鲁登巴赫综合征的外科治疗

目的 :回顾分析 10例鲁登巴赫综合征 ,探讨该疾病在病理生理、诊断和治疗方面的特点。方法 :总结经外科治疗的鲁登巴赫综合征共 10例患者的临床资料。结果 :全组病例无围手术期死亡 ,无顽固性心力衰竭、肺动脉高压危象及恶性心律失常发生。结论 :鲁登巴赫综合征应及早手术治疗 ,修补房间隔缺损同时有效的解除二尖瓣狭窄 ,探查并处理合并的三尖瓣关闭不全 ,术后积极治疗肺动脉高压 ,支持心功能 ,可以取得满意的手术效果     

鲁登巴赫综合征的外科治疗

目的 :总结鲁登巴赫综合征外科治疗经验。方法 :自 1992年 12月至 2 0 0 0年 6月手术治疗鲁登巴赫综合征 12例 ,其中房间隔缺损均为先天性 ,二尖瓣病变先天性 2例 ,风湿性 10例。单纯二尖瓣狭窄 8例 ,二尖瓣狭窄合并闭锁不全 4例。房缺直接缝合 8例 ,补片修补 4例。二尖瓣换瓣 9例 ,直视成形 3例。三尖瓣Devega成形 10例。结果 :围术期无死亡。 12例随访 6～ 90个月 ,1例二尖瓣成形术后 3年再次手术换瓣 ,余 11例恢复顺利。心功能恢复为Ⅰ级者 8例 ,Ⅱ级者 4例。结论 :鲁登巴赫综合征应尽早手术。正确选择处理二尖瓣病变手术方式 ,加强围术期处理 ,防治肺动脉高压危象和左心室功能衰竭是手术成功的关键     

鲁登巴赫综合征的外科治疗

目的总结10例鲁登巴赫综合征的外科治疗经验。方法我院自2006年6月至2010年6月共收治鲁登巴赫综合征10例,男性2例,女性8例。其中房间隔缺损均为先天性,二尖瓣病变先天性1例、风湿性9例。术中10例均行房间隔缺损心包补片修补术,行二尖瓣置换术8例、二尖瓣成形术2例。同期行三尖瓣Devega成形术6例、左心耳结扎术4例。结果10例患者均无围手术期死亡,术后均未出现顽固性心力衰竭、肺动脉高压危象及恶性心律失常等严重手术相关并发症。结论早期行房间隔缺损修补术同期解除二尖瓣狭窄及处理合并的三尖瓣关闭不全,加强围手术期处理,可取得满意的手术效果。     

介入治疗鲁登巴赫氏综合征二例

例1　女性,30岁。3年前查体发现心脏杂音,近2年活动后心悸、气短。查体:胸骨左缘2～3肋间闻及ＩＩＩ级吹风样杂音,心尖部隆隆样舒张期杂音,Ｐ2亢进。心电图:右心室肥厚。Ｘ线平片:肺血多,右房室增大。超声心动图检查:右房室增大,ＩＩ孔型房间隔缺损,缺损直径7.5ｍｍ。二尖瓣狭窄,瓣口面积1.5ｃｍ2,瓣叶活动好。右心导管检查:肺动脉压力32/12(21)ｍｍＨｇ,左房平均压17ｍｍＨｇ,ＱＰ/ＱＳ=2.1,提示大量左向右分流。介入治疗:局麻下,Ｓｅｌｄｉｎｇｅｒ技术穿刺右股动脉、静脉,先行左、右心导管检查。经房间隔缺损送入球囊导管,用26ｍｍ直径扩…     

鲁登巴赫氏综合征的外科治疗

本文报告17例鲁登巴赫氏综合征的外科治疗结果,并就其临床特点、手术方法和左心功能的维护等问题进行讨论。     

经导管介入治疗鲁登巴赫综合征一例

鲁登巴赫综合征是指先天性房间隔缺损（ASD）合并二尖瓣狭窄，该综合征传统依靠外科手术治疗。随着ASD介入治疗和经皮二尖瓣成形术开展，部分病例可经介入治疗。介入治疗可以避免外科手术的痛苦以及因二尖瓣再狭窄需要再次开胸的困扰，是一种较为理想的方法。现报道我院成功经皮介入治疗鲁登巴赫综合征一例。鲁登巴赫综合征是指先天性房间隔缺损（ASD）合并二尖瓣狭窄，该综合征传统依靠外科手术治疗。随着ASD介入治疗和经皮二尖瓣成形术开展，部分病例可经介入治疗。介入治疗可以避免外科手术的痛苦以及因二尖瓣再狭窄需要再次开胸的困扰，是一种较为理想的方法。现报道我院成功经皮介入治疗鲁登巴赫综合征一例。     

鲁登巴赫综合征1例报道

1病例资料 患者,男,63岁,以“反复胸闷、气短、心悸20余年,加重伴咳嗽,咳痰1周”为主诉入住大连医科大学附属第二医院。20年前无明显诱因出现活动时胸闷、气短、心悸,休息可以缓解,伴双下肢水肿．就诊外院诊断“风湿性心脏病、全心衰竭、心律失常、心房颤动”,     

彩色多普勒血流显像诊断鲁登巴赫综合征

应用CDFI诊断5例鲁登巴赫综合征,均显示出三种异常血流信号,即过IAS左向右五彩镶嵌分流束血流信号,过MO五彩镶嵌射流束血流信号和过MO、TO五彩镶嵌返流束血流信号,其中4例有心导管资料并经手术证实,表明CDFI对本病具有特异性诊断价值。可与心血管造影相媲美。     

鲁登巴赫综合征1例报告

患者女,48岁。因阵发性胸闷、心悸20a,加重0．5a,于2005年6月23日收入院。无风湿热史。查体：Bp135／76mmHg,心率（HR）100次／min,体重54kg。平卧位,二尖瓣面容,口唇紫绀,双侧颈静脉怒张．双肺呼吸音清。右心浊音界扩大,心律绝对不齐,心尖区可闻及m／6级杂音,三尖瓣区可闻及Ⅲ／6级收缩期杂音,P2亢进,固定分裂。腹软,肝颈静脉回流征阳性,肝大平脐．双下肢静脉曲张。X线片示,心胸比0．70,双肺纹理增粗紊乱,心影呈二尖瓣一普大型,肺动脉段明显膨凸,左右心缘明显隆凸,右室段食管受压,左房、右室增大。     

Lutembacher Syndrome and Rheumatic Pulmonary Stenosis

We discribed a case of a 55-yr-old woman diagnosed with Lutembacher syndrome and rheumatic pulmonary stenosis. Congenital atrial septal defect was found in age 7 and rheumatic fever in age 34. As the patient developed pulmonary hypertension with calcified mitral valve leaflet and pulmonary stenosis so surgery was not indicated. So the patient was managed by medical therapy alone.     

Successful percutaneous management of Lutembacher syndrome

Background

The surgical management of Lutembacher syndrome is straight forward but percutaneous management, though technically demanding, is always desirable.

Methods

A 17 year old unmarried female presented with severe Mitral stenosis and a 19 mm almost circular Ostium secundum ASD with moderate pulmonary artery hypertension and dilated right sided chambers. She was managed in a staged manner. Percutaneous trans mitral commissurotomy (PTMC) was done first, using a 26 mm Inoue balloon catheter set, and after 48 h, ASD was closed with a 20 mm Cocoon Septal Occluder.

Results

The mitral valve area increased after PTMC from 0.8 cm² to 2.1 cm² and Q_P/Q_S decreased from 4.9 to 2. ASD was successfully closed under echocardiographic and fluoroscopic guidance.

Conclusion

Percutaneous management of the Lutembacher syndrome (PTMC and ASD device closure) is an effective and low risk procedure and avoids considerable morbidity and mental trauma for the patients.     

1岁以下婴儿室间隔缺损伴肺动脉高压的外科治疗

目的　探讨婴儿室间隔缺损 (VSD)伴肺动脉高压 (PH)外科治疗的结果和经验。方法　对 6 0例 1岁以下婴儿室缺伴肺高压的手术治疗进行总结。年龄 2～ 11个月 ,平均 (6 2 3± 2 6 0 )个月 ,体重 3～ 10kg,平均 (5 5 0± 1 2 6 )kg。术前中度以上肺高压 5 3例 (88 8% )。结果　全组手术死亡3例 (5 % ) ,手术死亡的主要原因是肺动脉高压危象和急性心包填塞。术后主要并发症为肺动脉高压危象 ,术后出血和肺部感染。结论　婴儿室缺伴肺高压应尽早手术治疗。术前、术后支持疗法 ,术中加强心肌保护、提高手术技巧 ,术后注意呼吸、循环监测 ,加强呼吸道管理 ,防治可能出现的并发症是手术治疗成功的关键。     

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。     

Association between aortic calcification and stable obstructive coronary artery disease

Aims

Atrial fibrillation and flutter remain an important cause of morbidity in adults with atrial septal defect (ASD). This study aimed at investigating predictors for late (≥ 1 month after repair) atrial arrhythmia.

Methods

Patients who underwent ASD closure after the age of 18 years, were selected through the databases of three medical centres in Belgium. Preprocedural, periprocedural and follow-up data were extracted. Univariate and multivariate Cox-regression analysis was performed. Kaplan-Meier analysis was performed for any independent predictor of late atrial arrhythmia.

Results

A total of 155 patients (38 men and 117 women) was included. Twenty-four patients (median age 48.3 years, range 19.9-79.8) underwent surgical and 131 (median age 57.6 years, range 18.2-86.9) underwent transcatheter closure. Thirty-nine patients (25.2%) presented with late atrial arrhythmia. Male gender (P = 0.008), creatinine (P = 0.002), atrial arrhythmia before (P < 0.0001) and within 1 month after repair (P = 0.001) and a mean pulmonary artery pressure (mPAP) ≥ 25 mm Hg (P < 0.0001) correlated with late atrial arrhythmia in univariate Cox-regression analysis. Multivariate analysis showed that mPAP ≥ 25 mm Hg (HR 3.72; 95%CI 1.82-7.59; P < 0.0001) and the presence of atrial arrhythmia before (HR 3.22; 95%CI 1.56-6.66; P = 0.002) and within 1 month after repair (HR 2.08; 95%CI 2.08-15.92; P = 0.001) were predictive of late atrial arrhythmia. Kaplan-Meier analysis showed that patients with a mPAP ≥ 25 mm Hg had a higher risk at developing late atrial arrhythmia (P < 0.0001).

Conclusion

In patients with ASD type secundum, a mPAP ≥ 25 mm Hg is an independent predictor of late atrial arrhythmia. The presence of pulmonary hypertension before repair should raise awareness for atrial arrhythmias and may be used to guide therapy.     

Net atrioventricular compliance can predict persistent pulmonary artery hypertension after percutaneous mitral balloon commissurotomy

Background

Pulmonary hypertension is a common complication of rheumatic mitral stenosis (MS). Patients with similar mitral valve (MV) areas may have different pulmonary artery pressures. Net atrioventricular compliance (Cn) was found to play an important role in the development of pulmonary hypertension.

Partial anomalous pulmonary venous connection associated with Lutembacher's syndrome

Lutembacher's syndrome is a rare combination of congenital atrial septal defect and mitral stenosis (almost always rheumatic). The hemodynamic effect of this combination makes the clinical diagnosis difficult due to a wide variation of clinical presentation. Echocardiography and cardiac catheterization are useful tools for proper diagnosis and planning of the therapeutic strategy. We present a 28-year-old female with Lutembacher's syndrome associated with partial anomalous pulmonary venous connection (PAPVC), which was surgically corrected. To our knowledge, this combination has never been reported in the literature.