小儿二尖瓣双孔成形术

目的 探讨二尖瓣双孔成形术治疗儿童特别是婴幼儿二尖瓣关闭不全的临床可行性及有效性.方法 对16例重度二尖瓣关闭不全的小儿行二尖瓣双孔成形术,并在术前、术中、术后利用超声心动图对瓣膜进行评价.结果 全组体外循环时间(74.4±23.3)min,主动脉阻断时间(51.3±17.1)min.术中及术后超声心动图检查提示,患儿二尖瓣口均无中度以上反流,无患儿须立即再次手术,术后常规使用血管活性药物,所有患儿均在术后24 h内脱离呼吸机.全组患儿均存活,无灌注肺、术后重度心衰等严重并发症发生.随访10个月～4年,所有患儿心功能(NYHA)为Ⅰ级,超声心动图显示双孔成形瓣膜活动良好,无瓣口狭窄,跨瓣压差均≤5 mmHg,术后14例无瓣膜反流、无左房增大,1例二尖瓣轻度反流,1例中度反流,无近远期死亡.术后常规行阿司匹林抗凝治疗3～6个月.结论 双孔瓣膜成形术治疗儿童重度二尖瓣关闭不全,修补后瓣膜形态稳定,早中期疗效满意,双孔成形术指数是一种简便而有效实用的参数.     

小儿二尖瓣关闭不全瓣膜成形术的临床疗效分析

目的回顾性分析二尖瓣成形术在治疗小儿二尖瓣关闭不全中的临床疗效。方法收集2012年1月至2019年1月南京医科大学附属儿童医院心胸外科202例采用二尖瓣成形术治疗的二尖瓣关闭不全(排除房室间隔缺损)患儿作为研究对象。采用超声心动图评价患儿手术前后左心室功能及二尖瓣反流程度的改善情况,分析随访结果。本组患儿中男童84例,女童118例,手术年龄为(21. 2±33. 3)个月,体质量为(9. 92±6. 75) kg。瓣叶活动正常型112例,瓣叶脱垂型54例,瓣叶活动异常型36例。所有患儿在中低温体外循环下行二尖瓣成形术(瓣叶修补、瓣环扩大、二尖瓣双孔成形等),体外循环时间(75. 89±51. 36) min,平均主动脉阻断时间为(45. 65±17. 89) min,合并其他心脏畸形者行同期矫治术。结果所有患儿中,无术中死亡病例;术后早期死亡5例;术后晚期死亡3例;术后多次行二尖瓣成形术15例,其中死亡1例。平均随访时间(19. 49±17. 48个)月。所有患儿行超声心动图评估手术前后左心室以及二尖瓣功能,其中左心室舒张末期内径(left ventricular end diastolic dimension,LVDD)、左房内径(left atrial dimension,LAD)、主肺动脉内径(main pulmonary artery dimension,MPA)与术前相比改善明显(P 0. 05)。术后1年心功能分级(New York Heart Association,NYHA)与术前相比亦有明显改善(P 0. 05)。结论二尖瓣成形术是一门综合技术,根据病情不同选择的不同方法可以很好纠正小儿二尖瓣关闭不全,临床疗效满意。     

儿童先天性二尖瓣疾病的外科治疗及中期随访研究

目的探讨儿童先天性二尖瓣疾病外科手术的疗效及中期随访结果分析。方法回顾分析2009年1月至2017年12月在复旦大学附属儿科医院心血管中心接受二尖瓣成形术的患儿92例。其中,二尖瓣狭窄患儿26例(二尖瓣狭窄组),二尖瓣关闭不全患儿66例(二尖瓣关闭不全组);男44例,女48例;手术年龄为(42.55±40.57)个月,范围为1个月至14岁,22.8%(21/92)患儿的年龄<1岁。结果4.3%(4/92)的患儿于手术早期死亡,3.3%(3/92)的患儿在随访期间死亡,总病死率为7.6%。术后所有患儿的心功能分级改善明显,Ⅲ级3例,余均在Ⅱ级及以上,随访时间为(42.72±29.66)个月,随访时间范围为3~104个月。随访期间11例患儿共接受了17次再手术,所有患儿的再手术率为12.0%(11/92)。二尖瓣狭窄组与二尖瓣关闭不全组在生存率及免除再手术率的Log Rank分析中,差异无统计学意义。所有患儿中<1岁的患儿较>1岁的患儿具有更高的病死率(P=0.045)及再手术率(P=0.039),差异均具有统计学意义。结论二尖瓣成形手术对于治疗先天性二尖瓣疾病的效果显著。但婴幼儿,特别是年龄<1岁的患儿,先天性二尖瓣疾病的修补手术仍面临着巨大挑战。虽然部分患儿需多次手术,瓣膜成形术仍是再手术时首要考虑的手术方式。     

儿童先天性二尖瓣疾病的外科治疗及中期随访研究

目的探讨儿童先天性二尖瓣疾病外科手术的疗效及中期随访结果分析。方法回顾分析2009年1月至2017年12月在复旦大学附属儿科医院心血管中心接受二尖瓣成形术的患儿92例。其中,二尖瓣狭窄患儿26例(二尖瓣狭窄组),二尖瓣关闭不全患儿66例(二尖瓣关闭不全组);男44例,女48例;手术年龄为(42.55±40.57)个月,范围为1个月至14岁,22.8%(21/92)患儿的年龄<1岁。结果 4.3%(4/92)的患儿于手术早期死亡,3.3%(3/92)的患儿在随访期间死亡,总病死率为7.6%。术后所有患儿的心功能分级改善明显,Ⅲ级3例,余均在Ⅱ级及以上,随访时间为(42.72±29.66)个月,随访时间范围为3～104个月。随访期间11例患儿共接受了17次再手术,所有患儿的再手术率为12.0%(11/92)。二尖瓣狭窄组与二尖瓣关闭不全组在生存率及免除再手术率的Log Rank分析中,差异无统计学意义。所有患儿中<1岁的患儿较>1岁的患儿具有更高的病死率(P=0.045)及再手术率(P=0.039),差异均具有统计学意义。结论二尖瓣成形手术对于治疗先天性二尖瓣疾病的效果显...     

儿童主动脉瓣瓣叶扩大成形术的近中期疗效分析

目的分析应用自体心包行主动脉瓣瓣叶扩大成形术治疗儿童主动脉瓣疾病的近中期效果。方法回顾性分析2017年9月至2021年6月在阜外华中心血管病医院行自体心包主动脉瓣成形术的26例主动脉瓣疾病(狭窄或关闭不全)患儿的临床资料, 收集近中期随访时资料, 比较术前和术后主动脉瓣最大跨瓣压差、主动脉瓣下反流面积、左心室舒张末期容积(LVEDV)和左心室射血分数(LVEF)等相关指标, 并采用配对t检验分析自体心包主动脉瓣扩大成形术的近中期疗效。结果 26例患儿手术均获得成功, 术后随访无死亡和严重并发症病例, 随访时间(22.96±6.45)个月。术后近中期主动脉瓣狭窄或狭窄合并轻中度关闭不全患儿中与术前主动脉瓣跨瓣压差比较, 术后1个月(t=7.85, P<0.05)、6个月(t=6.43, P<0.05)、1年(t=6.16, P<0.05)、2年(t=4.22, P<0.05)差异均有统计学意义;单纯主动脉瓣狭窄9例患儿随访结果, 术前舒张期主动脉瓣反流面积(8.87±3.57) cm2, 术后1个月、6个月、1年和2年主动脉瓣反流面积与术前比较差异均有统计学意义(...     

二尖瓣成行术后二尖瓣特征参数的三维量化分析

目的量化分析二尖瓣成形术前、术后二尖瓣特征参数,明确二尖瓣成形术后残留二尖瓣反流（MR）的发生机制。方法选取行二尖瓣成形术患儿30例,根据患儿术后MR改善程度,分为反流改善组与未改善组,量化分析和比较患儿二尖瓣装置三维参数术前、术后的改变。结果二尖瓣成形术后,MR改善组与MR未改善组相比,瓣环扩大程度均较术前改善,差异有统计学意义（P<0.05）;后瓣活动度幅度、前后组乳头肌至瓣环的空间三维距离、前后组乳头肌之间的空间距离、乳头肌与瓣环夹角、左室容量及射血分数等参数,MR改善组较术前明显改善（P<0.01）,而MR未改善组,除后瓣活动度外,其余参数术前、术后差异无统计学意义（P>0.05）。结论MR改善组患儿术后二尖瓣装置参数较术前有明显好转,而MR未改善组除二尖瓣环大小与瓣叶活动度较术前好转外,乳头肌位置、瓣下牵拉力、左室功能等无明显改变,而这些因素可能是术后残余反流的关键所在。     

小儿室间隔缺损合并二尖瓣关闭不全的外科治疗

目的探讨小儿室间隔缺损（VSD）合并二尖瓣关闭不全（MR）的外科治疗经验。方法26例VSD＋MR（部分合并肺动脉高压），平均年龄（4．1±1．2）岁，体重（1．6±1．7）kg，均在低温（22．5～26．5℃）、低中流量体外循环下行二尖瓣成形和心内其他畸形纠治。结果全组术后无一例死亡，术后左房平均压（PLA）为（1．3±0．2）kPa，肺动脉压力恢复至正常水平。结论小儿VSD伴MR应尽早行二尖辩成形术，其中大部分需行瓣环环缩，同时应注意监索和乳头肌的状况而予相应处理；停体外循环后即应行食道超声检查（ECHO）；术后早期必须控制液体的输入和避免过高的体循环压力。     

改良Carpentier法治疗Ebstein畸形

目的 探讨改良Carpentier法治疗Ebstein畸形的疗效与经验.方法 回顾性分析2003年6月至2009年10月本院手术治疗的18例Ebstein心脏畸形患儿的临床资料.18例均予改良Carpentier法手术矫治,在Carpentier法手术矫治的基础上,后瓣叶边缘对合缝在隔瓣叶或前瓣,以加大面积;同期矫治合并畸形.结果 17例存活,1例因术后并发心律失常持续低氧血症而死亡.存活患儿术后心功能Ⅰ级,均痊愈出院,其中1例术后出现Ⅲ°房室传导阻滞,3个月后转窦性心律.存活患儿均获随访,随访时间6～62个月,生长发育正常.结论 运用改良Carpentier法行三尖瓣成形术治疗Ebstein畸形,右心室功能恢复良好,效果满意,保全了右心室心尖至心底部的长度,是目前较为理想的手术方式.     

自体心包修复儿童主动脉瓣狭窄9例病例系列报告

目的：总结并分析以自体心包修复儿童先天性主动脉瓣狭窄的手术方法和早期预后。方法：纳入2013年7月至2015年6月在复旦大学附属儿科医院行自体心包主动脉瓣成形术的先天性主动脉瓣狭窄患儿,收集患儿的一般资料,围手术期情况,并发症,术前、术中、术后和随访时的超声心动图资料。结果：符合本文纳入标准的9例患儿进入本文分析,男6例,女3例,年龄4月龄至9岁。术前超声提示,三叶式和二叶式主动脉瓣分别为3例和6例;重度狭窄6例,中度狭窄1例,重度狭窄伴中度返流2例。9例均以自体心包再造或扩大主动脉瓣瓣叶。术后即刻与术前超声心动图比较：主动脉瓣最大跨瓣压差［（31.6±9.4）mm Hg vs（73.0±22.2）mm Hg, P=0.000］和主动脉瓣平均跨瓣压差［（15.8±18.3）mmHg vs （35.8±18.3）mmHg, P= 0.004］均下降。术后随访24～48（32±8）月,无死亡和再干预病例,未见严重不良事件,未见主动脉瓣重度狭窄或重度反流、升主动脉狭窄或扩张、主动脉瓣瓣环狭窄与扩张、瓣膜脱垂或瓣膜赘生物病例;3例患儿分别在随访18、24和12个月时出现瓣叶活动僵硬,瓣叶开放不完全。术后左室后壁厚径均呈下降趋势;末次随访时,8例患儿左室后壁厚径Z值（Z-LVPWd）均下降至正常水平（＜2）。结论：以自体心包修复主动脉瓣可改善先天性主动脉瓣狭窄患儿的血流动力学,手术风险低,术后早期疗效可,避免或延缓儿童主动脉瓣置换术,减少施行ROSS手术机会。     

小儿法乐四联症围术期的治疗策略

目的总结法乐四联症（TOF）患儿手术治疗经验,探讨手术时机、手术适应证和围术期处理。方法回顾性分析我科2004年1月至2010年6月收治的132例小儿TOF的临床资料,其中男98例,女34例;年龄4个月至12岁,平均12．85岁。术前均行超声心动图或心导管造影检查确诊,均在体外循环下行根治性手术,部分病例以自体心包或带瓣自体心包扩大右室流出道和（或）肺动脉,对合并畸形作相应处理。结果本组围术期死亡4例（3．03％）,其中2例死于术后多器官功能衰竭,2例死于低心排出量综合征。随访129例,随访时间12～90个月;失访3例。6例残余微量分流,生长发育正常,无不适。结论对症状显著的TOF患儿应尽早手术矫治,提高手术水平,加强术后监护是确保手术成功的关键。     

Postoperative mitral leaflet rupture in an infant with Loeys–Dietz syndrome

This report describes an infantile case of Loeys–Dietz syndrome (LDS) with spontaneous mitral leaflet rupture. The patient was diagnosed with a type B interruption of the aortic arch. Bilateral pulmonary artery banding was performed 5 days after birth. On the 53rd day, intra‐cardiac repair was performed without valvuloplasty. Although the operation was successful, mitral regurgitation deteriorated at 4 weeks after operation. On the 88th day, a mitral valvuloplasty was performed and a severely ruptured anterior leaflet was observed. Seven days after valvuloplasty, the mitral valve insufficiency again worsened and a fourth operation was performed. Two tears were observed in the anterior and posterior mitral valve leaflets, and a mitral valve replacement was required. Subsequently, the patient was diagnosed with LDS according to gene mutational status. LDS is known to have a poor prognosis with cardiovascular complications, but valve rupture has not been previously reported in other cases.     

Severe mitral insufficiency in mucopolysaccharidosis type III-B (Sanfilippo syndrome)

Summary A 6-year-old girl with mucopolysaccharidosis (MPS) III-B (Sanfilippo syndrome) who developed severe mitral regurgitation and congestive heart failure requiring surgery (valvuloplasty) is reported. One year after surgery the patient remains well, with marked improvement in her physical activity, and without signs of heart failure. This is only the second report of severe mitral regurgitation in MPS III, and is the first report of a successful repair (valvuloplasty) of a dysplastic mitral valve in the MPS. Mitral valvuloplasty should be considered instead of valve replacement in any MPS patient with mitral valve regurgitation requiring surgery.     

Percutaneous transvenous balloon mitral valvuloplasty: mid-term results in adolescents

Six patients with mitral valve stenosis underwent percutaneous balloon mitral valvuloplasty (PBMV) in our department between November 1992 and December 1997. Five patients had rheumatic mitral valve stenosis and one had congenital mitral valve stenosis and Eisenmenger's syndrome with patent ductus arteriosus (PDA). Functional status before PBMV was class IV in two patients, class III in two patients, and class II-III in two patients, as classified by the New York Heart Association (NYHA). The mean diastolic pressure gradient across the mitral valve measured during heart catheterization before and immediately after PBMV was 18.8 +/- 10.42 and 9.4 +/- 7.7 mmHg, respectively (p < 0.01). The patients were followed for a mean period of 36.6 +/- 8.5 months (range 12 to 72 months) after the procedure. During follow-up, post PBMV mean diastolic transmitral gradient measured by color Doppler echocardiography decreased from 19.3 +/- 11.16 to 7.43 +/- 7.3 mmHg (p < 0.01) and the mitral valve area increased from 1.09 +/- 0.7 to 3.1 +/- 0.9 cm2 (p < 0.002). Functional capacity showed improvement to NYHA class I in four patients, to class II-III in the patient with congenital mitral valve stenosis and Eisenmenger's syndrome with PDA and to class II in one patient with severe mitral valve calcification in whom restenosis occurred three years after PBMV. Percutaneous balloon mitral valvuloplasty PBMV can achieve very good short- and mid-term results in relieving symptomatic rheumatic mitral valve stenosis.     

A case of mitral papillary muscle rupture due to blunt chest trauma

Mitral regurgitation due to papillary muscle rupture after blunt chest trauma is uncommon. Sudden onset severe mitral regurgitation may lead to death due to heart failure if surgical repair is delayed. A previously healthy 12-year-old girl underwent splenectomy and chest tube insertion for pneumothorax after a traffic accident in a vehicle 15 days before. She was discharged from the hospital after a nine-day follow-up. She was presented to our hospital due to respiratory distress. On physical examination, an apical holosystolic murmur radiating to the axillary region was recognized. Transthoracic echocardiogram showed severe mitral regurgitation with freely moving posterior mitral chordae and prolapse of the posterior mitral valve leaflet. She received reimplantation of the complete ruptured posteromedial papillary muscle of the mitral valve. Her medical condition improved after the operation. On the postoperative echocardiogram, the left ventricular systolic function was normal with no mitral regurgitation.     

Valve Repair in Children with Congenital Mitral Lesions: Late Clinical Results

Mitral valve repair may be performed without ring support with advantages related to results and complications. The objective of this study was to analyze the long-term clinical results following surgical repair and reconstruction without the use of rings in cases of congenital mitral lesions in children less than 12 years of age. Twenty-one patients who had undergone surgery during the period from 1975 to 1998 were evaluated. The mean age was 4.6 +/- 3.4 years. Females represented 47.6% of the total. Mitral regurgitation was present in 57.1% (12 patients), stenosis in 28.6% (6 patients), and the mixed lesion group represented 14.3% (3 patients). Perfusion time was 43.1 +/- 9.5 minutes and ischemic time 29.4 +/- 10.5 minutes. Follow-up time was 41.5 +/- 53.6 months for the regurgitation group, 46.3 +/- 32.0 months for the stenosis group, and 39.41 +/- 37.51 months for the mixed lesion group. Echocardiographical follow-up time was 37.17 +/- 39.51 months for the regurgitation group, 42.61 +/- 30.59 months for the stenosis group, and 39.41 +/- 37.51 months for the mixed lesion group. Operative mortality was 9.5% (two cases). There were no late deaths. In the regurgitation group, 10 patients (83.3%) were asymptomatic (p = 0.004). In the echocardiographical follow-up, most of the patients had minimal regurgitation. In the clinical follow-up of the stenosis group all patients were in functional class I (NYHA). The mean transvalvular gradient measured by echocardiography was from 8 to 12 mmHg with a mean gradient of 10.7 mmHg. In the mixed lesion group there was one reoperation at postoperative month 43. There were no cases of endocarditis or thromboembolism. Mitral valve repair in congenital lesions is associated with good late results. The majority of cases in the regurgitation group remain asymptomatic and do not require reoperation. Rings or annular support are not necessary in such cases. Satisfactory repair is more difficult to achieve in cases of mitral stenosis due to valvular abnormalities and the seriousness of the associated lesions.     

婴幼儿原发性心脏肿瘤的诊断与治疗

目的:总结婴幼儿原发性心脏肿瘤的诊断和治疗经验。方法:回顾性分析2013年8月至2019年2月大连医科大学附属大连市儿童医院心脏中心收治的7例疑似原发性心脏肿瘤婴幼儿的诊治过程,患儿均行超声心动图等检查明确诊断,根据肿瘤的大小和位置选择治疗方案。出院后行随访观察。结果:7例患儿均经超声心动图诊断为原发性心脏肿瘤,其中2...     

Disappearance of a cardiac rhabdomyoma complicating congenital mitral regurgitation as observed by serial two-dimensional echocardiography

Summary A cardiac tumor was diagnosed by two-dimensional (2D) echocardiography in a four-month-old infant who also had congenital mitral regurgitation. At 18 months of age, histological examination of a biopsy specimen of the cardiac tumor, obtained during surgery for mitral valve replacement, revealed a rhabdomyoma. In serial postoperative 2D echocardiograms, this tumor decreased in size until total disappearance after 6 months.