Pulmonary atresia with obstructed ventricular septal defect

Summary Two patients with pulmonary atresia had a loud holosystolic murmur and an obstructed ventricular septal defect. Case 1 simulated pulmonary atresia with intact ventricular septum, presenting at 3 months of age with venous congestion, severe tricuspid regurgitation, and suprasystemic right ventricular pressure. Case 2 had a right ventricular systolic pressure that was initially at the systemic level but had increased to a suprasystemic level by 3 years of age. Autopsy showed that the ventricular septal defect was large in each case but was obstructed by tricuspid valve tissue in case 1 and by a hypertrophied septal band in case 2.Supported in part by the University of Illinois Foundation Goodenberger Medical Research Grant (2-44-33-66-3-14)     

肺动脉闭锁合并室间隔缺损104例诊断分析

目的　分析肺动脉闭锁合并室间隔缺损 (PA/VSD)的解剖类型及血流动力学改变 ,探讨其适宜的手术方式。方法　对 1992年 6月至 2 0 0 2年 5月在广东省心血管病研究所儿科住院的PA/VSD患儿共 10 4例 ,采用超声心动图结合心血管造影术 ,确定闭锁的部位、肺动脉的发育情况及血供来源。结果　右室流出道及瓣膜闭锁、有肺动脉总干 31例 ;肺动脉总干闭锁、左右肺动脉有汇合 5 0例 ;左肺动脉闭锁 10例 ,右肺动脉闭锁 8例 ;左右肺动脉均闭锁 5例。肺动脉的血供来源 :大的主肺动脉侧支血管 5 1例 ,动脉导管未闭 2 7例 ,多支小的侧支血管 2 6例。合并畸形有卵圆孔未闭、房间隔缺损、大动脉转位、完全性房室间隔缺损、右室双出口、镜面右位心、右旋心及左旋心。结论　合并室间隔缺损的肺动脉闭锁可发生在不同部位 ,肺动脉的血供来源多样化     

肺动脉闭锁合并室间隔缺损的临床病理分析

目的 研究肺动脉闭锁合并室间隔缺损的解剖类型和血流动力学改变,并探讨其适宜的手术方式.方法 回顾性分析233例肺动脉闭锁合并室间隔缺损的造影结果,分析肺血来源、肺动脉发育情况和合并畸形及其与手术预后的影响.结果 233例中中央肺动脉存在,肺血单纯由未闭的动脉导管供应者112例(48.1%),其中1例为双侧动脉导管(0.5%);中央肺动脉和大的主一肺动脉侧支血管(MAPCA)均存在者104例(44.6%);无中央肺动脉,仅有MAPCA供应肺血者17例(7.3%).肺动脉闭锁部位以右室流出道和瓣膜闭锁最多见(48.1%).侧支血管的来源包括直接的主动脉一肺侧支动脉,间接的主动脉一肺侧支动脉和支气管动脉.合并畸形包括心脾综合征、房室连接不一致、心室大动脉连接不一致、多发室间隔缺损、右位主动脉弓、房间隔缺损、左上腔静脉残存、内脏异位症、上下心室、肺静脉异位引流和冠状动脉起源异常等.结论 肺动脉闭锁合并室间隔缺损患者肺血来源多样化,肺动脉发育程度不一,并可合并多种心内、心外畸形,影响手术方法的选掸和手术结果.     

Pulmonary blood supply by a branch from the distal ascending aorta in pulmonary atresia with ventricular septal defect: Differential diagnosis of fifth aortic arch

Summary A patient with pulmonary atresia and a ventricular septal defect is described in whom an arterial branch from the distal ascending aorta supplied segments of both lungs. The branch is considered to represent a persistent fifth aortic arch. The possible morphogenesis and differential diagnosis of a communication between the ascending aorta and the pulmonary artery in pulmonary atresia with ventricular septal defect are discussed.     

Outcome of pulmonary atresia and ventricular septal defect during infancy

We evaluated 54 patients with pulmonary atresia and ventricular septal defect who were referred during the first year of life between 1972 and 1992. Particular emphasis was given to the nature of the pulmonary blood supply and its influence on outcome. Ductal supply of confluent pulmonary arteries was present in 30 patients (55.6%, group I), whereas 24 patients (44.4%, group II) had a pulmonary blood supply that was entirely (31.4%) or predominantly (13.0%) dependent on systemic collateral arteries. Over the 20 years these was no significant difference in actuarial survival between the two groups. Corrective surgery was performed in 8 of 30 patients in group I (26.7%)-significantly more than in group II (4 of 24, 16.7%). Arborization abnormalities of the pulmonary arteries (stenosis of unbranched and intrapulmonary arteries) were almost exclusively present in patients with systemic collateral arteries (p<0.03), accounting for the lower probability of undergoing corrective surgery in group II patients. During the first decade of this study (1973–1983) corrective surgery was attempted in 9.6% of patients, with 42% mortality; and during the second decade (1983–1993) surgery was performed in 39.1% of patients, with 26% mortality, a significantly lower figure. Improving surgical results, complete preoperative demarcation of the pulmonary blood supply, and a more aggressive approach with early unifocalization of the pulmonary blood supply may invalidate comparison with retrospective data on the advisability of attempting to correct this anomaly. The present paper provides data against which treatment of infants with pulmonary atresia and ventricular septal defect presenting during the next decade can be compared.     

Clinical relevance of monosomy 22q11.2 in children with pulmonary atresia and ventricular septal defect

The purpose of our study was to describe the prevalence and the clinical spectrum of monosomy 22q11.2 in a population of patients with pulmonary atresia and ventricular septal defect. We examined all 44 patients with this conotruncal cardiac malformation who presented to our institution from January 1994 until December 1997. The type of collateral lung perfusion was recorded including anomalies of the pulmonary arteries as well as facial and immunological abnormalities. Molecular-cytogenetic testing for a 22q11.2 microdeletion was performed using the probes D22S75 and cHKAD26. Statistical differences were evaluated with the Fisher's Exact Test. Monosomy 22q11.2 was present in ten children (23%) with major aortopulmonary collateral arteries (group 1). The remaining 13 children (29%) with major aortopulmonary collateral arteries (group 2) and all 21 children (48%) with ductus arteriosus (group 3) were negative for this microdeletion. All children in group 1 had facial anomalies, six had mild immunological abnormalities including decreased CD 4+ or CD 8+ cells. Anomalies of the pulmonary vascular bed were significantly more frequent in children of group 1 (9/10) than in children of group 2 (4/13) or group 3 (0/21). Due to these pulmonary vascular anomalies, corrective surgery had been accomplished in fewer children with monosomy 22q11.2 (none in group 1) as compared to 7/13 children in group 2 and 14/21 children in group 3. Conclusion In children with pulmonary atresia and ventricular septal defect, monosomy 22q11.2 is preferentially associated with major aortopulmonary collateral arteries. Due to the higher incidence of pulmonary arterial abnormalities, successful surgical repair will require a different therapeutic approach in most patients with this microdeletion. Received: 3 June 1998 / Accepted in revised form: 11 September 1998     

An overview of pulmonary atresia, ventricular septal defect, and multiple aorta pulmonary collateral arteries

The management of infants and children with pulmonary atresia, ventricular septal defect, and multiple aorta pulmonary collateral arteries has proven to be challenging. Therapeutic approaches have included unifocalization, shunting, coiling of collateral vessels, and heart/lung transplantation. Results have been variable and frustrating. This review will cover the anatomy, physiology, initial medical management, subsequent surgical management, and controversies surrounding various therapeutic approaches. The evolving understanding of genetic considerations in this condition will also be explored.     

肺动脉闭锁合并室间隔缺损与大型主肺侧支动脉的外科治疗及研究进展

肺动脉闭锁合并室间隔缺损与大型主肺侧支动脉是一类少见的先天性心脏病.目前,随着外科治疗的不断进步以及对该疾病的更深入的认识,分期手术的治疗方案已经得到了越来越多外科医生的认同.     

Failure of color flow mapping to identify coronary artery to pulmonary artery fistula

Summary A case of coronary artery to pulmonary artery fistula presented with the typical clinical findings of asymptomatic precordial continuous murmur and small left-to-right shunt. Echocardiography with color flow mapping failed to demonstrate the fistula on three occasions, including one study performed after angiographic demonstration of the fistula site. This case illustrates the importance of angiography in patients with unexplained continuous murmurs, even when echocardiographic and color flow mapping results are normal.     

Unifocalization and repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries

Aim: To correlate anatomic and genetic features of paediatric patients with pulmonary atresia, ventricular septal defect (VSD) and multiple aortopulmonary collateral arteries with surgical outcome.
Methods: 44 consecutive patients aged 33±40 mo underwent either primary one-stage unifocalization ( n =32) or palliative right ventricular outflow tract reconstruction ( n =12) followed by secondary unifocalization and repair ( n =10) based on preoperative morphometric and functional evaluation of pulmonary blood sources. Chromosome 22q11.2 microdeletion occurred in 41% of cases. Combined VSD closure during one-stage procedures was guided by an intraoperative pulmonary flow study. Complete repair was accomplished in 35 cases (83%, 95% CI 72–95%). Variables examined included occurrence of confluent intrapericardial pulmonary arteries, central pulmonary arteries, confluent intraparenchymal pulmonary arteries, dominant collateral or pulmonary arteries, and chromosome 22q11.2 microdeletion. The sensitivity and specificity of the pulmonary flow study in predicting postoperative pulmonary haemodynamics were also tested.
Results: Eight-year actuarial survival and freedom from reoperation were 85% and 63%, respectively. Sensitivity and specificity of the pulmonary flow study were 94% and 100%, respectively. None of the anatomical variables examined was significantly related to the outcome of treatment. The only statistically relevant association was detected between survival and occurrence of 22q11.2 microdeletion ( p <0.003). Logistic analysis showed an increased likelihood of positive outcome in relation to first- ( p <0.02) or second-stage ( p <0.04) complete correction.
Conclusion: Morphology of pulmonary blood supply has no major impact on surgical outcome. Pulmonary flow study is a highly specific and sensitive intraoperative test. Chromosome 22q11.2 microdeletion remains the only variable significantly affecting survival.     

Two-dimensional and Doppler echocardiographic features of left circumflex coronary artery to right ventricle fistula: Case report and literature review

Summary We report the case of a seven-month-old infant with a clinical diagnosis of patent ductus arteriosus whose two-dimensional and Doppler echocardiographic examinations were consistent with a coronary artery fistula. At angiography, a left circumflex coronary artery to right ventricle fistula was diagnosed. Echo-Doppler techniques for the noninvasive diagnosis of this lesion are discussed and the literature is reviewed.     

Pulmonary arterial changes in patients with ventricular septal defects and severe pulmonary hypertension

Summary In 25 patients, aged eight months to 31 years, with ventricular septal defect (VSD; isolated in 15, the others with atrial septal defect, PDA, coarctation or patent ductus arteriosus + coarctation), each with severe pulmonary artery hypertension (pulmonary artery systolic pressure [Ppa] at least 75% of systemic and an elevated pulmonary vascular resistance), we related morphologic and morphometric data from open-lung biopsy to hemodynamic measurements obtained at cardiac catheterization during the same hospital admission. Of the hemodynamic features measured, only the ratios of pulmonary-to-systemic flow and pulmonary-to-systemic resistance correlated significantly with structure. Neither pulmonary artery pressure (Ppa) nor pulmonary vascular resistance correlated significantly with any structural feature studied. The increased external diameter of respiratory bronchiolar arteries in those with the more advanced Heath-Edwards grades reflects dilatation and suggests that it is in the small arteries of the distal arterial bed that the changes of pulmonary hypertension are most significant. Neither age nor body weight correlated significantly with the degree of structural or hemodynamic abnormality. In the ten patients who underwent VSD closure, Ppa was measured postoperatively. The Heath-Edwards grade (no more than one grade-III lesion) and arterial density (at least one-half that normal for age) were the best correlates of the difference between preoperative Ppa and Ppa immediately after corrective surgery. The presurgical catheterization data, including pulmonary resistance and the resistance ratio, did not correlate significantly with change in Ppa following VSD closure. Lung biopsy is an important diagnostic aid because it helps in predicting the early response in postoperative Ppa in patients with VSD and elevated pulmonary vascular resistance.     

The influence of ventricular morphology and coronary artery dominance and morphology on early mortality in patients with pulmonary atresia and intact ventricular septum

Coronary artery abnormalities are known to be present in a certain percentage of patients presenting with pulmonary atresia and intact ventricular septum. These abnormalities range in severity from small fistulae to complete right ventricular dependence of components in the coronary circulation. These abnormalities are known to complicate therapy preoperatively, during palliation, and during definitive correction. This study will explore the influence of right ventricular morphology and coronary artery dominance and morphology on early mortality in infants with pulmonary atresia and intact ventricular septum, something which has not been previously explored.     

婴幼儿室间隔缺损并肺动脉高压的外科治疗

目的 评估婴幼儿VSD并肺动脉高压(PH)的纠治方法及效果.方法 收集河北医科大学第一医院心外科VSD并PH婴幼儿215例.男109例,女106例;月龄1～36个月;体质量1.5～10.0 kg.膜周型VSD 156例,嵴内型18例,干下型41例.VSD直径0.8 ～2.1 cm.并继发性ASD 16例,卵圆孔未闭8例,PDA15例,并PDA、ASD 14例,并PDA和主动脉瓣下隔膜3例,肺动脉瓣狭窄14例,右心室流出道狭窄33例,二尖瓣关闭不全22例,三尖瓣关闭不全42例,永存左上腔静脉12例.肺动脉压力4.66～11.31 kPa.患儿均在全麻体外循环下行一期根治术.结果 全组手术顺利.术后死亡3例.术后并发症:15例出现短暂低心排出量综合征,其中死亡2例;10例右束支传导阻滞;6例室上性心动过速;5例短暂Ⅲ度房室传导阻滞;5例术后发生PH危象,其中死亡1例;3例气胸;2例肺不张.随访期均行X线胸片、ECG、心脏彩超检查,术后心功能明显改善.结论 婴幼儿VSD并PH病情发展快,易发生低心排出量综合征和低氧血症,早期手术效果良好,治疗的关键在于加强心肌保护,提高手术操作技术,缩短手术时间;术后加强监护,及时处理各种术后并发症.     

Criss-cross heart—a case with horizontal septum,complete transposition,pulmonary atresia and ventricular septal defect

Summary Criss-cross heart is a recently described anomaly in which the systemic and pulmonary blood streams cross at the atrioventricular (AV) level, without mixing. A case of criss-cross heart is described in which the right atrium, in a solitus position, communicated with a left-superior positioned, morphologically right ventricle, and the left atrium communicated with a normally located, morphologically left ventricle. The interventricular septum occupied a horizontal plane. Associated defects were complete d-transposition of the great arteries with l-positioned aorta, pulmonary atresia, venticular septal defect, atrial septal defect, and patent ductus arteriosus. To the best of our knowledge this is the first angiocardiographic demonstration of this rare combination of lesions. The literature on criss-cross heart and horizontal septum is reviewed. It is stressed that regardless of whether the criss-cross phenomenon is an anatomical fact or an angiocardiographic illusion, it is an established angiocardiographic entity and should be recognized as such.     

Hypoplasia of the intrapulmonary arteries in children with right ventricular outflow tract obstruction,ventricular septal defect,and major aortopulmonary collateral arteries

Summary Postmortem injection studies have been carried out on the pulmonary vasculature of four children dying with pulmonary atresia and ventricular septal defect or severe tetralogy of Fallot with major aortopulmonary collateral arteries, in which nearly all bronchopulmonary segments had more than one source of blood supply. Despite regional variations in the source of blood supply, there was remarkable uniformity of arterial size and number within the respiratory unit throughout each case. In all cases, there was a normal number of arterial pathways, but both pre- and intraacinar arteries were considerably smaller than normal. The need for early operative intervention to ensure growth of pre- and particularly intraacinar arteries is emphasized.     

Closure of ventricular septal defect through the pulmonary artery

Summary A transpulmonary arterial approach to the closure of a high ventricular septal defect (VSD) has been used, between 1978 and 1982, in eight patients. The reasons were ease of access and the wish to overcome the problems associated with right ventriculotomy. The patients' ages ranged from three weeks to 15 months, their weight from 2.9 kg to 9 kg. The approach was used both when the VSD was an isolated anomaly and when there were major associated defects. It is in this latter group, four with aortic arch anomalies, two with additional double outlet right ventricle (DORV), that avoidance of ventriculotomy was most helpful. It was especially important in the two patients with DORV and a perimembranous, outlet subpulmonary VSD, where it was possible to close off the left ventricular outflow tract and pulmonary valve using a patch, without opening the right ventricle, which was subsequently to become the systemic ventricle. This technique obviates the need for ventriculotomy in the closure of some perimembranous outlet and doubly committed subarterial VSDs, and is the approach of choice for the closure of a perimembranous, outlet, subpulmonary VSD in DORV.     

Necrotizing arteritis in uncorrected tetralogy of fallot with pulmonary atresia

Summary A 10-year-old girl with uncorrected tetralogy of Fallot with pulmonary atresia presented with fevers of unknown origin and left lung infiltrates. At autopsy, necrotizing vascular changes resembling those of severe pulmonary hypertension (grade VI in the Heath-Edwards classification) were confined to the left lung. Pulmonary blood flow and pressure were greater in the left lung and were provided by an enlarged collateral artery arising directly from the descending thoracic aorta. To our knowledge, this is the first report of necrotizing arteritis of the pulmonary arteries in uncorrected tetralogy of Fallot with pulmonary atresia.The opinions or assertions contained herein are the private views of the authors and are not to be construed as official or reflecting the views of the Department of the Army or the Department of Defense.     

Membranous septal aneurysm: an unusual cause for right ventricular outflow tract obstruction in a malaligned ventricular septal defect with aortomitral discontinuity (double-outlet right ventricle) associated with visceral heterotaxy

The development of a septal aneurysm in the natural history of membranous ventricular septal defects usually makes the defect hemodynamically less significant. This report describes a case of severe right ventricular outflow obstruction produced by a membranous septal aneurysm in a patient who had an anterior malaligned ventricular septal defect with aortomitral discontinuity (double-outlet right ventricle). This patient did not have pulmonary stenosis other than the dynamic obstruction produced by the septal aneurysm. In this patient, the septal aneurysm produced both favorable and unfavorable hemodynamic effects. A reduction in the size of the ventricular septal defect produced a favorable effect, whereas a right ventricular outflow obstruction led to the unfavorable situation of right ventricular hypertension and hypertrophy. The large septal aneurysm in the presence of an already compromised right ventricular outflow tract related to an anteriorly malaligned septum resulted in severe obstruction.     

Congenital left coronary artery — right ventricular fistula

ABSTRACT. The case of a neonate with heart failure and myocardial ischaemia due to a large coronary artery fistula is reported. ECG monitoring and Thallium — 201 imaging indicated pre-operative myocardial ischaemia. The diagnosis was confirmed by cardiac catheterization. Successful surgical ligation of the fistula was performed at the age of 10 days and to our knowledge is the first successful ligation in the neonatal period. Follow up Thallium — 201 imaging was normal. Repeat cardiac catheterization demonstrated complete closure of the fistula and normal left ventricular function.