Severe pulmonary hypertension in ostium secundum atrial septal defect

Between 1969 and 1999, 149 patients underwent cardiac catheterization for ostium secundum atrial septal defect, in hemodynamics department. Age average was 21.5 +/- 16 years (2-67). Systolic pulmonary arterial pressure average was 35 +/- 15.6 mm Hg (15-140). The atrial septal defect was closed in 108 patients (by surgery in 107 cases, and by interventional catheterization in one case). Mean follow-up was 8 +/- 7 years (1-20). Overall mortality rate was 2.7%. Severe pulmonary hypertension (systolic pressure > or = to 50 mm Hg) was seen in 13.4% of cases. It was present at all ages, but more frequent above 20 years. Obstructive pulmonary vascular disease was noticed in 2% of patients. Clinical and haemodynamic data analysis showed that, severe pulmonary hypertension is a frequent, and unpredictible complication of secundum atrial septal defect; it might occur at any age and worsen considerably the cardiac disease prognosis. Because of atrial septal defect hemodynamic profile, and current rise of percutaneous closure technics, invasive investigation should be more and more routinely performed, in order to define the best therapeutic option. Early and systematic closure of secundum atrial septal defects, should prevent, or at least minimize pulmonary vascular lesions.     

Coexistent congenital aortic defects, aneurysm of sinus of valsalva, atrial septal defect and infective endocarditis: a case report

Coarctation of the Aorta is frequently associated with bicuspid aortic valve. This is a risk factor for infective endocarditis. Aneurysm of a sinus of Valsalva is a rare defect with a prevalence of 0.09%. They are associated in 10% of cases with a bicuspid aortic valve and less frequently with coarctation of the aorta and atrial septal defect. It is extremely rare the association of coarctation of the aorta with an atrial septal defect. This is one of the first cases reported in Puerto Rico of an adult patient with coarctation of the aorta in association with a bicuspid aortic valve, a ruptured aneurysm of a sinus of Valsalva and an atrial septal defect. The patient is a 22 year old male with coarctation of the aorta diagnosed since childhood who was admitted at the Cardiovascular Center of Puerto Rico with signs of heart failure due to infective endocarditis secondary to a teeth infection. Upon evaluation with transthoracic and transesophageal echos, he was found to have a coarctation at the aortic isthmus, aortic root dilatation, bicuspid aortic valve with vegetation, severe aortic and tricuspid regurgitation, aneurysm of the non coronary sinus of Valsalva with perforation to the right atrium, biatrial enlargement and a dilated right ventricle. Successful antibiotic treatment of endocarditis was achieved followed by surgical replacement of the aortic valve and ascending aorta with closure of the non coronary sinus of Valsalva was done. An secundum atrial septal defect was found and was also closed. Surgical correction of the coarctation of the aorta was postponed for a future time. The patient had a successful postsurgical recovery and was discharged home with anticoagulation treatment.     

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。     

先天性房间隔缺损对新生儿心脏结构和血流动力学的影响

目的:通过检测先天性房间隔缺损(atrial septal defect,ASD)新生儿心脏结构和血流动力学的变化,探讨其对新生儿的潜在危害。方法:利用彩色多普勒超声心动图检测68例先天性房间隔缺损患儿与82例正常新生儿心脏各腔室的大小、肺动脉内压力、三尖瓣反流等。结果:进行心脏彩超检查的原因以心脏杂音、紫绀、宫内窘迫或新生儿窒息、呼吸急促、肺动脉瓣第二心音亢进最常见。房间隔缺损有房间隔水平存在异常分流但探测不到回声失落的无缺损型34例,回声失落直径<5mm的小缺损型21例,回声失落直径≥5mm的大缺损型13例。三种类型对室间隔、左心室、主动脉、左室后壁的影响差异无显著性,但对右心房、右心室、肺动脉的影响程度与缺损直径有显著正相关;只有大型房间隔缺损型导致左心房扩张;缺损和分流束的直径与肺动脉压力增高、三尖瓣反流率、反流面积也有显著正相关;但分流方向与缺损直径无关。结论:新生儿先天性ASD以轻型最多见,可导致新生儿心脏结构和血流动力学的异常改变。     

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.     

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。     

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.     

Contracted endocardial fibroelastosis in children: report of a case

Endocardial fibroelastosis is un uncommon disease and it has a very bad prognosis since fatal evolution is usual before 2 years old. We report the case of a 20 years old woman who is affected with the contracted form of this disease associated with atrial septal defect (ASD) and mitral regurgitation. This disease was discovered by endocardial biopsy when she was 4 years old and underwent surgical resection of endocardial fibrosis, a patch on the ASD and mitral valve replacement. She was rehospitalised 15 years later with heart failure although continuous digitoxin therapy.     

单向活瓣及肺动脉注入前列腺素E1(PGE1)治疗室缺合并肺动脉高压

目的探讨基层医院室缺合并重度肺动脉高压(PH)的外科治疗方法。方法 41例室缺合并肺动脉高压患者采用单向活瓣补片修补室间隔缺损,同时肺动脉内留置导管泵入前列腺素E1(PGE1);术后超声心动图动态监测分流情况,并比较术前术后肺动脉压(PAP)及血气分析指标变化。结果 41例患者无1例死亡,术后2个月内单向活瓣关闭、分流消失;术后PAP明显下降,血气分析指标明显改善。结论单向活瓣补片及肺动脉置管泵入前列腺素E1(PGE1)能有效提高基层医院室缺合并肺动脉高压的治疗效果,增加围手术期安全性。     

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。     

Aortic endocarditis complicated with a large ventricular septal defect and septic pulmonary embolism

BACKGROUND: Infectious complications of the aortic valve can lead to severe cardiac failure and widespread contiguous lesions by the involvement of subaortic structures such as aorto-left ventricular discontinuity, destruction of the aortic ring, aortic abscesses, true or false aneurysms and shunts. AIM: Report a new case of a large ventricular septal defect due to infection. CASE REPORT: Abnormal communications occurring during acute aortic valve endocarditis are rare but they are very serious complications. We report a case of a 58 year-old-man, referred to our hospital with the diagnosis of aortic endocarditis with complicating root abscesses, acquired ventricular septal defect (VSD) and pulmonary septic embolism. Surgery was indicated and the whole procedure was performed through the aortic root. The patient underwent a radical resection of the abscesses, reconstruction of the aortic ring, closure of the ventricular septal defect and prosthetic replacement of the aortic valve. The immediate postoperative course was marked by persistent sepsis. Transoesophageal echocardiography showed vegetation's in the right side of the patch without signs of dehiscence. The outcome was fatal due to hemodynamic collapse. CONCLUSION: The authors would like to select and analyze some weaknesses of their procedure like using only the transaortic access that often limits exposure of possible right-side lesions, not removing the pulmonary obstruction and using an important amount of foreign material to reconstruct subvalvular lesions.     

Late postinfarction rupture of the interventricular septum

Postinfarction ventricular septal rupture complicates 1 to 2% of cases of acute myocardial infarction and accounts for 5% of early mortality. This severe complication usually occurs within two weeks after acute myocardial infarction, and the elderly are more susceptible. We present a case of late rupture of the septum. Case report: In a 75-year-old man, a ventricular septal defect developed more than two months after an extensive inferoseptal myocardial infarction due to occlusion of the right coronary artery. After more than two months of no symptoms he was referred to hospital due to symptoms of right ventricle failure. The diagnosis was made by echocardiography, pulmonary artery catheterization and ventriculography. Coronarography was also performed. Intraaortic balloon pump was introduced and the patient was transferred to the operating room. The defect was repaired using a circular polytetrafluoroethylene patch supported by buttressed interrupted sutures from both sides. Due to significant mitral valve regurgitation mechanical bileaflet mitral valve was implanted with preservation of the entire mitral apparatus and the left descending artery was revascularized using a saphenous graft. Conclusion: This case is reported to emphasize that later postinfarction rupture of the ventricular septum may occur with symptoms of right ventricle failure dominating the clinical course.     

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。     

心内结构无异常胎儿的心脏瓣膜返流超声检查分析

【摘要】目的观察心内结构尤异常的胎儿瓣膜返流出现情况,分析不同孕间符个瓣膜返流愉出率的不同和原闵。方法总结296例往我院做胎儿超声筛查的胎心,除先天性心内结构畸形、动脉导管收缩或早闭病例外,分析不同时期各个瓣膜返流的检出率、结果20～24孕周胎儿,三尖瓣返流检出率10％,1例为巾度,余均为轻度;肺动脉瓣返流检出率3．4％．均为轻度;无二尖瓣和主动脉瓣返流胎心。25～29孕周胎儿,三尖瓣返流检出率53．36％,中度返流18例;二尖瓣返流检出率7．92％,均为轻度返流;肺动脉瓣返流检出率7．03％,均为辑度;主动脉瓣返流枪出率0．014％,均为轻度：30～34孕周胎儿,：三尖瓣返流检出率59％．中度返流4例：二尖瓣轻度返流检出率13％,肺动脉瓣轻度返流检出率14％．无主动脉瓣返流榆出。35～38孕周胎儿,三尖瓣轻度返流检出率58．79％,均为轻度返流;二尖瓣返流检出牢0．17％;肺动脉瓣返流检出牢0．07％;无主动脉瓣返流柃出。结论心内结构无异常的胎儿,出现瓣膜返流的情况很普遍,以—尖瓣返流检出率最高,瓣膜返流与孕阍大小有密切相父性.     

Descriptive epidemiology of selected congenital heart defects, Hawaii, 1986-1999

Congenital heart defects are the most common type of birth defect and contribute the most to infant mortality due to birth defects. This study examined the relationship between several demographic factors and selected congenital heart defects among the unique multiethnic population in Hawaii during 1986-99, using data from a population-based birth defects registry. Rates were significantly higher in 1993-99 than in 1986-92 for transposition of the great arteries and Ebstein's anomaly, and significantly lower for tetralogy of Fallot. Significantly elevated rates were found with maternal age of > or =35 years for ventricular septal defect, atrial septal defect, endocardial cushion defect, and hypoplastic left heart syndrome. When cases with a known chromosomal abnormality were excluded, elevated rates among the older maternal age group remained for ventricular septal defect, atrial septal defect, and hypoplastic left heart syndrome. Whites had significantly higher rates than one or more of the other racial/ethnic groups for Ebstein's anomaly and coarctation of aorta, and significantly lower rates for tetralogy of Fallot, atrial septal defect, pulmonary valve atresia/stenosis, tricuspid valve atresia/stenosis, and anomalous pulmonary venous return. Significantly higher rates were found among males for transposition of great arteries, aortic valve stenosis, and interrupted aortic arch and, among females, for ventricular septal defect, endocardial cushion defect, and anomalous pulmonary venous return. Some of these differences were consistent with the literature while others were not.     

二尖瓣成形术治疗二尖瓣关闭不全的疗效分析

目的分析二尖瓣瓣膜成形术(MVP)治疗中重度二尖瓣关闭不全(MR)患者的临床疗效。 方法以2013年6月至2017年6月徐州医科大学附属沭阳医院心胸外科收治的28例行MVP的MR患者为研究对象行回顾性分析,其中男性17例,女性11例;年龄50～72岁,平均(61.5±10.6)岁。所有患者术前均存在中重度MR。术前根据患者的合并症、病变部位的定位和合并腱索断裂与否,评估选择可行的MVP术式及合并手术：所有患者均采用正中切口、体外循环下手术治疗,术中行二尖瓣楔形切除、矩形切除及缘对缘缝合方法修补二尖瓣,合并腱索断裂或腱索冗长的行e-PTFE线人工腱索植入,常规行二尖瓣成型环植入,术中注水观察评估瓣膜成行效果。术中采取的二尖瓣成形方法统计：7例(25.0%)植入1～3根腱索,行二尖瓣矩切除术9例(32.1%),二尖瓣楔形切除术8例(28.6%),二尖瓣缘对缘缝合4例(14.3%),所有患者均置入鞍形二尖瓣成形环。对于合并心房颤动的患者同期行单纯左心耳切除术(LAA),或心房颤动射频消融术(AB)＋LAA;合并冠心病,则同期行冠状动脉搭桥术(CABG);合并中度及以上的三尖瓣关闭不全,则同期行三尖瓣成形环植入手术(TVP)。1例(3.6%)患者行MVP＋CABG,1例(3.6%)行MVP＋TVP＋LAA;3例(10.7%)行MVP＋TVP＋AB＋LAA;5例(17.9%)行MVP＋TVP;18例(64.3%)行单纯MVP。术后予华法林抗凝治疗3～6个月,合并心房颤动者终身抗凝治疗。统计所有患者采取的手术方式,包括合并手术、二尖瓣成形方法;对比患者术前及术后2年的返流、心功能改善情况以及LAD、左心室舒张末内径(LVEDD)、左心室射血分数(LVEF)水平的差异。 结果所有患者术前均为中重度返流,术后2年复查心脏彩超：21例(75.0%)无明显二尖瓣返流,6例(21.4%)二尖瓣轻度返流,1例(3.6%)二尖瓣中度返流。且所有患者的心功能较术前均提升1 ～ 2级。术前LAD[(49.42±12.58)mm],术后2年LAD[(38.17±9.84)mm],术前LVEDD[(50.91±7.93)mm],术后LVEDD[(44.37±7.42)mm],术后均较术前明显缩小;术前LVEF(51.69±9.71)%,术后LVEF(62.79±8.53)%,术后LVEF较术前明显增加。 结论MVP治疗MR安全有效、疗效显著,但远期效果还待进一步研究随访。     

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.     

Changing P waves after mitral valve replacement

A 55-year-old man is convalescing from mitral valve replacement five days earlier. He had severe calcific mitral stenosis and moderately severe hypertension, with repeated attacks of acute pulmonary edema. He had always been in normal sinus rhythm, except for a single episode of atrial fibrillation associated with one of his episodes of acute pulmonary edema. He had been taking digoxin (0.25 mg daily) but this was stopped the day before the operation. The cardiac rhythm has been mostly regular since the operation, but occasional irregularities have been noted. An ECG on the fifth postoperative day is shown.     

Cardiovascular abnormalities in Marfan syndrome]

During the period February to December 1990, 52 adult patients were referred to our clinic for evaluation of the presence of the Marfan syndrome. In 24 out of 52 patients the Marfan syndrome was diagnosed. Cardiac abnormalities were found in all patients: mitral insufficiency because of mitral valve prolapse (83%), aortic dilatation (67%), aortic insufficiency (38%), tricuspid valve insufficiency with or without tricuspid valve prolapse (17%) and atrial septal defect (4%). In 3 patients an aneurysm of the ascending aorta was found. Early recognition of the Marfan syndrome is relevant for prevention of the life threatening complication of aortic dissection. In patients with valve abnormalities endocarditis prophylaxis is advised. A Marfan outpatient clinic offers optimal diagnostic possibilities.