted to our hospital for treatment of a thoracic aortic aneurysm. On admission, there was marked inflammatory response, but blood culture was negative and there was no significant accumulation of gallium scintigraphy. The aorta was shaggy and there were two aneurysms in the descending aorta. We performed endovascular aortic repair for this aneurysm in consideration of the inflammatory aortic aneurysm. After treatment, the patient had paraparesis, however he underwent physical rehabilitation to regain function. He was followed up for 1 year and remains in good clinical condition without anastomotic aneurysm, dilatation or aneurysm at another site.

Secondary abdominal fascial closure by abdominal vacuum-assisted closure (VAC) therapy is required for abdominal organ protection and prevention of infection due to abdominal compartment syndrome (ACS) developing after the surgery. In this paper, we present our experience with abdominal VAC therapy for two cases that required open abdominal management after surgical repair for ruptured abdominal aortic aneurysm, with favorable outcomes. Case 1 involved a 72-year-old man who underwent endovascular aortic repair for ruptured abdominal aortic aneurysm. Abdominal VAC therapy was started after decompression laparotomy because he developed ACS immediately after surgery. Secondary abdominal fascial closure was performed on day 4 postoperatively, and he had no complications. Case 2 involved a 71-year-old man who underwent emergency Y-graft replacement for ruptured abdominal aortic aneurysm. We considered secondary abdominal fascial closure necessary because of prominent intestinal edema and massive retroperitoneal hematoma, and performed abdominal VAC therapy. We changed the VAC system on day 4, postoperatively and performed secondary abdominal fascial closure on day 7, postoperatively. Abdominal VAC therapy is considered effective and safe for patients requiring secondary abdominal fascial closure after abdominal surgery.