房室间隔缺损矫治术后二尖瓣功能的长期随访

目的　探讨房室间隔缺损 (AVSD)矫治术后二尖瓣功能的远期转归。方法　收集 1992年 10月至 2 0 0 3年3月在我院手术的AVSD患儿 135例 ,男 6 0例 ,女 75例。年龄 :11个月至 2 3岁 (平均 10 3岁± 5 7岁 ) ,手术年龄 :2个月至 14岁 (平均 5 9岁± 4 2岁 ) ,体重 :3 5～ 5 6 0kg(平均17 3kg± 9 7kg)。完全型房室间隔缺损 (CAVSD) 32例 (占2 3 7% ) ,不完全型房室间隔缺损 (PAVSD ) 82例 (占6 0 7% ) ,过度型房室间隔缺损 (TAVSD) 2 1例 (占 15 6 % )。局限二尖瓣返流 (MR) 9例 (占 6 7% ) ,轻度MR 4 5例 (占33 3% ) ,中度MR 5 5例 (占 4 0 7% ) ,重度MR 2 6例 (占19 3% )。应用彩色多普勒超声心动图对二尖瓣功能进行长期随访。随访时间 :6个月至 10年 (平均 4 3年± 2 7年 )。结果　全组术后无二尖瓣狭窄发生。术后MR减轻 4 7例(占 34 8% ) ,维持术前状态 79例 (占 5 8 5 % ) ,加重 9例 (占6 7% )。随访中 ,术前轻度MR者 ,13%于术后 6个月内进展为中度 ;术前中度MR者 ,14 %于术后 6个月内进展为重度 ;术前重度MR者 ,15 %于术后 1个月内进行性加重。大部分MR术后维持稳定状态。中、重度MR患儿需长期口服强心、利尿及血管扩张剂。 4例重度MR伴顽固性心力衰竭分别于术后 0 5、2、3及 3 5年行二     

经胸房间隔缺损堵闭术对房室瓣反流的影响

目的探讨经胸房间隔缺损封堵术对房室瓣反流的影响。方法回顾性分析2002年1月至2011年3月在南方医科大学珠江医院经胸微创房间隔缺损堵闭术患者的临床资料,其中资料完全者43例,40例在食道超声、2例在经胸超声辅助下行房间隔缺损堵闭术。患者术前、术后1个月及6个月经超声心动图检查,观察心脏各指标的变化和房室瓣反流程度。结果41例手术成功,手术成功率95．3％（41／43）;1例术中改为右侧开胸小切口体外循环下房间隔缺损修补术,1例术中并发心搏骤停。1例术后并发肾功能衰竭：12例术后即时有少量残余漏,1个月后超声复查消失。术后超声随访显示：右心室、右心房直径较前缩小,左心室直径较前增大,肺动脉瓣血流速度明显降低,差异有统计学意义（P〈0．05）;室间隔厚度、二尖瓣血流速度、主动脉瓣血流速度无明显改变,差异无统计学意义（P〉0．05）。房间隔缺损堵闭术后1个月、6个月,二尖瓣瓣膜反流程度较术前加重,差异有统计学意义（平均秩次：2．01VS．2．17vs1．77,x2=10．78,P=0．04）;而三尖瓣的瓣膜反流程度术前与术后1个月、6个月比较,差异无统计学意义（平均秩次：1．88vs2．11US．2．01,X2=4．23,P=0．134）。结论房间隔缺损封堵术后,可引起二尖瓣反流程度的加重,但对三尖瓣的反流程度近期影响不明显;二尖瓣中度以上或三尖瓣重度反流的患者或不适宜行单纯房间缺损封堵术。     

Percutaneous edge‐to‐edge repair for common atrioventricular valve regurgitation in a patient with heterotaxy syndrome,single ventricle physiology,and unbalanced atrioventricular septal defect

Congenital heart disease patients, specifically with unbalanced atrioventricular septal defects and common atrioventricular valves requiring single ventricle palliation, have substantial morbidity and mortality. Atrioventricular valve regurgitation (AVVR) is associated with poor outcomes in single ventricle patients, and many of them require surgical treatment of AVVR in their lifetimes. We describe a unique case of transcatheter edge‐to‐edge valve repair using the MitraClip system (Abbott, Chicago, IL) in a single ventricle patient with severe common AVVR.     

部分型房室间隔缺损外科治疗的临床总结

目的 总结部分型房室间隔缺损手术治疗的经验,以提高治疗效果.方法 回顾性分析32例部分型房室间隔缺损患者的治疗经过,以缝合瓣裂、Reed法环缩、运用成形环等方法矫正房室瓣关闭不全,分别采用Kirklin法和McGoon法修复原发房间隔缺损.结果 术后早期死亡1例,死亡率3.13％.McGoon法修补发生Ⅲ度房室传导阻滞2例,而Kirklin法则无一例发生.26例术后随访6个月至5年,术后3年内心功能均有改善.结论 根据二尖瓣畸形的病变解剖差异,选择个体化处理方法,采用Kirklin法修补原发孔型房间隔缺损更为安全可靠.     

Stenotic double-orifice mitral valve after surgical repaired partial atrioventricular septal defect

Double-orifice mitral valve (DOMV) is an uncommon congenital anomaly account for 1% of congenital heart disease. However, accurate diagnosis and evaluation of valve stenosis or regurgitation and other concomitant congenital anomalies due to DOMV are required to obtain suitable treatment. Two- and three-dimensional echocardiography can contribute valuable functional and anatomic information that can support to reach this goal. Here, we present a case of complete bridge-type DOMV that causes mitral stenosis after surgical repair of the partial atrioventricular septal defect in childhood.     

Added value of three‐dimensional transthoracic echocardiography in assessment of an adult patient with atrioventricular septal defect

Transthoracic echocardiography is the principal imaging modality for assessment of patients with atrioventricular septal defects. Three‐dimensional echocardiography streamlines and simplifies data acquisition offering a unique realistic en‐face display of heart valves and septal defects and enables accurate evaluation of the cardiac anatomy, dynamic, and function. We demonstrated an added value of three‐dimensional echocardiography in assessment of an adult patient with atrioventricular septal defect and its advantages over conventional echocardiography.     

Atrial septal defect repair gone wrong

Isolated atrial septal defect (ASD) accounts for 13% of congenital heart disorders. The anatomic location, size, and coexistence of other cardiac anomalies determine outcomes of repair. Surgical closure was the first‐choice treatment until the 1990s and remains the only treatment for large defects. We describe a case of a 64‐year‐old woman who underwent surgical repair for an ASD as a child in 1959. She presented with dyspnea to the hospital almost 53 years after the surgery. Diagnostic cardiac imaging revealed interesting anatomy of the repair surgery. Transthoracic echocardiography showed areas of flow signal across the patch consistent with surgical perforation of the patch to reduce symptoms of superior vena cava (SVC) syndrome. Despite intervention, severe dilation of the SVC along with a thrombus is seen. CT angiography of the heart showed the ASD patch occluding the ostium of the SVC instead of patching the ASD. Transesophageal echocardiography showed malpositioned patch allowing the sinus venosus ASD to remain patent.     

室问隔缺损介入术后并发三度房室阻滞

目的分析国内室间隔缺损患者介入封堵术后三度房室阻滞的发生特点和原因。方法通过中国生物医学文献数据库和清华同方医学期刊数据库文献检索系统,以“室间隔缺损”和“传导”为任意字段进行检索,在纳入的文献中,重点对患者年龄、性别、术后三度房室阻滞的发生时间、恢复时间、处理措施等进行总结分析。结果总计纳入23篇文献,共报道了73例术后出现三度房室阻滞的患者,女性和儿童多见。88％的三度房室阻滞发生于术后3～7d。80％的病例均在症状出现后的7—15d恢复窦性心律。有3例患者植入了永久起搏器。结论室间隔缺损介入术后的三度房室阻滞有其发生发展的规律,多数为一过性,需植入永久性起搏器的患者极少。     

房间隔缺损经导管介入封堵术发生房室传导阻滞的分析

目的:分析房间隔缺损（ASD）经导管介入封堵术发生房室传导阻滞（AVB）的情况。方法:521（男189,女332）例,年龄1.0～65（27±19）岁住进我科行ASD封堵术的患者,对封堵术后发生AVB的患者进行分析。结果:13（男5,女8）例（2.5%）封堵术后发生AVB,年龄4～44（23±12）岁。其中2例在发生AVB后撤出封堵器,其余11例均成功植入封堵器。3例为Ⅰ°AVB,4例Ⅱ°ⅠAVB,4例Ⅱ°ⅡAVB,2例Ⅲ°AVB。7例植入的封堵器在38mm以上;3例4～8岁幼儿,植入的封堵器在22～28mm;1例为15岁青少年,所植入封堵器为34mm;另2例为成人,植入的封堵器分别为30mm和34mm。所有患者AVB均恢复正常。结论:ASD介入封堵术有发生AVB的可能,AVB多见于植入大型号封堵器的患者,多数AVB是能够恢复的。     

完全性房室隔缺损的手术治疗(附36例报告)

目的探讨完全性房室隔缺损的治疗方法。方法在中度低温体外循环下采用双补片法修复36例完全性房室隔缺损。结果手术死亡2例,余患者术后均恢复为窦性心律,无完全性房室传导阻滞。复查心脏彩超二尖瓣轻度反流8例,中度反流2例。结论完全性房室隔缺损应尽早手术。双补片法效果较好。术中应注意预防左室流出道狭窄和矫治二尖瓣关闭不全。     

双心室矫正法洛四联症合并完全性房室间隔缺损的手术技巧

目的:评价双心室矫治法洛四联症合并完全性房室间隔缺损(TOF/CAVSD)的效果。方法:2007年1月至2012年12月,共行TOF-CAVSD解剖矫正术16例,年龄1~13岁,平均3.3岁,体质量7~36kg,平均9.1kg,其中6例为Down综合征;16例均采用右心房、右心室纵切口,二片法补片修补房室间隔缺损,右心室流出道加宽补片或外管道。结果:术后早期无死亡,无因为室缺残余分流、左侧房室瓣反流和右心室流出道梗阻而再手术。随访6个月至5年,无晚期死亡及再手术;NYHA心功能Ⅰ级或Ⅱ级。结论:双心室法矫正TOF-CAVSD效果良好。     

Late complete atrioventricular block after closure of an atrial septal defect with a gore septal occluder (GSO™)

Temporary intermittent complete heart block (CHB) occurred the day after interventional closure of an ASD with a 30 mm Gore Septal Occluder (GSO?) in a 2 years and 11‐month‐old female. CHB disappeared without further treatment and stable sinus rhythm recovered within 3 days. Only short episodes of 2nd degree AV‐block (Wenckebach periodicity) at rare intervals were documented in Holter‐monitors the following 2 months. Eleven months after device implantation the patient suffered from long lasting episodes of CHB. Surgical removal of the device resulted in incomplete recovery of AV‐conduction. Histopathological work‐up of the explanted GSO showed complete endothelialization of the device and regular scar formation. One year after surgery, the child had sinus rhythm during daytime but needed VVI‐pacing while sleeping. Young age, inferior localization of the defect, and use of a large device have been individual risk factors for CHB in this patient. Clinical course and histologic findings indicate that mechanical compression was the only cause for CHB. The cumulative number of reports of CHB after use of different ASD‐devices supports the recommendation to postpone the intervention in asymptomatic patients to preschool‐age. Early removal of a pushing device may increase the chance of complete recovery from CHB. © 2015 Wiley Periodicals, Inc.     

Real-time three-dimensional echocardiography is useful in the evaluation of patients with atrioventricular septal defects

OBJECTIVE: We sought to determine whether three-dimensional echocardiography (3DE) is useful in the evaluation of patients with atrioventricular septal defect (AVSD). BACKGROUND: Recent advances in 3DE have enhanced its practicality. We assessed whether 3DE provided new information compared to 2DE among patients with AVSD. METHODS: We retrospectively reviewed 52 3DE datasets from 51 patients (median age: 4.6 years, range 0-30 years; median BSA: 0.6 m2, range 0.2-1.9 m2) with any type of AVSD during a 1-year period. 3DE findings were compared to 2DE and surgical reports. For each study, AVSD was classified by 2DE as one of the following: unrepaired balanced defect, repaired balanced defect with residual lesions, repaired balanced defect without residual lesions, or unbalanced defect. 3DE was graded as (1) Additive: 3DE resulted in a new finding or changed diagnosis; (2) Useful: While useful, 3DE did not result in new findings or changed diagnosis; or (3) Not useful. RESULTS: 3DE on unrepaired balanced AVSD and repaired AVSD with residual lesions was more often additive/useful (33/36; 92%) than on repaired AVSD without residual lesions or unbalanced AVSD (9/16 (56%), P=0.009). 3DE was additive or useful in all three patients with unbalanced AVSD being considered for biventricular repair. Useful information obtained by 3DE included: precise characterization of mitral regurgitation and cleft leaflet, substrate for subaortic stenosis, valve anatomy, and presence and location of additional septal defects. CONCLUSION: 3DE provides useful and additive information in unrepaired balanced AVSD, repaired AVSD with residual lesions, and unbalanced AVSD under consideration for biventricular repair.     

Near miss sudden cardiac death on a young patient with repaired atrioventricular septal defect

Patients with congenital heart disease often face the prospect of long-term haemodynamic or arrhythmic complications for which lifelong follow-up in specialist adult congenital heart disease (ACHD) centres is required. We describe the case of a 25-year-old man with repaired atrioventricular septal defect who was referred to our centre after a ventricular fibrillation arrest. Serial echocardiograms in previous years had shown progressive severe left ventricular outflow obstruction, but the patient had not been operated on as he was deemed asymptomatic and reluctant to consider surgery. Management and criteria for further intervention in ACHD patients often differ from those of patients with acquired heart disease and reliance on symptoms alone is not good practice and may prove catastrophic.     

室间隔缺损介入封堵术中及术后房室传导阻滞的转归与防治

目的探讨经导管介入封堵治疗室间隔缺损(VSD)术中及术后房室传导阻滞(AVB)的发生、发展、转归与防治。方法2005年3月至12月,共行VSD介入封堵术157例,术中及术后发生Ⅱ度及以上AVB8例。其中术中发生Ⅲ度AVB2例,术后予静脉注射糖皮质激素治疗;术后发生Ⅱ度AVB3例,Ⅲ度AVB3例,发生时间为术后4h至术后第8天,其中2例伴阿-斯综合征,1例给予临时起搏治疗,其余给予静脉注射糖皮质激素、利尿、脱水等治疗。结果2例术中发生Ⅲ度AVB的患者术后未再出现AVB;3例术后发生Ⅲ度AVB的患者分别于术后第7、8、18天恢复窦性心律,但有2例出院后再次出现Ⅲ度AVB,1例经转回我院积极治疗后恢复窦性心律,另外1例因院外治疗不及时,未能恢复;3例Ⅱ度AVB分别于术后第5、7、8天完全恢复,期间均出现Ⅱ度Ⅰ型和Ⅱ度Ⅱ型交替现象。结论AVB是VSD介入封堵术中及术后的常见并发症。对于AVB应积极治疗,若治疗不及时可能会转为永久性AVB。改进和提高导管技术及操作方法,可在一定程度上减少AVB的发生。     

完全房室间隔缺损心内矫正术的麻醉处理

目的总结完全房室间隔缺损心内矫正术的麻醉处理。方法对6例3～10岁完全房室间隔缺损心内矫正术患儿实施中小剂量舒芬太尼、咪哒唑仑并辅以七氟醚吸人维持麻醉，术中应用血管活性药物和降低肺血管阻力药物，并采用碱化血液、低血细胞比容、适宜的吸入氧浓度（FiO2）、维持正常的功能残气量和经食管超声等方法。结果脉搏氧饱和度（SpO2）由术前平均（91．5±4．6）％升到（99．3±0．5）％；碱剩余（BE）由术前平均（-4．667±3．559）升到（0．667±2．875），差异有统计学意义（P〈0．05），全组无一例死亡。结论适宜的麻醉深度、循环功能的维护、维持低状态肺血管阻力、加强呼吸管理、及时和适度补充容量等措施是维持平稳麻醉的关键，尤其值得注意的是经食管超声的应用。     

Double aortic arch, tetralogy of Fallot with pulmonary atresia and atrioventricular septal defect.

A double aortic arch is usually an isolated abnormality. We describe a case with a previously undescribed combination of tetralogy of Fallot with pulmonary atresia, complete atrioventricular septal defect, and left patent arterial duct in association with a double aortic arch. A complete diagnosis was made by echocardiography. Meticulous suprasternal echocardiography must be employed to avoid overlooking an unsuspected aortic arch abnormality.     

房室间隔缺损的心电图分析

目的 探讨心电图诊断对房室间隔缺损的临床应用价值。方法对照分析120例房室间隔缺损患儿(部分型、过渡型、完全型各40例)和177例其他先天性心脏病(紫绀型57例、房间隔缺损61例、室间隔缺损59例)(对照组)的心电图表现。结果房室间隔缺损患儿同时出现P-R间期延长、不完全性右束支传导阻滞、左前分支阻滞的阳性率明显高于对照组，但3种不同类型的房室间隔缺损间心电图表现差异均无显著性意义。结论心电图同时出现P-R间期延长、不完全性右束支传导阻滞和左前分支阻滞高度提示房室间隔缺损存在，结合临床可作出初步诊断，但心电图对房室间隔缺损的分型诊断无意义。     

Congenital tricuspid valve stenosis with atrial septal defect and left anterior fascicular block

Congenital tricuspid valve stenosis in the absence of other valvular abnormalities is rare. In this report we describe a patient with congenital tricuspid valve stenosis, ostium secundum atrial septal defect, and electrocardiographic left anterior fascicular block, who presented with paradoxical emboli. This case, as well as previous case reports, suggests that congenital tricuspid stenosis with ostium secundum atrial septal defect is associated with left anterior fascicular block.