Atrial flutter in grown-up congenital heart (GUCH) patients. Clinical characteristics of affected population

OBJECTIVE: To define occurrence, lesions and clinical characteristics of grown-up congenital heart (GUCH) patients who develop atrial flutter (AFL). DESIGN: All GUCH patients who presented as inpatients or outpatients with documented sustained AFL between 1996 and 1998 were studied prospectively. Retrospective review of case notes for basic data relating to underlying anomaly, prior surgery and age at onset of AFL, enquiry into events before the first attack. Clinical state was assessed by Ability Index before AFL and at last visit. Setting: Designated quaternary service for GUC in a tertiary referral centre. RESULTS: From October 1996 to April 1998, 100 consecutive patients (49 female) aged 17-77 (mean 35) years, who presented to the GUCH Unit at Royal Brompton Hospital with a sustained attack of AEL documented by a 12 lead electrocardiogram were studied. Four basic cardiac anomalies accounted for 75% patients: one ventricle (26), atrial septal defect (ASD) (19), transposition of great arteries (TGA) (17) and Tetralogy of Fallot (13). AFL occurred occasionally in small ventricular septal defect (VSD), congenital corrected TGA (CC-TGA), pulmonary stenosis and pulmonary atresia with or without VSD. 86/100 patients had undergone cardiac surgery: Fontan 19 (22%), reconstruction of right ventricular outflow tract 17 (20%), closure of ASD 15 (17%), Mustard for TGA 13 (15%), and other palliative surgery 22 (26%). AFL occurred in 'natural history' (unoperated) in 14 (14%) mostly in CC-TGA, ASD and Fallot. Age at first attack was 6-64 (mean 28) years with the first attack occurring at younger age after Mustard (22+/-7 years) and Fontan (24+/-7), than in un-operated ASD (46+/-13) and CCTGA (31+/-10). Haemodynamic abnormalities from anatomical causes were present in 62/74 (84%) patients who had undergone reparative surgery and included venous pathway obstruction, pulmonary regurgitation and pulmonary hypertension. Additional factors which could have precipitated AFL in prone patients were present in 63. New symptoms appeared in 96 patients with the first attack of AFL. Ability Index prior to onset in 90 patients who have been followed-up for more than 1 year since the first onset was 1 in 52, 2 in 31, 3 in 6 and 4 in 1 patients. At the last visit (mean time from the first onset 6.6+/-4.7 years), only 9 patients remained with Ability Index 1, 43 in 2, 20 in 3 and 18 in 4 despite return to sinus rhythm. CONCLUSION: One ventricle heart, ASD, transposition of great arteries and Tetrology of Fallot are the most common underlying anomalies in GUCH patients who develop AFL. It is less commonly seen in unoperated patients. When occurs AFL compromises patients' activities and deteriorates the clinical condition. Residual or developed haemodynamic abnormalities and precipitating factor are often present in this patients, hence full investigation and close follow up are necessary once AFL develops.     

Down综合征并发先天性心脏病的临床分析

目的:总结分析Down综合征(Down’s syndrome)并发的先天性心脏病畸形及血流动力学资料。方法:2008年7月~2012年10月,采用经胸二维超声心动图并彩色多普勒显像及右心导管/心血管造影检查方法,诊断36例并发先心病的Down综合征患者,本文通过36例临床资料分析,探讨Down综合征并发的先心病畸形及其血流动力学。结果:36例患者中室间隔缺损(VSD)10例,房室间隔缺损(AVSD)6例,动脉导管未闭(PDA)6例,房间隔缺损(ASD)2例,ASD+PDA 2例,ASD+VSD 1例,ASD+PDA+VSD 1例,VSD+PDA 4例,PDA+二叶主动脉瓣(BAV)1例,法洛四联症(TOF)2例,TOF+ASD 1例,18例有肺动脉高压者,其中5例为阻力型肺动脉高压。结论:①Down综合征并发的心血管畸形中,以VSD、AVSD和PDA最为常见,并常并发ASD、TOF。②在无肺动脉狭窄的患者中,约50%并发有肺动脉高压。     

Spectrum of congenital heart defects and extracardiac malformations associated with chromosomal abnormalities: results of a seven year necropsy study.

OBJECTIVE: To analyse the spectrum of congenital heart malformations, the frequency of extracardiac malformations, and the proportion of chromosome aberrations among fetuses sent for necropsy. MATERIAL: Necropsies were performed on 815 fetuses-448 induced abortions (55%), 220 spontaneous abortions (27%), and 147 stillbirths (18%)-during a seven year period (1991-97) in the department of pathology of the Charité Medical Centre in Berlin. A congenital heart defect was identified in 129 cases (16%). For all 129 fetuses, karyotyping and an ultrasound examination had been performed. RESULTS: Congenital heart defects were present in 22% of induced abortions (99 cases), 9% of spontaneous abortions (20 cases), and 7% of stillbirths (10 cases). The heart malformations were classified into 13 categories. A fetus with more than one defect was included only in the category of the most serious defect. The malformations in order of frequency were: ventricular septal defect (VSD) (28%), atrioventricular septal defect (AVSD) (16%), hypoplastic left heart (HLH) (16%), double outlet right ventricle (DORV) (12%), coarctation of the aorta (CoA) (6%), transposition of the great arteries (TGA) (4%), aortic valve stenosis (AoVS) (4%), tetralogy of Fallot (TOF) (3%), truncus arteriosus communis (TAC) (3%), pulmonary valve stenosis/pulmonary valve atresia (PaVS/PaVA) (3%), tricuspid atresia (TA) (3%), single ventricle (SV) (1.5%), and atrial septal defect (ASD) (0.5%). The most common congenital heart defects were VSD, AVSD, HLH, and DORV, which made up 72% of all the cases. In 11 cases the heart defect was isolated (no other cardiovascular or extracardiac malformations present), 85 cases (66%) were associated with additional cardiac malformations, 85 cases (66%) were associated with extracardiac malformations, and chromosome anomalies were detected in 43 cases (33%). CONCLUSIONS: Fetal congenital heart malformations are common. These defects are often associated with other cardiovascular and extracardiac malformations, as well as with chromosome anomalies. Complex heart defects such as AVSD, HLH, and DORV are frequent in fetuses, as they often lead to spontaneous abortion or stillbirth or, after prenatal diagnosis, to deliberate termination of pregnancy.     

Neonatal anatomical repair of transposition of great vessels associated with atrial septal defect. Apropos of 42 cases

The good results obtained by anatomic correction of simple transposition of the great arteries (TGA) in the neonatal period have incited some surgical teams to widen the indications to neonates with TGA associated with ventricular septal defect (VSD). The classical management of these patients is a two stage procedure: banding of the pulmonary artery followed by detransposition, which carries a certain risk. Between January 1985 and June 1990, 42 neonates with TGA and VSD underwent a combined procedure consisting in anatomic correction of the TGA and closure of the VSD. The average age of these patients was 16 days, and the average weight was 3.3 kg. Ten patients had coarctation and 6 underwent a complete one stage correction by an anterior approach. The surgical technique consisted in closing the VSD from the right atrium in 20 patients, from the right ventricle in 11 patients and from the pulmonary artery in 11 patients, associated with detransposition of the great arteries and coronary artery reimplantation. Three children died in the preoperative period (7.1%). In two cases, death was related to malposition of the coronary artery. The third fatality was the result of haemorrhage. There has been one late death three years after surgery. Four patients have been reoperated for stenosis of the right ventricular outflow tract (1 case), recurrence of coarctation (2 cases) and stenosis of the superior vena cava (1 case) and have survived. All patients were followed up for an average period of 26.4 +/- 19 months. They are all in the NYHA Class I without treatment. One patient has mild aortic regurgitation.(ABSTRACT TRUNCATED AT 250 WORDS)     

Tetralogy of Fallot: influence of surgical technique on survival and reoperation rate.

BACKGROUND: The influence of different surgical techniques in patients with Tetralogy of Fallot (TOF) on long-term survival and reoperation rate is still a matter of controversy. PATIENTS AND METHODS: The data of 266 patients (152 male, 114 female, mean body weight: 13.4 +/- 11.9 kg, mean age 4.1 +/- 6.0 years) with TOF, including 30 patients (11.2 %) with TOF/pulmonary atresia, were analyzed retrospectively. A palliative surgical procedure preceded definite correction between 1975 and 1999 in 46 patients (17.5 %). The surgical technique consisted of a transannular patch in 103 patients (40.1 %); in 133 patients (51.8 %), a non-transvalvular technique was used. In 33 patients (12.3 %), a conduit was implanted into the right ventricular outflow tract (RVOT). For surgical access to the cardiac defects, a transventricular approach was used in 138 patients, and a transatrial/trans-pulmonary approach in 26 patients. The mean follow-up time of all patients was 6.6 years. RESULTS: Kaplan-Meier survival for the entire group was 86.2 % at one year and remained stable at 85.6 % after 5 years. The 30-day mortality was 10.1 % (27 patients). Fatal risk factors were: previous cardiac surgery, preoperative oxygen saturation (SO(2)) < 80 %, diagnosis of pulmonary atresia, prolonged aortic clamping or bypass time, postoperative infections, and early revisions. Freedom from reoperation was 91.6 % after 5, 84.5 % after 10, and 52.6 % after 20 years. There was no correlation between the different surgical techniques (transannular patch, non-transvalvular techniques, transventricular approach) applied and the mortality or the rate of reoperations. CONCLUSIONS: In our series of TOF patients operated over a period of 25 years, we were unable to assess an influence of the surgical technique used for definite correction on mortality or reoperation rate. Pulmonary atresia in patients with TOF is associated with a higher risk of death and reoperation.     

Range and distribution of NT-proBNP values in stable corrected congenital heart disease of various types

Background

Elevated levels of N-terminal pro-brain natriuretic peptide (NT-proBNP) are an expected finding in many adults with congenital heart disease (ACHD) but no reports have described the range of values within different diagnostic groups.

Methods

Between the years 2003 and 2011 we measured NT-proBNP in consecutive ACHD patients attending the outpatient department. Further NT-proBNP analysis was performed on 705 blood samples from 394 stable patients with the defect corrected. The results were compared among different diagnostic groups and with a control group of healthy volunteers.

Results

The median value of NT-proBNP in the whole cohort was signifinatly higher than in the control group (211 vs 42 pg/mL; P < 0.0001). The median value of NT-proBNP in pg/mL and the percentage of normal NT-proBNP values in the listed diagnostic groups were as follows: coarctation of the aorta (COA), 97 (64%); pulmonary stenosis (PS),160 (48%); secundum atrial septal defect (ASD), 254 (44%); incomplete atrioventricular septal defect (AVSD), 211 (32%); ventricular septal defect (VSD), 154 (58%); tetralogy of Fallot (TOF), 177 (38%); transposition of the great arteries (TGA) after Mustard, 237 (20%) or Senning correction, 143 (39%); Ebstein anomaly 287, (26%); pulmonary atresia (PA), 327 (18%); and complex defects after Fontan procedure, 184 (31%).

Conclusions

Normal levels of NT-proBNP were found most often in patients after the correction of coarctation of the aorta and ventricular septal defect with the highest levels found in pulmonary atresia and Ebstein anomaly. Our results may serve as reference values for different ACHD groups.     

Residuae, sequelae, and complications of surgery for congenital heart disease

Definitive, if not curative surgery is available for the eight most common congenital cardiac defects—ductus arteriosus, ASD, VSD, coarctation, pulmonary valve stenosis, aortic valve stenosis, tetralogy of Fallot, and transposition.The results of surgery for uncomplicated cases of DA, ASD, VSD, and coarctation usually can be determined by clinical means (including chest radiogram and ECG). Postoperative heart catheterization is recommended for evaluation of the patient who has had surgery for pulmonary valve stenosis or aortic stenosis and is necessary after tetralogy of Fallot or transposition of the great arteries repair to identify the important postoperative residua and sequelae.The term “curative” surgery probably should be reserved for operation for division of ductus arteriosus unassociated with pulmonary hypertension and performed in childhood.After closure of ASD, patients should continue to be observed for late development of arrhythmias and persistent cardiac enlargement, although the incidence of these problems is low.After VSD closure the patient is still followed at intervals for possible ill effects of the ventriculotomy scar, manifest as arrhythmias, ventricular aneurysm or myocardial insufficiency.The patient with coarctation repair must be observed for a possible late complication from one of the several clinically silent cardiovascular or cerebrovascular anomalies as well as for the chance of restenosis or unrelieved hypertension.Patients who have had an effective relief of the pressure gradient from pulmonary valve stenosis have a good outlook but should be observed for signs of myocardial insufficiency or the possible effects of increased load on the right heart resulting from pulmonary insufficiency.The high incidence of some degree of aortic insufficiency after valvotomy for aortic valve stenosis as well as the slight chance of restenosis necessitates follow-up examinations for these patients.After tetralogy of Fallot repair partially unrelieved infundibular stenosis or pulmonary annular stenosis and/or persistent VSD may require some limitation of physical activities even though the patient feels much improved because of disappearance of the cyanosis. Whether there are residual defects or not, patients should continue to be re-examined annually for an indefinite period of time because of possible myocardial insufficiency or arrhythmias.The problems after transposition repair include arrhythmias, right ventricular myocardial insufficiency, tricuspid insufficiency, persistent communications around the atrial baffle, and vena caval and/or pulmonary vein stenosis. Despite the frequent residua and sequelae of surgery, most patients with transposition obtain remarkable relief of symptoms.The use of exercise stress testing should increase the reliability of clinic assessment of the operative result. In those patients at risk for arrhythmias after surgery, electrophysiological studies at the time of postop cath may contribute to the management of the patient. The addition of ultrasound in the pre and postoperative evaluation of patients will aid the identification of certain clinically silent associated defects and some of the postoperative sequelae.The risk of subacute bacterial endocarditis remains in most patients after intracardiac repair of defects and, with few exceptions, patients who have had definitive surgery require long-term follow-up despite relief of symptoms and the disappearance of clinical manifestations of cardiovascular dysfunction.It is reasonable to expect that with improvement in diagnostic and surgical technique, many of the currently acceptable residua and sequelae of surgery for congenital heart disease will, in the future, be designated as avoidable complications in these patients. With the current healthy interest in long-term postoperative results there is a good chance that more precise information will become available to guide in the selection for surgery of the patient with congenital heart disease.     

Down综合征并发先天性心脏病的临床分析

目的：总结分析Down综合征（Down’s syndrome）并发的先天性心脏病畸形及血流动力学资料。方法：2008年7月-2012年10月,采用经胸二维超声心动图并彩色多普勒显像及右心导管／心血管造影检查方法,诊断36例并发先心病的Down综合征患者,本文通过36例临床资料分析,探讨Down综合征并发的先心病畸形及其血流动力学。结果：36例患者中室间隔缺损（VSD）10例,房室间隔缺损（AVSD）6例,动脉导管未闭（PDA）6例,房间隔缺损（ASD）2例,ASD＋PDA2例,ASD＋VSD1例,ASD＋PDA＋VSD1例,VSD＋PDA4例,PDA十二叶主动脉瓣（BAV）1例,法洛四联症（TOF）2例,TOF＋ASD1例,18例有肺动脉高压者,其中5例为阻力型肺动脉高压。结论：①Down综合征并发的心血管畸形中,以VSD、AVSD和PDA最为常见,并常并发ASD、TOF。②在无肺动脉狭窄的患者中,约50％并发有肺动脉高压。     

先天性心脏病门诊患者的分布特征

目的:调查和描述先天性心脏病（先心病）门诊患者的分布特征。方法: 从2005年1月起,由先心病内科专家门诊负责,对先心病明确诊断的初诊患者连续登记至2011年1月,共11 780例,由26种畸形组成。统计分析26种先天畸形构成比以及其中8种常见畸形（VSD、ASD、PDA、TOF、PS、AVSD、AS、TGA）在婴幼儿、儿童和成年人等阶段比例和单病种先心病中不同年龄段所占比例;4种常见先心病（VSD、ASD、PDA、AS）的3种并发症（肺动脉高压,心房颤动,感染性心内膜炎）呈现特点。结果: ①不同年龄段疾病构成比不同, VSD,TOF和TGA婴幼儿期明显高于成年人,VSD,TOF和TGA在婴幼儿和成人比例分别为53.8%和26.6%,8.6%和2.5%,5.1%和0.2%,而ASD和AS则成年人显著高于婴幼儿期,分别为41.1%和5.7%,6.8%和0.4%;②并发症呈现特点:艾森曼格综合征在VSD、ASD和PDA患者中绝大多数系成人,占97%,存活达50岁以上不足10%;心房颤动在ASD比较常见（2.4%）,其次是PDA（1.1%）,而VSD少见（0.2%）;感染性心内膜炎在AS中较常见,发生率为6.1%,VSD和PDA分别为0.52%和0.55%。 结论: 先心病在各种不同年龄阶段的构成比不同;婴幼儿期或儿童期手术可预防致残或影响生活质量的并发症。     

外科手术治疗先天性心脏病介入封堵术后并发症-附10例分析

目的:总结外科开胸手术方法治疗介入封堵动脉导管未闭(PDA)、房间隔缺损(ASD)和室间隔缺损(VSD)术后并发症。方法:对1995年至2010年10例PDA、ASD、VSD采用介入封堵后出现并发症而进行外科手术治疗的病例进行回顾性分析。其中严重主动脉瓣反流、三尖瓣反流各1例,Ⅲ°房室传导阻滞2例,封堵器移位5例,介入装置释放困难1例。10例患者均采用全麻体外循环下心脏直视手术,取出堵闭器、矫治心脏畸形。结果:10例患者均成功取出堵闭器,心脏畸形矫治满意,外科手术中开放升主动脉全部自动复跳,顺利脱机。10例患者中9例术后恢复良好,1例因肾功能衰竭及多脏器功能衰竭死亡。结论:PDA、ASD及VSD介入封堵治疗创伤小,术后恢复快,疗效肯定,但也存在一定的手术风险和出现严重并发症的可能。及时地行外科手术可以避免病情恶化、矫治心脏畸形,术后心功能恢复良好。     

先天性心脏病婴幼儿外科治疗对策分析(附115例报告)

目的 探讨婴幼儿先天性心脏病外科围术期处理对策.方法我院于2008年6月至2011年12月对115例患先天性心脏病的婴幼儿实施外科手术治疗,男性73例,女性42例,年龄3个月至3岁,体重3.5～20.0 kg;其中室间隔缺损(VSD)54例,法洛四联症(TOF)14例,室间隔缺损合并房间隔缺损(VSD+ASD)10例,房间隔缺损(ASD)9例,动脉导管未闭(PDA)8例,肺动脉瓣狭窄(PS)7例,室间隔缺损合并动脉导管未闭(VSD+PDA)5例,完全型肺静脉异位引流(TAPVC)4例,右室双出口(DORV)3例,主动脉缩窄合并室间隔缺损(CoA+VSD)1例.结果全组死亡5例,死亡率为4.3%.死亡原因:低心排出量综合征1例,肺高压危象2例,呼吸道梗阻2例.结论先天性心脏病患儿在婴幼儿期外科治疗可以取得满意疗效,根据婴幼儿生理、病理特点采取个体化治疗对策是成功的关键.     

Congenital heart disease: diagnostic approaches, surgical indications and operative results

Our management policies of the main congenital cardiac defects which require emergency surgical intervention in neonate and infancy were reported. Total anomalous pulmonary venous drainage (TAPVD): Recent refinements in two-dimensional (2-D) echocardiography have made it possible to operate for this anomaly using only this modality. When a combination of coarctation is suspected and/or the location of the drainage of the pulmonary vein is obscure, catheterization and angiocardiography are added for detailed diagnosis. It is, however, our policy not to perform right-sided angiocardiography for patients younger than three months of age. Symptomatic cases are operated urgently, although not always on an emergency basis. In our experience, a 12 approximately 24 hour delay with intensive cardiorespiratory and metabolic care may improve the preoperative conditions considerably and increase the chances of a successful surgical repair. Coarctation of the aorta (CoA) and interruption of the aortic arch (IAA): Diagnosis of CoA by echocardiography and aortography by radial artery injection is well established. In neonates and infants with CoA or CoA + patent ductus arteriosus (PDA) and/or ventricular septal defect (VSD), emergency repair of coarctation (usually with subclavian flap aortoplasty) without pulmonary artery banding (PAB) is undertaken on the day of established diagnosis. If a combination of complex cardiac anomalies such as transposition of the great arteries (TGA) and Taussig-Bing anomaly is suspected, catheterization and angiocardiography are added for the detailed diagnosis. Repair of coarctation combined with PAB has been our choice of procedure in these infants. If IAA is suspected, catheterizaton, angiographic and detailed echocardigraphic studies are performed to define the precise anatomy of the aortic arch and associated intracardiac lesions, paying particular attention to the left ventricular outflow tract, as soon as such patients become clinically stable by intensive medical treatment using prostaglandin E1 (PGE1), digitalis and diuretics. Once the diagnosis is established surgical treatment should be carried out without delay. Initial palliation by aortic arch reconstruction with PAB followed by two-stage definitive intracardiac repair have been our choice of procedure in neonates and infants with IAA without severe left ventricular outflow tract obstruction.(ABSTRACT TRUNCATED AT 400 WORDS)     

介入技术和外科手术同期"复合"治疗先天性心脏病的临床研究

目的结合介入器械和外科手术,实施“复合（Hybrid）”技术对先天性心脏病实施治疗。方法2005年3-10月,20例先天性心脏病患者接受了术中Hybrid技术治疗。球囊扩张组包括3例室间隔完整型肺动脉闭锁的新生儿和4例婴儿重度肺动脉瓣狭窄。缺损封堵组13例,包括10例房间隔缺损和3例多发室间隔缺损。正中或右侧腋下小切口进胸,在超声引导下经右室流出道置入球囊扩张管扩张肺动脉瓣或经右心房置入封堵器。2例多发室间隔缺损于体外循环下经三尖瓣置入封堵器。合并的其他心脏病变同期常规外科矫正,术式包括部分性肺静脉异位引流矫正、动脉导管结扎、冠状动脉旁路移植术等。术后心脏超声随访。结果患者全部顺利出院。1例婴儿重度肺动脉瓣狭窄术后2个月接受常规右室流出道成形术,1例多发肌部室间隔缺损因无法置入封堵器而转为常规术式。其余患者均顺利实施Hybrid术式。随访期内未发现介入器材相关的并发症。结论“复合”Hybrid技术可以避免体外循环,减少手术创伤,对于提高先天性心脏病的疗效具有重要的意受．     

Outcome of patients with double-inlet left ventricle or tricuspid atresia with transposed great arteries

OBJECTIVES: We sought to determine the long-term outcomes and risk factors for mortality in patients with double-inlet left ventricle (DILV) or tricuspid atresia with transposed great arteries (TA-TGA). BACKGROUND: Patients with DILV or TA-TGA are at risk of systemic outflow obstruction and a poor outcome. The impact of various management strategies on the long-term outcomes of these patients remains unknown. METHODS: We reviewed the outcomes of 164 consecutive pediatric patients with DILV or TA-TGA who underwent surgical palliation between 1983 and 2002. Patients with a Holmes heart or heterotaxy syndrome or who were lost to follow-up (n = 24) were excluded. Risk factors for mortality or the need for orthotopic heart transplantation (OHT) were assessed by multivariate analysis. RESULTS: There were 105 patients with DILV and 35 patients with TA-TGA. The overall mortality rate, including OHT, was 29%. Patients with DILV had a lower mortality rate than patients with TA-TGA (23% vs. 49%, p = 0.007). Multivariate analysis showed the presence of arrhythmia and pacemaker requirement as independent risk factors for mortality, whereas pulmonary atresia or stenosis and pulmonary artery banding were associated with decreased mortality. Gender, era of birth, aortic arch anomaly, and systemic outflow obstruction were not risk factors. The perioperative and overall mortality were similar between patients who underwent the Damus-Kaye-Stansel procedure beyond the neonatal period and those had subaortic resection. CONCLUSIONS: The mortality of patients with DILV or TA-TGA remains high. The outcomes of these patients are influenced by restriction of pulmonary blood flow, arrhythmia, and pacemaker requirement. Surgical palliation to relieve systemic outflow obstruction is not associated with a poor outcome.     

A new strategy for the surgical treatment of aortic coarctation associated with ventricular septal defect in infants using an absorbable pulmonary artery band.

OBJECTIVES: We propose a new strategy using coarctation repair together with a polidioxanone absorbable pulmonary artery banding to limit operative risk and to spare infants with aortic coarctation subsequent operations. BACKGROUND: The alternative for the surgical management of aortic coarctation associated with ventricular septal defect (VSD) is single-stage repair versus coarctation repair with or without banding of the pulmonary artery. METHODS: Eleven infants (mean weight 2,560 +/- 1,750 g, range 1,320 to 3,800 g) underwent a coarctation repair with a polydioxanone banding. Seven had a trabecular and four a perimembranous VSD. The mean size of the VSD was 5 +/- 0.7 mm (range 4 to 7 mm). The systolic pulmonary pressure was >80% of the aortic pressure in all. The pulmonary band was tightened until the systolic pulmonary pressure fell below 50% of the aortic pressure. RESULTS: There were no hospital deaths. The reabsorption of the banding was complete after 5.7 months in all patients (3 to 6.5 months). The VSD closed completely in four infants and partially in six, in whom the pulmonary artery pressure was normal without evidence for significant left-to-right shunt. One patient with a large trabecular VSD underwent surgical closure of his defect after four months. Finally, a subsequent open-heart surgery could be avoided in 91% (10/11) of patients. CONCLUSIONS: Provided the VSD belongs to types prone to close spontaneously, this policy may reduce the number of surgical procedures per infant as well as in-hospital mortality and morbidity rates. It should be proposed as an alternative to more complex procedures.     

Association of 22q11 deletion with isolated congenital heart disease in three Chinese ethnic groups

BACKGROUND: Congenital heart disease (CHD) is the most common type of heart disease among children. About 75% of DiGeorge syndrome (DGS) and velo-cardio-facial syndrome (VCFS) includes CHD. A deletion within chromosome 22q11.2 has been identified in the majority of patients with DGS and VCFS. And 22q11.2 deletion has become one of the markers used to study CHD in these syndromes. Whether 22q11.2 deletion is associated with isolated CHD is not known and was the topic of this study. METHODS AND RESULTS: We studied the 22q11.2 deletion in three Chinese ethnic groups (Tai, Bai and Han people) with 19 sporadic, isolated CHD by genotype and haplotype analysis with D22S420 etc. 11 consecutive polymorphic microsatellite markers. Among 19 isolated CHD patients, four had Tetralogy of Fallot (TOF), five exhibited Ventricular Septal Defect (VSD), five showed Atrial Septal Defect (ASD) and 5 had Patent Ductus Arteriosus (PDA). In some isolated CHD patients, 3 Mb and 1.5 Mb deletion to chromosome 22q11.2 was found. 2 of 4 TOF (50%) and 1 of 5 VSD (20%) and 1 of 5 PDA (20%) respectively were found to have deletions at D22S944. CONCLUSIONS: 22q11.2 deletion can be detected in isolated TOF, VSD and PDA of three Chinese ethnic groups, without detectable 22q11.2 deletion in those isolated ASD patients examined thus far. Our finding may be the first to show the 22q11.2 deletion in sporadic, isolated PDA/VSD patients whose family members are without CHD. In addition, D22S420 etc. 11 consecutive polymorphic microsatellite markers are very useful for the determination of 22q11.2 deletion in isolated CHD in China.     

Mitral valve atresia with normal aortic valve and pulmonary atresia. A case with pulmonary collateral vascular perfusion by systemic pulmonary arteries

A case of mitral atresia with normal aortic valve, situs solitus, d-loop, ASD, VSD, DORV and atresia of the pulmonary valve is presented. Systemic-pulmonary collateral arteries were the single source of collateral lung perfusion. These vessels are often found in patients with tetralogy of Fallot and pulmonary atresia with VSD. The case favours the idea that these vessels are not specific for the above mentioned cardiac anomalies. It supports the theory that they represent true embryonal vessels which can persist in cases of an inadequate or completely missing connection of the sixth aortic arch derivates with the pulmonary vascular bed.     

Congenital heart surgery in adults: An Australian experience of 379 cases

Background: Congenital heart disease (CHD) may present for the first time in adult life and may require operative repair. Due to technical advances in CHD surgery, an increasing population of children with CHD are surviving into adult life, some of whom need further surgery for residual or complicating lesions. Little is known about the outcomes of CHD surgery in adult patients. Aim: To assess the frequency, types and outcomes of congenital heart surgery in adults. Methods: Retrospective review of hospital records. Long-term outcome data were obtained by questionnaires and from the State Registry of Births, Deaths and Marriages. Results: From 1982–1995, 379 adults aged 34±16 (range, 16–73) years, comprising 144 males and 235 females, were operated on for CHD. In 328 patients, this was their first operation: closure of atrial septal defect in 212 (64%, secundum type in 180, sinus venosus in 14, primum in 18); closure of a persistent ductus arteriosus in 30; repair of coarctation of the aorta in 30; closure of ventricular septal defects in 17; repair of tetralogy of Fallot in 10, and 29 others. Fifty-one cases were reoperations and included Fontan repair in 8, revision of coarctation of the aorta in 8, and complete repair of pulmonary atresia/ventricular septal defect after earlier palliation in 5 subjects. There were 7 perioperative deaths (2%) within 30 days of surgery. After median follow-up of 8 years, there were 27 late deaths (7%). Most survivors are in functional Class I. Conclusions: Congenital heart disease surgery is often required in adults, both for first operations and redo procedures, many of which are for complex heart disease. Despite this, good perioperative and long-term results may be obtained.     

Mode of death in adults with congenital heart disease

An increasing number of patients with congenital heart disease (CHD) are entering adulthood. Although prior studies have focused on the causes of death in the pediatric population, the modes of death for adults with CHD have not been well defined. In a cross-sectional study performed on a population of 2,609 consecutive adults assessed at a CHD specialty clinic, there were adequate information available in 197 of 199 deceased patients. Mean age at death was 37 +/- 15 years. Mortality was highest in patients with congenitally corrected transposition of the great arteries (26%), tricuspid atresia (25%), and univentricular connection (23%). Youngest mean age at death was observed in patients with tricuspid atresia (27 +/- 5 years), complete transposition of the great arteries (27 +/- 7 years), pulmonary atresia (27 +/- 6 years), and aortic coarctation (29 +/- 6 years). Sudden death (26%) was the most common cause of death followed by progressive heart failure (21%) and perioperative death (18%). Postmortem examinations were performed in 77 of 197 deceased patients (39%) and provided incremental data on the mechanism of death in 22% of autopsies. Thus, the 3 major causes of death in the growing population of adults with CHD are sudden, perioperative, and progressive heart failure.     

先心病合并重度肺动脉高压双向分流的外科对策

目的:探讨先天性心脏病伴重度肺动脉高压双向分流的外科对策及疗效。方法:回顾分析32例患者的临床资料,其中,室间隔缺损(VSD)22例,VSD合并房间隔缺损(ASD)5例,动脉导管未闭5例。术前均有不同程度劳力后紫绀,心脏B超提示双向分流,术中测量平均肺动脉压/平均体循环压>0.85,平均0.90±0.15,采用心脏停跳手术6例,不停跳手术26例。房间隔均采用自体心包片做成单向活瓣,以减轻术后早期右心负荷。结果:早期死亡3例(9.4％,3/32)。术后早期均有明显低氧血症,呼吸机支持,平均35.26±17.59小时,无气管切开病例。随访2～86(平均23.6±17.2)月,晚期死亡2例(6.3％,2/32),顽固右心衰3例(9.4％,3/32),其余24例(75％,24/32)恢复良好。结论:先心病伴重度肺动脉高压部分双向分流的病例,采用浅低温心跳不停心内直视手术有利于心肺功能保护,术中作房间隔单向活瓣有利于其早期恢复。