右外侧小切口剖胸矫治小儿先天性心脏病1972例

目的:总结右外侧小切口剖胸矫治先天性心脏病(先心病)的经验,探讨右外侧切口在先心病的应用与推广。方法 :2002年1月至2011年10月,本手术组经右外侧剖胸小切口完成1 972例小儿先天性心脏畸形矫治。其中男性1 143例,女性829例。年龄平均38.7个月(3～489个月),体质量平均11.6 kg(4.8～69 kg)。主要病种:室间隔缺损、房间隔缺损及法洛四联症等。结果 :术后并发症81例(4.1%):低心排出量综合征(低心排)26例(死亡3例)、严重肺部感染22例(死亡2例)、二次开胸止血8例(死亡1例)、多脏器功能衰竭4例(死亡1例)、一过性脑功能障碍5例、术后残余分流6例(经原切口再次手术1例)、右膈神经麻痹5例(1例膈肌折叠)、术后房室传导阻滞4例(1例置永久起搏器)、乳糜胸2例。随访3～108个月,3例法洛四联症存在残余梗阻26～50 mmHg(1 mmHg=0.133 kPa)观察中,1例二尖瓣成型术后大量反流行二尖瓣置换。结论:先心病可以在右外侧剖胸小切口下完成,该入路安全可靠、创伤小、暴露好并恢复快。     

389例先天性心脏病右外侧小切口剖胸心内直视手术

目的:总结经右胸外侧小切口体外循环下行先天性心脏病心内直视手术经验。方法:1996年11月至2011年12月,我科室应用右胸小切口完成各类先天性心脏病(先心病)的心内直视手术389例。年龄1.5～57岁,平均13.5岁。体质量7.6～68 kg,平均24.5 kg。手术切口后缘自右腋中线第3肋间处,向前下斜行达锁骨中线第6肋间处做8～12 cm弧形切口,第4肋间入胸。手术方式:房间隔缺损修补157例,室间隔缺损修补150例,部分房室隔缺损矫治8例;右心室流出道疏通术24例;法洛三联症矫治32例,法洛四联症矫治5例以及其他畸形矫治13例。随机选择同期常规手术(胸正中切口)患者100例作为对照。结果:研究组切口长度、手术时间及术后引流量都显著低于对照组;研究组术后呼吸机辅助时间明显低于对照组;术后监护时间各组间差异无统计学意义。研究组手术死亡4例(1.03%),2例死于术后脑栓塞,另外2例死于低心排出量综合征(低心排)。结论:右胸小切口手术入路,可安全有效地行常见先心病的矫治。该技术创伤小、恢复快、美观,并提高了患者的生活质量,值得进一步推广应用。     

右胸部小切口矫治先天性心脏病641例分析

目的探讨右胸部小切口在先天性心脏病心内直视手术中的临床应用评价。方法自1995年12月至2008年1月，共经右胸部小切口行先天性心脏病手术641例，其中男性347例，女性294例，经右胸前外侧小切口587例，右腋下小切口54例。选择同期正中切口心内直视手术治疗的先天性心脏病患者350例为对照组。结果小切口组手术切口长度（13．7±4．7）cm，术后呼吸机辅助时间（4．2±2．2）h，术后引流量（175±95）ml，术后输血量（350±101）ml，术后切口感染及胸骨畸形发生率均明显少于对照组（P〈0．05）。小切口组的手术死亡率（0．47％）、手术时间及术后住院时间和对照组相比差异无统计学意义。结论右胸部小切口适用于简单的先心病手术，具有切口隐蔽美观、术后渗血少、切口感染发生率低、无胸骨畸形等优点，但必须要选择适合的病例，应用必要的措施改善显露和熟练的手术技巧，才能取得良好的手术结果。     

右外侧小切口剖胸行婴幼儿法洛四联症根治术治疗体会

目的:总结0～3岁婴幼儿经右外侧小切口剖胸行法洛四联症(TOF)根治手术的结果及治疗体会。方法:回顾性分析2011年1月至2011年12月完成的0～3岁TOF根治术142例,经右外侧小切口剖胸进行的TOF根治术47例,经正中剖胸进行的TOF根治术95例,对比2组患儿的术前血氧饱和度、血红蛋白、McGoon比值,术中体外循环时间和主动脉阻断时间以及术后当日胸腔引流量、左心房压、正性肌力药物评分、呼吸机使用时间,监护室停留时间和术后住院时间。结果:经右外侧小切口剖胸进行的TOF根治术47例,其中跨环补片15例(占32%)。本组平均年龄(13.34±6.41)个月(5～34个月),平均体质量(9.58±1.17)kg(6～14 kg),术前血氧饱和度(87.57±9.02)%(69%～99%),McGoon比值2.06±0.48(1.11～3.36),术中体外循环时间(95.95±26.31)min(40～170 min),主动脉阻断时间(67.95±17.57)min(30～117 min),术后当日胸腔引流量(10～850 mL),中位数胸腔引流量110(80,167)mL,左心房压(9.19±2.12)mmHg(3～14 mmHg),正性肌力药物评分8.57±3.97(2～19),呼吸机使用时间(4～165 h),中位数呼吸机使用时间21.5(14.2,36.5)h,监护室停留时间(2.86±1.94)d(0～9 d)。术后因胸腔引流过多二次开胸探查2例。残余分流2例(2 mm)。与同期相匹配95例正中剖胸TOF根治术相比,术前临床资料无差异,术中体外循环时间和主动脉阻断时间无差异,术后右外侧小切口剖胸组监护室停留时间明显短于正中剖胸组,正性肌力药物评分及术后左心房压明显低于正中剖胸组。其余各项均与正中剖胸组无差异。结论:在熟练掌握手术技巧,严格手术适应证选择的基础上,右外侧小切口剖胸可以在TOF根治手术中应用。     

右胸前外侧小切口心脏不停跳治疗房间隔缺损

目的:总结右胸前外侧小切口治疗单纯房间隔手术经验。方法:右胸前外侧小切口体外循环心脏不停跳下行房间隔缺损ASD补片修补术。结果:术后早期并发症有右胸壁皮下气肿、活动性出血、Ⅲ°房室传导阻滞及右胸腔积液等。1例迟发性右膈肌麻痹,给予药物治疗后痊愈。全部患者均痊愈出院。结论:右胸前外侧小切口对于单纯性ASD是一个良好的手术途径,具有切口小、恢复快、术后疤痕小及平滑等优点。     

右腋下微创小切口在先天性心脏病手术中的应用

目的 总结右腋下微创小切口在先天性心脏病手术中的应用及体会.方法 经右腋下微创小切口剖胸矫治先天性心脏病194例,其中单纯室间隔缺损76例、房间隔缺损92例、房间隔缺损合并室间隔缺损16例、室间隔缺损合并右室流出道狭窄10例.结果 手术顺利,全组无死亡,无并发症发生.本组患者体外循环时间(48.7±29.5)min,术后机械通气时间(4.8±1.4)h,术后引流量(170.5±82.2)ml.结论 右腋下小切口剖胸具有损伤小、恢复快、切口隐蔽美观、不破坏胸廓连续性等优点,符合微创外科的原则.     

112例经右外侧小切口下行房间隔缺损修补术的体会

目的:通过分析右外侧小切口行房间隔缺损修补术的病例,探讨右外侧小切口在房间隔缺损修补术的应用与推广。方法:2011年1月至2011年9月,112例房间隔缺损修补术在右外侧小切口下完成。其中男性44例,女性68例。年龄平均58.7个月(6～489个月),体质量平均16.6kg(4.8～69kg)。20例合并心脏畸形如三尖瓣关闭不全,部分肺静脉异位引流,永存左上腔静脉。结果:术后2例出现早期血痰,1例出现神经系统并发症,1例引流偏多。均获得定期随访,随访时间3～12个月,无死亡,超声心动图示无残余分流,无胸廓畸形。结论:房间隔缺损修补术可以在右外侧小切口下完成,该入路安全可靠,创伤小,暴露好,恢复快及美观效果强。     

右外侧小切口剖胸矫治合并心底部畸形的室间隔缺损体会

目的:总结经右外侧小切口剖胸矫治合并心底部畸形的室间隔缺损(VSD)手术方法及临床疗效。方法:总结经右侧剖胸入路矫治合并简单心底部畸形(动脉导管未闭和/或永存左上腔静脉)的VSD 16例(R组),同期经右侧剖胸入路矫治不合并心底部畸形的VSD 144例(C组),R组在矫治心内畸形的同时,游离结扎动脉导管或游离阻断左上腔静脉。通过对比体外循环时间、主动脉阻断时间、术后机械通气时间、术后引流量、监护室时间以及术后住院时间,观察处理简单心底部畸形对手术的影响和治疗效果。结果:与C组相比较R组体外循环时间[(65.31±17.10)vs.(55.02±15.97)min P<0.05]明显延长,但主动脉阻断时间[(33.44±8.02)vs.(31.40±10.12)min]、术后机械通气时间4.25 hvs.13.08 h、术后引流量88.44 mL vs.89.83 mL、监护室时间2.38 d vs.1.95 d、术后住院时间[(7.88±1.82)vs.(7.88±2.30)d],差异无统计学意义。2组均无死亡及严重并发症,C组有8例患儿术后血痰,1例肺部感染;R组2例血痰。结论:经右外侧小切口剖胸入路矫治合并简单心底部畸形的VSD安全可行。     

经右腋下小切口及胸骨正中切口手术治疗先天性心脏病对比观察

体外循环心脏直视手术多采用胸骨正中切口。随着微创心脏外科技术的发展,许多小切口入路越来越多地用于治疗先天性心脏病。其中,右腋下小切口是常用的手术人路之一。2002年5月至2005年2月,我们对经右腋下小切口及胸骨正中切口手术治疗儿童先天性心脏病进行了对比观察。现将结果报告如下。     

右外侧小切口行法洛四联症根治术346例临床分析

目的:总结右外侧小切口剖胸行法洛四联症根治的经验及技术关键。方法:1997年1月至2013年10月,本手术组经右外侧小切口根治法洛四联症346例。其中男性159例,女性187例。年龄4个月~5岁;体质量6~15kg,平均体质量(9.7±2.4)kg。合并卵圆孔未闭43例、房间隔缺损22例、动脉导管未闭10例、永存左上腔静脉10例、主动脉瓣下隔膜7例、二尖瓣关闭不全1例。跨环补片205例,右心室流出道补片141例。结果:术中体外循环时间(90±24)min;主动脉阻断时间(64±17)min,术后机械通气时间4~165 h,监护室停留时间(3.2±1.7)d,术后当日胸腔引流量(138±91)mL,平均带胸管(2.5±0.9)d。术后并发症36例(1.04%):低心排出量综合征(低心排)17例(死亡5例),严重肺部感染2例(死亡1例),灌注肺5例(死亡1例),右肺损伤7例,膈神经损伤4例,室间隔缺损残余分流2例,乳糜胸2例。死亡共计7例,病死率2.02%。结论:经右外侧小切口行法洛四联症根治安全可靠。     

经右腋下微创直切口体外循环下直视心脏手术治疗小儿先天性心脏病

目的总结经右腋下直切口行体外循环直视心脏手术的临床经验,探讨其适应证及技术要点。方法2008年6月至2011年1月我们共完成79例经右腋下直切口的sJ,JL体外循环心脏直视手术,患者年龄6个月至12岁,平均（7．4±3.3）岁,体重6．5-42．0（17．8±8．7）kg。其中房间隔缺损19例,同期行三尖瓣成形5例;室间隔缺损55例,其中合并动脉导管未闭2例;部分房室管畸形5例。所有手术均在全身麻醉、体外循环下进行（房间隔缺损修补在不停跳下进行）。患者取左侧卧位,切口位于右侧第3肋间腋中线与第5肋间腋前线之间,皮肤切VI长度3．5-6．0（4．7±1．3）12111,经第3或第4肋间进胸,切开并悬吊心包,行升主动脉及上、下腔静脉插管建立体外循环,经主动脉根部灌注心脏停搏液,单纯房间隔缺损采用心脏不停跳下手术,经右心房切口修补房、室缺及三尖瓣成形。结果全组手术均顺利完成,无手术死亡;1例因术后心内膜炎致室缺残余漏,再次正中开胸行室缺残余漏修补术。体外循环时间（63．4±18．2）min,主动脉阻断时间（37．6±14-3）min,术后24h胸腔引流40-130（85．3±26．5）ml。68例患者术后未输血。全组无其他严重并发症。结论右腋下小切口直视心脏手术安全、可靠,可减少用血量,术后切口隐蔽美观,患儿及家属满意率高,值得临床椎广。     

Cardiovascular surgery in Turner syndrome-early outcome and long-term follow-up

BACKGROUND Cardiovascular disease is the leading cause of death in patients with Turner syndrome(TS), and cardiovascular surgery is frequently required for management of these patients. TS is associated with medical comorbidities than can complicate the care of this patient population.AIM To describe the cardiovascular surgical outcomes of patients with TS.METHODS A retrospective case series was compiled of 51 consecutive TS patients who had at least one cardiovascular surgery at Mayo Clinic Rochester from 1977-2017. The baseline clinical data of these patients were reviewed including demographics,medical comorbidities, congenital heart disease history, and medications.Echocardiographic reports were analyzed in detail. Operative reports and surgical hospital courses were reviewed. Long-term mortality was determined using medical records and the Social Security Death Index. Survival analysis was performed with the Kaplan Meier method.RESULTS The cohort comprised 51 TS patients, average age at the time of surgery at Mayo Clinic was 28(8-41) years, and 23(45%) patients were under the age of 18. At the time of first Mayo Clinic surgery, 18(35%) patients had previously undergone cardiac surgery at another institution. The most common procedures were repair of aortic coarctation in 14(28%) patients, aortic valve replacement in 6(12%)patients, and composite aortic root/ascending aorta replacement in 7(14%)patients, with 7 patients undergoing repair of more than one lesion. Aortic dissection required operative intervention in 5 patients. After initial Mayo Clinic surgery, subsequent operations were required in 6(13%) patients. Average hospital length of stay was 6 ± 2 d. There were 4(8%) early surgical deaths.Freedom from death was 97% and 89% at 10 and 20 years, and the freedom from reoperation was 93% and 81% at 10 and 20 years.CONCLUSION Cardiovascular surgery is associated with 8% early mortality given the medical complexity of TS patients. Those who survive to dismissal have good survival.Later cardiovascular reoperations are not rare.     

腹膜透析在小儿先天性心脏病术后的应用

目的:探讨腹膜透析在婴幼儿先天性心脏病(先心病)术后的应用。方法:13例行先心病手术的患儿术后因出现急性肾损伤(acute kidney injury,AKI)或低心排出量综合症(低心排)行腹膜透析。①总结手术时间、体外循环时间、主动脉阻断时间及腹膜透析持续时间。②比较透析前后SCr、乳酸(Lac)、中心静脉压(CVP)、尿量、血管活性药物评分及左心室射血分数。③根据死亡与否分为2组:死亡组;非死亡组,比较各组术后循环及肾功能转归情况。④根据急性肾损伤程度分组,比较SCr下降情况。结果:本组患儿手术时间90~420min,平均(206±90)min。8例患儿进行了体外循环,运转时间90~196min,平均(129±40)min,其中有7例患儿阻断升主动脉,并于主动脉根部灌入停跳液使心脏停跳,时间为52~126min,平均(85±24)min。腹透持续时间为72~360h,平均(138±80)h。腹膜透析后第7天Lac,CVP,正性肌力药平分(IS)降低,与透析前比较,P0.05,差异有统计学意义。肾损伤程度越重,SCr下降越不明显。透析后第3天和第5天AKI-3级患儿的SCr水平高于AKI-1级和AKI-2级患儿水平,P0.05,差异有统计学意义。结论:先心病手术后发生AKI或低心排时行腹膜透析可以排出体内多余水分及代谢产物,促进心功能恢复。肾损伤程度越重,SCr下降越不明显且恢复时间越长。     

单向活瓣补片在合并重度肺动脉高压先心病治疗中的应用

为探讨合并重度肺动脉高压先心病的有效治疗方法 ,采用单向活瓣补片对 30例本病患者进行手术治疗。术后 5例肺动脉压力下降明显 ,未出现跨单向活瓣补片分流 ;余 2 5例 72小时内出现分流 ,其中 1例术后 72小时死于低心排综合征 ,1例 4周死于右心衰竭 ;2 8例康复出院。对术后出现分流的 2 0例随访 3～ 4 1个月 ,其中术后 3个月仍出现跨单向活瓣补片分流 6例 ,右向左分流 3例 ,双向分流 3例 ;半年以上仍存在单向活瓣补片分流 4例 ,其中 3例肺动脉收缩压 (PP) /主动脉收缩压 (PS) >0 .75 ,2例右向左分流 ,1例双向分流 ;另 1例 PP/ PS降至 0 .4 5 ,存在 3mm跨单向活瓣补片左向右分流。 18例自觉症状及生活质量较术前改善明显。表明对于合并重度肺动脉高压的先心病应用单向活瓣补片行手术修补 ,允许右向左、阻止左向右分流 ,术后右心压力高于左心者右心血流可通过单向活瓣补片活瓣孔分流到左心系统 ,为右心起到泄洪减压作用 ,同时以可以耐受的体循环血氧饱和度降低为代价增加左心系血容量 ,保证左心排出量 ,有利于保持体肺循环相对平衡 ,使患者度过术后危险期 ,减少围术期死亡     

先天性心脏病手术后生长激素-胰岛素样生长因子轴的变化

目的 探讨先天性心脏病患儿手术后生长激素（growth hormone）和胰岛素样生长因子（insulin-like growth factor，IGF）轴的变化及意义。方法 先天性心脏病25例，采用放射免疫测定法测定治疗前、手术治疗后3个月、6个月和1年空腹血IGF-1及胰岛素样生长因子结合蛋白-3（insulin-like growth factor binding protein-3，IGFBP-3）;对照组为正常儿童20名。结果 先天性心脏病患儿手术前血IGF-1和IGFBP-3浓度较正常儿童减低（P〈0.01）;先天性心脏病手术治疗后患儿的IGF-1和IGFBP-3浓度随着术后时间增加而逐渐提高，至1年时与同龄儿童持平，差异无统计学意义（P〉0.05）。结论 先天性心脏病患儿存在生长发育迟缓及生长激素-胰岛素样生长因子轴功能的紊乱能通过手术治疗纠正，从而改善先天性心脏病患儿生长发育。     

An alternate technique to pacing in complex congenital heart disease: assessment of the left thoracotomy approach

BACKGROUND: Pacing in children with congenital heart disease often requires alternate approaches to standard transvenous pacing. The surgical approach used to implant the pacemaker leads has been shown to impact lead survival. There is a paucity of pediatric literature describing the experience using a left thoracotomy approach. OBJECTIVES: To report on short- and mid-term experiences with pacemaker implant via the left thoracotomy approach in children with complex congenital heart disease. METHODS and RESULTS: Data were abstracted retrospectively from patients' hospital charts. To date, the left thoracotomy technique has been used in 11 patients with complex heart disease, with a median of three prior cardiac operations. The median patient age was five years (range of two months to 23 years of age). The pacing indications were acquired postoperative atrioventricular block (n=5), sinus node dysfunction (n=5) and long QT syndrome (n=1). There were no intraoperative complications or long-term complications from this approach. The pacing thresholds at implant and follow-up were acceptable in all patients. One patient died in follow-up for reasons unrelated to the pacemaker or arrhythmia. CONCLUSIONS: The placement of epicardial pacemaker leads via the left thoracotomy approach is a safe and effective alternative to transvenous pacing in pediatric patients with complex congenital heart disease.     

Effect of prostaglandin E1 inhalation on pulmonary hypertension following corrective surgery for congenital heart disease

BACKGROUND:

Intravenous infusion of prostaglandin E1 (PGE1) has been used to treat pulmonary arterial hypertension (PAH); however, the efficacy and safety of inhaled PGE1 is unclear.

OBJECTIVES:

To investigate the effect of inhaled PGE1 on PAH following corrective surgery for congenital heart disease.

METHODS:

Sixty patients with postoperative residual PAH following corrective surgery for congenital heart disease were randomly assigned to a control group, a PGE1 infusion group (intravenous PGE1 infusion; 30 ng/kg/min daily for 10 days) or a PGE1 inhalation group (100 μg nebulized PGE1 every 8 h for 10 days). Systolic blood pressure, mean pulmonary arterial pressure, arterial oxygen pressure, oxygen saturation and serum endothelin-1 level were measured before and after the treatment.

RESULTS:

At the end of the study, the mean pulmonary arterial pressure in the two PGE1 groups were lower than in the control group (P<0.01), whereas the mean arterial oxygen pressure was higher (P<0.01). Compared with the PGE1 infusion group, the mean pulmonary arterial pressure in the PGE1 inhalation group was lower (P<0.01) whereas the arterial oxygen pressure was higher (P<0.01). The mean endothelin-1 levels in the two PGE1 groups were lower than in the control group (P<0.01), but there was no statistically significant difference in endothelin-1 levels between the PGE1 inhalation and infusion groups (P>0.05).

CONCLUSIONS:

In pediatric patients with PAH, PGE1 inhalation was associated with a reduction in pulmonary arterial pressure and improvement in arterial blood oxygen levels.     

Long-term natural history and postoperative outcome of double-chambered right ventricle—Experience from two tertiary adult congenital heart centres and review of the literature

Background

Double-chambered right ventricle (DCRV) is a rare form of congenital heart disease. Little is known about the outcome during adult life. Here we report the combined experience of two tertiary Adult Congenital Heart Disease Centres and systematically review the published literature.

Results

50 patients (32 female, median age 39 years [IQR: 27;53]) with DCRV under follow-up could be identified. A retrospective review of medical records was performed. Almost all patients (96%) had ventricular septal defects as underlying diagnosis. Eight patients remained completely asymptomatic during follow-up and have been managed conservatively thus far. The remaining patients developed symptoms at a median age of 26 years. Surgical correction was performed in 33 patients (median age at operation 27 years). No residual intraventricular gradient was present at the latest follow-up in 91% of operated patients and functional class improved significantly with only 6 patients remaining in NYHA class 2. There was no early or late operative mortality and no patient required re-operation for DCRV during a median follow-up of 8 years.

Conclusions

Contemporary adult DCRV patients have good survival prospects and low long-term morbidity. Despite occasionally presenting with considerable intraventricular gradients we could not identify any case of sudden death in our unoperated adult sub-population. Although asymptomatic adults may be encountered even with severe obstruction, symptom development is common during adult life. Cardiac surgery in this cohort is inherently low risk and offers good long-term haemodynamic and functional results justifying early intervention in consideration of the progressive nature of this rare congenital lesion.     

Abnormal right ventricular tissue velocities after repair of congenital heart disease--implications for late outcomes

BACKGROUND: Although repair of Tetralogy of Fallot (TOF) and transposition of the great arteries (TGA) has facilitated survival into adulthood, many survivors have residual haemodynamic abnormalities, including exercise intolerance and late right ventricular (RV) failure. METHODS: We studied 40 asymptomatic adult subjects (31.3+/-1.5 years) after congenital heart disease (CHD) surgery during childhood, using tissue Doppler echocardiography (TDE). We compared systolic (S') and diastolic (E' for early filling) RV and LV velocities, with 40 age matched controls (29.5+/-1.0 years). RESULTS: Both RV S' and E' velocities were significantly slower in the CHD group compared to controls (6.3+/-0.4 cm/s vs. 9.3+/-0.3 cm/s; 8.5+/-0.5 cm/s vs. 10.9+/-0.4 cm/s, respectively, p<0.001 for both). By contrast, LV S' and E' velocities were similar in both groups. Interestingly, in 50% of CHD subjects where RV function was reported as 'normal', both RV S' and E' velocities were significantly slower compared with controls (6.5+/-0.6 cm/s vs. 9.3+/-0.3 cm/s, p<0.0001 and 9.4+/-0.7 cm/s vs. 10.9+/-0.4 cm/s, p<0.05 respectively). CONCLUSIONS: RV S' and E' velocities are frequently abnormal in asymptomatic survivors of TOF and TGA repair, even where RV function appears 'normal'. Hence TDE during follow up may be a sensitive means of detecting pre-clinical abnormalities in RV performance.