A Double Connection of Mixed Total Anomalous Pulmonary Venous Connection : Successful Patch Closure of Residual Shunt / 日本心臓血管外科学会雑誌

Double drainage sites from a common venous confluence of the pulmonary veins of a mixed total anomalous pulmonary venous connection is a rare condition that is called a “double connection.” There have been very few reports of reoperation for minor drainage of a double connection. A 28-year-old male with double connection type (Ia: major drainage + IIa: minor drainage) mixed total anomalous pulmonary venous connection (TAPVC) was referred to our institution. He had undergone TAPVC type Ia (major drainage) repair at the age of 1 year. Postoperative enhanced computed tomography showed residual TAPVC IIa (minor drainage) at the age of 15 years. Therefore, the definitive diagnosis was double connection-type mixed TAPVC. Residual shunt gradually increased, and cardiac catheterization revealed an increased pulmonary blood flow/systemic blood flow ratio. Echocardiography showed enlarged shunt vessel and mild tricuspid regurgitation. Catheter intervention was considered too risky and, therefore, we performed patch closure of the residual shunt through a right atriotomy. The postoperative course was uneventful, and follow-up computed tomography showed shrinkage of the shunt vessel without any thromboembolic events.

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>

Total Aortic Arch Replacement in a Patient with a Patent Right Internal Thoracic Artery Graft Crossing between the Sternum and the Ascending Aortic Aneurysm / 日本心臓血管外科学会雑誌

We report a case of 76 year-old woman who had previously undergone coronary artery bypass grafting (CABG) with the right internal thoracic artery (RITA) bypassed to the left anterior descending artery. Six years after CABG, she developed acute type A aortic dissection, and she was medically treated because the false lumen was thrombosed and it was considered that surgical intervention would be high risk for the patent RITA graft crossing between the sternum and the ascending aorta. During follow-up, her aortic aneurysm enlarged to 57 mm in diameter, and finally she was referred to our hospital for surgical intervention. In this case, preservation of the patent RITA graft was thought to be critical because the RITA graft was the only blood source for the left anterior descending artery. Prior to re-median sternotomy, we performed a right anterior minithoracotomy to make sufficient space between the sternum and the RITA graft, and then instituted peripheral cardiopulmonary bypass to decompress the heart. After re-sternotomy, we ensured minimum dissection of the RITA graft, and we successfully accomplished graft replacement of the ascending aorta to the aortic arch without injuring the patent RITA graft. In cases with a patent RITA graft and an ascending aortic aneurysm close to the sternum, our strategy is considered to be efficient for re-median sternotomy.

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.

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.

A Survey of the Awareness and Ability of Health Care Providers to Cope with Language Barriers at Medical Facilities in Hyogo, Japan / 国際保健医療

<B>OBJECTIVE:</B><br>This study aims to examine the awareness and ability of health care providers to cope with language barriers at medical facilities in Hyogo Prefecture, Japan, and to clarify the issues concerning health care for foreigners.<br><B>METHODS:</B><br>In total, 2100 copies of self-report questionnaires on issues of health care for foreigners were mailed to health care providers of 352 hospitals in Hyogo. The survey was conducted from February to March 2010.<br><B>RESULTS:</B><br>The response rate of medical facilities was 21.6% (76/352), whereas that of the health care providers was 15.2% (320/2100). Approximately 10% hospitals handled foreign patients at least once per month, and they dealt with patients using several languages, including English, Chinese, Korean, and others. The providers' main issue was communication with their foreign patients. The documents and booklets for guidance regarding some health care procedures at the hospitals were also poorly prepared. It is therefore exceedingly necessary for hospitals to provide common documents in different languages; moreover, the government or local authorities should arrange for public medical interpretation services.<br><B>DISCUSSION:</B><br>Medical facilities in Hyogo have been struggling to improve their linguistic capabilities, and it is difficult to indicate whether public organizations concerned with medical interpretation are being recognized. Efforts on the part of medical facilities and local and national governments, as well as cooperation of nonprofit organizations, are immensely essential to resolve the issue of multilingual health care. This survey suggests that to help people with diverse languages, the health care system in Japan should be improved rapidly, particularly with regard to the establishment of licensed professional medical interpreters.

Improving Outcomes in Right Atrial Isomerism / 日本心臓血管外科学会雑誌

Surgery for right atrial isomerism usually has a poor outcome because of complex congenital cardiac malformations. Here we rearn the outcomes of all 71 consecutive patients with right atrial isomerism whom we treated from their initial operation at Shizuoka Children's Hospital between January 1987 and October 2006. We categorized 34 patients treated between 1987 and 1996 as the ‘early’ group, and 37 patients between 1997 and 2006 as the ‘late’ group. The early group was more commonly associated with pulmonary stenosis (<i>p</i>=0.010), and the late group was more commonly associated with neonatal status (<i>p</i>=0.010), body weight less than 3.0kg (<i>p</i>=0.037), and pulmonary atresia (<i>p</i>=0.013). All 71 patients were scheduled for single ventricular repair. Survival in the early group was 52.9% at 1 year, and 32.4% at 5 years, and this poor outcome was related to 2 factors; cardiac dysfunction from volume loading and inappropriate lung perfusion area for Fontan completion. We therefore changed our surgical strategy in the late group as follows: earlier right heart bypass operation and aggressive atrioventricular valvoplasty to prevent volume overloading, and central pulmonary artery (PA) strategy (central PA plasty and blood flow source anastomosed to the central PA) and improvement of anastomosis of total anomalous pulmonary venous connection (TAPVC) to preserve appropriate lung perfusion area. If severe unbalanced pulmonary blood flow occurred, it was treated with a novel surgical approach consisting of ‘intrapulmonary-artery septation’. Survival in the late group was 66.8% at 1 year, and 53.1% at 5 years, which was better than the early group, but not with statistical significance (<i>p</i>=0.102). Univariate analysis identified significant risk factors for mortality as neonatal status (<i>p</i>=0.036), extracardiac TAPVC (<i>p</i>=0.049), and preoperative pulmonary vein obstruction (PVO) (<i>p</i>=0.001) in the early group, and mixed TAPVC (<i>p</i>=0.001) in the late group. Multivariate analysis identified preoperative PVO (<i>p</i>=0.038) in the early group, and mixed TAPVC (<i>p</i>=0.007) in the late group as significant risk factors for mortality. Outcome is improving with our current strategy of preventing volume overloading and preserving an appropriate lung perfusion area, even in the late group with more severe cases, and neonatal status, extracardiac TAPVC, and preoperative PVO are no longer risk factors for mortality, but mixed type TAPVC remains a serious problem and is associated with high mortality.

A Case of Total Right Heart Bypass Operation for Polysplenia with Diffuse Bilateral Pulmonary Arteriovenous Fistula and Left Pulmonary Vein Obstruction by Anterior Translocation of the Hemiazygos Vein and Direct Anastomosis of the Hemiazygos Vein to the H / 日本心臓血管外科学会雑誌

Diffuse bilateral pulmonary arteriovenous fistula (PAVF) developed in a 19-month-old girl with polysplenia, single atrium, single right ventricle, left superior vena cava and interrupted inferior vena cava (IVC) after total cavopulmonary shunt (TCPS) operation. In addition, left pulmonary vein obstruction (PVO) by compression between the dilated hemiazygos vein and the atrium was identified. We performed total right heart bypass operation with anterior translocation of the hemiazygos vein for release of PVO and direct anastomosis of the hemiazygos vein to the hepatic vein to divert hepatic venous flow to the bilateral pulmonary circulation. Follow-up cardiac catheterization 9 months later showed disappearance of PAVF and no stenotic region. The merits of this operation are: 1) to provide balanced distribution of hepatic venous flow through the hemiazygos vein to the pulmonary circulation, 2) to release the PVO caused by the dilated hemiazygos vein, and 3) to allow for potential growth. This operation can be performed to Fontan candidates with interrupted IVC.