室间隔缺损合并重度肺动脉高压的外科治疗

目的总结室间隔缺损(VSD)合并重度肺动脉高压病例的外科治疗经验。方法全组76例病人,心导管检查的16例,平均肺动脉压力65±17mmHg,其余经多普勒测定为50mmHg。于体外循环下手术修补室间隔缺损,术前、术后应用血管紧张素转换酶抑制剂(ACEI)。结果 术后3例死亡,占3.9％,余73例治愈。术后心功能明显改善。结论手术治疗室间隔缺损合并重度肺动脉高压可以取得满意疗效。     

先天性心脏病合并重度肺动脉高压诊断性治疗的临床研究

目的:评估诊断性治疗在选择伴艾森门格综合征的左向右分流型先天性心脏病(先心病)外科根治手术的临床价值。方法:回顾性分析2011年1月至2015年7月,行手术治疗的先天性心脏病合并重度肺动脉高压患者32例(男性8例,女性24例)。年龄9~52岁,中位年龄23岁,体质量25~60kg,平均(45.9±10)kg,其中房间隔缺损8例,室间隔缺损18例,部分型心内膜垫缺损2例,单纯动脉导管未闭2例,动脉导管未闭合并房间隔缺损、室间隔缺损各1例。首诊时经皮血氧饱和度(Sp O2)86%~94%,平均(91.5±2.2)%。均予强心利尿治疗的基础上,加用西地那非或波生坦等选择性降低肺动脉压靶向药物8周到24周,行右心导管检查后在全麻体外循环下行外科手术根治,术中放置4/5#SwanGanz漂浮导管监测术中及术后肺动脉压。术后残余肺动脉高压患者继续给予降低肺动脉压力药物治疗。术后1个月、3个月、6个月及以后每年超声心动图检查随访。结果:患者经药物靶向治疗后Sp O2均95%,平均(97±1.7)%,术前右心导管检查肺动脉平均压(m PAP)47~93mm Hg,平均[(75±13)mmHg,1mm Hg=0.133k Pa],肺血管阻力指数(PVRI)平均(10.2±3.1)Wood unit·m-2(4.6~15.3),肺循环血流量与体循环血流量比值(Qp/Qs)1.30~2.98,平均(1.84±0.44);导管检查中行急性肺血管扩张实验阳性31例,阴性1例。术后随访6个月~4年,全组术后至随诊无一例死亡,无远期并发症。结论:本组病例提示,诊断性靶向药物治疗作为慢性肺血管扩张实验,为错过良好手术时机的艾森门格综合征前期患者提供了可能根治的机会。术后残余肺动脉高压患者继续降低肺动脉压力药物治疗仍有一定疗效。     

室间隔缺损合并重度肺动脉高压围手术期处理

目的 :评估室间隔缺损合并重度肺动脉高压手术治疗围手术期处理的效果。方法 :选择 5 5例室间隔缺损合并重度肺动脉高压病例 ,在手术时放置Swan ganz导管 ,术后进行肺动脉压监测 ;2 0例用单向活瓣补片修补室缺 ,术后用超声心动图监测分流情况 ;用镇静 ,肌松 ,过度通气 ,血管扩张剂等治疗肺动脉高压危象。结果 :发现 7例持续肺动脉高压 ,死亡 2例 ;2 7例有肺动脉高压危象 ,死亡 2例 ;在严重肺动脉高压时 ,单向活瓣补片有右向左分流 ,肺动脉压下降后分流停止 ,未发现左向右分流。结论 :室间隔缺损合并重度肺动脉高压应把握好手术适应症 ,有不可逆的肺动脉高压患者禁止手术 ;Swan ganz导管在术后肺动脉高压处理上有重要作用 ;单向活瓣补片修补室缺能在术后发生重度肺动脉高压时发挥良好的右向左单向分流作用 ,肺动脉压下降后又能自动关闭。     

靶向药物联合缺损修复治疗成人先天性心脏病相关肺动脉高压的预后研究

目的:分析靶向药物联合缺损修复(Treat-Repair策略)治疗成人先天性心脏病合并肺动脉高压患者的有效及安全性。方法:回顾分析2010年12月至2019年9月,于我中心接受缺损修复的54例,平均年龄(31.07±10.09)岁的成人左向右分流先天性心脏病合并肺动脉高压[平均肺动脉压力(mPAP)≥25 mmHg,1mmHg=0.133 kPa],肺小动脉阻力(PVR≥3WU)患者的临床资料,其中28例在缺损修复前接受了靶向药物治疗,26例直接接受了缺损修复。对所有患者进行电话随访,观察患者不良事件发生情况并绘制生存曲线。不良事件包括:死亡,进行肺移植,出现右心功能不全表现:晕厥、水肿、WHO心功能分级Ⅲ～Ⅳ级、6 min步行距离减少15%或BNP100 ng/L。结果:初次就诊时,术前有靶向药物治疗组患者有更高的PVR [(11.30±4.91)vs.(7.25±2.20)wood units,P0.01]。经过靶向药物治疗后,术前有靶向药物治疗组患者PVR降至(7.13±3.80)wood units(P0.01)。远期随访中,两组患者的生存曲线差异无统计学意义。结论:"Treat-Repair"治疗策略为超手术适应证的左向右分流先天性心脏病合并肺动脉高压的患者带来了新的治疗希望,使患者成功接受缺损修复的同时改善中远期生存状况。     

组织多普勒速度成像评价先天性心脏病合并肺动脉高压患者左心室功能的研究

目的:探讨组织多普勒速度成像技术(TVI)评价先天性心脏病(先心病)合并肺动脉高压(PAH)患者左心功能的价值。方法:选取先心病合并肺动脉高压患者30例分为3组,轻度肺动脉高压12例,中度10例,重度8例,其中4例临床诊断为艾森曼格综合征失去手术机会,畸形为室间隔缺损20例,动脉导管未闭8例,房间隔缺损合并室间隔缺损2例。全部病例均为在我院确诊为先心病并在我院拟行手术治疗的患者。分别对以上病例进行常规超声测量及进行TVI成像模式下参数测定。结果:3组患者相比:重度肺动脉高压组收缩期室壁前向运动速度较轻中度肺动脉高压组明显减低,舒张早期运动速度(Ve)减低,二尖瓣环运动速度降低。结论:TVI组织速度成像技术可无创有效的评估肺动脉高压患者左心功能的变化。     

37例室间隔缺损并重度肺动脉高压外科治疗分析

目的：总结37例室间隔缺损（VSD）并重度肺动脉高压的手术治疗经验。方法：37例VSD合并重度肺动脉高压的患，应用包括休息、吸氧、口服卡托普利及静滴前列腺素E1等在内的降肺动脉压综合治疗，治疗后若杂音增强，血氧饱和度（SaO2)升高，心脏彩超示左右向右分流增加，则进行手术治疗；若变化不明显，重复用药。全组病例均在中度低温体外循环下手术修补缺损。分别观察术前、后的SaO2，肺动脉压以及心功能，随访心功能的改善情况及死亡率。结果：术后2例死于低心排，1例死于呼吸衰竭，其他病例恢复顺利。经随访1例患于术后8月死亡，其他患心功能均有明显改善。结论：合理的围手术期治疗及严格掌握手术适应症是降低室间隔缺损合并重度肺动脉高压死亡率的关键因素，血氧饱和度可作为判断是否手术的简单易行指标。     

10例先天性心脏病合并重度肺动脉高压联合靶向药物创造手术时机疗效观察

目的：评价三类靶向药物对于扩大合并重度肺动脉高压患儿手术适应证的临床研究。方法：回顾性分析2018年1月至2021年6月，我院收治的左向右分流型先天性心脏病合并重度肺动脉高压患儿18例，根据三类降肺动脉高压药物诊断性治疗，氧饱和度改善和肺动脉压力下降情况，分为手术治疗组和非手术治疗组，经过药物治疗后评估手术指征，对符合手术患儿行外科根治手术，术后继续靶向药物治疗，并定期随访评估肺动脉压力；非手术治疗组需继续靶向药物治疗。结果：本研究10例先天性心脏病合并重度肺动脉高压，平均年龄(9±2)岁，联合靶向药物诊断性治疗后经皮氧饱和度(SpO₂)由88%～94%，平均(91.2±1.5)%上升至96%～99%，平均(97.9±0.5)%，均行根治性外科手术。另外8例患儿SpO₂用药后维持在76%～89%，平均(87.1±2.2)%，未达到手术指征继续靶向药物治疗。全组10例手术患儿随访6个月至1年，7例患儿经靶向药物治疗后肺动脉压力恢复正常；3例患儿降至轻中度肺动脉高压。结论：对于合并重度肺动脉高压的先天性心脏病，经过联合靶向药物诊断性治疗后，SpO...     

室间隔缺损外科治疗821例报告

目的 :探讨室间隔缺损并肺动脉高压及其合并畸形的诊断、术式选择及围手术期处理。方法 :手术治疗室间隔缺损 82 1例 ,其中补片修复 2 92例 ,缝合修补 5 2 9例。合并畸形 14种 (142例 ) ,需行矫正者 134例均给予手术矫正。结果 :早期死亡 16例 (1 9% ) ,死因主要为心力衰竭、呼吸衰竭和心律失常。结论 :室缺合并重度肺动脉高压者强调合理掌握手术指证及围手术期心肺支持与保护 ;对于合并主动脉瓣关闭不全、动脉导管未闭、右室双腔心等畸形者强调术前明确诊断 ,术中详细探查及正确处理。     

5例完全性房室间隔缺损的外科治疗

报告５例完全性房室间隔缺损的外科治疗．５例均为ＲａｓｔｅｌｌｉＡ型，３例合并重度肺动脉高压；在中度低温体外循环下根治手术，早期死亡１例．随访２月～４年，无晚期死亡，３例恢复良好，ｌ例合并中～重度二尖瓣返流及中度肺动脉高压．作者主张尽早在婴儿期行根治术，术中精确修补室间隔缺损及原发孔房间隔缺损，精确重建二尖瓣功能防止术后二尖瓣返流，加强术后肺动脉高压的处理。     

复杂先心病术后远期同种移植物衰败的再手术治疗

目的 回顾性总结复杂先心病术后远期同种异体移植物衰败再狭窄的手术方法和疗效。方法 2010年1月至2016年1月,同种异体主动脉重建肺动脉术后远期肺动脉再狭窄的手术治疗共23例。年龄20.6±4.7岁(13～31岁),男15例(65.2%)。病种包括右心室双出口 室间隔缺损 肺动脉狭窄11例,肺动脉闭锁/室间隔缺损6例,矫正性大动脉转位 室间隔缺损 肺动脉狭窄3例,永存动脉干3例。首次手术时均使用同种异体主动脉重建肺动脉,直径16.6±1.6 mm(14～19 mm,其中4例不详)。本次手术前峰值肺动脉压力阶差111.7±21.8 mmHg (86～170 mmHg),本次手术与前次手术的间隔时间为15.0±2.3年(12～19年)。术前通过心脏彩超和心脏及周围大血管CTA明确诊断。术中以涤纶人造血管(以牛心包缝制抗反流活瓣,开胸前缝制好)重建肺动脉,使用的人造血管直径20.6±1.5 mm(18～22 mm)。体外循环时间145.7±31.4 min(106～182 min),升主动脉阻断时间85.3±17.4 min(70～118 min)。术后每6个月门诊随访一次,平均随访时间30(6-78)个月。结果 无死亡。术后并发症有纵隔感染1例(4.3%),术后低心排综合症2例(8.7%),呼吸机使用时间大于72小时4例(17.4%),均痊愈。无再次开胸止血。术后ICU时间6.0±2.6 d(3～15 d),术后住院时间13.7±4.8 d(7～30 d)。术后复查心脏彩超峰值肺动脉压力阶差12.6±5.4 mmHg(3～25 mmHg)。 随访中无死亡,肺动脉压力阶差无明显增加,无需要再手术患者。结论 对于同种异体主动脉重建肺动脉后远期肺动脉再狭窄患者,可采用带瓣人造血管再次重建肺动脉,手术安全可靠,早期疗效良好。     

Cardiopathies in trisomy 21. Therapeutic indications

Approximately 50% of patients with Trisomy 21 have congenital heart lesions and they account for 2.7% of the admissions to the Pediatric Cardiac Unit of the Cardiac Hospital of Lyons. In a series of 91 cases observed over a 10 year period (1070-1980) undergoing hemodynamic and angiographic investigation, 9 out of 10 cases - as previously reported - had the following abnormalities: endocardial cushion defect, 42.7% (39% of which were complete atrioventricular canals), ventricular septal defect, 33%, and Fallot's tetralogy, 12%. These lesions were characterised by the high incidence and precocity of pulmonary hypertension (69 out of 72 cases with a left-to-right shunt . A comparison of the hemodynamic data of children with Trisomy 21 with ventricular septal defects, and children without Trisomy 21 of the same age with ventricular septal defect showed the pulmonary arterial resistances to be significantly higher in the group with Trisomy 21, suggesting that Trisomy 21 plays a role in the pathogenesis of severe early pulmonary hypertension. The operative risk is much higher in these patients, especially with openheart surgery (4 operative deaths out of 12 cases of closure of VSD). The surgical indications are difficult to define because of the context of malformation and mental retardation, the high incidence of pulmonary hypertension and the high opertive risk requiring a large and detailed dialogue with the family.     

Long-term outcome of patients operated for large ventricular septal defects with increased pulmonary vascular resistance

BACKGROUND: There is a paucity of data regarding the long-term outcome of patients operated for ventricular septal defect with severe pulmonary arterial hypertension and elevated pulmonary vascular resistance. METHODS AND RESULTS: We evaluated the long-term follow-up results of a selected cohort of patients with nonrestrictive ventricular septal defect and elevated pulmonary vascular resistance (>6 Wood units). Thirty-eight patients, median age 7.5 years (range 6 months-27 years), with nonrestrictive ventricular septal defect with severe pulmonary hypertension were operated between 1985 and 1996 at our institute. Preoperative pulmonary vascular resistance, ratio of pulmonary blood flow to systemic blood flow, and ratio of pulmonary vascular resistance to systemic vascular resistance were 7.63+/-1.8 Wood units, 1.9+/-0.48, and 0.41+/-0.12, respectively. The majority (68.4%) had perimembranous ventricular septal defect. Thirty patients (79%) had a good outcome and were asymptomatic at a mean follow-up of 8.7 years, with significant reduction in pulmonary artery pressures. Eight patients (21%) had a poor outcome, which included 5 immediate postoperative deaths, 1 late death and 2 surviving patients with persistent severe pulmonary arterial hypertension. There was no significant difference regarding hemodynamic parameters at baseline between those who had a good outcome and those who did not. Eleven patients with a preoperative pulmonary blood flow to systemic blood flow ratio of <2:1. who had a good outcome following surgery, underwent repeat catheterization at follow-up. There was a significant reduction in their mean pulmonary vascular resistance (8.03+/-1.4 v. 4.16+/-1.6 Wood units, p=0.001) and pulmonary vascular resistance to systemic vascular resistance ratio (0.41+/-0.12 v. 0.19+/-0.06, p=0.05). CONCLUSIONS: The late results of surgery on this selected group of patients with nonrestrictive ventricular septal defect with high pulmonary vascular resistance are encouraging. Operative correction of the ventricular septal defect should be actively considered in all children presenting with nonrestrictive ventricular septal defect with a significant left-to-right shunt, despite moderately elevated pulmonary vascular resistance. Even among older patients with ventricular septal defect and moderately elevated pulmonary vascular resistance, there is a specific group that does well after operation.     

小于4岁先天性心脏病患儿急诊手术41例临床报告

目的 探讨婴幼儿先天性心脏病急诊手术原因及疗效。方法 收集我院外科1998年1月-2000年11月收治的41例小于4岁的患儿资料。分析急诊心脏手术的原因及手术效果。结果 41例患儿中，法鲁四联症25例，房间隔缺损合并肺动脉瓣狭窄5例。完全型肺静脉异位引流4例。大动脉转位2例，室间隔缺损合并肺动脉高压3例。三尖瓣闭锁合并室间隔缺损1例，室间隔缺损合并右室赘生物1例。院内手术死亡7例。死亡率17.1%。结论 病情危重，内科治疗效果不佳的先天性心脏病患儿接受急诊手术治疗，可以取得较好效果。     

Acute haemodynamic effects of nifedipine in patients with ventricular septal defect

The haemodynamic effects of nifedipine were studied in 14 patients (aged 8-14 years, seven male and seven female) with ventricular septal defect with and without pulmonary hypertension. All underwent left and right heart catheterisation. In each patient the pressures and heart rate were measured and blood samples were taken for oximetry before and after sublingual administration of 10 mg nifedipine. In eight patients with ventricular septal defect without pulmonary hypertension (mean pulmonary artery pressure less than 20 mm Hg) nifedipine significantly reduced the mean aortic pressure and systemic vascular resistance, and significantly increased heart rate. The other haemodynamic indices did not change significantly. In six patients with ventricular septal defect complicated by pulmonary hypertension (mean pulmonary artery pressure greater than 20 mm Hg) nifedipine significantly increased systemic output, stroke volume, and heart rate, and significantly reduced systemic vascular resistance and the pulmonary to systemic flow ratio. The other haemodynamic indices did not change significantly. Nifedipine had a beneficial effect in patients with ventricular septal defect complicated by pulmonary hypertension. It reduced the left to right shunt and increased the stroke volume. This effect was not seen in patients with ventricular septal defect uncomplicated by pulmonary hypertension.     

Acute haemodynamic effects of nifedipine in patients with ventricular septal defect.

The haemodynamic effects of nifedipine were studied in 14 patients (aged 8-14 years, seven male and seven female) with ventricular septal defect with and without pulmonary hypertension. All underwent left and right heart catheterisation. In each patient the pressures and heart rate were measured and blood samples were taken for oximetry before and after sublingual administration of 10 mg nifedipine. In eight patients with ventricular septal defect without pulmonary hypertension (mean pulmonary artery pressure less than 20 mm Hg) nifedipine significantly reduced the mean aortic pressure and systemic vascular resistance, and significantly increased heart rate. The other haemodynamic indices did not change significantly. In six patients with ventricular septal defect complicated by pulmonary hypertension (mean pulmonary artery pressure greater than 20 mm Hg) nifedipine significantly increased systemic output, stroke volume, and heart rate, and significantly reduced systemic vascular resistance and the pulmonary to systemic flow ratio. The other haemodynamic indices did not change significantly. Nifedipine had a beneficial effect in patients with ventricular septal defect complicated by pulmonary hypertension. It reduced the left to right shunt and increased the stroke volume. This effect was not seen in patients with ventricular septal defect uncomplicated by pulmonary hypertension.     

提高先天性心脏病并发肺动脉高压外科治疗效果的综合方案

目的:探讨先天性心脏病并发肺动脉高压的手术适应证,包括术前综合评价病情、充分降肺动脉压、术中综合处理、术后重症监护,以提高其外科治疗成功率。方法: 对我院2009年1月～2010年12月在全麻低温体外循环下手术治疗室间隔缺损（VSD）并发肺动脉高压（pulmonary hypertension,PH）患者280 例进行回顾性分析。术前综合评价有无手术适应证及充分降肺动脉压准备:所有患者均接受氧疗,经静脉微泵静脉注射扩血管药［硝普纳和（或）前列地尔注射液］,必要时行心导管检查。术中综合处理:术中加强体外循环心肺保护及降肺动脉压的药物应用,精细操作,对部分极重度肺动脉高压的患者留单向活瓣,术中特别注意探查三尖瓣的处理同时矫正其他并发畸形。术后处理:术后呼吸机辅助呼吸时间适当延长,用呼气末正压（PEEP）、过度通气、一氧化氮治疗等。结果: 本组术后死亡5例,病死率1.8%,发生并发症者18 例（发生率9%）。入院后经术前降肺动脉压力及氧疗后,平均肺动脉压力由(71±14) mmHg降至手术开始前的(58±11) mmHg（P<0.05）,手术后肺动脉压力进一步下降至出院前的平均肺动脉压力 (38±5) mmHg（P<0.05）。术后1年复查超声心动图265例,缺损处残余分流2例,三尖瓣中度反流2例、重度返流1例。结论: 我院术前正确评估、积极降压准备、术中综合处理、术后重症监护的综合外科治疗措施,明显提高了先天性心脏病并发肺动脉高压的治疗效果。     

Transatrial closure of postinfarction ventricular septal defect

While operative approaches to postinfarction ventricular septal defect emphasize repair through the infarcted area, we present a case that illustrates the transatrial approach in an acute setting in which no discrete infarct or other abnormality of the free ventricular wall is apparent. In such a setting, transatrial repair of mid-muscular or posterior defects can avert unnecessary compromise of right ventricular function. Avoidance of a right ventriculotomy might also benefit patients with chronic defects complicated by severe lung disease or pulmonary hypertension. However, successful transatrial closure requires full visualization of the defect, avoidance of tricuspid valve damage, thorough débridement of necrotic tissue, and the taking of wide, deep sutures.     

Surgical management of coarctation of aorta with ventricular septal defect. Multivariate analysis.

Optimal management of coarctation with ventricular septal defect is difficult. Should one treat the coarctation, the ventricular septal defect, or both? This dilemma was investigated by reviewing 39 successive patients, aged less than 4 months, undergoing coarctation repair without pulmonary artery banding. Twelve hospital deaths occurred. Ventricular septal defect size was graded "blind" according to preoperative angiocardiographic and haemodynamic findings. Of 14 patients classified as having a large ventricular septal defect, necropsy and operative findings in eight showed defects 7 to 12 mm in diameter. Factors associated univariately with significantly increased mortality were young age, raised atrial and ventricular end-diastolic pressures, low weight, high admission blood urea, preoperative ventilation, and a large ventricular septal defect. All but the last two were also closely associated with each other. A jack-knifed discriminant function based upon ventricular septal defect size, blood urea, pulmonary venous oxygen content, and inferior caval oxygen saturation correctly predicted outcome in 78.9% of patients. Combinations of these four giving a probability greater than 0.9 of survival were rare with blood ureas above 8 mmol/litre. Raised blood urea was associated with low descending aortic pressure and subsequent dialysis. Only patients with a large ventricular septal defect stand to benefit from pulmonary artery banding at initial operation. Preoperative treatment to increase renal blood flow (prostaglandins, dopamine) may improve overall survival.     

Is it possible to operate four heart valves in a patient with heart failure,congenital heart disease and pulmonary hypertension?

We describe a 60-year old man with the history of radical correction of the Tetralogy of Fallot (TOF) in the year 1964. This patient has had a long lasting decompensation of a severe right heart failure with ascites and pulmonary hypertension. On echocardiography he had residual mild pulmonary stenosis (PS) and severe pulmonary and tricuspid regurgitation (TR), moderate aortic and mitral regurgitation. He also had residual ventricular septal defect (VSD) and severe pulmonary hypertension with the maximal gradient on TR 83 mmHg. He was considered unoperable by his cardiologist, however patient decided to undergo a high-risk operation. The operation comprised pulmonary and aortic valve replacement with bioprosthesis, mitral and tricuspid repair, closure of ventricular septal defect, bilateral MAZE and volume reduction of both atria. After a very complicated postoperative course with multiorgan failure he recovered and was discharged home 2 months after operation. The NYHA class improved from IV before operation to II. Twenty months after this operation he experienced infective endocarditis with a leak on the aortic bioprosthesis. He was reoperated with reimplantation of a new aortic bioprosthesis and with a very complicated postoperative course. Sildenafil was added to his therapy due to the persistent severe pulmonary hypertension. He survived and was discharged home.The presented unique high-risk complex reoperation of a congenital heart disease with pulmonary hypertension can be performed only by a very experienced cardiosurgical and cardio-anesthesiological team with a high level of the early and late postoperative care.     

Coronary artery fistula as source of pulmonary circulation in pulmonary atresia with ventricular septal defect

Four patients are described with pulmonary atresia and ventricular septal defect, in whom the pulmonary circulation was dependent on a fistula from the left coronary artery to the pulmonary artery. The issue in this complex anomaly is complete preoperative diagnosis, including anatomic information on the coronary artery fistula and the pulmonary vasculature. This was achieved in the last 2 patients. In the last patient echocardiography turned out to be an important diagnostic tool in this rare anomaly and facilitated selective angiocardiography. All 4 patients were successfully operated by closing the fistula, closing other aortopulmonary connections and inserting a valved conduit between right ventricle and pulmonary artery. The ventricular septal defect was closed in 3 patients with a patch. In the setting of an already existing pulmonary hypertension and a possibly inadequate pulmonary arterial system at surgery, a perforated patch was inserted in the ventricular septal defect of the remaining patient.