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.     

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.     

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.     

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.     

Redo procedures for correction of aortic valve diseases in infants and children. The Bentall-Konno procedure

On an infant with critical valvular aortic stenosis balloon-valvoplasty, and 3 years later because of the aortic valve regurgitation Ross operation was performed. In the early postoperative period an aortic-root abscess occurred due to an infective endocarditis; the aortic root was corrected by homograft implantation. Due to a relatively small, calcified aortic valve, with aortic valve regurgitation grade III at the age of 12 years, a Bentall-Konno procedure was performed successfully. This is the first case when this complex surgical procedure was performed successfully on a child in Hungary.     

Aortic root replacement with pulmonary allograft (Ross procedure) in children. Early results

INTRODUCTION: For infants and children with congenital aortic valve disease root replacement with pulmonary allograft (Ross procedure) is the preferred method of choice. PATIENTS/RESULTS: The authors have successfully applied this operation in 12 children (age range from 2.5 to 17 years--mean 9 years, body weight from 12 to 58 kg--mean 46 kg), one of whom has also required a Konno extension for long segment left ventricular outflow tract obstruction. The operation was complicated by early postoperative endocarditis in one case, and the child required redo homograft root replacement on the ninth postoperative day. All patients, including this one survived, and are doing well at present. CONCLUSIONS: In the Hungarian literature this is the first report on the Ross and Konno procedure in children. On the basis of our excellent early results, Ross procedure is the method of choice in aortic valve disease in children.     

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.     

干下室间隔缺损型法洛四联症的手术治疗

目的 探讨干下室间隔缺损型法洛四联症独特的解剖特点、手术方法及预后.方法 2002年11月至2009年4月共手术治疗法洛四联症患者150例,其中干下室间隔缺损型法洛四联症13例.结果 本组13例患者的右室流出道及肺动脉狭窄类型:单纯右室流出道狭窄2例,右室流出道+肺动脉瓣狭窄3例,右室流出道+肺动脉瓣+瓣环狭窄8例;室间隔缺损为干下型,直径1.5～3.0 cm;主动脉骑跨率30%～50%.全组治愈12例,死亡1例,病死率7.7%(1/13),死亡原因为低心排血量综合征.随访1个月至6年,紫绀消失,活动能力明显改善,复查心脏超声,4例右室流出道残余梗阻,1例室水平残余分流,1例主动脉瓣轻中度关闭不全;9例右室流出道未见残余梗阻.结论 干下室间隔缺损型法洛四联症术后低心排血量综合征发生率高,精确的室间隔缺损补片远端缝合技术、右室流出道漏斗部的充分疏通,尽量减少跨瓣环补片和较小的室间隔缺损补片可预防其发生.     

主动脉窦瘤破裂合并感染性心内膜炎的外科治疗

目的 总结主动脉窦瘤破裂合并感染性心内膜炎的易感因素及治疗经验.方法 对2000年1月至2008年3月收治的30例主动脉窦瘤破裂中10例合并感染性心内膜炎的患者的临床资料进行回顾性分析.9例手术治疗,其中6例同时行主动脉瓣替换术.结果 9例行手术治疗患者均康复出院.随访2个月至8年,心功能恢复到Ⅰ级7例,Ⅱ级2例.死亡1例系未行手术治疗者,死于多脏器功能衰竭.结论 合并室间隔缺损是主动脉窦瘤破裂易感因素之一;主动脉窦瘤破裂合并感染性心内膜炎一经确诊,应足量有效抗炎治疗并尽早手术;合并中度以上主动脉瓣关闭不全应果断换瓣治疗.     

Congenitally absent pulmonary valve--analysis of ten prenatally diagnosed cases and review of the literature

OBJECTIVES: To assess in a population of 10 fetuses diagnosed with absent pulmonary valve the incidence of associated cardiac extracardiac and chromosomal anomalies and the outcome of pregnancies. METHOD: Retrospective analysis of data of prenatally diagnosed absent pulmonary valve cases between 1 January 1993 and 31 December 2005 in Fetal Echocardiography Laboratory of Ist Department of Obstetrics and Gynecology, Semmelweis University, Budapest, Hungary. The diagnosis was controlled with autopsy or postnatal examinations. The time of the prenatal diagnosis, indications, connected cardiac, extracardiac and chromosomal anomalies were studied. RESULTS: Absent pulmonary valve was diagnosed prenatally in 10 pregnancies. The mean gestational age at the time of the diagnosis was 23.1 weeks (18-33 weeks). In 6 cases the malformation was diagnosed before the 24th gestational week, in 4 cases after the 24th gestational week. In 6 cases pregnancies were terminated. Fetal hydrops were found in 2 cases. Among 4 continued pregnancies one intrauterine fetal demise and two neonatal death occurred, one baby was successfully operated and survived. The Botallo duct was normally developed in five cases, in this group the absent pulmonary valve was isolated at 3 fetuses, at 1 fetus absent aortic valve and at 1 fetus tricuspid valve dysplasia were diagnosed. The Botallo duct was absent in five cases, in this group at 3 fetuses tetralogy of Fallot, at 1 fetus atrioventricular septal defect and at 1 fetus subaortic ventricular septal defect were diagnosed. Extracardiac malformations associated with cardiac abnormality at 3 fetuses. The indication for fetal echocardiography was positive family history in 3 cases, abnormal four chamber view in 2 cases, suspicion of thoracal cyst in 3 cases, fetal hydrops in 1 case, extracardiac malformation in 1 case. CONCLUSION: The absent pulmonary valve is a severe malformation, the prognosis is poor and highly dependent on associated malformations. The correct early prenatal diagnosis is possible. The recurrence risk is higher than in multifactorial inheritance.     

产前超声对矫正型大动脉转位的诊断价值

目的 探讨产前超声应用心脏三节段分析法对诊断矫正型大动脉转位(CTGA)的价值。方法 采用心脏三节段分析法对胎儿进行筛查,检出或疑似CTGA胎儿6例,对其超声病例资料进行回顾性分析。结果 6例检出或疑似CTGA的胎儿中,经生后超声心动图或尸检证实有1例为单纯性CTGA,3例为CTGA合并室间隔缺损,1例为CTGA合并室间隔缺损、肺动脉狭窄,1例为CTGA合并室间隔缺损、主动脉弓缩窄,其中1例漏诊肌部室间隔缺损,1例漏诊主动脉弓缩窄。结论 房室连接不一致和心室-大动脉连接不一致是诊断CTGA的关键,仔细观察四腔心切面和左、右室流出道切面是诊断CTGA的重要步骤。心脏三节段分析法是产前超声诊断胎儿CTGA的可靠方法,具有很好的临床应用价值。     

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.     

Konno aortoventriculoplasty: indications and results. 13 cases

BACKGROUND: Konno procedure is used to enlarge the aortic root and increase the size of the aortic valve implanted. AIM: The aim of this study is to present the indications of the Konno procedure in congenital and acquired aortic lesions, in young patients with normal or hypoplastic aortic annulus or adults with small aortic annulus. METHODS: We reported our experience of this technique with a retrospective study of 13 patients operated on between 1998 and 2004. RESULTS: The mean diameter of aortic annulus measured in echocardiography is 16.2 mm, and the mean prosthetic valve diameter in post operative control is 20.3 mm. The early Death rate was 16%. In the late follow up of 2 years, the only predictor of myocardial hypertrophy regression was the presence of aortic insufficiency before the operation. In the literature, Konno procedure is reported as a good surgical option for the enlargement of the left ventricular outflow tract, but the results with the Ross-Konno operation are more promising.     

Porcine bioprosthetic aortic valve endocarditis with ring abscess and aortic stenosis

Porcine bioprosthetic valve endocarditis is an infrequent but serious complication of valve replacement surgery. Ring (or annular) abscess is a frequent finding in mechanical valve endocarditis. In contrast, porcine valve endocarditis most often involves the cusps, and annular infection is uncommon. Porcine valvular dysfunction secondary to endocarditis usually takes the form of incompetence, whereas stenosis is less frequent. We report a case of a 76-year-old female who developed endocarditis with Staphylococcus epidermidis nine months after placement of a Carpenter-Edwards porcine aortic valve. Her initial presentation included complete heart block and moderate aortic stenosis. Transesophageal echocardiography aided the diagnosis by demonstrating large vegetations, while transthoracic echocardiography showed only slight thickening of the valve leaflets. At operation, there was a circumferential abscess around the sewing ring causing valve dehiscence and virtual discontinuity of the aorta from left ventricle. Valve degeneration and organisms within the cusps were observed on microscopy. This case illustrates two infrequent complications of porcine aortic valve endocarditis, namely massive annular abscess with invasion of the conducting system and aortic stenosis. It also demonstrates the utility and limitations of transesophageal echocardiography in the diagnosis of this disorder.     

Anatomic correction of the congenitally corrected transposition of the great vessels by the first successful "double switch" operation in Hungary

Congenitally corrected transposition of the great arteries is a rare defect characterized by discordant atrioventricular and ventriculoarterial connections. Symptoms result from one or a combination of associated cardiovascular malformations, including ventricular septal defect, pulmonary stenosis or atresia, tricuspid valve dysfunction, dextrocardia, hypoplastic left or right ventricle. Correcting exclusively the associated defects, leaving the morphologic right ventricle in systemic position, will determine the patient's life-long prognosis. Anatomic repair by double switch technique may improve survival of patients with congenitally corrected transposition of the great arteries by establishing the morphologic left ventricle in the systemic circulation. A 3-year-old girl with congenitally corrected transposition of the great arteries, ventricular septal defect, hypoplastic right ventricle, and previous palliative procedure was corrected by double switch technique and patch closure of ventricular septal defect. The authors prefer the double switch procedure inspite of its many surgical challenges because it has very good long-term outcome. The more simple surgery namely the operation of only associated cardiac defects will involve the possibility of deterioration of right ventricular function.     

成年人小主动脉瓣环者机械瓣膜置换术后远期疗效

目的 探讨成年人小主动脉瓣环者机械瓣膜置换术后的远期疗效,以指导临床实践.方法 2003年7月至2005年2月对36例小主动脉瓣环(直径≤19mm)的成年患者行人工机械瓣膜置换术.分别采用改良Manougnian法瓣环扩大后植入23 mm CarboMedics机械瓣膜14例(CM组);瓣环上主动脉瓣置换法直接植入19 mm CarboMedics Top Hat Supra-Annular Aortic机械瓣膜22例(CMSA组),于术前和术后6年评价两组患者的心功能,超声心动图检测左心室舒张末内径(LVDD)、室间隔厚度(IVST)、左心室后壁厚度(LVPWT)、收缩期主动脉瓣平均跨瓣压差(PGav)、左心室短轴缩短率(LVFS),计算左心室射血分数.并抽取20例健康成年人的超声心动图数据作为对照组,对三组资料进行对比分析.结果 术后6年与术前比较,除CMSA组的IVST[(10.37±2.06)mm比(11.03±2.45)mm]和LVPWT[(10.53±2.18)mm比(11.24±3.09)mm]差异无统计学意义外,CMSA组的心功能分级、LVDD、PGav、LVEF、LVFS和CM组的全部检测指标均较术前有不同程度改·善(P＜ 0.01或＜0.05).术后6年CM组的PGav较CMSA组下降更明显[(9.24±5.93)mm Hg(1mmHg=0.133 kPa)比(24.30±12.50)mmHg],差异有统计学意义(P＜ 0.05);CM组术后6年各项指标与对照组比较差异无统计学意义,而CMSA组的IVST、LVPWT、PGav与对照组比较差异仍有统计学意义(P＜0.05).结论 成年人小主动脉瓣环者机械瓣膜置换术后远期左心室功能多恢复良好,但行瓣环扩大术应植入较大内径的瓣膜,将有利于左心室形态的逆转.     

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。     

主动脉窦瘤破裂手术治疗的临床体会

目的 总结主动脉窦瘤破裂患者破裂类型及外科治疗经验.方法 对19例主动脉窦瘤破裂患者手术治疗情况进行回顾性分析.合并室间隔缺损10例,主动脉瓣关闭不全6例,其他畸形6例(有的同时合并几种畸型),并发感染性心内膜炎2例.所有患者均及时施行了手术治疗.结果 发生于右冠状动脉窦者15例,其中12例破入右室,3例破入右房;发生于无冠状动脉窦者3例,其中1例破入右室,2例破入右房;发生于左冠状动脉窦者1例,破入左室.全组无手术死亡,1例出现主动脉瓣轻至中度返流,全部患者均痊愈出院.结论 主动脉窦瘤以右冠状动脉窦瘤最常见,且多数破入右室;主动脉窦瘤破裂一经确诊,应尽早手术治疗,以免延误手术时机,合并感染性心内 膜炎尤其如此.

Abstract：

Objective To summarize the ruptured types and surgical treatment experience for rupture of aortic sinus aneurysm. Methods Nineteen cases with aneurysm of aortic sinus were retrospectively analyzed. There were 10 cases with ventricular septal defect,6 cases with aortic valvular incompetence,6cases with other diseases,2 cases complicated by infective endocarditis. All cases underwent operational treatment. Results There were 15 cases of right aneurysm of aortic sinus,including 12 cases among them ruptured into right ventricle,3 cases rupture was penetrated into right atrium.There were 3 cases of null aneurysm of aortic sinus,including 1 case among them ruptured into right ventricle,2 cases ruptured into right atrium. There was 1 case of left aneurysm of aortic sinus, which ruptured into left ventricle. No operative death occurred in all, 1 case with aortic regurgitation light to moderate,and all cases were recovered.Conclusions The right aneurysm of aortic sinus is the most common types,and always rupture into right ventricle. It is important that aneurysm of aortic sinus should be operated as early as possible in order to avoid losing the chance of operation, particular in the patients complicated by infective endocarditis.     

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。     

Berry syndrome: successful neonatal repair of a rare complex cardiac malformation

Aortopulmonary septal defect, interrupted aortic arch, aortic origin of the right pulmonary artery, intact ventricular septum, and patent ductus arteriosus (Berry Syndrome) is a rarely reported malformation. A 6-day-old girl underwent successful one-stage complete repair of direct anastomosis between the ascending and descending aorta, reimplantation of the right pulmonary artery augmented with pericardium patch. Stenosis of the origin of the reimplanted right pulmonary artery was treated by balloon angioplasty at the age of 3 months. The authors emphasize the additive nature of surgical correction and interventional cardiology. Review of the literature reveals this patient to be the 25th reported case with Berry syndrome, and the first of that in Hungary.