婴儿室间隔缺损的外科治疗

目的探讨婴儿室间隔缺损外科治疗适应证、手术技巧以及围手术期处理要点.方法回顾总结作者自1997年1月至2000年1月连续70例婴儿室间隔缺损手术治疗的临床资料.患儿年龄3～12月(平均9月),体重4～10kg(平均7kg),全组手术在体外循环下进行,室间隔缺损位于膜周部57例、肺动脉瓣下8例、肌部流出道4例、膜周干下混合型1例.67例用补片修复,3例直接缝合.结果全组均存活出院,无重要的手术并发症,心功能和健康状况恢复良好.结论婴儿室间隔缺损的手术治疗安全,效果良好.室间隔缺损对心肺功能和健康已造成损害以及缺损难以自愈的患儿均应在婴儿期手术修复.     

右腋下剖胸小儿心内直视术

目的 :探讨右侧剖胸小切口的手术要点及手术指征。　　方法 :本组 10 2例患者经右腋下小切口剖胸及体外循环下行小儿心内直视术 ,其中房间隔缺损 2 4例 ,室间隔缺损 6 8例 ,法乐四联症 5例 ,室间隔缺损合并房间隔缺损 3例 ,右心室双出口、Ebstein畸形各 1例。合并畸形 :左上腔静脉 4例 ,右心室双腔及动脉导管未闭各 1例。　　结果 :全组患者无死亡。　　结论 :切口的正确选择是术野良好显露的前提 ,主动脉插管是手术成功的关键。该切口适用于大部分常见先天性心脏病的矫治。     

主动脉瓣下狭窄合并室间隔缺损、右室异常肌束的诊断和治疗

本文报告4例小儿主动脉瓣下狭窄伴室间隔缺损、右室异常肌束(或双腔右心室)的手术治疗和良好结果,结合文献对该症的病理生理、临床表现、诊断和手术等同题进行了讨论。     

双腔右心室的诊断和治疗

１９８８年１月至１９９４年３月，手术治疗双腔右心室３３例。其中合并室间隔缺损３１例，室间隔缺损伴肺动脉瓣狭窄及单纯性各１例。均在体外循环下行心内直视手术，效果满意。彩色多普勒超声心动图有助于本病的术前诊断。并从病理解剖学角度对右心室异常肌束进行讨论，提出诊断与手术要点。     

双腔右心室的手术治疗

本文报告5例小儿双腔右心室并存室间隔缺损的手术治疗结果。重点描述由这类异常肥厚肌束形成的心内病变表现。手术矫治须防止损伤室间隔和三尖瓣,避免残余分流。     

室间隔缺损合并主动脉瓣关闭不全的外科治疗

目的探讨室间隔缺损合并主动脉瓣关闭不全的外科治疗时机和手术方法。方法自1987年9月至2005年12月期间,我们共施行外科手术治疗室间隔缺损(VSD)合并主动脉瓣关闭不全(AI)87例,其中属动脉干下型室间隔缺损者60例、膜周型者27例。手术均采用全身中度低温体外循环。4例直接修补室间隔缺损,余均采取补片修补术。对主动脉瓣病变,施行瓣膜成形术31例,10例无法做者则施行主动脉瓣置换手术,其余46例单纯行室间隔补片修补术,对主动脉瓣未做特殊处理。结果手术效果满意。术后随访6个月至10年,全组病例无死亡,心功能Ⅰ级55例,Ⅱ级32例。结论对室间隔缺损合并主动脉瓣关闭不全的患者进行同期外科治疗可以取得良好的治疗效果。     

30例双腔右心室的外科治疗

目的 探讨过去10年我院双腔右心室的诊断及外科治疗经验。方法 回顾分析2006年1月至2016年7月我院接受外科治疗的30例双腔右心室的诊治情况。术前经超声心动图确诊27例,术中确诊3例。其中肌隔型18例,肌束型12例,单纯右室双腔心2例,合并室间隔缺损25例,房间隔缺损2例,动脉导管未闭2例,合并主动脉瓣下隔膜1例。手术予切除右心室异常肥厚肌束,修补室间隔缺损,未予补片加宽右室流出道8例,用自体心包加涤纶补片加宽右室流出道22例,同时矫治心内其他畸形。结果 手术早期死亡1例,为成人双腔右心室合并三尖瓣中－重度关闭不全,术前有右心功能不全,出现腹胀及双下肢浮肿等临床症状,术后出现低心排综合征,2天后死亡。其余患者手术效果满意,康复出院。死亡率为3.33%(1/30),生存29例,无近期或中期死亡及相关并发症。术后29例随访,随访率100%,主要以门诊及电话随访。随访3个月－10年,本组2例术前三尖瓣轻度关闭不全,术后早期心脏彩超提示三尖瓣轻-中度关闭不全,考虑术中损失乳头肌所致,通过门诊心脏彩超随访,目前三尖瓣仍为轻-中度关闭不全,无右心功能不全表现。右室流出道疏通满意,目前暂无患者因右室流出道狭窄而再次手术。全组患者术后心功能明显改善,活动量明显增大,生长发育改善。结论 严格把握手术指征,术前充分评估,熟悉掌握病理解剖,有效疏通右室流出道并充分矫治心内其他畸形,手术治疗近、中期效果满意,远期待进一步随访。     

食道超声引导经胸室间隔缺损封堵术的临床体会

【】 目的 总结食道超声引导经胸非体外循环下微创封堵术治疗室间隔缺损(VSD)的临床经验。方法 自2011年3月至2015年3月我院经食道超声引导室间隔缺损封堵术患者45例,男28例,女17例;年龄7个月～9岁(4.7±1.9)岁;体重4.9～25.6(13.5±5.8)kg。膜部VSD30例,嵴下型VSD6例,干下型VSD4例,肌部VSD5例。VSD直径4.7～11.5(5.8±1.2)mm。全组均采用胸骨下端3～5cm小切口进胸,术中食道超声引导下,送入导引钢丝及输送鞘管经右心室-VSD进入左心室,沿输送轨道送入封堵器完成VSD封堵术,术毕立即经食道超声全面评估手术效果。结果 全组43例封堵成功(95.6%),2例术中转为体外循环下常规手术(4.4%)。术后封堵器边缘少量残余分流2例(4.4%),3个月后复查消失。术后40例随访12个月(88.9%,40/45),无传导阻滞及主动脉瓣返流,无近期死亡。 结论 食道超声引导室间隔缺损封堵术是一种简单有效的治疗方法,多次操作后即可掌握操作要点,术中需结合超声对手术效果进行评估。     

新疆地区107例低体质量婴儿先天性心脏病的外科治疗

目的:探讨新疆地区体质量5 kg以下少数民族婴儿,先天性心脏病(先心病)的外科治疗方法及围术期处理。方法:2006年7月至2011年12月,本院手术治疗5 kg以下先心病患儿107例,年龄11 d至13个月,体质量2.6～5 kg。病种包括:动脉导管未闭(PDA)4例,室间隔缺损(VSD)和(或)房间隔缺损(ASD)54例,其中部分伴动脉导管未闭(PDA)和(或)肺动脉高压(PH),肺动脉瓣狭窄(PS)1例,法洛三联症3例,法洛四联症(TOF)9例,完全性心内膜垫缺损(ECD)9例,右心室双出口(DORV)9例,完全性大血管转位(TGA)8例(室间隔完整4例,室间隔缺损4例),完全性肺静脉异位引流(TAPVC)3例,主动脉弓缩窄(COA)并室间隔缺损、房间隔缺损3例,肺动脉闭锁(PA)2例,三尖瓣闭锁(TA)1例,房室连接不一致1例。本组中I期根治手术96例,减状手术11例(肺动脉环缩术6例,中心分流术4例,右心室流出道疏通术1例);体外循环下手术93例,其中深低温停循环3例。结果:本组术后早期死亡6例(5.6%),死于低心排出量综合征(低心排)1例,多脏器衰竭2例,呼吸衰竭2例,凝血功能障碍1例。术后主要并发症:低心排9例,多脏器损害5例,肾衰竭3例,肺部感染28例,心律失常3例,切口感染3例,心包积液2例,残余分流1例及乳糜胸1例。随访2个月～5年,81例无中期死亡,再手术3例。结论:严格评估低体质量先心病患儿手术适应证和时机,加强围手术期处理,是提高治疗效果的关键。     

室间隔缺损介入治疗的中远期疗效观察

目的评价介入方法治疗室间隔缺损的中远期效果。方法对采用国产室间隔缺损封堵器治疗的89例先天性心脏病室间隔缺损患者进行随访研究,比较术后和随访期间的室间隔残余分流、心律失常、右心室流出道狭窄、主动脉瓣关闭不全和三尖瓣关闭不全的发生率。结果 89例患者无死亡、脑梗死、封堵器脱落或移位发生;术后和随访期间的室间隔残余分流、心律失常和右心室流出道狭窄的发生率分别是14例(15.73%)、10例(16.85%)、10例(11.24%)和1例(1.12%)、4例(4.49%)、3例(3.37%),两组差异有统计学意义(P<0.05),而术后和随访的主动脉瓣和三尖瓣关闭不全的发生率分别是5例(5.62%)、15例(16.85%)和3例(3.37%)、9例(10.11%),两组差异无统计学意义(P>0.05)。结论应用介入方法治疗先天性心脏病室间隔缺损的中远期疗效良好,无严重并发症发生,但术后三尖瓣关闭不全的发生率较高,应引起大家的重视。     

小儿双腔右心室的外科治疗（附10例报告）

本文报告了１９９２年收治的１０例双腔右心室病人，男性９例，女性１例。年龄３～１２岁，平均７岁，均合并有其它心内畸形，包括房缺、室缺、肺动脉瓣狭窄等。术前明确诊断双腔右心室者８例，２例误诊为法鲁四联症及单纯室间隔缺损。１０例病人全部经右心室切口切除异常肌束，疏通右室流出道，４例用牛心包补片扩大流出道。术后９例恢复良好，１例死于脑部并发症。作者在文章中描述了双腔右心室的病理解剖学特征，强调术前诊断和术中识别本病的重要性。同时，就手术时机、手术方法及术中应注意的重要问题进行了讨论。     

Color flow Doppler in the diagnosis of double-chambered right ventricle: a demographic and echocardiographic study

The purpose of this study was to evaluate the demographic and echocardiographic data of patients diagnosed with double-chambered right ventricle and attempt to explain a perceived rise in the incidence. Definition: Double-chambered right ventricle (DCRV) is a division of the right ventricle into two chambers by a hypertrophied muscle bundle. Methods: The medical records of patients diagnosed with DCRV were reviewed, and demographic, echocardiographic, and catheterization data were tabulated. Annual incidence of DCRV, based on year of birth, was compared to yearly detection rate, based on year of DCRV diagnosis. To evaluate the influence of color flow Doppler on the frequency of diagnosis of DCRV, demographics of patients born prior to September 1986 (when utilization of color Doppler began in our institution) were compared to those born after that date. Results: Despite an unchanged annual incidence of DCRV, yearly detection rate of this lesion rose significantly following the introduction of color flow Doppler to our institution (September 1986). DCRV was diagnosed earlier and was accompanied by earlier catheterization, which also showed lower right ventricular body gradients after September 1986. Associated anomalies, both cardiac and noncardiac, in our population differed from those reported in previous series. Conclusion: This study infers that the advent of color flow Doppler significantly enhanced the diagnosis of DCRV in our pediatric patients and led to a perceived rise in incidence.     

A Case Report of Double‐Chambered Right Ventricle Associated with Subaterial Ventricular Septal Defect and Rupture of Right Coronary Sinus Aneurysm

Double‐chambered right ventricle (DCRV) is a rare congenital heart disease in which the right ventricle (RV) is divided into two chambers by anomalous muscle bundles. Here, we report a case of DCRV associated with subarterial ventricular septal defect (VSD) and rupture of right coronary sinus aneurysm (RCSA); the patient was diagnosed by echocardiography and later confirmed by surgical operation.     

双腔右心室的术前与术中诊断对比分析

对１７ 例双腔右心室（ＤＣＲＶ） 患者于术前行经彩色多普勒血流显像（ＣＤＦＩ） 检查, 诊断为单纯ＤＣＲＶ４ 例, ＤＣＲＶ 合并室间隔缺损 （ＶＳＤ） ８ 例、肺动脉瓣狭窄 （ＰＳ） １ 例、房间隔缺损（ＡＳＤ） １ 例, 单纯ＶＳＤ２例, 法乐氏四联症１ 例。均经手术治疗, 术中确诊为单纯ＤＣＲＶ４ 例, ＤＣＲＶ合并ＶＳＤ１１ 例、ＡＳＤ１例、ＰＳ１ 例。认为ＣＤＦＩ对ＤＣＲＶ的诊断有较高的准确性, 但对不典型ＤＣＲＶ的诊断仍有一定困难。     

16例双腔右心室合并室间隔缺损的手术治疗

双腔右心室（ＤＣＲＶ）多合并室间隔缺损（ＶＳＤ），本文对ＤＣＲＶ合并ＶＳＤ的发病、病理、手术矫治进行讨论。强调流出道疏通要彻底，ＶＳＤ修补要确切，避免误将右心室狭窄孔当作ＶＳＤ修补。     

Clinical, echocardiographic, and angiographic profile of patients with double-chambered right ventricle: experience with 48 cases

Forty-eight patients with double-chambered right ventricle (DCRV) were prospectively studied over a 3-year period. Clinical, echocardiographic, hemodynamic, and angiographic studies were done in all. Three patients were studied postoperatively also. Fifteen patients underwent surgery confirming the diagnosis. Echocardiography detected anomalous muscle bundles (AMBs) in 39/41 patients in whom this study was carried out. Angiographically AMBs were best demonstrated in anteroposterior views in a systolic frame. Common associated anomalies found with DCRV were ventricular septal defects (69%), pulmonary valve stenosis (38%), and double-outlet right ventricle (10%).     

The right ventricular pouch: a proposed explanation for the electro-vectorcardiographic pattern of double chambered right ventricle

The presence of a specific deformity of the right ventricle in association with the double-chambered right ventricle (DCRV) has been discovered in 8 of 14 individuals undergoing detailed study for DCRV. Investigational study employing multiple angiographic projections with selective angiography has allowed precise morphological identification of this deformity as a contractile pouch located arising from the outflow tract of the right ventricle. Comparison of the angiographic data with the electro-vectorcardiographic findings of DCRV suggests that these highly specific electrical features of DCRV correlate with the position and location of the contractile pouch.     

Not all obstructive cardiac lesions are created equal: double-chamber right ventricle in pregnancy

Double-chambered right ventricle (DCRV) is a rare form of right ventricular outflow tract (RVOT) obstruction accounting for approximately 1% of patients with congenital heart disease. It consists of an anomalous muscle bundle that divides the right ventricle usually between the sinus (inlet) and the infundibulum (outlet). This division creates a proximal chamber with high pressure and a distal chamber with low pressure. The hemodynamic obstruction of the RVOT is usually an acquired phenomenon, however the substrate for the anomalous muscle bundle is likely congenital. The diagnosis of DCRV should be considered in the young patient with an elevated right ventricular systolic pressure and intracavitary gradient. Echocardiography and cardiac MRI are the principal diagnostic tools for the assessment of DCRV. This entity is often misdiagnosed as pulmonary hypertension in the young patient, and can often go overlooked and untreated for many years. Definitive therapy involves surgical resection of the muscle bundle. This can often be curative and if done in a timely fashion, may prevent right ventricular remodeling. We describe the unique diagnostic dilemma, the course and management of a young adult with DCRV during pregnancy.     

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.     

Pulmonary valve-moderator band distance and association with development of double-chambered right ventricle

Double-chambered right ventricle (DCRV), a form of right ventricular outflow obstruction that sometimes accompanies a ventricular septal defect (VSD), is associated with superior and rightward displacement of the septal insertion of the moderator band. It was hypothesized that this superior displacement is present and identifiable by echocardiography in patients with a VSD even before right ventricular outflow tract obstruction develops. Eight patients who had a previous echocardiographic study showing a VSD alone were echocardiographically diagnosed as having DCRV. Their initial echocardiographic studies were reviewed, and superior displacement of the moderator band was quantified by measuring the distance between the pulmonary valve and moderator band, normalized to tricuspid anulus diameter. These measurements were compared with those from the initial studies of the following 3 other groups: (1) an age-matched group of 10 patients with no structural heart disease; (2) an age-matched group of 10 patients with a VSD who did not develop DCRV; and (3) a group (not age-matched) of 10 patients with VSD and DCRV in whom subpulmonary obstruction was present on the initial study. The 8 patients who eventually developed subpulmonary obstruction had significant superior displacement of the moderator band at the time of their initial echocardiogram compared with that of the 2 age-matched control groups (p < 0.01). In contrast, there was no significant difference in moderator band displacement between these patients and the 10 with DCRV who already had right ventricular outflow obstruction at their initial study (p = not significant).

Superior displacement of the moderator band is quantifiable by 2-dimensional echocardiography and may help to predict which infants with a VSD are at risk to develop subpulmonary obstruction.