超声心动图诊断法洛四联症的临床价值评估

目的评价超声心动图诊断法洛四联症（TOF）的准确性、可靠性和效益,探讨法洛四联症无创伤性术前诊断方法及手术指征。方法以手术后诊断为金标准,评价234例TOF患儿的二维超声心动图（2-DE）诊断及心导管诊断结果。结果对于TOF的诊断,2-DE诊断灵敏度97.9%,特异度99.8%,符合率99.6%;心导管诊断灵敏度99.5%,特异度100%,符合率99.9%;两者的差异无统计学意义。在TOF合并心血管畸形的诊断中,2-DE诊断CA横跨ROVT、侧支血管、PDA的灵敏度、特异度、符合率与手术后诊断存在明显差异。结论2-DE可替代心导管造影检查用于TOF的诊断。如果术前2-DE检查发现冠状动脉显示不清或怀疑走行异常、存在侧支血管、PDA与侧支血管难以鉴别及肺动脉分支发育不良时,需进一步行心导管造影检查。     

多排螺旋CT在小儿法乐四联症诊治中的应用价值

目的 探讨多排螺旋CT血管造影(MUCTA)三维重建应用于小儿法乐四联症(TOF)诊断及治疗中的价值.方法 2004年12月至2008年12月38例经胸二维超声心动图(TEE)诊断TOF患儿,为进一步明确肺动脉发育状况行MUCTA检查,均经外科手术证实.MUCTA扫描后应用三维重建处理,测算全肺动脉指数(Nakata),确定肺动脉发育状况,冠状动脉走向,主、肺动脉侧枝循环位置,设计手术方案.结果 38例患儿检查过程顺利均为TOF,其中合并房间隔缺损(ASD)3例、动脉导管未闭(PDA)2例、室间隔缺损(VSD)嵴下型38例,单纯右室流出道狭窄7例、合并肺动脉狭窄31例,冠状动脉走向异常2例、主、肺动脉侧枝循环19例、其中粗大侧枝循环3例.35例康复出院,3例术后死亡.结论 MUCTA对于诊断TOF心外畸形的显示清晰度超过其它影像学检查,特别是对观察肺动脉发育形态、肺动脉狭窄的部位、冠状动脉起源与走向异常、PDA、主肺动脉侧枝循环,精确测算Nakata指数、McGoon指数均有明显帮助.与TEE相结合能达到优势互补、能够更加完整、准确的诊断TOF,为手术决策,方案的制定提供了有效的依据,对提高TOF患儿的治疗效果有重要价值.     

Septal Course of Left Anterior Descending Artery From the Right Aortic Sinus in Tetralogy of Fallot: A Benign Anomaly and Important Lessons Learned

The proximal course of an anomalously arising coronary artery is a decisive factor in the surgical approach for tetralogy of Fallot (TOF). Studies have shown that echocardiography provides a good anatomic definition of the ostium and proximal epicardial course of coronary arteries [1, 2]. This report describes a case of TOF with an atrioventricular canal defect whose preoperative echocardiography showed abnormal origin of the left anterior descending artery (LAD) from right aortic sinus, which was interpreted as crossing the right ventricular outflow tract. Perioperative inspection did not show any abnormal vessel crossing the outflow, and corrective surgery was performed. At the echocardiographic evaluation after surgery, it was noted that the abnormal LAD arising from right aortic sinus was taking a septal course in relation to the posterior aspect of the pulmonary annulus. It is important to recognize this anomalous course because it is benign with no surgical implications.     

法洛四联症伴肺动脉瓣缺如3例报道并文献复习

目的总结法洛四联症伴肺动脉瓣缺如患儿的临床特点和诊治经验。方法收集复旦大学附属儿科医院2007年11月至2009年2月收治的法洛四联症伴肺动脉瓣缺如3例患儿的临床资料,总结其临床表现、影像学特征 、治疗和预后。结果男1例,女2例,年龄39 d至4个月。临床均表现为气急、咳喘及轻度发绀;体检可闻及胸骨左缘来回杂音。超声心动图和心导管检查除法洛 四联症表现外,可见肺动脉瓣环发育不良或缺如,伴肺动脉重度反流、肺动脉瘤样扩张,均未见动脉导管未闭,其中1例左肺动脉不连接伴发育 不良。2例临床症状严重的患儿施行手术根治,其中1例手术效果满意,另1例左肺动脉不连接伴发育不良患儿术中死亡;1例患儿临床症状尚不 明显,仍在密切随访中。 结论法洛四联症伴肺动脉瓣缺如尽管少见,但由于在婴儿期甚至新生儿期即可引起严重气道受压从而危及生命,临床中遇到难以控制的呼吸窘 迫新生儿和小婴儿应考虑本病的可能。尽早手术根治解除气道受压呼吸困难症状是唯一的治疗方法,无呼吸道受压症状可考虑6个月左右择期手 术。手术病死率高于单纯法洛四联症,尤其是伴一侧肺动脉不连接或发育不良的患儿。     

Imaging of congenital coronary artery anomalies

Imaging of the coronary arteries is an important part of the evaluation of children with congenital heart disease and isolated congenital coronary artery anomalies. Echocardiography remains the main imaging modality and is complemented by MRI and CT angiography in the older or difficult-to-image child. We review echocardiography, MRI, and CT angiography for coronary artery imaging, with emphasis on techniques. The clinical implications of isolated congenital coronary artery anomalies are also addressed, along with a discussion about the current consensus on optimal management of these anomalies.     

How is pulmonary arterial blood flow affected by pulmonary venous obstruction in children? A phase-contrast magnetic resonance study

Background: Hemodynamic changes within a stenosed pulmonary vein might not reflect the severity of the obstruction if redistribution of pulmonary artery flow occurs. Objective: The purpose was to investigate flow changes in branch pulmonary arteries in the presence of pulmonary vein stenosis. Materials and methods: Twelve children (age range 3–180 months) who had undergone MRI for pulmonary vein stenosis were identified. The severity of vein stenosis was assessed from percentage diameter reduction. Pulmonary artery flow distribution was correlated with the severity of pulmonary vein stenosis. Nine of the patients had unilateral stenosis; three had bilateral involvement. Results: Unilateral vein stenosis was associated with diastolic reversal in the ipsilateral branch pulmonary artery (mean flow reversal 12.3%, range 2.4–42%) and continuous diastolic forward flow in the contralateral pulmonary artery in seven of nine patients. Severe stenosis was associated with decreased systolic flow to the ipsilateral lung. The ratio of net forward flow through pulmonary arteries correlated well with the cross-sectional area ratio of pulmonary arteries (r=0.75, P=0.006). Conclusions: Severe pulmonary vein obstruction results in redistribution of pulmonary arterial flow. When investigating pulmonary vein stenosis by MR, an evaluation of the pulmonary arterial system should be included to assess the functional importance of an obstruction.     

The role of magnetic resonance imaging in the assessment of suspected extrinsic tracheobronchial compression due to vascular anomalies.

AIMS: To evaluate the role of magnetic resonance imaging (MRI) in the assessment of children with suspected extrinsic tracheobronchial compression due to vascular anomalies. METHODS: Retrospective case note review in a tertiary referral centre. Twenty nine children who underwent dynamic laryngotracheobronchoscopy (DLTB) and were found to have a clinical suspicion of extrinsic tracheobronchial compression were evaluated. All subsequently underwent thoracic MRI within 10 days. The findings on endoscopy were compared to those of MRI, and where performed, echocardiography, aortography, and surgery. RESULTS: There were 17 males and 12 females (mean age 5 months, range 28 weeks gestation to 60 months). The most common presenting features were stridor and cyanotic episodes. MRI showed abnormalities in 21 patients. There were five vascular rings (three double aortic arches and two right aortic arches) and 11 cases of innominate artery compression. Other vascular anomalies noted included aberrant right subclavian artery and aneurysmal left pulmonary artery. Echocardiography was generally found to be unhelpful in the diagnosis of extra-cardiac vascular abnormalities. Angiography was subsequently conducted in eight children; findings agreed with those shown on MRI. Surgery was performed on all five vascular rings, one innominate artery compression, and one aneurysmal left pulmonary artery. Surgical findings were also compatible with the preoperative MRI. CONCLUSIONS: This study shows the successful use of MRI as the initial imaging modality in endoscopically suspected extrinsic vascular compression of the upper airway. It enables accurate delineation of vascular anomalies and, unlike aortography, is non-invasive and does not require the use of contrast media.     

Cardiac Catheterization Is Necessary Before Bidirectional Glenn and Fontan Procedures in Single Ventricle Physiology

There are significant mortality and morbidity in bidirectional Glenn (stage II) and Fontan (stage III) procedures for congenital heart diseases with single ventricle physiology. In order to minimize the mortality and morbidity, the presence or absence of hemodynamic and anatomical abnormalities, such as poor ventricular function, coarctation of the aorta, pulmonary artery distortion, high pulmonary resistance, and abnormal collateral vessels, should be evaluated. Echocardiography and magnetic resonance imaging (MRI) can visualize ventricular size and coarctation of the aorta but may fail to visualize pulmonary artery distortion. Furthermore, cardiac catheterization is needed to measure pulmonary resistance. If the pulmonary resistance is >3 Wood units·m² before stage II, the risk for the bidirectional Glenn operation may be high. Abnormal aortopulmonary collateral vessels may develop after the stage II procedure and echocardiography and MRI may not visualize these vessels. Coil embolization may be required to treat these vessels. In conclusion, cardiac catheterization is required to evaluate abnormalities, which may be treated by interventional catheterization, and to reduce mortality and morbidity of stage II and III procedures.     

单侧肺动脉异常起源于升主动脉的产前超声心动图诊断

目的 探讨单侧肺动脉异常起源于升主动脉（AOPA）的产前超声心动图特征，提高对本病产前诊断的准确率。方法 回顾性分析2012年6月1日至2018年10月30日经首都医科大学附属北京安贞医院（我院）产前诊断的6例AOPA胎儿的超声心动图特点，总结该病胎儿期的超声心动图特征。结果 5例胎儿为右肺动脉异常起源于升主动脉（AORPA），其中3例为孤立型AORPA，2例合并Berry综合征；1例为左肺动脉异常起源于升主动脉（AOLPA），同时合并先天性肺动脉瓣缺如综合征。6例胎儿均为近端型AOPA，其中2例胎儿引产（1例AOLPA，1例合并Berry综合征）。出生的4例患儿中，1例Berry综合征患儿于出生后63 d因反复肺炎、心衰死亡；2例行AORPA根治手术，术后均恢复良好；1例刚出生，仍在随访中。近端型AOPA胎儿超声心动图特征：①多切面显示正常的肺动脉主干分叉处“八”字型结构消失,正常的肺动脉分支内径增宽；②在三血管气管切面(3VT)及左室长轴切面二维及彩色多普勒显像（CDFI）可显示升主动脉发出一粗大血管向肺内走行，该血管发出位置靠近主动脉瓣左后或右后侧壁的升主动脉；③ CDFI显示异常肺动脉分支血流孤立，与主肺动脉缺乏确切连接；④频谱多普勒显示向肺内走行的血管为肺动脉血流频谱；⑤不合并其他心内畸形时，心内结构正常。结论 超声心动图可对胎儿期的近端型AOPA做出准确的诊断，3VT及左室长轴切面对诊断近端型AOPA至关重要。     

以反复喘息为表现的肺动脉吊带畸形2例报告并文献复习

目的 提高对肺动脉吊带畸形(PAS)的认识和早期诊疗水平,减少漏诊、误诊.方法 分析2例婴幼儿PAS的临床资料,评价超声心动图、支气管镜、螺旋CT、MRI等方法在早期诊断方面的作用.结果 2例患儿主要临床表现为反复喘息、肺部感染.初次发病年龄＜3个月,且多次住院,纤支镜检查示气管狭窄,1例经螺旋CT及超声心动图确诊,1例经手术诊断.结论 婴儿早期出现反复喘息、肺部感染及气道梗阻表现时,应考虑PAS可能.超声心动图、螺旋CT、MRI足确诊的最佳方法.     

Branch Pulmonary Artery Growth After Blalock–Taussig Shunts in Tetralogy of Fallot and Pulmonary Atresia with Ventricular Septal Defect: A Retrospective, Echocardiographic Study

We performed a retrospective echocardiographic study in tetralogy of Fallot (TOF) or pulmonay atresia with ventricular septal defect (PA&VSD) to evaluate the effects of Blalock–Taussig shunt on branch pulmonary artery growth. There were 35 patients with TOF and 11 with PA&VSD. We measured the right and left pulmonary artery area index and also the combined pulmonary artery area index, both before and after shunt operation. The mean ± SD of these three variables before the shunt operation in the TOF group were 63.5 ± 22.5, 57.8 ± 24.9, and 121.4 ± 42.8 mm²/m²; after shunt operation they were 98.5 ± 33.6, 85.9 ± 31.9, and 184.0 ± 59.8 mm²/m², respectively (p values <0.0001, <0.0002, and <0.0001, respectively). In the PA&VSD group the comparable values before shunt operation were 66.5 ± 16.0, 55.4 ± 10.6, and 120.9 ± 26.9 mm²/m² and after shunt operation were 90.5 ± 22.9, 77.2 ± 24.1, and 166.6 ± 44.4 mm²/m², respectively (p values <0.0006, <0.014, and <0.002, respectively). We also examined the effect of distensibility of pulmonary arteries by comparing the percentage change in size of the combined pulmonary artery area index in the first 4 months after shunt with those after this time (p < 0.023). There were no significant differences between left- and right-sided shunts, origin and distal pulmonary artery growth, and the TOF and PA&VSD groups.     

Repair of Tetralogy of Fallot with Anomalous Coronary Arteries Coursing Across the Obstructed Right Ventricular Outflow Tract

Anomalous coronary artery (ACA) anatomy occurs in 2–9% of patients with tetralogy of Fallot (TOF), in which the left anterior descending coronary artery (LAD) originates from the right coronary artery (RCA) crossing the right ventricular outflow tract. The purpose of this study was to review our results of repair for TOF with ACA. Between 1978 and 2001, 43 ACA patients (mean age, 4.8 years; range, 5 months–41 years) underwent repair for TC. The ACA anatomy was classified as the single LAD from the RCA (n = 20), a significant conal branch (dual LAD) from the RCA (n = 13), paired anterior descending arteries originating from the left and right coronary arteries (n = 7), and single RCA from the LAD (n = 3). In cases in which the anomalous LAD crossed the obstructed infundibulum, thinning or coring of the endocardium was done. Patch infundibuloplasty was performed in 39 patients, with 10 needing separate RV and pulmonary artery patches, and the pulmonary valve was preserved. Nine patients had the addition of a monocusp to a transannular incision. Two patients had a main pulmonary arterioplasty alone. There was one early (2.3%) and no late deaths. Mean early and late postoperative gradients were 21.5 ± 10.5 mmHg (4 patients had ≥30 mmHg) and 27.1 ± 13.7 mmHg (5 patients had >30 mmHg; p = 0.12), respectively. There were four reoperations during a mean follow-up of 4.8 years (range, 6 months to 18 years). Actuarial freedom from reoperation was 90% at 5, 10, and 15 years. At the latest follow-up, all patients were in NYHA functional class I. TOF repair for an ACA can be performed without disturbing the native coronary anatomy and without the use of conduits in most cases. Outcomes are similar to those of other patients with TOF. The presence of ACA does not impose increased risk after this surgical strategy. Paper presented in part at the annual meeting of the Southeastern Surgical Congress, Savannah, GA, February 2003     

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     

超声心动图和64层螺旋CT冠状动脉成像在川崎病冠状动脉瘤随访中的应用价值

目的探讨超声心动图和64层螺旋CT冠状动脉成像（64SCTCA）在川崎病（KD）冠状动脉瘤临床诊断和随访中的应用价值。方法选择2006年12 月至2007年5月在首都医科大学附属北京儿童医院门诊随访的KD并发冠状动脉瘤患儿作为研究对象。患儿观察终点随访时依据病变程度除行超声 心动图检查外,同时行64SCTCA检查,对各项检查结果进行详细描述,并分析其应用价值。结果研究期间纳入15例患儿,急性期KD超声心动图检 查显示急性期累及冠状动脉48/60支（80％）。患儿观察终点随访9个月至8.6年,平均（2.8±2.0）年,超声心动图示14/48支(29.2％)冠状动 脉瘤或扩张消退,其余进一步扩张或无明显变化,累及冠状动脉34/60支（56.7%）,同时检测出2处血栓,未发现狭窄及钙化。64SCTCA示累及 冠状动脉30/60支（50％）,主要累及右冠状动脉和左前降支,分布部位与超声心动图检查基本一致,64SCTCA与超声心动图检查对冠状动脉瘤 最大内径的测量值具有较好的相关性（r =0.837,P<0.001）,64SCTCA发现4处血栓、5处钙化及3处狭窄。结论64SCTCA和超声心动图在KD并发 冠状动脉瘤患儿的随访中具有各自的应用价值,两种方法的联合应用可增加冠状动脉病变的检出率。     

MRI of surgically created pulmonary artery narrowing in the dog

Narrowing of the pulmonary arteries was created surgically in twelve dogs. In six of the dogs the narrowing was central (main pulmonary artery), and in the remaining six the narrowing was located peripherally at the hilar level of the right pulmonary artery beyond the pericardial reflection. MRI and angiography were performed in all dogs. MRI clearly delineated the site of the pulmonary band and the caliber of the pulmonary artery at the site of the band in all dogs (N=6). MRI was not able to visualize any of the stenosis of the right pulmonary arteries at the hila, beyond the pericardial reflection. In addition, optimal imaging planes to depict each segment of the central pulmonary arteries were determined. The capability to image in oblique planes is essential in evaluating the morphology of the central pulmonary arteries.     

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.     

Unusual Cardiac Associations with Tetralogy of Fallot��A Descriptive Study

We present a large single-center series (>2200 cases) operated for Tetralogy of Fallot (TOF). We analyzed the incidence of associated unusual and uncommonly described cardiac lesions and their diagnostic and therapeutic implications in TOF patients. This retrospective study was conducted by reviewing records of patients operated for TOF at a large tertiary care pediatric cardiac centre. From 2002 to 2008, a total of 2235 cases of TOF were evaluated with echocardiography, cardiac catheterization, and/or cardiac computed tomography followed by cardiac surgery. Known and well-described associations were excluded from the study. Unusual associations were tabulated. Several unusual associations having an incidence >0.1% were detected. These included subaortic membrane (1%), pulmonary venous abnormalities (0.5%), small left ventricle (0.5%), interrupted inferior vena cava (0.5%), mitral valve abnormalities (0.4%), hemitruncus (0.4%), tricuspid valve abnormalities (0.4%), biventricular dysfunction (0.3%), retroaortic innominate vein (0.3%), bicuspid aortic valve (0.2%), and pericardial effusion (0.2%). This series describes unusual, not previously routinely reported cardiac lesions associated with TOF that may affect management and should be sought on preoperative evaluation. We demonstrate that more unusual associations do exist in not infrequent numbers, i.e., a TET is not just a TET.     

Pediatric Stress Echocardiography

Traditional health evaluations are performed while the patient is at rest. Stress echocardiography extends these examinations by providing data in a physiologic setting more closely mimicking the typically active state of children. The test represents a fusion of the fields of two-dimensional echocardiography and cardiovascular stress testing and can be used to assess myocardial perfusion in patients with suspected coronary artery pathology or to evaluate cardiac gradients or functional reserve in patients with noncoronary artery pathology. Testing should be performed with a trained sonographer and attending physician and in collaboration with adult cardiology colleagues. Stress can be administered to the patient through either exercise or pharmacologic agents. Echocardiography is used to assess regional wall motion abnormalities when evaluating myocardial perfusion or gradients and/or function when assessing the patient without coronary artery issues. Conditions with potential coronary artery pathology for which stress echocardiography is appropriate include Kawasaki disease, transplant graft vasculopathy, arterial switch operation for transposition of the great arteries, anomalous coronary artery origins or courses, pulmonary atresia with intact ventricular septum, hyperlipidemia, insulin-dependent diabetes mellitus, and supravalvar aortic stenosis. Stress echocardiography can also be helpful in determining the behavior during activity of gradients in conditions such as hypertrophic cardiomyopathy or aortic and pulmonic stenosis, of cardiac pressures in pulmonary hypertension and of ventricular function in conditions such as dilated cardiomyopathy or mitral and aortic regurgitation.     

Pulmonary atresia and ventricular septal defect: a therapeutic challenge in pediatric cardiology and cardiac surgery

From 1986 to 1997, 41 newborn infants and 19 children (1–6 years) with pulmonary atresia and ventricular septal defect were treated at the Heart Center North Rhine-Westphalia. The infants' birth weights ranged from 1140 g to 4100 g (mean 2990 g). Eighteen of these 60 patients had significant additional cardiovascular malformations and they were excluded from this study. The results of surgical treatment in the remaining 42 patients are presented and discussed here. Twenty patients had a main pulmonary arterial trunk with confluent branch pulmonary arteries and nine had an atretic trunk with confluent pulmonary arteries. In nine patients without confluence, one branch pulmonary artery originated normally from the main pulmonary trunk and the other originated abnormally. In four patients the intrapericardial vessels were completely missing. The Nakata index measured by cineangiography ranged from 50 to 240 mm²/m² (mean 150 mm²/m²). All newborns with a patent ductus arteriosus received prostaglandin intravenously. In eight of them oral prostaglandin was used to provide long-term stability before surgery. A total of 65 surgical aortopulmonary anastomoses were created, primary repair was carried out in six patients, and secondary corrective surgery was performed in 21 patients. After the first anastomosis, oxygen saturations increased by 5–15% (mean 8%), depending on the pulmonary vascular pathology. When a second shunt was created in the same patient, the increase in oxygen saturations ranged from none to 7%. After a mean interval of four years following the last operation, inspiratory vital capacity and intrathoracic gas volume were measured in 22 patients. Values of both studies decreased progressively as more thoracotomies were performed. All 42 patients were treated surgically and 13 died (31%). A primary or secondary repair was performed in 27 with 23 survivors (85%), and 15 had palliative procedures with six survivors (40%). Twenty of the 29 survivors (69%) are in a good clinical condition.     

A Case of Congenitally Protected d-Transposition of the Great Arteries in a Very Low-Birth-Weight Infant

An arterial switch is the corrective procedure of choice for d-transposition of the great arteries but may be associated with increased morbidity and mortality when performed in low-birth-weight infants. Conversely, delaying surgery often leads to left ventricular "deconditioning" as pulmonary arteriolar resistance decreases. We present an infant with a birth weight of 940 g with d-transposition of the great arteries with an intact ventricular septum whose bilateral pulmonary artery branch stenosis allowed for maintenance of near systemic left ventricular pressure, thereby protecting against deconditioning. This case also represents the smallest reported patient to undergo a successful balloon atrial septostomy.