29例原位心脏移植近期疗效报告

目的:总结29例原位心脏移植的近期疗效。方法:2006年10月至2012年12月,对29例终末期心脏病患者行原位心脏移植治疗。受体肺血管阻力为3.6~5.2 wood单位,平均(4.2±1.8)wood单位;应用4℃的HTK液保护供心,供心冷缺血时间为63~360 min,平均(95.5±26.7)min;29例均采用双腔静脉吻合法原位心脏移植手术;术前使用免疫诱导治疗,术后采用环孢素A、霉酚酸酯和泼尼松预防急性排斥反应。结果:术后死亡2例,1例死于术后低心排出量综合征(低心排),1例死于急性右心衰竭。术后早期并发症有急性右心衰竭3例,急性肾衰竭2例,心包大量积液4例。结论:心脏移植近期疗效满意。选择肺血管阻力较低的患者、妥善的供心心肌保护、熟练的手术操作、合理应用免疫抑制剂和正确处理术后肾功能不全是提高心脏移植近期疗效的重要措施。     

同种原位心脏移植（附3例报告）

目的观察3例同种原位心脏移植患者的近、远期疗效,总结心脏移植的经验。方法3例心肌病患者施行原位心脏移植,手术方法采用标准法1例、双腔静脉法2例,供心保护液为4℃的HTK液,术后免疫抑制治疗采用赛尼哌加＂三联＂方案。结果3例手术均成功,2例术后未发生明显感染及其他并发症,心功能Ⅰ级,已恢复正常工作,1例于术后13 d死于急性排斥反应。结论选择合适的供心及保护方法是同种原位心脏移植手术成功的前提,合理应用免疫抑制剂、正确处理并发症是手术成功的关键。     

原位心脏移植5例初步经验

目的总结原位心脏移植成功的初步经验。方法2000年9月至2004年9月为5例扩张型心肌病患者行原位心脏移植术,其中标准原位心脏移植手术2例,双腔静脉吻合法3例。结果本组5例无手术死亡,2例术后因右心衰竭和真菌感染致肾功衰竭分别于术后第14天和第76天死亡,其余3例至今(2005年5月)存活,最长生存时间近5年,生活质量良好,心功能0～I级。结论合适的受体选择,良好的心肌保护,术后并发症的有效防治和合理的免疫治疗,是心脏移植成功的重要因素。     

原位心脏移植长期存活原因分析（附34例报告）

目的探讨原位心脏移植长期存活的原因和价值。方法选自2004·10～2012·12在广西壮族自治区人民医院和解放军181医院进行同种异体心脏移植患者34例。男性29例,女性5例;年龄12～56岁。扩张型心肌病30例,肥厚型心肌病2例,冠心病2例。心脏功能Ⅲ一Ⅳ级。全部病例均采用双腔静脉吻合法进行原位移植。对患者的个体因素、供心保护、手术过程、围手术期情况、免疫抑制剂治疗和术后检测情况、生活质量进行分析。随访1—99个月。结果早期死亡3例,其中2例死于肺动脉高压危象一右心功能衰竭。出院的31例患者全部存活,心脏功能恢复I～Ⅱ级。存活3年以上13例,其中8年以上1例,心功能I级,已生育一小孩。免疫抑制剂血液浓度监测和B型超声检查未发现异常。64排CT检查未发现冠状动脉有狭窄表现。结论组织配型、供心保存、缺血．再灌注损伤、手术过程、免疫抑制剂合理使用是移植后长期存活的关键因素,防治免疫排斥、代谢、感染等是防治心脏移植物血管病变的重要措施。64排CT等非创伤性检查可作为常用的监测手段。     

原位心脏移植8例报告

目的探讨影响心脏移植手术效果的主要因素。方法2003年3月至2004年12月8例患者施行了原位心脏移植术,其中扩张型心肌病7例,肥厚型心肌病1例。采用标准原位心脏移植手术1例,双腔静脉吻合法原位心脏移植手术7例。4例术前存在中度肺动脉高压,NO吸入等措施降肺动脉压力。抗排异治疗采用环孢素A(CsA)+皮质激素(Pred)+骁悉(MMF)三联方案。结果手术均获成功,1例术后第9天出现急性排异反应,给予大剂量泼尼松龙冲击治疗3d缓解;1例术后第3天出现肾功能衰竭,给予血液透析治疗;1例于术后35天死于肾功能衰竭及抗排异药物神经毒性反应,其余病例心功能恢复至Ⅰ￣Ⅱ级。术后5例已恢复正常工作和生活。结论严格掌握受体适应证、供体的心脏保护、合适的手术方法及围术期患者管理,是心脏移植手术成功的关键。     

原位心脏移植术的围术期临床经验及效果

目的总结心脏移植围术期的心肌保护方法和经验。方法2003年3月至2005年11月为11例终末期心脏病患者实施了原位心脏移植,其中心-肺联合移植、心-肾联合移植各1例。采取标准法1例,双腔静脉法10例。结果全组热缺血时间8￣12min,冷缺血时间90￣292min,术中心肌自动复跳10例,1例电除颤复跳。术后1周超声心动图示各心腔大小、EF值均在正常范围。死亡3例:心-肺联合移植术后气管吻合口瘘合并重度感染1例,心-肾联合移植、单纯心脏移植术后急性肾功能衰竭各1例。随访1￣29个月,8例存活患者心功能均恢复至Ⅰ￣Ⅱ级。结论心脏移植术中除在供心的正确切取过程中注意供心的心肌保护外,还应在术中吻合、术后管理过程的整个围手术期都要重点加强心肌保护,这些均是手术成功的重要基础。     

同种异体原位心脏移植(附11例报告)

目的总结同种异体原位心脏移植的手术经验。方法对11例终末期心脏病患者施行同种异体原位心脏移植术。其中标准原位心脏移植术1例,双腔静脉吻合法原位心脏移植术10例,心肾联合移植术1例。5例术前存在中度肺动脉高压者,术中及术后给予一氧化氮（NO）吸入等措施治疗。抗排异治疗采用环孢素A（CsA）＋甲基强的松龙（MP）＋霉酚酸酯（MMF）三联方案。结果2例术后出现急性排异反应．给予大剂量MP冲击治疗3d缓解;1例术后第3天出现肾功能衰竭．给予血液透析后缓解。1例行心肾联合移植术者术后18d死于肺动脉栓塞。随访6～45个月,晚期死亡2例,其余病例心功能正常,恢复正常工作和生活。结论严格掌握受体适应证、合适的手术方法及严格的围术期管理可提高同种异体原位心脏移植术的疗效。     

全心脏原位移植(附4例报告)

目的总结4例全心脏原位移植的经验。方法我院于2005年4月至2006年12月对4例扩张型心肌病患者施行了全心脏原位移植手术,其中男性2例,女性2例,年龄32～57岁。供体均为急性脑死亡者。供心心肌保护3例为UW,1例为HTK液。全心脏移植吻合次序依次左肺静脉、右肺静脉、下腔静脉、上腔静脉、肺动脉及主动脉,其中2例由于受体全心脏扩大,按常规保留的受体上、下腔静脉长度无法与供体腔静脉吻合,为此,截取一段长4cm的供体肺动脉,一端与受体下腔静脉吻合,另一端与供体下腔静脉吻合。吻合时间78～104min,主动脉阻断时间136～197min,体外循环时间202～261min。主动脉开放后,1例电击复跳,3例自动复跳,均为窦性心律,未安置心脏临时起搏器。结果患者术后3～6h神志清楚,14～19h拔除气管插管,6d拔除所有侵入性监测管道,两周后从ICU迁至普通病房,1例术后52d出现急性排异反应,经处理得到控制,均痊愈出院。术后随访心功能均为I级,窦性心律,2例有轻度三尖瓣反流,2例已经恢复工作。结论全心脏原位移植法不仅保存左右心房解剖上完整性,有利于心功能恢复,减少移植后血栓的发生率,而且使房、室间隔传导系统的完整性免遭破坏,降低了心律失常的发生率,这是一种值得推荐的心脏移植方法。     

同种异体原位心脏移植的临床研究（附5例报道）

目的　总结 5例心脏移植经验 ,探讨心脏移植的近远期疗效。方法　 1998年 10月至 2 0 0 3年 12月施行5例原位心脏移植 ,5例均为心肌病 ,其中 3例为扩张型 ,2例为限制型 ,手术方法采用标准法 4例、双腔静脉法 1例 ,供心平均冷缺血时间为 (16 5 .6± 2 6 3)min ,术后定期行心内膜活检 ,使用 4联免疫抑制剂。结果　第 1例存活 5d ,死于低心排及主动脉内球囊反搏 (IABP)的并发症 ,第 4例存活 18月 ,死于中———重度的急、慢性并存的排斥反应 ,余 3例至今存活 ,至今存活时间分别为 5年 8个月、4年 5个月、6月。结论　心脏移植是治疗终末期心脏病的有效手段 ,充分的术前准备 ,良好的心肌保护 ,合理使用免疫抑制剂 ,能取得良好的近远期疗效 ,长期生存病例需注意慢性排斥反应。     

心脏移植术后的监护与治疗

目的:总结同种异体原位心脏移植术后的监护与治疗经验。方法:对我院2006年10月至2008年3月开展的8例原位心脏移植手术的临床资料进行回顾性分析。结果:全组存活7例,死亡1例,7例受体术后呼吸机辅助时间18～32h,术后2～5d下床活动,监护室停留时间6～22d,术后住院时间10～30d。术后并发症有急性右心功能不全3例,低心排综合征1例,急性肾功能衰竭1例,大量心包积液1例,肺不张1例。结论:心脏移植术后早期加强监护,有效的抗排斥治疗,积极防治右心衰竭,维护重要脏器功能是心脏移植成功的关键。     

11例原位心脏移植成功的初步经验

目的 报告11例原位心脏移植成功的初步经验。方法 2000年5月－2001年5月连续为11例患者行原位心脏移植术，其中扩张型心肌病10例，复杂性先天性心脏病1例。标准原位心脏移植手术10例，双腔静脉吻合法1例。结果 所有病例均存活，心功能恢复至I－Ⅱ级（NYHA），围术期无感或严重排异反应发生。随访期间有1例巨细胞病毒感染，1例排反应发生。结论 良好的心肌保护、术后合理的监测与抗排异治疗是心脏移植成功的关键。     

Specific cardiomyopathy caused by high dose cyclophosphamide immediately after bone marrow transplantation

An 11-year-old girl with CML in chronic phase was treated with allogeneic bone marrow transplantation (BMT). She had had no episode of heart disease, and her chest roentgenogram and electrocardiogram were within normal ranges. She had no anthracycline, but 120 mg/kg (2.01 g/m2/d x 2 days) of cyclophosphamide (CY) for conditioning of BMT. She suffered from an acute heart failure on 14 days after BMT and died 2 days later. Autopsy revealed "specific heart muscular disease" with apparent degeneration and necrosis of heart muscle cells. More than 30 fatal cases of CY cardiotoxicity after BMT have been reported, and most patients developed acute heart failure within 10 days after CY dosing. Goldberg et al. reviewed 14 cases of CY cardiotoxicity, and considered more than 1.55 g/m2/day to be the critical doses for the onset of fatal cardiomyopathy. This case was considered to be first case in Japan. CY cardiomyopathy is an early fatal complication of BMT when CY used for conditioning. The dosing schedule may be considered.     

From discrete dilated cardiomyopathy to successful cardiac transplantation in congenital disorders of glycosylation due to dolichol kinase deficiency (DK1-CDG)

Congenital disorders of glycosylation are a growing group of inborn errors of protein glycosylation. Cardiac involvement is frequently observed in the most common form, PMM2-CDG, especially hypertrophic cardiomyopathy. Dilated cardiomyopathy, however, has been only observed in a few CDG subtypes, usually with a lethal outcome. We report on cardiac pathology in nine patients from three unrelated Israeli families, diagnosed with dolichol kinase deficiency, due to novel, homozygous DK1 gene mutations. The cardiac symptoms varied from discrete, mild dilation to overt heart failure with death. Two children died unexpectedly with acute symptoms of heart failure before the diagnosis of DK1-CDG and heart transplantation could take place. Three other affected children with mild dilated cardiomyopathy at the time of the diagnosis deteriorated rapidly, two of them within days after an acute infection. They all went through successful heart transplantation; one died unexpectedly and 2 others are currently (after 1–5 years) clinically stable. The other 4 children diagnosed with mild dilated cardiomyopathy are doing well on supportive heart failure therapy. In most cases, the cardiac findings dominated the clinical picture, without central nervous system or multisystem involvement, which is unique in CDG syndrome. We suggest to test for DK1-CDG in patients with dilated cardiomyopathy. Patients with discrete cardiomyopathy may remain stable on supportive treatment while others deteriorate rapidly. Our paper is the first comprehensive study on the phenotype of DK1-CDG and the first successful organ transplantation in CDG syndrome.     

Pharmaceutical management of decompensated heart failure syndrome in children: Current state of the art and a new approach

Prompt initiation of appropriate and intensive treatment in children with decompensated heart failure is crucial to avoid irreversible end-organ dysfunction. Initial management of these children includes transfer to the pediatric cardiac intensive care unit, basic hemodynamic monitoring, and establishment of intravenous access. Inotropic support should be instituted peripherally before obtaining central venous and arterial access. The team should be prepared for emergent intubation and initiation of mechanical circulatory support. Two experienced physicians should work together to obtain vascular access and manage sedation, airway control, and cardiovascular support. Acute heart failure syndrome in children may be related to cardiomyopathy, myocarditis, congenital heart disease, and acute rejection post heart transplantation. Each of these causes requires a different approach. Fulminant myocarditis may lead to severe morbidity and requires intensive support, although its outcome is considered to be good. Acute heart failure related to newly diagnosed dilated cardiomyopathy may represent end-stage heart failure; therefore, long-term mechanical circulatory support and heart transplantation may be considered to avoid other end-organ dysfunction. Hypertrophic cardiomyopathy may lead to acute decompensation due to 1) left ventricular outflow obstruction, 2) restrictive physiology leading to pulmonary hypertension, or 3) myocardial is chemia associated with coronary artery bridging. Decompensated heart failure associated with congenital heart disease usually represents end-stage heart failure and requires thorough evaluation for heart transplantation. Children with single-ventricle physiology who develop decompensated heart failure after a Fontan procedure are not candidates for mechanical circulatory support and therefore may not survive to heart transplantation. Acute heart failure due to posttransplantation acute rejection requires aggressive antirejection treatment, which places these patients at significant risk for overwhelming opportunistic infections. In our opinion, mechanical circulatory support should be initiated early in children who present with end-stage heart failure associated with hemodynamic instability to avoid end-organ damage.     

Heart transplantation in hypertrophic cardiomyopathy

Heart transplantation (HT) is the sole therapeutic option for selected patients with hypertrophic cardiomyopathy (HC) and refractory heart failure. However, the results of HT have not been systematically investigated in HC. We assessed the pathophysiologic profile of HT candidates and the outcome after transplantation in 307 patients with HC consecutively evaluated at our tertiary referral center from 1987 to 2005; follow-up was 9.9+8.2 years. Outcome of recipients with HC was compared with that of 141 patients who underwent transplantation for idiopathic dilated cardiomyopathy at our center over the same period. Of 21 patients with HC who entered the transplantation list, 20 had end-stage evolution with systolic dysfunction and 1 had an extremely small left ventricular cavity with impaired filling and recurrent cardiogenic shock during paroxysmal atrial fibrillation. Of 33 study patients with HC who showed end-stage evolution during follow-up, the 23 who were included on the waiting list or died from refractory heart failure (2 patients) were significantly younger than the 10 patients who remained clinically stable (37+/-14 vs 57+/-17 years, p=0.004). Of the 21 HT candidates, 18 underwent transplantation during follow-up. In heart transplant recipients, 7-year survival rate was 94% and not different from that of the 141 patients who received transplants for idiopathic dilated cardiomyopathy (p=0.66). In conclusion, long-term outcome after HT in patients with HC is favorable and similar to that of patients with idiopathic dilated cardiomyopathy. In patients with end-stage HC, young age is associated with more rapid progression to refractory heart failure.     

肥厚型心肌病原位心脏移植后心肌再肥厚初探

目的 探讨原位心脏移植治疗肥厚型心肌病(hypertrophic cardiomyopathy,HCM)的经验和疗效以及移植后心肌再肥厚分析.方法 9例晚期HCM患者接受同种异体原位心脏移植,男7例,女2例,采用免疫诱导方案,维持治疗采用环孢素A+霉酚酸酯+泼尼松三联方案.结果 1例移植后因肾动脉栓塞发生急性肾功能衰竭、急性排斥反应,行持续肾脏替代治疗及冲击治疗后好转,移植后32个月心跳骤停死亡.1例并发额顶叶脱髓鞘病变经内科治疗后好转.8例长期存活,3例移植后半年发现心肌再肥厚,未发现移植物血管病.结论 心脏移植是治疗晚期HCM的最佳方法之一,临床疗效良好,移植后心肌再肥厚可能与心肌微环境等有关.

Abstract：

Objective To investigate the outcome of orthotopic heart transplantation for patient with end-stage hypertrophic cardiomyopathy. Methods This retrospective review analyzed the clinical data of nine patients (7 males ) undergoing orthotopic heart transplantation for end-stage hypertrophic cardiomyopathy in our center. All patients received induced therapy protocols peri-operative and standard triple maintenance immunosuppressive therapy postoperative. Results One recipients developed acute renal failure due to renal artery embolism and allograft rejection in the early posttransplantive course, symptoms and signs were improved under continuous renal replacement therapy and steroid-pulse therapy, this patient died of sudden cardiac arrest at 32 months post transplantation. Another recipient developed demyelinating disease in frontal and parietal lobe and finally recovered with medical therapy. Eight patients survived the operation with good quality of life and there was no episode of rejection or infection or chronic graft arteriosclerosis during follow-up time. Three recipients developed left ventricular hypertrophy and there were no signs of grapg-vessle diseases in the survivals. Conclusion Heart transplantation is the best therapeutic option for selected patients with end-stage hypertrophic cardiomyopathy.     

原位心脏移植5例报告

目的总结5例同种异体原位心脏移植的治疗经验,探讨手术方式及术后围术期处理。方法2005年1月至2005年6月连续为5例终末期心脏病患者实施了原位心脏移植手术。术后免疫抑制剂应用环饱素A+骁悉+泼尼松"三联疗法。结合临床表现、超声心动图、化验检查及心肌内心电图,对心脏移植术后急性排斥反应的监测进行分析。结果5例手术均顺利,其中1例存活时间仅为9 d,其余均近期存活,生活质量良好。结论原位心脏移植是治疗终末期心脏病的有效方法。作为一种监测排斥反应的无创方法,心肌内心电图可以明显减少心肌活检的次数。低血管阻力受体的选择和合理的免疫抑制治疗方案的应用是心脏移植成功的关键。