婴儿先天性心脏病1387例外科治疗结果分析

目的 回顾性分析我院近11年来外科治疗的≤6个月小婴儿先天性心脏病(先心病)1387例,探讨其病种、手术时机和治疗观念的变迁,以期进一步提高小婴儿先心病的就诊和救治率.方法 1997年1月至2007年12月,在我院行手术治疗的≤6个月先心病患儿1387例,主要病种包括:室间隔缺损合并肺动脉高压(VSD/PH)、法洛四联症(TOF)、完全性大动脉转位(TGA)、完全性肺静脉异位引流(TAPVC)、主动脉缩窄或主动脉弓中断合并室间隔缺损[CoA(IAA)/VSD]、右心室双出口(DORV)、合并室间隔缺损的肺动脉闭锁(PA/VSD)、室间隔完整的肺动脉闭锁(PA/IVS)等,根据病情采取相应的手术方法矫治,部分复杂型先心病进行了随访.结果 手术死亡110例,总手术死亡率7.9%.从历年手术治疗分析,手术死亡率1997至2003年为11.5%～14.4%,2004至2005年降至8.6%～&9%,2006至2007年降至3.3%～3.8%.对TGA、TAPVC、TOF、PA/VSD、PA/IVS患儿进行了随访,随访率分别为83.8%(98/117)、87.8%(79/90)、48.2%(68/141)、65%(13/20)和95%(19/20),随访期限为3～86个月.晚期死亡16例.随访中绝大多数患儿无症状,心功能和生长发育正常.结论 绝大部分早期出现症状的危重先心病可以在小婴儿期进行矫治,手术效果接近国际水平.不能进行一期矫治的可以先做姑息手术,改善缺氧、促进肺动脉发育,为以后的根治手术创造条件.     

Bjork Surgery for Tricuspid Atresia—Revisited

We present a 19-year-old college student completely asymptomatic who underwent successful Bjork surgery (right atrium-to-right ventricle connection) in infancy with excellent results. Surgical approach for tricuspid atresia is done in three stages. Blalock Tuassig shunt soon after birth or pulmonary artery band depending on the size of the ventricular septal defect (VSD) and the main pulmonary artery, Glenn surgery (bidirectional cavopulmonary shunt) at 3–6 months of age, and Fontan surgery at 2–3 years age. There are many short- and long-term complications after Fontan surgery and patients require multiple medications throughout life. For tricuspid atresia with normally related great arteries, large VSD, and normal pulmonary valve, Bjork surgery offers the advantages of right ventricular growth, biventricular function, and good outcome without any medications.     

Cyanotic congenital heart disease with decreased pulmonary blood flow in children

Of the "five T's" of cyanotic congenital heart disease--tetralogy of Fallot, TGA, TAPVC, truncus, and tricuspid valve abnormalities (tricuspid atresia, stenosis, and displacement)--the first and last are commonly associated with diminished PBF. The four features that comprise tetralogy of Fallot--right ventricular hypertrophy, VSD, overriding aorta, and subpulmonary stenosis--are all secondary to a single morphogenetic defect: failure of expansion of the subpulmonary conus. This also explains the variability in clinical presentation. When neonates need intervention, shunts are usually performed. Coronary arterial anatomy must be defined before repair, which is usually done after these infants are 3 months of age. Although children with repaired tetralogy of Fallot are not completely "normal," markedly increased longevity and improvement in quality of life can be achieved. When major associated defects are present, such as atrioventricular canal defect, diminutive pulmonary arteries or collateral vessels, or left heart lesions, the prognosis changes from excellent to merely good. Tetralogy of Fallot with absent pulmonary valve syndrome is physiologically different from other tetralogy of Fallot conditions and characterized primarily by airway obstruction from massive dilatation of the central and perihilar pulmonary arteries; repair with pulmonary artery reduction is necessary. Tricuspid valve abnormalities include atresia, hypoplasia (i.e., pulmonary atresia with intact ventricular septum), and displacement (i.e., Ebstein anomaly). The pathophysiology that dictates these children's clinical condition (and prognosis) relates to three factors: (1) status of the tricuspid valve, (2) presence and size of a VSD, and (3) TGA or normally related great arteries. Virtually all children with tricuspid valve abnormalities can be palliated; reparative options include repair using two-ventricle, one-ventricle, or 1-1/2 ventricle repair. Children with critical pulmonary stenosis generally have a normal tricuspid valve and right ventricle. Balloon dilation is usually the only therapy necessary.     

Children with congenital heart disease: Probability of natural survival

Summary The age distribution of death in all children with congenital heart disease (CHD), who died in a 27-year period in Central Bohemia (population of 1.2 million), and the data on the incidence of CHD in children born in Bohemia (population of 6.3 million) in 1980 were used to calculate the probability of survival of a child born with CHD. Eighty-six percent of these children survived to the first month of life—mostly those with pulmonary stenosis (PS, 99%), aortic stenosis (AS, 95%), ventricular septal defect (VSD, 92%), and atrioventricular septal defect (AVSD, 91%). Seventy-one percent of patients survived the first year of life—mostly those with PS (97%), AS (91%), atrial septal defect (ASD, 89%), VSD (80%), and persistent ductus arteriosus (PDA, 78%). In total, 67% of CHD patients can be expected to survive childhood. The highest survival rates were found in PS (94%), AS and ASD (84%), VSD and PDA (70–80%), and coarctation of the aorta (COA, 68%). The survival rate for the remaining forms of CHD was less than 50%.The highest mortality rate (10% of all children born with CHD) can be expected in the first postnatal week. The lowest survival in the first week was found among those with hypoplastic left heart (HLHS, 39%), double-outlet right ventricle (DORV, 50%), truncus arteriosus (TrA, 57%), pulmonary atresia (PA, 70%), and transposition of the great arteries (TGA, 83%). In addition, total anomalous pulmonary venous connection (TAPVC) and single ventricle had the highest risk of death in the first year of life.     

Absent Pulmonary Valve with Tricuspid Atresia or Severe Tricuspid Stenosis: Report of Three Cases and Review of the Literature

Absence of the pulmonary valve occurs usually in association with tetralogy of Fallot and occasionally with an atrial septal defect or as an isolated lesion. Very rarely it occurs with tricuspid atresia, intact ventricular septum, and dysplasia of the right ventricular free wall and of the ventricular septum. We present the clinical, anatomic, and histologic findings of a new case, and for the first time, the data from two patients with absent pulmonary valve and severe tricuspid stenosis, who exhibited similar histologic findings. We also reviewed the clinical and anatomic data of 24 previously published cases and compared them with the new cases. In all three new cases, the myocardium of the right ventricle was very abnormal. In the two cases with tricuspid stenosis, large segments of myocardium were replaced with sinusoids and fibrous tissue. In the case with tricuspid atresia, the right ventricular free wall contained only fibroelastic tissue. The ventricular septum in all three patients showed asymmetric hypertrophy and in two of the three patients, multiple sinusoids had replaced large segments of myocardial cells. The left ventricular free wall myocardium and the walls of the great arteries were unremarkable. Our data indicate that myocardial depletion involving the right ventricular free wall and the ventricular septum and its replacement by sinusoids and fibroelastic tissue occur not only in cases of absent pulmonary valve with tricuspid atresia but also in cases of absent pulmonary valve with tricuspid stenosis. The degree of myocardial depletion varies and is more severe when the tricuspid valve is atretic. Received April 8, 1999; accepted July 12, 1999.     

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     

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)     

Surgical management of pulmonary atresia with intact ventricular septum. Right ventricular size as a guideline for surgical intervention.

61 infants with critical valvular pulmonary stenosis (21 cases) or pulmonary atresia with intact ventricular septum (40 cases) were operated on between 1975-1989 in the Semmelweis University Medical School. Right ventricular volume, area, outflow tract dimension and tricuspid anulus diameter was measured by angiocardiography and echocardiography to evaluate right ventricular size, and to predict the operative outcome using these data. Our results suggested, that if the normalized right ventricular volume was less than 3 ml/m2, the normalized right ventricular area was less than 2.5 cm2/m2, and the normalized right ventricular area was less than 2.5 cm2/m2 and the normalized tricuspid anulus diameter was less than 1.2 cm2/m2/3 only a systemopulmonary shunt procedure is needed. In all other cases pulmonary valvotomy is necessary to decompress the right ventric and to help increase the right ventricular size. Where a different part of the right ventricle is hypoplastic a systemopulmonary shunt procedure is needed too. In the follow up period the right ventricular dimension and tricuspid valve diameter was measured by echocardiography. This noninvasive assessment can predict the preoperative diagnosis, postoperative outcome and demonstrates an adequate growth of the right ventricle after pulmonary valvotomy.     

Tricuspid atresia with special emphasis on cases with increased pulmonary blood flow

Summary Though an average case of tricuspid atresia offers no diagnostic challenge, the clinical picture of the relatively less common form of tricuspid atresia with increased pulmonary blood flow is not properly defined. The problem of suitable surgery for this type of lesion has not been given serious consideration. On the basis of clinical, radiological and electrocardiographic findings in twenty-six proven cases of tricuspid atresia (six of whom had increased pulmonary blood flow) we feel that a child with mild cyanosis, biatrial hypertrophy, left ventricular hypertrophy and/or absent normal right ventricular potentials in the precordial leads and the clinical picture of a large left to right shunt is likely to have tricuspid atresia with increased pulmonary blood flow. The presence of counter clock wise loop and electrical axis around +90° in the frontal plane is of further help. In the present state of our knowledge pulmonary artery banding seems to be a useful palliative procedure for these children.     

先天性心脏病术后残余室间隔缺损介入治疗及中长期随访

目的 评价先天性心脏病（先心病）术后残余室间隔缺损（VSD）介入治疗的效果及中长期随访结果。 方法 回顾性分析2002年9月至2014年5月复旦大学附属儿科医院介入治疗的先心病术后残余VSD患者的临床资料,对辅助检查、介入治疗情况和随访行描述性分析。结果 21例患者进入分析,男11例,女10例,平均年龄8.8（2.7～21）岁,平均体重26.3（10.5～53.5） kg。13例为VSD修补术后残余漏,其中2例合并主动脉弓缩窄修补术后再缩窄;5例为法洛四联症（TOF）术后残余VSD,其中1例合并频发室性早搏及短阵室性心动过速;2例为右室双出口术后残余VSD;1例为完全性大动脉转位术后残余VSD。介入治疗距离外科手术时间为11～80个月。①介入治疗中左心室造影测得VSD右心室面直径为(3.73±1.56) mm,4例右室面有多处分流,1例为左室-右房瘘;肺循环与体循环血流量之比为1.53±0.23;无中度以上肺动脉高压者。②21例患者中,1例介入治疗中建立轨道时反复发生Ⅲ度房室传导阻滞而放弃堵闭行外科手术治疗;20例（95.2%）封堵成功,其中1例应用2个堵闭器。1例合并频发室性早搏及短阵室性心动过速患儿同时行射频消融治疗成功;2例合并主动脉弓再缩窄患儿经球囊扩张后缩窄解除。介入治疗中无不良事件发生。介入治疗后即刻造影和经胸超声心动图显示少量残余分流3例。ECG均无异常发现。③20例随访1～79个月,均无明显临床症状。2例在1年随访中ECG分别出现不完全性右束支传导阻滞及Ⅰ度房室传导阻滞,继续随访6~12个月无改变;2/3例即刻有残余分流患儿在随访中残余分流消失,1例介入治疗后20个月时仍有残余分流3.4 mm,心腔大小和功能正常;无新发的三尖瓣或主动脉瓣反流,3例堵闭前存在的中重度三尖瓣反流在随访中反流均为轻度;未见堵闭器移位、脱落或血栓形成。结论 经导管堵闭介入治疗先心病手术后残余VSD是一项安全、有效和易行的方法,介入治疗后即刻和中长期随访效果好,应成为此类患儿首选的治疗方法。     

2085例小儿先天性室间隔缺损手术纠治的临床分析

分析影响小儿先心病室缺纠治术的因素，以提高手术成功率。回顾性分析２０８５例小儿先心室缺纠治术，手术死亡４８例，病死率２．３％。手术死亡与患儿的年龄、体重有关。年龄小于６个月，体重低于６ｋｇ者手术病死率为３５．７％和３０．０％，而年龄大于３岁，体重超过１６ｋｇ者手术病死率低于１．０％。先心室缺的手术时间取决于室缺的位置、大小和心内分流量。室缺伴肺动脉高压患儿必须在２岁前手术，对肺动脉瓣下型室缺患儿应早期手术，防止主动脉瓣脱垂导致主动脉瓣关闭不全．术中避免右室切口，保护术后右心室功能和术后肺动脉高压的处理是手术成功的关键。     

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.     

Differential diagnosis of congenital heart disease in the first 3 months of life: Significance of a superior (left) QRS axis

The ECGs of 473 infants under the age of 3 months who were referred to a paediatric cardiological unit were analysed; 47 (10%) of the ECGs showed a superior axis (dominantly negative deflection S wave, in lead aVF). Of these, the majority of noncyanosed patients with plethora on chest x-ray proved to have either an atrioventricular canal defect or a large ventricular septal defect. When cyanosis and pulmonary plethora on x-ray were present, tricuspid atresia with increased pulmonary flow (types Ic or IIc) or d-transposition with ventricular septal defect accounted for most cases. With cyanosis and pulmonary oligaemia on x-ray, tricuspid atresia (types Ia and b) or pulmonary atresia with ventricular septal defect accounted for all cases. Finally, 2 patients with superior axis presenting in a shocked condition were found to exhibit the hypoplastic left heart syndrome.Recognition of superior axis in the ECG provides a useful diagnostic aid in congenital heart disease in early infancy.     

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.     

Plasma and right auricle concentrations of atrial natriuretic polypeptide in children with cardiac diseases

To investigate the atrial natriuretic polypeptide (ANP) levels in congenital heart diseases, we measured plasma immunoreactive ANP (ir-ANP) in 29 patients with left-to-righ shunt, and right auricle ir-ANP in 25 patients who underwent operation. The levels of ir-ANP in the pulmonary artery correlated with that in the femoral vein (r=0.95, P<0.001), mean pulmonary arterial pressure (r=0.74, P<0.001) and right atrial pressure (r=0.53, P<0.01). The right auricle ir-ANP level was high in patients with heart failure combined with pressure or volume overload of the right atrium, as observed in tricuspid atresia, complete endocardial cushion defect, and total anomalous pulmonary venous drainage. These results suggest that the production of ANP in the atrium increases as a compensatory or secondary response to persistent release of ANP into the circulation in cardiac diseases.Abbreviations ANP atrial natriuretic polypeptide - ir-ANP immunoreactive ANP - -hANP alpha human ANP - VSD ventricular septal defect - ASD atrial septal defect - PDA patent ductus arteriosus - TOF tetralogy of Fallot - DORV double outlet right ventricle - TAPVD total anomalous pulmonary venous drainage - cECD complete endocardial cushion defect - TA tricuspid atresia - MRAP mean right atrial pressure - MPAP mean pulmonary arterial pressure - Qp/Qs pulmonary-to-systemic blood flow ratio     

Intrauterine closure of membranous ventricular septal defects: Mechanism of closure in two autopsy specimens

Summary Anatomic evidence of intrauterine closure of ventricular septal defects (VSDs) has been reported rarely. Between 1985 and 1990, 112 autopsies were performed at the Mayo Clinic on third trimester stillborns and infants who died during the first week of life. There were 21 (19%) cases of congenital heart disease. VSD was found in 12 (11%) cases: in eight (7%) as a part of a more complex heart defect and in four (4%) as an isolated lesion. Two cases with membranous VSDs with tricuspid valve tissue partially occluding the ventricular septal defect were found. A 2280-g female infant (case 1) with trisomy 18 died at 4 days of age. Autopsy revealed bilateral superior venae cavae, a large atrial septal defect, cor triatriatum, an atypical tricuspid valve with large septal leaflet partially obstructing a large membranous VSD, a hypoplastic right ventricle, and severe pulmonic stenosis. A 2610-g female infant (case 2), born with congenital heart block died at 4 days of age. Autopsy revealed cor triatriatum dexter obstructing the tricuspid orifice, a large membranous VSD partially obstructed by the septal leaflet of the tricuspid valve, four small muscular VSDs, and pulmonic stenosis. These cases suggest that closure of membranous VSDs may gegin in utero and the mechanism of closure is similar to that reported postnatally.     

Prostaglandin E1 in infants with congenital heart disease: Indian experience

BACKGROUND: E-type prostaglandins (PGE1) can effectively maintain the patency of the ductus arteriosus in neonates. Its use, therefore and be life saving in infants born with ductus dependent congenital heart disease. Although PGE1 is available for over two decade in western world, it has been introduced in India only since April, 1995. OBJECTIVE: To assess the efficacy of PGE1 at our center. SETTING: Hospital based. METHOD: 65 infants with ductus dependent congenital heart disease were included. Age at time of starting PGE1 infusion ranged from 18 hours to 39 days. Forty two of these were more than a week of age, 19 were more than 14 days, and two were above one month. PGE1 was started in an initial dose of 0.05 microgram/kg/min, decreased to 0.005-0.01 microgram/kg/min for maintenance. The indications for use of PGE1 were to increase pulmonary blood flow in 33 cases with pulmonary atresia, tricuspid atresia or critical pulmonic stenosis (Group I); to increase systemic blood flow in 15 cases with coarctation of aorta, hypoplastic left heart and interruption of aortic arch (Group II); to improve mixing in 13 cases of transposition of great arteries (Group III) and for improving the left ventricular volumes by keeping the duct open in 4 cases of transposition of great arteries with intact ventricular septum (Group IV). The efficacy of the drug was assessed by a rise on PaO2 and SaO2% determined for Group I & III, and by appearance of lower limbs pulses in Group II. Left ventricular volumes were serially measured by echocardiography in Group IV cases. RESULTS: The drug was successful in 62 of the 65 cases. There were two failures. One was a 39 days old baby with Ebstein's anomaly of tricuspid valve and pulmonary atresia and other was an eight days old baby with coarctation of aorta and renal failure. In addition, PGE1 could not be continued in another baby due to development of a linear skin rash locally. Side effects included apnea in 5 (9%) of 56 spontaneously breathing patients. Necrotizing enterocolitis, hyperpyrexia and jitteriness was sent in one case each. Six patients died. Two were related to PGE1, one due to failure, another due to its side effects. Definitive procedure were performed in 51 cases electively. PGE1 was used upto 13 days with sustained benefit. CONCLUSIONS: PGE1 is an effective drug for keeping the ductus open in infants with ductus dependent congenital heart disease. It can be used for neonates beyond the first week of life with efficacy. Apnea is a major side effect and close monitoring is essential.     

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.     

Cross-sectional echocardiographic measurements of right ventricular size and growth in patients with pulmonary atresia and intact ventricular septum

Summary Fifteen patients with pulmonary atresia or critical pulmonary stenosis and intact ventricular septum were studied. All were operated on in the neonatal period, with valvotomy or a systemic to pulmonary arterial shunt, or both. In 12 patients, right ventricular to pulmonary arterial communication was established in the neonatal period. In three patients, only systemic to pulmonary arterial shunts were constructed. Six patients died. The median follow-up period for the surviving patients was 65 months (range, 12–87 months).Right and left atrial and ventricular dimensions and areas, the tricuspid annular diameter, and the cross-sectional area of the aortic root were measured in cross-sectional echocardiograms from the neonatal period, at the age of 1 year, and at the latest clinical follow-up. A classification of right ventricular morphology was made, based on identification of the inlet, the trabecular, and the outlet parts.Most of the patients had hypoplastic right ventricles at birth but at the latest follow-up, seven of nine surviving patients had right ventricles in the normal range. Right ventricular growth was better in patients who were given a right ventricular to pulmonary arterial communication in the neonatal period and those with complete right ventricular anatomy. The patients who died had severely hypoplastic right ventricles and small tricuspid valves.     

Uhl's anomaly associated with atresia of the pulmonary valve

A case of Uhl's anomaly in a newborn who died in congestive cardiac failure at 18 days of age is described. There was severe cardiomegaly on chest X-ray, right atrial hypertrophy on E.C.G., and a delayed tricuspid valve closure of 0,8 seconds on M-mode echocardiogram. The necropsy study showed a huge right atrial and right ventricular dilatation, a very thin right ventricular wall (1 mm.) with an important reduction in the number of muscular fibers, a pulmonary valve atresia, and a patent ductus arteriosus and foramen ovale. We have only three cases Uhl's anomaly associated with pulmonary atresia, and no one with such an important delay of the tricuspid valve closure on the echocardiogram.