Aortic Root and Pulmonary Artery Reconstruction in a Patient with Pulmonary Artery Intimal Sarcoma / 日本心臓血管外科学会雑誌

A 52-year old woman was referred to our hospital because of abdominal pain and restlessness. A chest contrast-enhanced CT showed huge pericardial effusion and intraluminal defects in the main pulmonary artery. We could not make a diagnosis based on the cytology of the pericardial effusion and histopathology of the mass with a sample taken by a catheter. Therefore, we undertook biopsies of the mass by median sternotomy, which led to the diagnosis of pulmonary intimal sarcoma. The tumor resection was performed to release the right ventricular outflow stenosis. We tried to resect the tumor as much as possible, and reconstructed the pulmonary artery and aortic root. She was discharged to home and survived 5 months after surgery.

Cardiovascular Surgery in Patients with Connective Tissue Disease / 日本心臓血管外科学会雑誌

<p><b>Background</b> : Connective tissue disease (CTD) is an idiopathic autoimmune disorder which causes systemic chronic inflammation. Inflammation causes various cardiovascular diseases. Systemic steroid use, which is usually the sole treatment for CTD, also causes arteriosclerosis. Although cardiovascular surgery is often necessary in patients with CTD, preexisting multiple organ dysfunction related to CTD, in addition to systemic administration of steroids or other immunosuppressants, is thought to increase the risk of surgery. However, little is known about how the disease process of CTD influences early and late cardiovascular surgery outcomes. <b>Methods</b> : To better understand these issues, we reviewed 31 patients with CTD (study group) and compared their outcomes to those of other patients (control group) who underwent cardiovascular surgery at our institution between April 2008 and November 2013. <b>Results</b> : There were 26 women and 5 men, and the average age was 64.4±16.7 years. CTD types included rheumatoid arthritis in 7 patients, systemic lupus erhythematosus in 6, aortitis syndrome in 6, polymyalgia rheumatica in 3, scleroderma in 3, polymyositis in 3, and others. The procedures included 10 valve cases, 10 coronary artery bypass grafting (CABG) or CABG-valve combination cases, and 11 isolated or complicated thoracic aortic surgery cases. Prior to undergoing these procedures, 24 patients (77.4%) were treated with steroids and/or immunosuppressant, and 6 patients had been diagnosed with interstitial pneumonia in the study group. Moreover, the rate of peripheral artery disease and carotid artery stenosis in the study group was significantly higher than that in the control group. There were no perioperative deaths in the study group. There were no significant differences in terms of major complications such as ischemic events, infection, acute kidney injury, lung injury, and others between the groups. We conducted a follow-up survey for the study group with an average period of 27.8±16.0 months. During the follow-up period, there were 4 late deaths. In addition, 8 patients required readmission, 6 for cardiovascular events and 2 for poor wound healing. All the survivors in the study group showed improved cardiac function and were in the NYHA functional class I and II. <b>Conclusion</b> : Cardiovascular surgery for patients with CTD can provide acceptable early and mid-term results.</p>

Staged Repair for a Patient with Infracardiac Total Anomalous Pulmonary Venous Connection Complicated by Hypoplastic Left Heart Complex / 日本心臓血管外科学会雑誌

Total anomalous pulmonary venous connection (TAPVC) is rarely associated with remarkably small left heart structures. In these types of cases, the hemodynamics resembles that of hypoplastic left heart syndrome, and the treatment strategy is controversial. We present the case of a 1-day-old girl with infracardiac TAPVC, small left heart structures (hypoplastic left heart complex), bilateral superior <i>vena cava</i>, and aberrant origin of the right subclavian artery. We performed a semi-emergent first-stage open palliation for repair of TAPVC, because of pulmonary venous obstruction. We concomitantly performed atrial septal defect (ASD) enlargement and bilateral pulmonary artery banding (BPAB). The postoperative course was uneventful and the left heart structures did not grow, so we performed the Norwood procedure and placed a right ventricle-pulmonary artery shunt with a 5.0 mm artificial graft. Subsequently, the left heart structures were not suitable for biventricular repair, so we chose univentricular repair. The patient underwent a bilateral bidirectional Glenn operation and Fontan completion at 6 and 23 months of age, respectively. TAPVC repair, BPAB, and ASD enlargement are reasonable surgical options for a patient with borderline small left heart structures and TAPVC, as they enable us to wait for growth in the left heart structures and to determine whether univentricular or biventricular repair is suitable.

Hemolytic Anemia after Mitral Valve Surgery / 日本心臓血管外科学会雑誌

<b>Objective</b> : The aim of this study is to describe a series of patients undergoing reoperation due to hemolytic anemia after mitral valve surgery and assess the mechanisms and surgical outcomes. <b>Methods</b> : Between 2009 and 2014, we performed redo mitral valve surgery in 11 patients who had refractory hemolytic anemia after mitral valve surgery at Kyoto University Hospital. The mean age of the patients was 72.2±6.8 years old, and there were 5 men. <b>Results</b> : Preoperative echocardiography demonstrated that only 3 patients had ≥ grade 3 mitral regurgitation (MR), the rest of the patients had only mild to moderate MR. The mechanisms of severe hemolysis included paravalvular leakage (PVL) after mitral valve replacement (MVR) in 8 patients, structural valve deterioration (SVD) after MVR using a bioprosthesis in one, and residual/recurrent mitral regurgitation after mitral valve plasty (MVP) in two. All the patients except one (re-MVP) underwent MVR. The mean interval between previous operation and current operation was 14.1±9.4 years in post-MVR cases, and 2.0±1.9 years in post-MVP cases. There were three late deaths, one of which was due to cardiac death (exacerbation of heart failure due to pneumonia). There was one patient who required re-MVR for recurrent hemolysis due to PVL after MVR. <b>Conclusion</b> : Although hemolytic anemia after mitral valve surgery is rare, it often requires reoperation regardless of the degree of MR at late follow-up period. Thus, patients after mitral valve surgery should be carefully followed-up.

A Case Report of Atrio-Esophageal Fistula Caused by Percutaneous Transcatheter Ablation of Atrial Fibrillation / 日本心臓血管外科学会雑誌

A 66-year-old man underwent percutaneous transcatheter ablation of the myocardium to treat chronic atrial fibrillation. Fifteen days after the procedure, he visited our hospital with a chief complaint of hematemesis. At that time, upper gastrointestinal endoscopy led to a diagnosis of esophageal ulcer. Oral food intake was suspended for approximately 1 month. Subsequently, 4 days after resumption of oral intake, he developed multiple cerebral infarcts. Moreover, massive hematemesis occurred, with resultant shock and cardiopulmonary arrest. At this point, a definitive diagnosis of left atrio-esophageal fistula resulting from the injury relating to the transcatheter ablation was made. Cardiopulmonary resuscitation was carried out, followed by emergency surgery. The operation was performed via median sternotomy and was done under cardiac arrest using complete extracorporeal bypass, and the fistula in the posterior left atrial wall and the middle esophagus were directly sutured for closure. Unfortunately, 3 days after this open heart surgery, the patient died from low cardiac output syndrome and multiple organ failure. Although rare, this complication may be fatal when it develops then its prevention is important. Once atrio-esophageal fistula develops after percutaneous transcatheter ablation, immediate surgical intervention seems essential.

Giant Subclavian Artery Aneurysm Associated with Airway Stenosis / 日本心臓血管外科学会雑誌

A 65-year-old man presented to our hospital with a chief complaint of hoarseness. Chest radiography and computed tomography detected a right subclavian artery aneurysm. The aneurysm had a maximum diameter of 85 mm, and was associated with a mural thrombus and displacement of the trachea to the left, which led to airway stenosis. In case ventilatory insufficiency developed during anesthesia induction, an extracorporeal membrane oxygenator was prepared, followed by administration of anesthesia. Careful administration of anesthesia allowed for anesthesia management without the extracorporeal membrane oxygenator. We approached the periphery and the proximal portion of the aneurysm through a right subclavicular incision and partial median sternotomy, respectively. After excision of the aneurysm, we performed EPTFE prosthesis implantation. The patient's postoperative course was uneventful, which led to postoperative improvement of the airway stenosis. The combination of a right subclavicular incision and partial median sternotomy is useful for the surgical treatment of large subclavian artery aneurysms such as the one in this case. Moreover, careful anesthesia management after close consultation with anesthesiologists is important for patients who exhibit preoperative airway stenosis.

Infected Abdominal Aortic Aneurysm Rupture due to <i>Listeria monocytogenes</i> / 日本心臓血管外科学会雑誌

A76-year-old man was transferred to our hospital for evaluation of continuous abdominal pain with systemic inflammation. Clinical examinations suggested the pressure of an adynamic ileus due to diverculitis and an infrarenal abdominal aortic aneurysm. Thirty-eight millimeter in diameter despite the commencement of antibiotic therapy, the symptoms progressively worsened and repeated CT scan demonstrated a rupture of the aneurysm without any enlargement in diameter. An emergency operation was done, and the patient was discharged on the 77th postoperative day. Blood culture and histological findings disclosed an infected aortic aneurysm due to <i>Listeria monocytogenes</i>. The possibility of aortic infection should be considered in patients showing a sudden rupture of an aortic aneurysm.