Language：Japanese   Publisher：(NPO)日本心臓血管外科学会  

症例は男児.在胎22週の胎児エコーで左腎嚢胞,先天性心疾患を疑われ母体紹介された.在胎36週5日体重2,282g,Apgar8/8点で出生し,ファロー四徴症,肺動脈弁欠損,肺動脈弁逆流,左上大静脈遺残,右側大動脈弓,cervical archと診断した.生直後に人工呼吸管理となったが日齢9に抜管した.日齢26頃よりSpO2の著明な低下を認め,気管支鏡検査で狭窄所見は軽度であることから,肺血流減少がチアノーゼの主因であると判断した.低体重であり単一冠動脈を認めたため姑息術を先行する方針となったが,cervical archであり通常のブラロック-タウジッヒシャント(BT shunt)は解剖学的に困難のため,日齢49にセントラルシャント(central shunt)+主肺動脈結紮術(main pulmonary artery(mPA)ligation)を行った.チアノーゼは改善し呼吸状態は安定していたが,造影computed tomography(CT)で左肺動脈拡大傾向と左主気管支の圧排所見を認め,気道症状増悪が予想されたため日齢87にRastelli型手術+両側肺動脈縫縮術を行った.術後呼吸状態は安定し術後10日に抜管した.日齢136に在宅高流量鼻カニューレを導入した上で自宅退院した.(著者抄録)

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本心臓血管外科学会  

Ebstein病の重症度はさまざまであるが,胎生期および生直後に発症する重症例においては循環維持が困難な危機的な状態となり,生直後に外科的介入が必要となることも多い.今回,肺動脈弁逆流を伴いcircular shuntの状態となった新生児Ebstein病の2例に対し,主肺動脈結紮術などを先行したRapid two-stage approachによるStarnes手術変法を施行した.(1)症例1.在胎39週に吸引分娩にて体重3,012gで出生.7生日に両側肺動脈絞扼術,主肺動脈結紮術,右房縫縮術を施行するも,術後に乳酸アシドーシスの進行を認めたため8生日に緊急Starnes手術を施行した.(2)症例2.胎児水腫を認めたため在胎37週に緊急帝王切開にて体重2,528gで出生.0生日に主肺動脈結紮術を施行.術後も循環動態は改善せずアシドーシスの進行を認めたため,1生日に緊急Starnes手術を施行した.どちらも良好な経過が得られたため報告する.(著者抄録)

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：日本胸部外科学会-関東甲信越地方会  

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Language：Japanese   Publisher：(一社)日本成人先天性心疾患学会  

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Language：Japanese   Publisher：日本胸部外科学会-関東甲信越地方会  

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Language：Japanese   Publisher：(一社)日本胸部外科学会  

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Language：Japanese   Publisher：(一社)日本胸部外科学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：日本胸部外科学会-関東甲信越地方会  

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OBJECTIVES: Complete atrioventricular septal defect with tetralogy of Fallot is a rare and complex heart disease. This study aimed to describe contemporary management approaches for this heart disease and the outcomes. METHODS: Data were obtained from 46 domestic institutions in the Japan Cardiovascular Database (2011-2018). Patients with a fundamental diagnosis of complete atrioventricular septal defect with tetralogy of Fallot, without other complex heart diseases, were included. The primary outcome was operative mortality (30-day or in-hospital mortality). RESULTS: A total of 119 patients underwent initial surgery for a complete atrioventricular septal defect with tetralogy of Fallot during this study period. Primary repair was performed in 40 (34%) patients (primary repair group), and palliative procedure was performed in 79 (66%) patients as part of a planned staged approach (staged group). Forty institutions (87%) experienced at least one case of staged repair. No institution experienced more than or equal to two cases/year on average during the study period. Overall, 11 operative mortalities occurred (9.2%). Operative mortality rates in the primary and staged groups were comparable (p = 0.5). Preoperative catecholamine use, repeat palliative surgeries, and emergency admission were significant risk factors for operative mortality in multivariate analysis (odds ratio, 95% confidence interval: 8.58, [0-0.11]; 12.65, [1.28-125.15]; 8.64, [1.87-39.32, respectively]). CONCLUSIONS: Staged approach for complete atrioventricular septal defect with tetralogy of Fallot was the preferred option. The outcomes of this complex disease were favorable for patients in centers with low cases of complete atrioventricular septal defect with tetralogy of Fallot.

DOI： 10.1007/s11748-022-01809-3

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Language：Japanese   Publisher：(NPO)日本心臓血管外科学会  

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Language：Japanese   Publisher：(一社)日本成人先天性心疾患学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

肺静脈閉塞を合併したFontan術後患者に対する有効な治療法はなく予後は不良である。今回我々はFontan術後に右肺静脈閉塞を来し、多量の胸水、浮腫、心房性不整脈が出現した急性心不全の3歳女児例に対して体肺側副血管と右肺動脈の塞栓後に開窓作成術を行った。体肺側副血管の塞栓は3回のセッションに分けて51本のコイルを使用した。右肺動脈塞栓は計5個のAmplatzer Vascular Plug(AVP)と計2本のコイルを用いて、手技時間は3時間程度で合併症なく完全閉塞できた。その後に開窓作成術を行い、中心静脈圧は閉塞術前の20mmHgから7mmHgへ低下した。手技に伴う合併症はなく、手技時間も短く施行し得た。しかし術後9日目に心タンポナーデを発症し死亡した。過去に肺静脈閉塞を合併したFontan術後症例に対して体肺側副血管と患側の肺動脈を完全に塞栓した報告はない。本治療は一時的に肺静脈閉塞を伴うFontan患者の循環動態を安定させた。(著者抄録)

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Systemic intravenous administration of opioids is the main treatment strategy for intraoperative and postoperative pain management in patients undergoing cardiac surgery with sternotomy. However, using lower doses of opioids may achieve the well-established benefits of the fast-track approach, with minimal opioid-related side effects. Postoperative pain is coupled with a long stay in the intensive care unit. Although neuraxial anesthesia has some benefits, its use remains controversial due to the potential development of epidural hematoma after anticoagulation for cardiopulmonary bypass and coagulopathy after cardiac surgery. Therefore, there is a need for other effective postoperative analgesic strategies, such as peripheral nerve blocks other than neuraxial anesthesia, for cardiac surgery with sternotomy. The effects of real-time ultrasound-guided transverse thoracic muscle plane (TTP) block on postoperative pain after sternotomy have been reported; however, the pain and discomfort in the epigastric area caused by chest drainage tubes placed through the rectus abdominis muscle also are major postoperative problems after cardiac surgery. Herein, the authors report on a preoperative combination of TTP block and rectus sheath block (RSB) for postoperative pain management after cardiac surgery with sternotomy that addresses pain in both the chest and epigastric areas. Considering previous studies, it is presumed that preemptive analgesic effects can be expected via a combination of the TTP block and RSB, and indeed, the preemptive effect was observed in the present study's patients. In this article, the procedure and tips for combining the TTP block and RSB are introduced.

DOI： 10.1053/j.jvca.2020.07.041

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(一社)日本胸部外科学会  

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Language：Japanese   Publisher：(NPO)日本心臓血管外科学会  

症例は37歳男性。三尖弁閉鎖の診断で左Blalock Taussigシャント手術原法、Glenn手術原法、左肺動脈形成術の既往あり。10歳時にBjoerk手術を施行したが、術後に循環不全となり心肺補助装置を装着された。上下大静脈間の著明な圧較差を認めたため、人工血管による右房・無名静脈バイパス術を追加し心肺補助装置を離脱し得た。術後他院にて経過観察されていたが、30歳頃より心房頻拍が出現、36歳時より蛋白漏出性胃腸症を発症した。内科的治療でも改善を認めないため当院再診した。心臓カテーテル検査にて右房・無名静脈間の人工血管の開存が確認され、左肺動脈が低形成のため下大静脈血流は一部が人工血管経由に無名静脈から右肺動脈へ還流しており、上下大静脈間の圧較差を認めた。手術は人工心肺・心停止下に右房アブレーション、心外導管を用いたTCPC転換術、大動脈前方での左肺動脈再建、右房-心外導管間のfenestration作成および心外膜ペースメーカーリード装着術を行った。術後34日に退院した。蛋白漏出性胃腸症は軽快し3年経過した現在も再発は認めていない。(著者抄録)

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The choice of graft material for reconstruction of the vena cava in pediatric patients remains controversial. We successfully treated an eight-month-old female patient with single ventricle physiology and long segment obstruction of the left superior vena cava using the right superior vena cava autograft at the time of bilateral bidirectional superior cavopulmonary anastomosis. Postoperative computed tomography confirmed the patency of the reconstruction.

DOI： 10.1177/2150135117706951

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BACKGROUND: In a Fontan candidate, univentricular pacing may cause delay in interventricular conduction, which induces asynchronous contraction. Cardiac resynchronization therapy is expected to be an effective mode of therapy in such a case. CASE PRESENTATION: A 7-month-old girl, diagnosed with dextrocardia, congenitally corrected transposition of the great artery [situs solitus, L-loop, and L-transposition], ventricular septal defect, infundibular and pulmonary valvular stenosis, and straddling of the tricuspid valve, was considered as a candidate for the Fontan procedure. She had undergone Blalock-Taussig shunt, and epicardial univentricular pacemaker implantation for persistent complete atrioventricular block. She underwent a bidirectional cavopulmonary shunt concomitant with ventricular lead translocation from the morphological left ventricle to the morphological right ventricle. After discharge, ventricular dyssynchrony was noted and cardiac failure persisted. She was converted to cardiac resynchronization therapy (CRT) at 13 months of age. Two-dimensional speckle tracking imaging was used by cardiologists to determine the most suitable pacing site. CRT rapidly corrected the heart failure; thus, she underwent the Fontan procedure after 1.5 years. Five years have passed since the cardiac resynchronization therapy; her interventricular synchrony is maintained well and the level of brain natriuretic peptide remains within normal range. CONCLUSION: We describe the successful conversion from single ventricular pacing to CRT, in a case of congenitally corrected transposition of the great artery indicated for the Fontan procedure. The long-term prognosis of cardiac resynchronization therapy is undetermined in the pediatric population; therefore, further follow-up is required.

DOI： 10.1186/s40792-020-00863-4

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Language：Japanese   Publisher：(株)日本臨床社  

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Language：Japanese   Publisher：(株)日本臨床社  

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Language：Japanese   Publisher：(NPO)日本心臓血管外科学会  

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Language：Japanese   Publisher：日本胸部外科学会-関東甲信越地方会  

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Language：Japanese   Publisher：(NPO)日本心臓血管外科学会  

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Language：Japanese   Publisher：(一社)日本成人先天性心疾患学会  

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A right-sided descending aorta with a left aortic arch is a rare congenital anomaly in which the aortic arch crosses the midline from the left side of the trachea coursing to the descending aorta in the right thoracic cavity. The surgical repair of an interrupted aortic arch with a right-sided descending aorta carries great risks of bronchial and oesophageal obstruction. Herein, we describe a case of successful surgical repair of an interrupted aortic arch with a right-sided descending aorta using the swing-back and trap-door techniques.

DOI： 10.1093/icvts/ivz175

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A 46-year-old man who had undergone atrial septal defect closure during childhood was referred for surgery for residual partial anomalous pulmonary venous connection. The anomalous pulmonary veins were connected to the higher (cephalad) segment of the superior vena cava. As the usual caval division technique was not applicable, we chose to utilize the anterior wall of the superior vena cava as a flap for anomalous pulmonary vein rerouting. Bovine pericardium was used to reconstruct the systemic venous pathway. Systemic or pulmonary venous stenosis was not detected. The patient was discharged with a sinus rhythm.

DOI： 10.1177/2150135119862596

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Language：Japanese   Publisher：日本胸部外科学会-関東甲信越地方会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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OBJECTIVES: The main goal of palliative procedures for congenital heart defects is adequate pulmonary blood flow (PBF), but precise intraoperative PBF evaluation is sometimes difficult. The purpose of this preliminary study was to investigate the usefulness of velocity time integral of the pulmonary vein (PV-VTI) measured by transoesophageal echocardiography (TOE) at the time of palliative procedure as a parameter for PBF. METHODS: Case histories of 63 patients who underwent palliative procedures (bilateral pulmonary artery banding in 18 patients, main pulmonary artery banding in 22 patients and systemic-to-pulmonary artery shunt in 23 patients) and whose intraoperative PV-VTI was measured by TOE from 2011 to 2017 at our centre were retrospectively reviewed. Low-body-weight infants, cases in which cardiopulmonary bypass was used and cases that were anatomically difficult to measure were excluded. RESULTS: PV-VTIs measured at 4 orifices of the pulmonary veins were all significantly decreased in both the bilateral pulmonary artery banding and main pulmonary artery banding groups and increased in the systemic-to-pulmonary artery shunt group immediately after the procedure. There were significant correlations between the velocity time integrals of both right and left pulmonary veins and arterial oxygen saturation (r = 0.564 and 0.703). Nine patients (6 bilateral pulmonary artery banding and 3 systemic-to-pulmonary artery shunt) required unplanned early reoperation due to inadequate PBF; their PV-VTIs were significantly different from those of patients not requiring reoperation. No major complications related to TOE occurred postoperatively. CONCLUSIONS: The PV-VTI measured by TOE during palliative procedures reflected the change of PBF and could help identify patients at higher risk of early reoperation due to inadequate PBF. This parameter may be a useful additional tool for evaluating intraoperative PBF.

DOI： 10.1093/ejcts/ezy465

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Language：English   Publisher：(一社)日本成人先天性心疾患学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：日本胸部外科学会-関東甲信越地方会  

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Background: To determine potential predictors of ventricular tachyarrhythmia and sudden cardiac death (SCD) occurring late after repair of tetralogy of Fallot (TOF). Methods: Since 1964, 415 patients had undergone total repair for TOF at Niigata University Hospital. Of these, 89 patients who were followed for more than 10 years at our institute were retrospectively reviewed. Results: The mean follow-up period was 24.3 years. During the study period, one patient died of cerebral bleeding, and two patients had SCD. The overall survival rates at 20, 30, and 40 years were 100%, 94.6%, and 94.6%, respectively. Eight (9.0%) patients required re-intervention during the late period associated with right ventricular outflow (n=4), tricuspid valve (n=3), aortic valve (n=2), and others (n=2). Ten (11.2%) patients had a history of ventricular tachycardia (VT) or ventricular fibrillation (VF), and six underwent implantation of an implantable cardiac defibrillator. Multivariate analysis selected the change rate of QRS duration [ms/year; odds ratio (OR), 2.44; 95% confidence interval (CI): 1.28-4.65; P=0.007] and the pressure gradient at tricuspid valve regurgitation on echocardiography (OR, 1.12; 95% CI: 1.02-1.22; P=0.017) as risk factors for VT/VF or SCD. Trans-annular patch (TAP) repair was not an independent risk factor for ventricular arrhythmia. Conclusions: The combination of rapid change rate of QRS duration and higher-pressure gradient at tricuspid regurgitation were risk factors for ventricular tachyarrhythmia late after TOF repair. Adequate surgical or catheter intervention for pressure and volume load in the right ventricle might decrease the prevalence of VT/VF and SCD.

DOI： 10.21037/jtd.2017.11.53

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Language：Japanese   Publisher：日本胸部外科学会-関東甲信越地方会  

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Language：Japanese   Publisher：日本胸部外科学会-関東甲信越地方会  

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We describe a case of complex left ventricular outflow tract obstruction, multiple muscular ventricular septal defects, aortic coarctation and a hypoplastic aortic arch, where staged biventricular repair was performed successfully using pulmonary root translocation.

DOI： 10.1093/icvts/ivw294

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Language：Japanese   Publisher：(一社)日本成人先天性心疾患学会  

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BACKGROUND: Non-bacterial thrombotic endocarditis (NBTE) is an uncommon pathological situation, which involves the presence of bland, fibrin-platelet thrombi. It usually occurs at the endocardium of cardiac valves, in association with endothelial injury and a hypercoagulative state. However, NBTE on the endocardium at the right atrial free wall in a patient without any apparent hypercoagulative background is rarely reported. CASE PRESENTATION: A girl aged 4 years with severe pectus excavatum was referred to our hospital for treatment of a recurrent right atrial tumor. The tumor was removed concomitant with pectus excavatum repair. The tumor was revealed as recurrent thrombus. Pathological findings showed that NBTE caused by an operative scar on the endocardium of the right atrium and sustained rheological stress in the right atrium due to compression from pectus excavatum lead to recurrent thrombus formation. Three years after the discontinuation of anticoagulation therapy, no sign of thrombus formation was found. CONCLUSIONS: To our knowledge, this is the first report of NBTE related to an interaction between sustained rheological stress from cardiac compression and endocardial injury. In such patients, we recommend concomitant chest wall repair when the operative scar is present at the site of the rheological force.

DOI： 10.1186/s40792-016-0236-4

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Atresia of the left coronary artery ostium is extremely rare. We report the case of a 13-year-old boy who played volleyball in school and collapsed with severe chest pain during practice. He was referred to our hospital, and imaging modalities showed atresia of the left main coronary artery ostium. Urgent coronary ostioplasty was performed using a patch of 0.6% glutaraldehyde-treated autologous pericardium. His postoperative course was uneventful, and he has had a normal everyday life without chest pain 8 months postoperatively. Physicians should be aware of the patient's history, as in this case, because prompt imaging diagnosis is essential when there is a high likelihood that the event is related to myocardial ischemia. Since long-term outcome is uncertain even after successful surgical revascularization, close follow-up is required.

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A 15-day-old boy after intracardiac repair was discharged from the intensive care unit with a low-flow nasal cannula for oxygen administration. The cannula was a 4-Fr multi-purpose tube with a side hole that was inserted into his left nostril. Next day, he suddenly developed pneumocephalus emerging from the right periorbital swelling and extending to his face and subcutaneous scalp over the next 6 h. A computed tomography (CT) scan revealed massive air pockets in the orbit, subdural space, subcutaneous scalp, and face. The nasal cannula was found to have been inserted deeper than we thought and was thus presumed to be the source of the air pockets. We immediately removed the cannula. Follow-up CTs revealed rapid resolution of the intracranial and subcutaneous air. The subcutaneous emphysema completely disappeared over the next 4 days, and he was discharged without any incident.

DOI： 10.1007/s11748-014-0454-2

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A neonate with transposition of the great arteries and intact ventricular septum presented without pulmonary over-circulation, and subsequently developed pulmonary haemorrhage after corrective surgery. Postoperative CT revealed an aortopulmonary collateral artery arising from the descending aorta, and we performed successful embolisation on postoperative day 9. Aggressive imaging modalities such as angiography and/or CT imaging with contrast can detect unexpected extra-pulmonary blood supply and guide further management.

DOI： 10.1017/S1047951115001328

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Language：Japanese   Publisher：日本胸部外科学会-関東甲信越地方会  

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Language：Japanese   Publisher：(NPO)日本心臓血管外科学会  

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Language：Japanese   Publisher：(NPO)日本心臓血管外科学会  

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Language：Japanese   Publisher：(NPO)日本心臓血管外科学会  

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Language：Japanese   Publisher：(一社)日本成人先天性心疾患学会  

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Language：Japanese   Publisher：日本胸部外科学会-関東甲信越地方会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：日本胸部外科学会-関東甲信越地方会  

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Language：Japanese   Publisher：日本胸部外科学会-関東甲信越地方会  

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Language：Japanese   Publisher：(NPO)日本心臓血管外科学会  

症例は男児。在胎38週3日、体重2,880gで仮死なく出生し、生直後より高度のチアノーゼを認め心エコー検査にて両大血管右室起始(DORV)と診断され同日当院NICUへ緊急搬送された。大血管関係は大動脈がやや右後方、肺動脈がやや左前方であり、心室中隔欠損は肺動脈弁下に存在するposterior TGA型DORVであった。漏斗部中隔は三尖弁側の心室漏斗部皺襞(VIF)側に挿入し、心室中隔にほぼ整列していた。手術は日齢25に胸骨正中切開・体外循環下に行った。心停止下に三尖弁経由に心室中隔欠損(secondary IVF)を閉鎖(心室内血流路作成)し、次に動脈スイッチ手術を行った。冠動脈パターンは1R2LCXのShaher 9型であり、trap-door法を用いた冠動脈移植を行った。肺動脈再建は前方転位を行わないJatene原法を行った。術後血行動態は早期から安定し、術後2日に人工呼吸器離脱、術後19日に退院した。(著者抄録)

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Other Link： https://search.jamas.or.jp/index.php?module=Default&action=Link&pub_year=2015&ichushi_jid=J01122&link_issn=&doc_id=20150128260005&doc_link_id=10.4326%2Fjjcvs.44.21&url=https%3A%2F%2Fdoi.org%2F10.4326%2Fjjcvs.44.21&type=J-STAGE&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00007_3.gif

Language：Japanese   Publisher：(NPO)日本心臓血管外科学会  

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OBJECTIVES: Bicuspid aortic valve (BAV) is a common risk factor for valve-related problems and occurs more frequently in patients with an interrupted aortic arch (IAA) or coarctation of the aorta (CoA), combined with a ventricular septal defect (VSD), than in the general population. We have been using conventional repair for patients with IAA/CoA+VSD, including those with a very small aortic valve (AV). We retrospectively investigated the outcomes of these patients from the perspective of valve morphology. METHODS: Between 2000 and 2012, 50 consecutive patients underwent conventional repair for CoA/IAA with VSD [one-stage repair, 44 (88%); staged repair, 6 (12%)]. The criteria for conventional repair were as follows: an AV annulus diameter (AVD) z-score of >-6.0; mitral valve annulus diameter z-score of >-3.0; without retrograde flow in the proximal arch. Sixteen (32%) patients had BAV (Group B); the remaining 34 (68%) patients had a tricuspid AV (Group T). The surgical outcomes in both groups were investigated. RESULTS: No mortality occurred in the cohort. The median follow-up times were 6 years and 3 months (6 months to 11 years and 8 months) and 6 years and 2 months (4 months to 11 years and 4 months) in Groups B and T, respectively (P > 0.05). The preoperative data (median age at repair, median body weight and median AVD) were comparable in the two groups (P > 0.05). Two patients (4%) underwent reintervention in the aortic arch: 1 patient underwent balloon angioplasty for re-coarctation; the other removal of the interposed graft because of somatic growth. In both groups, the AVD became significantly larger at the 1-year follow-up, approximating the normal value. Three (6%) patients underwent a total of eight valve-related reinterventions (balloon angioplasty, 6; Ross operation, 1; valve replacement, 1). All three had BAV, and the AVD was 3.8-5.6 mm (z-score, -3.4 to -1.6). The 5-year valve-related reintervention-free survival rate was 76% and 100% in Groups B and T, respectively (P < 0.01). CONCLUSIONS: The long-term outcomes after conventional repair under our criteria were acceptable. BAV was a significant risk factor for valve-related reinterventions after conventional repair for IAA/CoA with VSD.

DOI： 10.1093/ejcts/ezu078

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Language：Japanese   Publisher：日本胸部外科学会-関東甲信越地方会  

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OBJECTIVE: We adopted a policy of rapid-staged bilateral pulmonary artery banding (bPAB) before the Norwood (NW) procedure for all patients with hypoplastic left heart syndrome. We hypothesized that this strategy might mitigate some of the traditional risk factors and that postponing a major bypass procedure beyond the newborn period could have both short- and long-term benefits. The purpose of the present study was to evaluate the efficacy of this strategy with respect to the short-term outcomes. METHODS: From 2008 to 2010, 14 patients underwent bPAB and maintenance of ductal patency with prostaglandin E1 infusion before stage 1 NW. For reference, we also reviewed the data from patients who had undergone the primary NW procedure in the 2 years immediately before the study period. RESULTS: The bPAB was performed at a median age of 6 days (range, 2-39), gestational age of 38.5 weeks (range, 36-41), and weight of 2.75 kg (range, 2.3-3.6). The subsequent NW was performed at a gestational age of 43.5 weeks (range, 41-51) and weight of 3.2 kg (range, 2.2-4.9). When the NW procedure was eventually performed on the pBAB group, the maximum blood lactate levels within the first 24 hours after the NW were lower than those in the earlier primary NW group (2.8±0.9 vs 10.1±6.5 mmol/dL, P=.0002) and the urine output in the first 24 hours after the NW was greater in the pPAB group (4.1±2.1 vs 2.2±1.5 mL/kg/h; P=.0051). CONCLUSIONS: These data suggest that rapid-staged bPAB before NW can reduce the challenge of postoperative management in the early postoperative period after the NW procedure and have potential to improve the outcomes.

DOI： 10.1016/j.jtcvs.2013.11.053

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Language：Japanese   Publisher：(NPO)日本心臓血管外科学会  

症例は男児。在胎40週0日、体重3,465gで仮死なく出生した。生直後より高度のチアノーゼを認め心エコー検査にて両大血管右室起始・大動脈弓離断症と診断された。同日よりプロスタグランディン製剤の持続静注が開始され、当院NICUへ緊急搬送された。高肺血流に伴う急性心不全状態であったため、4生日に両側肺動脈絞扼術を施行した。術後に利尿が得られ全身状態は改善したが心不全状態が継続したため、9生日時に根治手術を行った。胸骨再正中切開下に上下半身分離体外循環を確立し、三尖弁経由にVSDから心室内血流路を作成し、次にtrap door法を用いて冠動脈移植を行った。Lecompte maneuverの後に大動脈弓再建(直接吻合)を行った。さらに右室流出路をパッチで拡大し、大動脈遮断解除後に肺動脈再建を行った。人工心肺離脱はとくに問題なく、開胸状態で手術を終了し4病日に閉胸し14病日に人工呼吸器を離脱した。術後に肺炎・乳び胸などを合併したが内科的治療にて改善し、78病日に退院した。(著者抄録)

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We introduce a simple, less invasive surgical technique for treating neoaortic valve regurgitation (neoAR) after the Norwood procedure, with the aim of delaying reoperation for neoAR. A 31-month-old girl, with hypoplastic left heart syndrome, previously underwent 4 median sternotomies and was admitted to our hospital for a fenestrated Fontan operation. She presented with moderate neoAR, originating from a tricuspid neoaortic valve (neoAV), with the regurgitation oriented from the centre. Her neoAV annulus was dilated to twice its normal size. With the aim of delaying future neoAV intervention and minimizing the surgical invasiveness, we performed extra-aortic commissuroplasties on the 2 commissures that could be approached from the front during the Fontan operation, without inducing cardiac arrest. We used direct echocardiography and transoesophageal echocardiography to confirm the feasibility before applying this procedure. Her postoperative course was uneventful, and the postoperative echocardiography did not reveal any residual neoAR 5 months postoperatively. We believe that this technique is a useful surgical option for patients with moderate neoAR oriented from its centre and well-balanced tricuspid native pulmonary valves, and it might help to delay future neoAV interventions, with minimal surgical risk.

DOI： 10.1093/icvts/ivu130

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：日本胸部外科学会-関東甲信越地方会  

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A 1-year-old boy weighing 10.4 kg underwent successful biventricular repair for transposition of the great arteries, a ventricular septal defect, and a left ventricular outflow tract (LVOT) obstruction with moderate pulmonary stenosis of the bicuspid pulmonary valve (z score of -4.4 for the pulmonary valve) by means of a modified Nikaidoh procedure with double root translocation by use of a valve-spared pulmonary root. The postoperative echocardiogram showed no LVOT obstruction, no aortic valve regurgitation, and mild pulmonary stenosis and pulmonary valve regurgitation. No reintervention has been required during the 6-year follow-up, with annular growth of the pulmonary valve.

DOI： 10.1016/j.athoracsur.2013.04.141

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Language：Japanese   Publisher：日本胸部外科学会-関東甲信越地方会  

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Language：Japanese   Publisher：(一社)日本外科学会  

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Language：Japanese   Publisher：(NPO)日本心臓血管外科学会  

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Language：Japanese   Publisher：(一社)日本成人先天性心疾患学会  

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Language：Japanese   Publisher：(NPO)日本心臓血管外科学会  

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Language：Japanese   Publisher：日本胸部外科学会-関東甲信越地方会  

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OBJECTIVES: As the surgical results for Fontan candidates improve, much attention has been given to the long-term cognitive outcomes. This study aimed to assess the neurocognitive outcomes after Fontan completion, and to determine the factors associated with an extremely low intelligence quotient (IQ <70). METHODS: A total of 70 local eligible school-aged patients were enrolled in the study (hypoplastic left heart syndrome, 17; right atrial isomerism, 15 and others, 38). Fontan operations were performed at a median age of 1.8 (range, 0.5-8.9) years (primary Fontan, 4 of 70 [6%]). The Wechsler Intelligence Test was taken at a median age of 9 (range, 5.1-14.4) years for the purpose of neurocognitive evaluation. Patients' data were collected from medical records, and a retrospective analysis of potential predictors for an IQ <70 was performed. RESULTS: The median full-scale IQ (FSIQ) for the entire cohort was 85 (range, 43-118). Of the lower order composites, the perceptual reasoning index and the working memory index were significantly lower in low-FSIQ patients (P < 0.05). Overall, 15 of 70 (21%) of the cohort had an IQ <70. Univariate analysis identified three significant risk factors for FSIQ <70 (15 of 70, 21%): body weight <2.5 kg at initial palliative surgery (P < 0.05), low 5-min Apgar score <4 (P < 0.05) and inter-stage events requiring cardiopulmonary resuscitation (CPR) (P < 0.05). No other patient-specific factors (e.g. cardiac morphology) or modifiable surgical factors (e.g. the use of hypothermic cardiac arrest) were associated with FSIQ <70. CONCLUSIONS: Low body weight (<2.5 kg) at initial operation, low 5-min Apgar score (<4) and inter-stage CPR were significant risk factors for impaired neurocognitive outcomes. An evolving strategy for preventing inter-stage CPR may improve cognitive outcomes.

DOI： 10.1093/ejcts/ezt062

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OBJECTIVES: Although pulmonary valve-sparing repair is preferable for patients with tetralogy of Fallot, the repair of very small pulmonary valves is challenging. The present study evaluates our modification for preserving severely hypoplastic pulmonary valves in patients with tetralogy of Fallot. METHODS: Sixty-eight consecutive patients who underwent complete repair of a tetralogy of Fallot between 2005 and 2011 were retrospectively reviewed. Patients with pulmonary atresia, absence of a pulmonary valve, atrioventricular septal defect and/or subarterial ventricular septal defect were excluded. There were 19 (28%) patients with a severely hypoplastic pulmonary annulus determined by preoperative echocardiography (z-score <-4). For these patients, we collected echocardiographic data and information about their postoperative course. RESULTS: Valve preserving was successful in 11 of 19 (58%) of the z < -4 group, compared with 48 of 49 (98%) of the z > -4 group. In the z < -4 valve-sparing subgroup (n = 11), the preoperative pulmonary valve diameter z-score was -4.9 (range -6.3 to -4.3), and an approach involving ventriculotomy with no transannular patch was employed at a mean age of 6.9 (range 2.2-16.1) months. In this subgroup, residual right ventricular outflow tract velocity was 2.4 ± 0.6 m/s at discharge from the hospital. During a mean follow-up of 2.6 ± 2.4 years, no reintervention was necessary. Late right ventricular outflow tract velocity was 2.2 ± 0.6 m/s, and there was no severe pulmonary regurgitation. The pulmonary valve annulus grew in relation to the patient's body surface area (z = -0.51, range -4.2-0.24) without any aneurysmal changes in the right ventricular outflow tract. CONCLUSIONS: Although our modification of valve-sparing repair for severely hypoplastic pulmonary valves in patients with tetralogy of Fallot could not be applied in all patients, this strategy enabled acceptable growth of the valve annulus, with only mild stenosis during the early to mid-term follow-up. This modification seems to be an option, even for a very small pulmonary valve.

DOI： 10.1093/icvts/ivt095

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BACKGROUND: Although the Fontan procedure is now being performed in younger patients, the influence of the early timing of Fontan on midterm to long-term results remains unclear. We investigated whether the timing of Fontan completion affects subsequent hemodynamics in patients with functional single ventricle followed for more than 3 years. METHODS: Between January 1997 and December 2008, 163 patients with functional single ventricle underwent extracardiac total cavopulmonary connection (TCPC) at a single institution. The survivors routinely underwent postoperative catheterization at 1 year and then every 5 years after TCPC and were divided into group A (weight < 10 kg; n = 65) and group B (weight > 10 kg; n = 97), and retrospectively reviewed. RESULTS: Mean follow-up was 6.6 ± 3.7 years. Mean weight and conduit size were 8.5 ± 0.8 kg and 17.1 ± 1.2 mm for group A versus 20.2 ± 13.1 kg and 18.8 ± 1.9 mm for group B, respectively (p < 0.001). There were 3 hospital deaths (group A, n = 1; group B, n = 2) and 5 late deaths (group A, n = 3; group B, n = 2). No TCPC was taken down. There were no thromboembolic events in either group. The end-diastolic volume of ventricle (eDV) (% of normal) was (232.7 ± 91.4 before TCPC versus 139.3 ± 57.2 5 years after TCPC; p < 0.001) in group A and (209.6 ± 77.7 before TCPC versus 136.7 ± 61.4 5 years after TCPC; p < 0.001) in group B. Ventricular ejection fraction and cardiac index at 5 years were similar in both groups. The end-diastolic pressure of ventricle (eDP) (mm Hg) at 1 year (p = 0.0037) and at 5 years (p = 0.047) was significantly lower in group A compared with group B. CONCLUSIONS: TCPC can be performed earlier with good intermediate results. Earlier unloading of a univentricular heart by means of TCPC might be advantageous for preservation of future ventricular function.

DOI： 10.1016/j.athoracsur.2012.06.022

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OBJECTIVES: Platelet dysfunction is a major cause of bleeding complications in patients undergoing cardiovascular surgery under cardiopulmonary bypass (CPB). Thromboelastography (TEG) can be used to assess post-CPB coagulopathy, but its utility in guiding platelet transfusion (PT) after CPB is unclear. This study assessed the utility of a TEG-guided PT protocol in patients undergoing cardiovascular surgery under CPB. METHODS: The platelet count and TEG maximum amplitude (MA) was measured in 100 patients undergoing valvular or thoracic aortic surgery under CPB. PTs were guided by an empiric protocol in 50 patients (group C) and by a TEG-guided protocol (MA <35 mm, platelet count <7 × 10(4)/mm(3)) in the other 50 patients (group T). RESULTS: PT was utilized significantly less in group T (11 patients; 22%) than in group C (24 patients; 48%) (P < 0.01). The difference in PT utilization was particularly marked in patients undergoing aortic arch aneurysm repair (20% in group T vs. 100% in group C; P < 0.01), yet there was no difference in bleeding complications between these two groups. CONCLUSIONS: Use of a TEG-guided transfusion protocol dramatically reduced PT after CPB, particularly in patients undergoing aortic arch aneurysm repair.

DOI： 10.1007/s11748-012-0070-y

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BACKGROUND: Patients with right atrial isomerism (RAI) have several cardiac malformations historically resulting in significant morbidity and mortality. We sought to assess whether current surgical strategies have improved the outcomes of patients with RAI. METHODS: A retrospective review of our database from 1997 to 2010 identified 60 consecutive patients with RAI who underwent initial palliation at Mt. Fuji Shizuoka Children's Hospital. All of the patients had a functional single ventricle. Of the 60 patients, 33 patients (51.7%) had obstructed total anomalous pulmonary venous drainage (TAPVD), 23 patients (40.3%) had significant (more than moderate) atrioventricular valvular regurgitation (AVVR), 7 patients (12.3%) had hiatus hernia, and 4 patients (6.7%) had major aortopulmonary collateral arteries. Pulmonary outflow atresia was present in 39 of the patients (65%), and 46 patients had systemic-to-pulmonary artery shunts. Of the 33 patients with TAPVD, 18 underwent repair of the condition at initial palliation, 9 others at the time of a Glenn operation, 2 more through a Fontan operation, and 4 at the interstage between palliative surgeries. Eight of the 23 patients with significant AVVR underwent atrioventricular valve repair at initial palliation, 8 others at the time of Glenn operation, 4 others at the time of Fontan operation, and 3 during the interstage between operations. An initial neonatal surgical procedure was performed in 30 patients (50%). The mean follow-up period for patients in the study was 53.1 months. RESULTS: Operative mortality after initial palliation was 15.4% (4 of 26 patients) before 2003 and 17.6% (6 of 34 patients) after 2004 (p=NS). Five-year survival was 53.8% before 2003 and 81.7% after 2004 (p=0.035, log-rank test). A multivariate analysis identified persistent AVVR of more than moderate degree (p=0.04) as a factor associated with late mortality. CONCLUSIONS: The outcomes of surgery for RAI are improving. Neonatal palliative surgery for RAI carries a high operative risk of early mortality, and persistent significant AVVR remains a risk factor for late mortality.

DOI： 10.1016/j.athoracsur.2011.05.110

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In this article, we report a rare case of rectal obstruction caused by bilateral internal iliac artery aneurysms that required open surgical repair. A 73-year-old man was admitted to our hospital complaining of abdominal pain and persistent constipation for >1 month. Computed tomography demonstrated bilateral internal iliac artery aneurysms, 5.0 and 7.0 cm each in diameter, which occupied the intrapelvic space. An urgent surgery was performed to reduce the volume of the aneurysms and release the obstructed rectum. The postoperative course was uneventful, in which he had good evacuation. Aneurysms in the iliac region can be a good indication for the use of newly developed endovascular devices; however, open surgery should be considered without delay to avoid ileus or subileus symptoms when the aneurysms cause space-occupying complications.

DOI： 10.1016/j.avsg.2010.07.028

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A 70-year-old woman without any previous history of heart disease was referred to our hospital for repeated chest discomfort. She had experienced temporary hemiparesis because of a cerebral infarction of unknown etiology. Clinical evaluations were all within normal limits except for echocardiography. There was a mobile tumor in her ascending aorta, about 15 mm in diameter, adhering to a part of the right coronary cusp on its aortic side. Urgent surgery was performed. The tumor was attached to the edge of the right coronary cusp and excised with a part of the right coronary cusp, which was then repaired. Histological examination revealed the tumor to be a papillary fibroelastoma. The chest discomfort responsible for admission disappeared after the operation. In patients with events that may be embolic in nature and are not explained by other cardiovascular or neurological diseases, a cardiac source of emboli should be considered.

DOI： 10.1007/s11748-010-0585-z

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Language：Japanese   Publishing type：Research paper (scientific journal)  

Vacuum-assisted closure (VAC) therapy is an efficacious modality for treating chronic and difficult wounds. We present 3 cases that responded well to VAC therapy after cardiovascular and thoracic surgery: 1 methicillin-resistant Staphylococcus aureus (MRSA) wound infection after Stony's incision, 1 inguinal lymphorrhea, and 1 empyema after a traffic accident The duration of VAC therapy was 9, 18, and 90 days, respectively, and all 3 wounds healed completely. Familiar equipment and supplies available on the hospital ward were used, and patients were able to leave their beds. In this report, the efficacy of VAC therapy, the problems encountered, and the steps that could be taken to address them are discussed.

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A 6-day-old girl underwent pacemaker implantation for complete atrioventricular block through a subxiphoid incision. Epicardial leads were sutured on the right ventricle, and the pulse generator was buried in a subcutaneous pocket on the abdomen. After 3 days, the pacemaker site became infected. The whole pacing system was extracted immediately and replaced by temporary pacing leads. After the infection was controlled, a new pacemaker was implanted through a left thoracotomy during which an original Blalock-Taussig shunt was established. The new pulse generator was buried in a subcutaneous pocket in the left precordial area. The patient's postoperative course was uneventful, and there have been no signs of lead problems or re-infection. Using temporary pacing leads as a bridge to implant a new system and choosing a precordial subcutaneous pocket for the implantation site following suturing of the leads on the left ventricle through a left thoracotomy were useful in this case.

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Language：Japanese   Publisher：(株)南江堂  

6生日女児。徐脈を主訴とした。胎児エコーで徐脈と先天性心疾患を指摘されており、出生直後の心エコーにて両大血管右室起始症、12誘導心電図にて完全房室ブロックと診断された。徐脈による心不全のため、剣状突起下切開でペースメーカ埋込み術を行ったが、術後3日目にペースメーカ感染をきたしたため、システムを完全に抜去して一時ペーシングリードを右室壁に縫着し、感染コントロールを図った。約1ヵ月で創がほとんど治癒したため、左側開胸で左Blalock-Taussig短絡手術を行うと同時に新しいペースメーカを感染巣と隔離して植込んだ。術後経過は良好で、術後15ヵ月経過後もペーシング不全やペースメーカ感染は認めていない。

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Language：Japanese   Publisher：日本胸部外科学会-関東甲信越地方会  

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We performed a right upper lobectomy with prosthetic replacement of the superior vena cava (SVC) through a posterolateral thoracotomy in a 65-year-old man undergoing complete resection of a locally advanced non-small-cell lung cancer with invasion of the SVC. Instead of using a vascular shunt, the right atrium and a right brachiocephalic vein (BCV) were anastomosed using a ringed polytetrafluoroethylene (PTFE) graft. During the anastomosis, vascular flow was maintained through the left BCV. By using this technique, SVC resection and reconstruction during lung cancer surgery can be safely performed through a posterolateral thoracotomy without blood flow interruption.

DOI： 10.1007/s00595-008-3927-z

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During follow-up of patients after primary lung cancer resections, small nodules or ground-glass opacities (GGOs) are sometimes detected on chest computed tomography. We report a case with multiple GGOs that were noted after primary lung cancer resection. A 76-year-old woman, who had undergone right upper lobectomy, middle lobe partial resection, and mediastinal lymph node dissection 3 years earlier, was admitted owing to five GGOs in the right lower lobe that had been increasing in size or density. A right S6+10 segmentectomy was performed. On histology, one adenocarcinoma and four bronchioloalveolar carcinomas (BACs), as well as two additional BACs that had not been detected preoperatively, were identified. No complications occurred postoperatively. Three years 4 months later, no tumor recurrence or new lesions have been found. Given the high possibility of malignancy, the appearance of new GGOs in patients with a history of lung cancer requires appropriate investigation.

DOI： 10.1007/s11748-008-0257-4

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