Large ostium secundum atrial septal defect discovered after severe mitral regurgitation. A case report

Mitral regurgitation is associated with ostium secundum atrial septal defect in about 22% of cases. mitral valve prolapse induced by atrial shunt is the main cause of this regurgitation. Ususually, atrial septal defect discovery precedes that of mitral regurgitation. The aim of this paper is to focus on clinical, hemodynamic and evolutive details of atrial septal defect and mitral regurgitation association. We report the case of large atrial septal defect in 37 years old girl referred for hemodynamic investigation of mitral regurgitation. The divergence of clinical data, electrocardiogram and echocardiography findings has led to atrial septal defect discovery. Hemodynamic data showed severe pulmonary arterial hypertension (medium pulmonary arterial pressure: 45 mmhg). Hence, mitral valve substitution by mechanical prosthesis and closure of atrial septal defect have been carried out. Ten hours after surgery, death occurred because of severe pulmonary arterial hypertension and heart failure. CONCLUSION: Association of severe mitral regurgitation and large ostium secundum atrial septal defect is an original anatomo-clinic entity caracterized by mitral valve lesions diversity and severe secondary pulmonary arterial hypertension. Danger of such a hypertension is due to progressive and infra clinical rise of pulmonary resistances and association of increased pulmonary blood flow and capillary pulmonary hypertension.     

Percutaneous closure of interatrial ostium secundum defects using the Amplatzer device. Apropos of the initial 5 Tunisian cases

We report our initial experience with percutaneous closure of ostium secundum atrial septal defect using Amplatzer device. Between september 2000 and July 2001, five devices were implanted in 5 patients, 4 female and 1 male. Age ranged from 18 to 66 years. A large atrial septal defect with significant shunting was diagnosed by trans thoracic echocardiography. Procedures were performed under general anesthesia with trans esophageal echocardiography guidance. Stretched diameter of atrial septal defects was determined by balloon sizing, ranged from 21 to 32 mm. Amplatzer devices of 20 to 32 mm were respectively deployed. No complications occurred during the procedure. Total occlusion of interatrial communication, was observed in one patient, immediately after the procedure, and in 3 patients 24 hours later. First 3 patients were controlled at months follow up were free of complications. Closure of atrial septal defect with Amplatzer device appears feasible and safe. However, long term results in comparison to surgery remain to be determined before definite conclusion regarding its use can be made.     

Percutaneous closure of ostium secundum atrial septal defect using an Amplazter device. Report of the first 5 Tunisian cases

We report our initial expérience with percutaneous closure of ostium secundum atrial septal defect using Amplatzer device. Between september 2000 and july 2001, five devices were implanted in 5 patients, 4 female and 1 male. Age ranged from 18 to 66 years. A large atrial septal defect with significant shunting was diagnosed by trans thoracic echocardiography. Procedures were performed under general anesthesia with trans esophageal echocardiography guidance. Stretched diameter of atrial septal defects was determined by balloon sizing, ranged from 21 to 32 mm. Amplatzer devices of 20 to 32 mm were respectively deployed. No complications occurred during the procedure. Total occlusion of interatrial communication, was observed in one patient, immediately after the procedure, and in 3 patients 24 hours later. First 3 patients were controlled at months follow up were free of complications. Closure of atrial septal defect with Amplatzer device appears feasible and safe. However, long term results in comparison to surgery remain to be determined before definite conclusion regarding its use can be made.     

Practical value of echo Doppler evaluation of aortic and mitral stenosis: a comparative study with cardiac catheterization

This retrospective analysis compares data derived by echocardiography and cardiac catheterization in the evaluation of aortic and mitral valve stenosis. Sixty-seven patients, aged 69 +/- 12 years, underwent 76 catheterization procedures. In all studies the Doppler recording was technically adequate. In 64 studies of patients with aortic stenosis, correlation was good between the gradient obtained at catheterization (peak 51 +/- 28 mm Hg, mean 48 +/- 24 mm Hg) and the Doppler gradient (peak 73 +/- 29 mm Hg, mean 41 +/- 17 mm Hg) (R = 0.78 peak, 0.77 mean). In 15 studies the aortic valve area, 0.8 +/- 0.2 cm2, calculated by the simplified continuity equation, correlated well with the catheterization valve area, 0.7 +/- 0.3 cm2, calculated by the Gorlin equation (R = 0.80). In 14 studies in mitral stenosis patients, the mean gradient at catheterization was 11 +/- 5 mm Hg compared to the Doppler gradient of 8 +/- 4 mm Hg (R = 0.58). The mitral valve area was 1.1 +/- 0.3 cm2 by the Gorlin equation and 1.2 +/- 0.3 cm2 by echo Doppler, using pressure half-time. When cardiac rhythm, the presence and severity of regurgitation, and the cardiac index were analyzed, none was shown to have demonstrable influence on the accuracy of the Doppler study. Doppler echocardiography can be used reliably to assess valvular stenosis in a clinical, noninvasive laboratory where routine tests are performed and interpreted by more than one individual.     

彩超对胎儿期卵圆孔闭合的诊断价值

目的：应用彩色多普勒超声观察胎儿心脏,观测胎儿时期卵圆孔的各项测量指标以预测其产后情况,并明确产后的重点随访对象,提高胎儿或新生儿继发孔型房缺的早期诊断。方法：选取胎儿期心脏结构、功能基本正常的病例,根据其产后房间隔闭合时间分为〈3个月闭合组、3～6个月闭合组、〉6个月尚未闭合等3组,比较各组产前的卵圆孔直径、卵圆孔流速、动脉导管流速、肺动脉内径、肺动脉流速。结果：随着房间隔闭合时间的延迟,胎儿时期的卵圆孔直径、动脉导管流速、肺动脉内径、肺动脉流速有增大趋势,卵圆孔流速有减慢趋势,对在6个月内闭合与未闭合的病例比较其差异,有统计学意义。结论：卵圆孔是胎儿心脏的重要血流通道．通过彩超对以上各项参数进行监测．能够对产后卵圆孔闭合的情况进行初步估测,从而对继发孔型房缺作出早期诊断。     

低体重婴幼儿先天性心脏病的外科治疗

目的总结低体重婴幼儿先天性心脏病外科手术的经验。方法自2000年1月～2004年12月共为133例15公斤以下的低体重婴幼儿(3岁以下)先天性心脏病患儿施行外科手术治疗;其中5～12个月32例(24.1%),13～24个月46例(34.59%),25～36个月55例(41.35%);体重6～15公斤,其中6～10公斤48例,11～15公斤85例。全组非体外循环动脉导管结扎术29例;体外循环手术104例,其中室间隔缺损修补术64例,常温心脏不停跳继发孔房间隔缺损修补术16例,常温心脏不停跳肺动脉瓣狭窄直视分离术7例,部分行房室间隔缺损矫治术2例,部分行肺静脉异位引流矫治术3例,法乐氏四联症一期矫治术5例,房间隔缺损并右室流出道梗阻4例,其它复合畸形一期矫治术3例。结果全组术后住院死亡6例,死亡率4.5%。术后主要并发症包括低心排综合征,呼吸衰竭,心律失常,肺部感染,出血等。死亡原因包括重度低心排综合征,严重室性心律失常,肺动脉高压危象等。结论心外科、心儿科、麻醉、体外循环和术后监护等人员的密切配合是小儿心脏外科迅速发展的重要前提,心内外科镶嵌治疗成为婴幼儿心脏外科手术成功的重要保证。     

67例继发孔房间隔缺损的外科治疗和围术期处理

目的总结继发孔房间隔的外科治疗和同术期的处理。方法共手术治疗继发孔房间隔缺损患者67例，其中男24例，女43例，年龄1．5～64岁；中度以上肺动脉高爪32例。所有病例均在全麻体外循环下采用直接缝合、心包或涤纶片修补房间隔缺损；合并畸形均同期手术矫治。结果全组无死亡，术后随访1～15个月，心功能均有明显改善。结论严格把握手术适应证、选择手术方式、确诊后应尽早手术，对有肺动脉高压增加手术危险者加强术后护理是提高手术疗效的关键。     

微创切口小儿心内直视手术56例临床分析

目的 报告一组小儿先天性心脏病采用微创切口手术治疗的经验。方法 全组56例病儿中继发孔房间隔缺损（ASD）25例,室间隔缺损（VSD）26例,右室双腔心（DCRV）2例,肺动脉狭窄（PS）3例。右前外侧小切口不停跳、不阻断主动脉心内直视手术23例,胸部正中小切口33例。结果 除1例因术中显露差改作常规切口,全组56例无手术死亡,无严重并发症。病儿术后恢复快,平均住院时间8d。结论 微创切口小儿心内直视手术创新小、美观、痛苦少、恢复快、住院时间短、安全有效,尤其适应于继发孔房缺及膜部或膜周室缺修补手术。     

A hazardous finding of a rare anomalous left main coronary artery in a patient with a secundum atrial septal defect

A 23-year-old male referred for evaluation of a "choking" sensation with exertion and a murmur. A transthoracic echocardiogram demonstrated right atrial and ventricular dilatation, right ventricular volume overload, and a large secundum atrial septal defect (ASD) with left to right shunt and a calculated pulmonary-to-systemic blood flow ratio (Qp/Qs) estimated at 2.3 to 1. Cardiac catheterization also demonstrated evidence of the ASD with Qp/Qs of 4.6 to 1 with a significant step-up in oxygen saturation at the right atrial level. Additionally, an anomalous left main coronary artery (ALMCA) origin from the anterior right coronary cusp was suspected. Using 64-slice multidetector computed tomography coronary angiography (CCTA) the left main coronary artery was seen to arise from the right coronary cusp then traverse between the pulmonary trunk and the proximal ascending aorta before bifurcating into the left anterior descending and circumflex arteries that followed their normal courses distally. Based on the high risk nature of associated sudden death from an anomalous left main coronary artery (ALMCA) coursing between the aorta and the pulmonary trunk, the patient underwent surgical re-implantation of the ALMCA to the left coronary cusp and repair of the ASD. This case highlights a rare finding of a hazardous ALMCA in a patient with a secundum ASD and the utility of CCTA in evaluating the course of coronary anomalies along with other cardiac pathology.     

Tetralogy of fallot with absent pulmonary valve

BACKGROUND: Absent pulmonary valve with ventricular septal defect is a rare cardiac malformation. The aim of our study is to specify the anatomic characteristics and the clinical and echocardiographic features of this cardiac malformation and to discuss its management. METHODS: We report 8 cases of absent pulmonary valve with ventricular septal defected collected over a period of 24 years. The diagnosis was made during the first year of life in all cases in the presence of respiratory symptoms and/or cyanosis. It was confirmed by cardiac catheterization in 4 cases and echocardiography in 4 cases. RESULTS: Pulmonary vascular obstructive disease, related to aortopulmonary collateral vessels, was noted in one patient who died at the age of 20 years. One patient was lost to follow up and 6 patients were operated. Closure of the ventricular septal defect, widening of the pulmonary tract and insertion of a pulmonary valve were performed in the 6 cases. Reduction procedure of the pulmonary arteries was performed in 3 cases. One death related to early postoperative infective endocarditis was noted. At a mean follow up of 2 years, the 5 survivors are going well with no significant pulmonary stenosis. CONCLUSION: Although named "tetralogy of Fallot with absent pulmonary valve", absent pulmonary valve with ventricular septal defect is different from tetralogy of Fallot by aneurysmal dilatation of the pulmonary arteries which may compress the bronchial tree and lead to respiratory symptoms that can be severe with respiratory distress. Pulmonary arterioplasty eliminate airways obstruction and its results are satisfactory. The need for insertion of a pulmonary valve is debatable.     

先天性心脏病复合畸形的临床介入治疗策略探讨及评价

目的研究先天性心脏病复合畸形经心导管介入治疗的病例选择、手术方法、疗效、安全性及评价。方法选择2008年1月至2008年12月在本院接受同期介入治疗的11例复合型先天性心脏病患儿为研究对象。其中,男性患儿为3例,女性为8例,平均年龄为（4.08±2．64）岁,最小为5个月,最大为10岁。本组先天性心脏病复合畸形类型包括：3例室间隔缺损（ventricular septal defect,VSD）合并动脉导管未闭（patent ductus arteriosus,PDA）,3例房间隔缺损（atrial septal defect,ASD）合并肺动脉瓣狭窄（pulmonary stenosis,PS）,2例房间隔缺损合并动脉导管未闭,1例房间隔缺损合并室间隔缺损,2例动脉导管未闭合并主动脉缩窄（coarctation of aorta,COA）,其中1例为同期介入治疗。介入治疗复合畸形的顺序为：肺动脉瓣球囊扩张术或主动脉球囊扩张术、室间隔缺损封堵术、动脉导管未闭封堵术、房间隔缺损封堵术（本研究遵循的程序符合本院人体试验委员会所制定的伦理学标准,得到该委员会批准,取得受试对象的知情同意,并与试验患儿监护人签署临床研究知情同意书）。结果11例患儿均成功封堵,手术顺利。术后X射线心脏造影及彩超提示,封堵器成形好,未见残余分流。术中、术后均未见严重并发症。其中,1例室间隔缺损术后心电图提示不完全右束支传导阻滞,给予地塞米松治疗后,心电图复查正常。结论介入治疗单纯性先天性心脏病的技术目前比较成熟,成功率高,但复合型先天性心脏病同期进行介入治疗,其操作较复杂,技术难度较高。本研究证明,若掌握恰当手术指征,制定合理操作策略,手术过程仔细、谨慎,介入治疗的可行性、安全性是较肯定的。     

The immediate effect of Rashkind's atrioseptostomy on systemic saturation in transposition of the great arteries

The purpose of this prospective study is to analyse the immediate effect of Rashkind's atrioseptostomy on systemic saturation in transposition of the great arteries (TGA). Thirteen neonates and infants (10 males and 3 females) with TCiA underwent balloon atrial septostomy (BAS) at a median age of 20 days (range 2 and 60 days). The mean atrial septal defect diameter after BAS was 6.5 +/- 1.1 mm. The right ventricular saturation increased from 37 +/- 17% to 67 +/- 13% (p < 0.001). There was no correlation between the atrial septal defect diameter and the increase of systemic saturation after BAS.     

The role of transesophageal echocardiography in atrial septal defect detected in adults

To assess the value of combined transesophageal echocardiography (TE) and transthoracic echocardiography (TTE) in the preoperative evaluation of atrial septal defect (ASD) 59 consecutive patients (aged 15-62, mean 38 yrs) with clinically or echocardiographically suspected ASD underwent TE and complete noninvasive hemodynamic study by TTE. ASD was found by TE in 48: fossa ovalis (FO) type in 36, sinus venosus (SV) type in 11 and primum type in 1. For FO and SV defect the sensitivity of clinical judgement was 83 and 0%, the specificity 39 and 100% resp., the sensitivity of TTE was 78 and 9%, the specificity 78 and 100%. The most frequently associated anomaly by TE was mitral valve prolapse (25%). SV defect was always associated with anomalous right upper pulmonary venous drainage, and left superior vena cava was found in 27% of SV defects. There was significant correlation between Qp/Qs measured by TTE and by catheterization (r = 0.64), but the Qp/Qs was not related to defect size. An excellent correlation was found between pulmonary artery systolic pressure measured by CW Doppler and catheterization (r = 0.98). In conclusion: 1) TTE combined with TE is reliable in the preoperative assessment of ASD in adults, 2) TE is not necessary if there are unequivocal clinical and TTE signs of an FO defect with significant left-to-right shunt, 3) TE is indicated in patients with signs of right ventricular overload or pulmonary hypertension if an ASD cannot be ruled out by TTE, 4) catheterization is necessary if all 4 pulmonary veins cannot be visualized by TE.     

Evolution after 5 years of percutaneous pulmonary valvulotomy: report of 47 cases

47 patients aged from 2 to 59 years affected by valvular congenital or mixed pulmonary stenosis including three fallot trilogies and one patient with right congestive cardiac failure are treated by percutaneous pulmonary valvulotomy between october 1986 and december 1990. All patients have been controlled with a mean follow-up of 6.5 +/- 1.1 years. The total gradient rate between pulmonary artery and right ventricule decrease from 112 +/- 55 mm Hg to 20 +/- 8 mm Hg on the last control with disappearance of infundibular inflammation and inter auricular shunt in all the concerned cases. We observe the regression of right cardiac failure symptoms with disappearance of tricuspid insufficiency in the cases of advanced pulmonary stenosis with right ventricular dysfunction. Percutaneous pulmonary valvulotomy by its simplicity and harmlessness, its long term efficiency is a good method of treatment of pure valvular or mixed pulmonary stenosis.     

Incidence and invasive treatment of congenital heart diseases in Hajdu-Bihar county

The incidence of congenital heart diseases was evaluated in the period 1994-1998. The number of those who went through therapeutic intervention and those infants dying of congenital heart disease were also assessed. Data were collected retrospectively. During the study period 26,932 live-births occurred in Hajdú-Bihar county and 421 congenital heart disease were diagnosed, 81% of whom were diagnosed under the age of one year. The most frequent diseases were secundum type atrial septal defect, ventricular septal defect and patent ductus arteriosus. 121 therapeutic procedures were performed (109 operations and 12 interventional heart catheterizations). 41 interventions occurred under the age of one and 13 before the age of 28 days. The mean age of children older than 1 year was 6 years at the time of the operation or interventional catheterization. The overall postoperative mortality within 30 days was 8.3%. During the study period 28 infants with significant heart disease died, 20 of whom also had an associated disease (most frequently prematurity). For congenital heart disease 4.5 operations or interventional heart catheterizations were required/1000 live births. The higher rate of diagnosed congenital heart disease is due to the development in diagnostic techniques, especially to Doppler-echocardiography. Owing to the operational waiting list the children's age at the time of operation is higher than optimal. The mortality among infants with heart disease is influenced by many factors, that is why stepping forward is a complex task in this field.     

先天性心脏病合并重度肺动脉高压的外科治疗

目的 探讨先天性心脏病合并重度肺动脉高压的外科治疗.方法 选择先天性心脏病合并重度肺动脉高压患者32例.动脉血氧饱和度0.84一0.94,左向右分流20例,双向分流10例,无分流2例.测肺动脉压65～120(82±14) mm Hg(1 mm Hg=0.133 kPa).所有病例均在体外循环下行心内畸形矫治手术,术前给予吸氧、西地那非等治疗,术中应用乌司他丁及低温肺保护液肺动脉灌注等手段,术后常规应用血管活性药物.结果 32例手术顺利,术后肺动脉压下降20～40 mm Hg.30例治愈出院,2例术后早期死于严重低心排血量综合征及致命性心律失常,病死率为6.25％(2/32).结论 先天性心脏病合并肺动脉高压经围手术期综合治疗后再行手术根治可获得良好的临床效果.     

经胸非体外循环下房缺微创封堵术的疗效评价

目的评价经胸非体外循环下房缺微创封堵术的疗效。方法15例患者,男7例,女8例,平均年龄5．5岁,平均体重20．8kg。经胸心脏超声显示Ⅱ度孔房间隔缺损,均为中央型。缺损直径8～26mm,平均14．5mm。气管插管全身麻醉后,经胸骨旁右第四肋间切口,长2～3em。在食道超声的引导下,经右房壁置人腰部直径12～32mm的国产房缺封堵器。结果15例患者均成功封堵,无中转体外循环病例。2例患者术后新发右束支传导阻滞。无Ⅲ度房室传导阻滞发生。患者术后住院时间3～8d,平均4．6d。随诊3～6个月,1例术后大量胸腔积液,经胸腔闭式引流后痊愈。余患者均恢复良好,无Ⅲ度房室传导阻滞,无残余分流,无封堵器晚期移位,无瓣膜关闭不全加重。结论经胸非体外循环下房缺微创封堵术创伤小、安全性高、近期疗效满意。远期疗效仍须进一步观察。     

经导管房间隔缺损封堵术后心脏形态大小变化的随访研究

目的评价房间隔缺损(ASD)患者经导管ASD封堵术后及随访中心脏形态和大小的变化。方法应用超声心动图分别测量36例ASD患者经导管ASD封堵治疗术前、术后3 d、术后3月和术后6月右室舒张末期前后径(RVEDD)、右房长径(RAD1)右房横径(RADt)、左室舒张末期前后径(LVEDD)、左室舒张末期容积(LVEDV)和左房前后径(LAD)。结果ASD封堵术后3 d及术后3月,RVEDD、RAD1、RAD t逐渐缩小;LVEDD、LVEDV和LAD逐渐增大。术后6月随访时,RVEDD继续缩小,LVEDD和LVEDV继续增大,RAD1、RADt和LAD无进一步变化。结论ASD封堵术治疗既减轻了右心容量负荷,使右房、右室内径缩小,也改善了左心的几何构型,使已经缩小的左房、左室逐渐增大恢复。     

唐山地区原发性高血压与Y染色体非重组区HindⅢ酶切位点多态性的研究

目的探讨Y染色体非重组区HindⅢ酶切位点多态性与唐山地区汉族人群原发性高血压的关系.方法入选男性研究对象412名,原发性高血压患者225例、正常对照人群187名.所有研究对象用常规方法提取白细胞DNA.采用多聚酶链反应结合限制性内切酶（HindⅢ）方法检测Y染色体非重组区HindⅢ酶切位点多态性.结果对照组和原发性高血压组Y染色体HindⅢ酶切位点多态性各基因型的差异有统计学意义（P=0.007）.HindⅢ（＋）基因型较HindⅢ（）基因型收缩压、舒张压、脉压和平均动脉压均明显降低（P＜0.05）.结论Y染色体HindⅢ酶切位点多态性与唐山地区汉族人群原发性高血压有关,可能是唐山地区汉族人群原发性高血压的一个遗传标志.     

实时三维超声心动图在房间隔缺损介入封堵治疗中的临床价值

目的:评价实时三维超声心动图(RT-3DE)在房间隔缺损介入封堵术前术后的应用价值。方法:RT-3DE检测187例房间隔缺损患者,分析缺损口的空间位置、大小和形态,并于封堵术后RT-3DE评估封堵器的位置及距主动脉瓣、二尖瓣和三尖瓣等周围组织和血管的距离。结果:RT-3DE能实时动态的显示房间隔缺损及其封堵器的位置、形态及其周围组织的关系,其中25例缺损口的下腔静脉端未见残端回声,5例缺损口最大间距为45 mm以上,且缺损口的心房顶部端和下腔静脉未见明显残端回声,1例因缺损处为筛孔状改行外科手术。结论:RT-3DE在明确房间隔缺损的解剖位置、观察封堵器释放后的形态结构、明确残余分流的性质及原因等方面较二维超声心动图具有明显优势。