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.     

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.     

Imaging of aortopulmonary collateral arteries with high-resolution multidetector CT

Background Precise visualization of the pulmonary vasculature is mandatory for adequate treatment of patients with pulmonary atresia and ventricular septal defect (PA-VSD). Aortopulmonary collateral arteries (APCs) can be visualized by selective injections of contrast agent in the catheterization laboratory.Objective To evaluate multidetector CT (MDCT) and different image postprocessing methods for analysis of complex pulmonary blood supply in patients with PA-VSD.Materials and methods Eight patients (6 weeks to 27.8 years of age) with PA-VSD and APCs underwent MDCT and cardiac catheterization. Using multiplanar reformatting, volume rendering and semiautomatic segmentation algorithms, the aorta, pulmonary arteries and APCs were displayed. MDCT and cardiac catheterization were analyzed by two independent observers.Results MDCT accurately imaged central pulmonary arteries (n=8), aortopulmonary shunts (n=2), right ventricular to pulmonary artery conduits (n=2) and origin, course and intrapulmonary connections of APCs (n=25), compared to X-ray angiography. A high correlation was found between the MDCT vessel diameter measurements by two independent observers (n=70, r=0.96, P<0.01) and between MDCT and angiographic vessel diameter measurements (n=68, r=0.96, P<0.01).Conclusions Using three-dimensional imaging software, a complex pulmonary blood supply can be non-invasively and accurately imaged with high-resolution MDCT. This technique may help to reduce the number of cardiac catheterizations or guide interventional or surgical therapy.Electronic supplementary material Supplementary material is available for this article at     

Growth of pulmonary artery after arterial switch operation for simple transposition of the great arteries

This retrospective study attempts to assess the size and growth pattern of the pulmonary artery about 1 year after neonatal arterial switch operation for simple transposition of the great arteries. Sixty-seven patients underwent cardiac catheterization, including catheterization of the right and left pulmonary arteries, and right ventricular angiography an average of 13.9 months after arterial switch operation. In 34 of these patients pre-operative right ventricular angiocardiograms were available. The diameter of the main pulmonary artery and that of its proximal right and left branches were measured. The values were compared to those of normal children matched for body surface area, taken from the literature and, for the branch values, related to the degree of branch stenosis and to the corresponding values, measured on pre-operative angiocardiograms. The cross-section of the main pulmonary artery after arterial switch operation with Lecompte manoeuvre becomes oval. The branches of the pulmonary artery are sometimes underdeveloped and this finding is related to branch stenosis. The ratio of the branch gradients is inversely proportional to the growth ratio of both branches while the pre- and postoperative Nakata indices are identical. Conclusion The Lecompte manoeuvre induces a flattening of the main pulmonary artery with concomitant reduction of its cross-sectional area. There is also frequently trivial or rarely moderate branch pulmonary stenosis which is accompanied by growth retardation of the concerned branch. Received: 15 April 1997 / Accepted: 14 July 1997     

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.     

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 Intrapulmonary Arterial Pattern in Normal Infancy And in Transposition of the Great Arteries

Microangiographic and histologic studies of the pulmonary and bronchial arterial systems were carried out on 52 normal human fetuses, infants and children and on a series of 17 infants with transposition of the great arteries, isolated or associated with an interventricular septum defect. Particular attention was paid to various types of aberrations from the basic arterial pattern: arterial bronchopulmonary anastomoses, pulmobronchial arteries and bronchopulmonary arteries.
The arterial bronchopulmonary anastomoses are of essentially the same type in normal cases as in transposition of the great arteries. The normal number of anastomoses is greater in infancy and early childhood than in the late fetal and neonatal period. From the age of a few months, however, many of the anastomoses become narrowed or obliterated. Abnormal pattern of the pulmonary arterial system, including abnormal pulmobronchial arteries, is a prominent feature in isolated transposition, less so in transposition associated with interventricular septum defect. In isolated transposition there is a considerable increase in the bronchial-artery supply of the pulmonary parenchyma proper via bronchopulmonary arteries. In transposition associated with interventricular septum defect, the bronchial-artery supply of the pulmonary parenchyma has a normal extent or is only moderately increased.     

Bronchopulmonary Dysplasia: A Morphometry Study with Emphasis on the Pulmonary Vasculature

Using morphometry, the pulmonary vasculature in lungs obtained at autopsy from 8 patients with bronchopulmonary dysplasia (BPD) was studied. In these specimens the axial arterial pathway was similar in length to those of equivalently aged fetuses and increased with lengthening survival. The internal diameter of axial arteries was variable—excessively wide in 2 patients and diffusely narrow in 3. Microscopically, the percent medial thickness of muscular pulmonary arteries was reduced compared to fetal values and tended to be less in older patients. Compared to normal fetuses, there were more muscularized intraacinar arteries, suggesting peripheral extension of smooth muscle. Arterial concentrations were elevated in 2 long-term survivors. The weight of the cardiac right ventricle was reduced in 4 patients. These findings indicate that in patients with BPD there is a complex dual process of pathological remodeling and an attempt at normal anatomic adaptation of the pulmonary vasculature to extrauterine life.     

Morphometric Study on Pulmonary Arterial Thickness in Pulmonary Hypoplasia

This study addresses the question of whether pulmonary hypoplasia is associated with structural changes of the pulmonary arteries. Quantitative (medial thickness, external radius) and qualitative (airway level) parameters of pulmonary arteries were assessed in eight hypoplastic lungs and eight lungs from age-matched fetuses without underlying primary pulmonary disease. The gestational ages ranged from 16 to 40 weeks. Relative medial thickness of the pulmonary arteries was related to the external radius and to the type of accompanying airway. Medial thickness was significantly higher in pulmonary hypoplasia than in normal fetal lungs. In contrast to normal fetal lungs, muscular arteries extended to the intra-acinar level in pulmonary hypoplasia. These arteries had medial thickness values up to a maximum of 80%.     

Clinical Investigation of Systemic-Pulmonary Collateral Arteries

Systemic-pulmonary collateral arteries are known to develop in children with congenital heart disease, chronic pulmonary infection, and prematurity. At present, these abnormal connections between the systemic and the pulmonary systems are thought to develop from the vascular plexus, which normally gives rise to the pulmonary and bronchial vasculature. The objective of this study was to review our patients with systemic-pulmonary collateral arteries and evaluate possible risk factors. The records of patients with systemic-pulmonary collateral arteries seen at our hospital over a 4-year period were retrospectively reviewed. They were grouped into one of the following five categories: premature, heart disease, pulmonary disease, healthy, and others. Age, gender, weight, and the results of echocardiography were recorded, as was the condition on follow-up. We reviewed the records of 284 patients: 130 premature, 13 heart disease, 30 pulmonary disease, 92 healthy, and 19 others. Over the same period, 3314 healthy 1-month-old infants had undergone echocardiography for health screening. Among the 92 healthy children with systemic-pulmonary collateral arteries, 52 were diagnosed at the health-screening exam. Therefore, we estimate the incidence of unsuspected systemic-pulmonary collateral arteries in healthy 1-month-old infants to be 1.57% (52/3314). We conclude that systemic-pulmonary collateral arteries may be present normally after birth and then gradually disappear. However, if there are certain predisposing factors, they may persist in order to augment pulmonary flow.     

Dual-Catheter Balloon Occlusion Aortography in Pulmonary Atresia with Ventricular Septal Defect and Major Aorto-Pulmonary Collaterals

Angiography remains the gold standard in the preoperative evaluation of complex pulmonary blood supply in patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries. In neonates, balloon occlusion aortography using a Berman balloon catheter is a very effective technique. However, in older patients this method is frequently limited due to failure to achieve distal balloon occlusion. A novel technique using a balloon valvuloplasty catheter and a standard angiographic catheter in combination is described. The technique allowed enhanced visualization of the complex anatomy in three patients scheduled for surgical intervention.     

Congenital pulmonary arteriovenous fistula producing pulmonary arterial steal syndrome

Summary This report describes a congenital pulmonary arterial steal syndrome manifested as cyanosis and acidosis in a newborn. A fistulous connection between the right pulmonary artery and a large, anomalous right common pulmonary vein stole blood from the pulmonary arteries. The anomaly was suspected because of a pericardiac shadow on frontal and lateral chest films, substantiated by M-mode echocardiogram, confirmed at cardiac catheterization with angiocardiography, and analyzed at postmortem examination. Supported in part by a grant from The Rose M. Badgeley Charitable Trust     

完全性大动脉错位动脉转位术后新主肺动脉发育的随访

目的 报道应用动脉转位术（ASO）治疗完全性大动脉错位（TGA）术后超声心动图的随访结果,评价TGA术后主、肺动脉的发育情况。方法 以2001年3月至2007年3月于复旦大学附属儿科医院心血管中心实施ASO治疗TGA术后存活患儿作为研究对象,于2008年8～9月进行随访,行超声心动图检查,测定新主动脉根部内径、新主动脉瓣环内径、新肺动脉内径和新肺动脉瓣环内径,获取的数据与正常参考值做比较。 结果 研究期间ASO治疗TGA患儿共72例,术中死亡6/72例（8.3％）,存活率为91.7%。失访10/66例,有效数据56例,其中室间隔完整的完全性TGA（TGA/IVS）32例,完全性TGA合并室间隔缺损（TGA/VSD）24例。随访距手术18～168(86.46±23.51)个月。新主动脉根部内径为(16.97±2.71) mm(t=6.936,P<0.001),12/56例(21.4%)>正常参考值90%CI的上限, 44/56例(78.6%)在正常参考值90%CI内,平均Z值为1.10±0.70。新主动脉瓣环内径为(16.27±2.38) mm(t=4.52,P<0.001),56例均在正常参考值90%CI内,平均Z值为0.66±0.65。新肺动脉内径为(14.29±1.92) mm(t=-3.2,P=0.005), 40/56例(71.4%)在正常参考值90%CI内,16/56例(28.6%)<正常参考值90%CI的下限,平均Z值为-0.95±1.33。 新肺动脉瓣环内径为(14.00±1.92) mm(t=0.132,P=0.897), 42/56例(75.0%)在正常参考值90%CI内,14/56例(25.0%)<正常参考值90%CI的下限,平均Z值为0.05±1.85。16/56例(28.6%)存有主动脉瓣轻度反流,均为TGA/VSD病例,平均随访(46.0±22.2)个月;40/56例（71.4％）未见主动脉瓣反流,平均随访(46.8±25.3)个月,两组随访时间差异无统计学意义（P=0.899）。随访中未见主动脉狭窄病例。肺动脉瓣上狭窄7/56例(12.5%),2/56例(3.6%)为轻度狭窄,5/56例(8.9%)为极轻度狭窄。结论 目前在复旦大学附属儿科医院心血管中心开展ASO治疗TGA术后新主动脉根部和瓣环内径均有所扩张,新肺动脉根部内径有所缩减,但其瓣环发育良好。主动脉瓣反流常见于TGA/VSD病例,但其反流程度较轻。肺动脉瓣上狭窄是TGA术后常见的并发症,但大多数病例狭窄程度极轻。     

Diagnosis and treatment of pulmonary atresia and ventricular septal defect

Surgical repair of the intracardiac anatomy in patients with pulmonary atresia and ventricular septal defect can be performed today with a low operative mortality. Diagnostic and therapeutic problems in these patients are almost exclusively related to the nature of collateral lung perfusion and associated anomalies of the pulmonary vascular bed. These anomalies are frequently found in patients with major aortopulmonary collateral arteries and include multifocal pulmonary blood supply, hypoplasia, stenosis, or arborization anomalies of the pulmonary arteries. Diagnostic methods must focus on an exact identification of the collateral pulmonary blood supply, the presence and size of central pulmonary arteries, and the connections of the arterial segments. Recent genetic studies have shown that monosomy 22q11.2 is found in 25–32% of children with pulmonary atresia and ventricular septal defect. This microdeletion is significantly more frequent in patients with major aortopulmonary collateral arteries and it seems to be associated with a higher percentage of pulmonary arterial anomalies. During recent years, efforts have concentrated on earlier treatment of patients with pulmonary atresia with ventricular septal defect with combined catheter and surgical interventions. Early establishment of antegrade flow to the central pulmonary arteries stimulates growth of the pulmonary arteries, optimizes the angiographic diagnosis of abnormalities of the pulmonary vascular bed, and allows for the possibility of balloon angioplasty or stenting of the central pulmonary arteries.     

Comparison of types of pulmonary stenosis with the state of the ventricular septum in complete transposition of the great arteries

Summary The pulmonary valve and left ventricular outflow tract of 214 hearts with d-transposition of the great arteries (d-TGA) were visually inspected (126 of 214 with intact ventricular septum and 88 of 214 with ventricular septal defect [VSD]). Pulmonary stenosis (PS), either valvular, subvalvular, or in combination, was present in 26 cases and was found to be more common in the presence of a VSD (20.5%) than intact ventricular septum (6.3%). PS occurred more commonly in the presence of a supracristal VSD than an infracristal VSD (70%, 7 of 10 vs 15%, 10 of 66). Further, infracristal or supracristal VSDs were associated with different types of pulmonary obstruction. In seven of ten cases with infracristal VSD and PS, the stenosis was caused by an anomaly of an atrioventricular valve. In six of seven cases with supracristal VSD and PS, the latter was caused by membranous encirclement of the left ventricular outflow tract. * Presented in part as an abstract at the American Heart Association Scientific Sessions, Fall 1987.     

肾上腺髓质素缓解低氧性肺动脉高压大鼠肺动脉中胶原堆积的研究

目的：观察长期应用肾上腺髓质素（adrenomedullin, ADM）对慢性低氧性肺动脉高压大鼠肺血管胶原代谢的影响,以探讨ADM对慢性低氧性肺血管结构重建的作用及其可能机制。方法：19只雄性Wistar大鼠随机分为对照组（n=6）、低氧组（n=7）、低氧+ADM组（n=6）。对于低氧+ADM组大鼠,通过微量渗透泵皮下持续给予ADM（300 ng/h）2周。低氧2周后,以右心导管法测定肺动脉平均压,检测右心室与左心室加室间隔比值,观测肺血管显微变化。用免疫组化法检测肺动脉中胶原I、胶原III和转化生长因子（TGF）-β的表达。结果：低氧2周后,大鼠肺动脉平均压明显升高(P<0.01),右心室与左心室加室间隔的比值明显增加(P<0.01),肺小血管肌化程度和肺动脉相对中膜厚度较对照组均明显增高（P<0.01）,同时肺动脉胶原I、胶原III和TGF-β表达增强。与低氧组大鼠相比,低氧+ADM组大鼠肺动脉平均压明显降低（P<0.01）,右心室与左心室加室间隔的比值明显下降（P<0.01）,肺小血管肌化程度和肺动脉相对中膜厚度较低氧组均明显降低（均P<0.01）,同时肺动脉胶原I、胶原III和TGF-β表达减弱。结论：外源性补充ADM可能通过抑制肺动脉TGF-β表达,减少肺动脉壁胶原异常堆积,对低氧性肺动脉高压和肺血管结构重建发挥调节作用。     

Ligating the ductus arteriosus before birth remodels the pulmonary vasculature of the lamb

The clinical syndrome of persistent pulmonary hypertension of the newborn includes a developmentally abnormal pulmonary microvasculature which contains excessive amounts of muscle and which cannot adapt to air breathing in the perinatal period. Surgical ligation of the ductus arteriosus of the fetal lamb has produced a physiologic model of pulmonary hypertension of the newborn. The aim of the present investigation is to determine whether surgical ligation of the ductus arteriosus in fetal sheep produces anatomic changes in the pulmonary blood vessels. The pulmonary vasculature of seven neonatal lambs that underwent surgical ligation of the ductus arteriosus from 6 to 17 d before birth was compared to that of five control lambs with a patent ductus arteriosus without fetal surgery and three control lambs with a patent ductus arteriosus that underwent sham surgery. Quantitative microscopic analysis of the barium gelatin-filled peripheral pulmonary vascular bed revealed an increase in the proportion of partially and fully muscularized pulmonary arteries at the level of the terminal bronchiole and within the acinus (p less than 0.0001). This finding demonstrates that medial muscle develops in areas of the distal pulmonary vascular bed where it is normally absent. Periadventitial fibrosis surrounding intraacinar pulmonary arteries was also present. No change in the number of small intraacinar arteries was detected. This structural remodeling of the peripheral pulmonary vascular bed was initiated in utero by ductus arteriosus occlusion.(ABSTRACT TRUNCATED AT 250 WORDS)     

����תλ���������ҹ��ܺʹ���������������ù۲�

目的观察动脉转位术(ASO)治疗复杂先天性心脏病术后早中期超声心动图的随访结果,评价该手术后左心室的功能和大动脉的发育。方法2000年10月至2004年3月,复旦大学儿科医院对实施ASO后4~37(19.6±11.1)个月的16例患儿进行超声心动图检查,其中室间隔完整的完全性大动脉转位(TGA/IVS)8例,完全性大动脉转位合并室间隔缺损(TGA/VSD)6例,右室双出口合并肺动脉瓣下室间隔缺损(Taussig-Bing畸形)2例。将获取的资料与正常参考值作比较。结果左室短轴缩短率(LVSF)为(38.10±5.90)%,其中14例(87.5%)在正常范围,2例(12.5%)大于正常范围;左室射血分数(LVEF)为(69.16±7.01)%,均在正常范围。主动脉根部直径平均较正常值扩大(5.5±3.2)mm,其中14例(87.5%)大于正常值95%可信区间的上限;瓣环直径较正常值平均扩大(3.6±3.3)mm,其中10例(62.5%)大于正常值95%可信区间的上限。主肺动脉直径较正常值平均小(1.3±1.5)mm,其中9例(56.25%)在正常值95%可信区间内;瓣环直径较正常值平均小(1.1±1.7)mm,其中10例(62.5%)在正常值95%可信区间内。6例存有主动脉瓣轻度反流;肺动脉瓣上狭窄极轻度3例,轻度狭窄1例;2例存有肺动脉瓣轻度反流。结论超声心动图随访结果证实目前在本中心开展ASO治疗TGA等复杂性先天性心脏病术后左心室功能良好,新主动脉根部和瓣环有所扩张,新肺动脉根部和瓣环发育良好。     

Pulmonary atresia, intact ventricular septum, right ventricle-dependent coronary artery circulation, and systemic collateral arteries supplying the left coronary artery.

We present a 14-day-old with pulmonary artery atresia with intact ventricular septum, right ventricle-dependent coronary circulation, a single aortic root coronary ostia (left), congenital collateral arterial supply to the left coronary artery from the left internal mammary artery, and bilateral paravertebral arteries, with obstructive coronary artery lesions.