Echocardiography in the preoperative evaluation of vascular rings.

Vascular rings may produce tracheal and/or esophageal compression in infants and children. Traditionally recognized fluoroscopically, the exact anatomy of the ring and appropriate surgical correction are determined intraoperatively. The role of preoperative echocardiography was examined. Twenty patients with symptomatic vascular rings were evaluated preoperatively with echocardiography at this institution. Their ages ranged from 10 days to 11 years (mean, 17 months). There were 9 boys and 11 girls. Most (17/20) presented with respiratory symptoms in the first year of life, although in 3 patients dysphagia was the primary complaint (at birth, 4 months, 9 years). All underwent initial evaluation with a barium esophagogram prior to the echocardiogram. Surgical correction was subsequently performed and the exact anatomy identified. All barium esophagograms were interpreted prospectively as demonstrating a "vascular ring." Although often suspected fluoroscopically, the actual type of ring was correctly identified by echocardiogram in all cases including determination of the dominant arch and associated anomalies. The types of vascular rings included double aortic arch (10), right aortic arch with left ligamentum arteriosum and/or aberrant left subclavian artery (6); aberrant right subclavian artery (2), and pulmonary artery sling (2). Barium esophagogram remains the best screening test for children in whom a vascular ring is suspected. However, echocardiography is a useful noninvasive complementary examination to confirm the diagnosis, clarify anatomy, and exclude other major intracardiac pathology prior to surgical correction.     

自体肺动脉组织一期矫治主动脉弓病变合并心内畸形

目的 探讨自体肺动脉组织一期矫治主动脉弓病变合并心内畸形的手术方法和治疗效果.方法 1993年4月至2009年11月,共25例主动脉弓病变合并心内畸形的患儿接受了一期矫治手术.男性17例,女性8例;年龄26 d～6.5岁,平均11个月;体质量3.0～14.5 kg,平均12.4 kg.其中6例患儿为主动脉弓缩窄,6例为主动脉弓中断,13例为主动脉弓发育不良.手术技术包括切开肺动脉前壁,切除动脉导管,应用部分自体肺动脉壁重建主动脉弓,重建肺动脉,并同时矫治合并心内畸形.结果 24例患儿术后恢复顺利,1例患儿死于围手术期肺动脉高压危象.随访心脏超声及心脏CT检查证实主动脉弓重建及心内畸形矫治满意.术后未发生神经系统及其他并发症.随访过程中,患儿生长发育正常,主动脉弓无再狭窄.结论 自体肺动脉具有生长潜力且柔韧性好,是一种理想的重建主动脉弓的材料.一期矫治手术治疗先天性主动脉弓病变合并心内畸形可以得到满意的结果.     

Congenital vascular rings: surgical management of 111 cases

Between October 1974 and May 1987, 111 congenital vascular rings were submitted to surgical correction. There were 83 infants (age: 5 days-12 months, median: 7 months; weight: 1.9-8.5 kg, median: 7.1 kg), and 28 children (age: 1-13 years, median: 3.5 years; weight: 7.5-48 kg, median: 26.5 kg). Patients were divided into five groups: (1) double patent aortic arch (44 cases), (2) double aortic arch with atresia in different parts of the left arch (36 cases), (3) right aortic arch with left ligamentum arteriosum (21 cases), (4) left aortic arch with aberrant right subclavian artery and truncus caroticus (8 cases), and (5) pulmonary artery sling (2 cases). We had no intraoperative mortality but in the postoperative period, 2 neonates died of severe bacterial infections of the respiratory tract.     

Trends in vascular ring surgery

OBJECTIVE: We sought to review our experience with infants and children with anatomically complete vascular rings (ie, double aortic arch and right aortic arch with left ligamentum) and define perioperative trends in diagnostic imaging, operative techniques, and clinical outcomes. METHODS: From 1946 through 2003, 209 patients (113 with double aortic arch and 96 with right aortic arch) underwent surgical repair. Mean and median ages at the time of the operation were as follows: double aortic arch, 1.4 +/- 2.4 years and 0.75 years, respectively; right aortic arch, 2.7 +/- 3.9 years and 0.9 years, respectively. Fourteen (14.6%) patients with right aortic arch had an associated Kommerell diverticulum. Cardiac diagnoses were present in 26 (12.4%) of 209 patients. RESULTS: There has been no operative mortality since 1959. In the past 30 years, mean hospital stay decreased from 8 to 3 days. Primary means of diagnosis has shifted from barium swallow and angiography to computed tomographic scanning or magnetic resonance imaging. In the past 10 years, 73% of patients had preoperative or intraoperative bronchoscopy. The technique of operation has shifted to a muscle-sparing left thoracotomy without routine chest drainage. In 7 recent patients with right aortic arch and a Kommerell diverticulum, the diverticulum was resected, and the left subclavian artery was transferred to the left carotid artery as a primary procedure. CONCLUSIONS: At our institution, computed tomographic scanning has replaced barium swallow as the diagnostic procedure of choice for vascular ring evaluation. We recommend both preoperative bronchoscopy and echocardiography. Use of a muscle-sparing thoracotomy without routine chest drainage has decreased mean hospital stay. For patients with a right aortic arch and associated Kommerell diverticulum, we recommend diverticulum resection with left subclavian artery transfer to the left carotid artery.     

Surgical therapy of congenital aortic valvular stenosis in neonates and infants

The preoperative evaluation, surgical course, and early follow-up results of 5 infants less than 4 months of age who underwent aortic valvotomy for severe valvular stenosis between 1983 and 1985 were reviewed to determine the early prognosis of these neonates. Two of the 5 patients had been used respiratory support due to severe congestive heart failure and dyspnea. Emergency aortic valvotomy was performed in all during cardio-pulmonary bypass which achieve maximal relief of the stenosis without significant causing aortic insufficiency. There was a single operative death and there was one late death at 2 months after surgery who was regarded to have a extensive endocardial fibroelastosis. The other three patients have had a favorable early prognosis during mean follow-up period of 1.78 (0.4-3.8) years. They had been followed by means of two-dimensional and doppler echocardiography, which inferred that the aortic pressure gradient had been kept under 41 mmHg and which indicated that there were no LV enlargement to prove significant aortic valve insufficiency. These results indicate that early infants with severe valvular stenosis can undergo sufficient valvotomy safely and have a favorable early prognosis.     

Outcome of unroofing procedure for repair of anomalous aortic origin of left or right coronary artery

BACKGROUND: Anomalous aortic origin of a coronary artery (AAOCA) from an incorrect sinus of Valsalva is a relatively rare congenital defect and is associated with sudden death. Several surgical techniques have been described to address this defect, but functional outcome has never been addressed. In this report we evaluate a consecutive series of patients treated with unroofing techniques using transthoracic echocardiography, graded exercise testing, and stress echocardiography to assess functional repair. METHODS: Nine consecutive patients (range 7 to 65 years) underwent surgical repair of AAOCA from 1995 to 2001. In all patients the intramural segment was at or below the level of the commissure. All patients underwent a modified unroofing procedure to move the coronary artery orifice to the appropriate sinus. In 2 patients, a new orifice was created without significant unroofing and disruption of the commissure. Patients were evaluated prospectively with exercise electrocardiography testing and by resting and stress echocardiography. RESULTS: Of the 9 patients, 8 presented with symptoms suggestive of ischemia (chest pain, dyspnea on exertion, or syncope). Six patients had anomalous left main coronary artery arising from the right sinus of Valsalva, and 3 patients had anomalous right coronary artery from the left sinus of Valsalva. Transthoracic echocardiography and graded exercise testing was performed in all 9 patients (mean 29 months, range 4 to 85 months) after repair. Of the 9 patients, 8 also underwent stress echocardiography. In 8 of 9 patients the newly created coronary artery ostium was visualized by either two-dimensional echocardiography or color flow Doppler. All patients were symptom free at the time of follow-up. Exercise stress echocardiography was negative in all patients. Of the 8 patients, 7 had normal left ventricular shortening. No patients had regional wall motion abnormalities suggestive of ischemia. All patients were intervention free except 1 patient who developed severe aortic insufficiency and underwent a subsequent Ross procedure 44 months after his initial procedure. CONCLUSIONS: Anomalous origin of a coronary artery from an incorrect sinus of Valsalva is known to be associated with increased risk of sudden death. Surgical correction can be carried out with minimal risk and good anatomic and functional results. Manipulation of the commissure can be avoided by creation of a neo-ostia without extensive unroofing of the intramural segment or manipulation of the intercoronary commissure. This may avoid aortic valve malfunction.     

Left ventricular approach for muscular ventricular septal defects in infant]

Two infants less than 4 months of age underwent repair of apical muscular VSD with left ventriculotomy. First case was 3 months old boy who had already undergone enlargement of the hypoplastic aortic arch with subclavian flap aortoplasty and pulmonary artery banding because of the associated aortic coarctation. Second case was 4 months old Down syndrome girl who associated with ASD and PDA. Both infants were diagnosed the apical muscular VSD with echocardiography and left ventriculography. We performed short longitudinal left ventriculotomy which was parallel to the left anterior descending coronary artery between the diagonal branch and left circumflex artery. VSD was closed with Gore-Tex patch using 6-0 prolene buttress stitches. Post operative echocardiography showed good LV contractile function (Ejection Fraction = 71%, 80%), and no residual interventricular shunt. Left ventriculotomy for the apical VSD closure allowed good exposure and did not reduce the LV function even in infants. Therefore, we concluded that left ventriculotomy was an useful procedure for the apical muscular VSD in infants.     

主动脉瓣下隔膜的外科矫治

目的总结主动脉瓣下隔膜的外科治疗经验及临床疗效。方法回顾性分析2009年3月至2013年9月北京安贞医院32例主动脉瓣下隔膜行主动脉瓣下隔膜切除术的临床资料,其中男22例,女10例;年龄0．5～14．0（3．6±3．2）岁;体重5．5～43．0（17．2±9．5）kg。32例中单纯主动脉瓣下隔膜7例,非单纯主动脉瓣下隔膜25例,其中合并室间隔缺损21例,合并二尖瓣关闭不全和动脉导管未闭各1例;动脉导管封堵术后和主动脉缩窄矫治术后再发主动脉瓣下隔膜各1例。18例合并不同程度的主动脉瓣关闭不全。结果术前超声心动图漏诊1例。全组体外循环时间（71．7±21．7）min,主动脉阻断时间（48．7±15．1）min,住ICU时间（2．2±1．7）d,术后住院时间（7．9±2．5）d。全组无手术死亡。术后发生Ⅱ°房室传导阻滞1例,于术后第6d恢复。所有患儿随访2～54个月,单纯主动脉瓣下隔膜患儿术后主动脉瓣关闭不全有6例好转,非单纯主动脉瓣下隔膜患儿主动脉瓣关闭不全术后有5例好转（P=0．003）;4例术前中度主动脉瓣关闭不全的单纯主动脉瓣下隔膜患儿中术后有3例转为轻度,1例转为微量反流;3例轻度反流中2例术后转为微量反流。8例术前轻度主动脉瓣关闭不全非单纯SM患儿中3例转为微量反流,术前3例中度反流中各有1例转为轻度和微量反流。随访期间全组均无主动脉瓣下隔膜再发。结论主动脉瓣下隔膜超声诊断易遗漏,一经诊断应尽早手术治疗;术中操作要求精细;术后因有再发可能,需定期随访观察。     

Ascending aortic origin of a branch pulmonary artery--surgical management and long-term outcome.

OBJECTIVE: Ascending aortic origin of a branch pulmonary artery (AOPA, hemitruncus arteriosus) is a rare congenital malformation. While there have been isolated case reports, larger series, relating to long-term outcomes following surgery are few. This article analyses the surgical results of a series of nine patients, over a period of 29 years. METHODS: Between 1974 and 2003, nine patients [neonates, 6; infants, 3; male, 5; female, 4] were operated on for AOPA. Median age at presentation was 14 days (range birth to 231 days). Six [corrected] patients (group 1) had associated simple lesions like patent ductus arteriosus or right aortic arch. Three patients (group 2) had complex lesions with right ventricular outflow tract obstruction. One patient (group 2) had DiGeorge syndrome. All patients except group 2 presented with congestive cardiac failure and, in addition one had pre-operative coronary ischemia. Diagnosis was established by angiocardiography in two patients and by echocardiography in seven [corrected] The median age at operation was 28 days (range 7-365). Follow-up period ranged from 7 months to 20.5 years (median 9 years). RESULTS: All nine patients had an anomalous right pulmonary artery (RPA) arising from the proximal ascending aorta, while the left branch was of right ventricular origin. All had evidence of pulmonary hypertension or elevated right ventricular pressure pre-operatively. There was no operative mortality. Of eight patients who had direct anastomosis of the RPA to the main pulmonary artery, one required patch enlargement and another required stenting of an anastomotic stenosis. One patient had a RV-RPA conduit, which required replacement 8, 13, and 14 years later. At follow-up, all patients were alive. All patients in group 1 had normal haemodynamic function and were in NYHA class I. In group 2, all were in NYHA class II with evidence of right ventricular hypertrophy. Four patients had post-operative ventilation-perfusion scans which showed satisfactory perfusion to both lungs. CONCLUSIONS: Early surgery is indicated in this lesion and is compatible with good long-term outlook. Surgical repair should not be deferred for corrective procedures of associated cardiac anomalies.     

Severe Subaortic Stenosis in Interrupted Aortic Arch in Infancy and Childhood

Thirteen out of a total 50 infants with interrupted aortic arch (IAA) seen between 1979-1988 had or developed severe subaortic stenosis (SAS). One had type A interruption and 12 type B. All had a large ventricular septal defect (VSD). The infundibular septum was displaced posteriorly in eight infants, severely narrowing the left ventricular outflow tract (LVOT). Three had fibromuscular narrowing of the LVOT, one each a subaortic muscle bar and membrane. The aortic root and subaortic area were small and measured between 3-8 mm. An anomalous right subclavian artery was noted in eight of the 12 type B IAA, the anomalous vessel arising from the descending aorta in seven. Thirteen infants with IAA and SAS were submitted to surgery, 12 having their subaortic area resected with three perioperative deaths early in the series and three late deaths where active treatment was ceased, including one infant with renal dysplasia who had had a successful establishment of arch continuity and pulmonary artery banding. Of the seven survivors, six have residual Doppler gradients of between 20-50 mmHg, two requiring a second resection and one a third resection. Alternate management programs are suggested based on anatomical evaluation and echocardiography. Any newborn presenting with IAA requires careful evaluation of the subaortic area, best seen on cross-sectional echocardiography. The improved surgical survival in infants following complete repair of IAA has led this important associated anomaly of subaortic stenosis to assume greater importance as a cause of mortality and long-term morbidity.     

Surgical management of congenital obstruction of the left main coronary artery with supravalvular aortic stenosis

OBJECTIVE: Stenosis of the left main coronary artery is a recognized complicating feature of supravalvular aortic stenosis. We have retrospectively identified three anatomic subtypes of left main coronary obstruction in patients with supravalvular aortic stenosis, each necessitating a distinct surgical approach. METHODS: From 1991 to 1998, 9 patients underwent surgical repair of supravalvular aortic stenosis and left main coronary stenosis. Five patients (group 1) had obstruction from near-circumferential thickening of the left main ostium, 2 patients (group 2) had restricted coronary flow due to fusion of an aortic valve leaflet to the supravalvular ridge, and 2 patients (group 3) had diffuse narrowing of the left main coronary artery. Group 1 patients were treated with patch aortoplasty encompassing the left main ostium and supravalvular aortic stenosis. Group 2 patients were treated with excision of the fused leaflet from the aortic wall and patch aortoplasty. Group 3 patients were treated with bypass grafting and aortoplasty. RESULTS: Surgical strategy was determined by coronary angiography and intraoperative assessment of coronary anatomy. There was 1 early death. All surviving patients underwent echocardiography with or without postoperative catheterization. The mean postoperative supravalvular gradient for 7 patients was 8 mm Hg (range 2-15 mm Hg). One patient required reoperation for a residual aortic gradient as a result of aortic arch involvement. No evidence of left main coronary artery stenosis was seen in groups 1 and 2; bypass grafts were patent in group 3 patients at a mean follow-up of 54.8 months. CONCLUSION: Three subtypes of left main coronary stenosis with supravalvular aortic stenosis are described. Each anatomic type mandates an individual surgical approach. Favorable surgical outcomes are achievable with each category.     

Vascular rings and slings. Diagnosis and surgical treatment of 49 patients

Forty-nine infants with symptomatic vascular rings and slings, ranging in age from 20 days to 12 months, required surgical intervention between 1973 and 1984. The following anomalies were present in our patients: double aortic arch with left descending aorta (14), double aortic arch with right descending aorta (6), anomalous innominate artery (13), right aortic arch with aberrant left subclavian artery (4), left aortic arch with aberrant right subclavian artery (10), aberrant left pulmonary artery (pulmonary sling) (2). All the babies had symptoms related to compression of the trachea and/or esophagus. Four patients required temporary tracheostomy in the early postoperative period; 1 patient, affected by a pulmonary sling, required tracheal resection and anastomosis, for severe tracheomalacia. There was one hospital death in a patient with severe tracheal compression from an anomalous innominate artery and brain damage as a result of metabolic problems. Forty-eight patients survived and follow-up ranged from 3 months to 11 years. For each type of vascular anomaly encountered, and based on personal experience, we have outlined a diagnostic scheme allowing an accurate morphological definition and a subsequent surgical procedure.     

Combined cardiac operation and carotid endarterectomy during aortic cross-clamping.

We present a surgical technique that we believe provides superior cerebral protection for simultaneous correction of carotid and cardiac pathology with low operative mortality and stroke rate. Our study population consists of 23 consecutive patients undergoing cardiac operation between August 1989 and April 1991 who also had associated critical (greater than 85%) carotid artery stenosis. Using 20 degrees C systemic hypothermia for cerebral protection, we performed simultaneous correction of both lesions during the aortic cross-clamp period, using continuous retrograde blood cardioplegia for myocardial protection. Mean patient age was 69.4 years; 83% were 65 years or older. Eighty-seven percent had angina, 35% had recent myocardial infarctions (within 30 days), and 52% had congestive heart failure. Asymptomatic bruit was found in 39%, and 61% had previous strokes, neurologic symptoms, or both. All had 85% or greater luminal narrowing on cerebral angiography, with 65% having severe or critical contralateral disease as well. Sixty-one percent had associated other vascular pathology, including peripheral vascular occlusive disease, renal artery stenosis, or abdominal aortic aneurysm. There were no postoperative strokes or neurologic events. One early vein graft occlusion resulted in postoperative myocardial infarction and subsequent death (4.3%).     

先天性心脏病合并右位主动脉弓的诊断与治疗

目的 探讨先天性心脏病合并右位主动脉弓的诊断与治疗。 方法 回顾性分析2012年1月1日至2013年4月1日北京儿童医院小儿心脏中心27例先天性心脏病合并右位主动脉弓行外科手术治疗患儿的临床资料,其中男20例,女7例;年龄（10.96±12.08） 个月,体重（7.70±3.13） kg。法洛四联症14例,室间隔缺损9例,动脉导管未闭1例;肺动脉吊带1例;单纯双主动脉弓2例;均合并右位主动脉弓。其中13例合并血管环畸形,包括迷走左锁骨下动脉畸形和双主动脉弓畸形。所有患儿均接受手术治疗,在矫治心内畸形的同时,进行左锁骨下动脉移植,矫治迷走左锁骨下动脉畸形,双主动脉弓主要是切断一侧非优势弓。 结果 围术期死亡3例,其中1例术后无法停体外循环死亡,1例未合并血管环的患儿术后7 d死亡,1例合并双主动脉弓患儿术后并发急性呼吸窘迫综合征（ARDS） 死亡;1例放弃治疗。随访23例,随访时间3～17个月,心脏超声心动图提示心内畸形矫治满意,恢复良好,双侧上肢动脉波动良好。 结论 对先天性心脏病合并右位主动脉弓患者行外科矫治时应慎重,术前尽量完善相关检查,选择增强CT、磁共振成像检查,明确诊断,制定手术计划。一期同期矫治血管环畸形及其它先天性心脏病,手术效果满意,近期效果良好。     

Nonvalvar aortic stenosis

Seven cases of discrete nonvalvar aortic stenosis, three supravalvar and four subvalvar were treated between 1992 and 1994. Their ages ranged from 6 to 21 (mean 11.3) years. One patient had an associated supravalvar mitral membrane whereas another patient had the classical components of Williams — Beuren syndrome. The patients were investigated by echocardiography. Cardiac catheterisation and cineangiography were undertaken in four patients. Peak preoperative gradient was 27 to 110 (mean 52.3) mm Hg. All patients underwent surgical correction. Mean postoperative gradient was 28.1 (range 18 to 100) mm Hg. In a follow up of five months to two years, there has been no incidence of aortic insufficiency.     

Reduced neurological injury during CABG in patients with mobile aortic atheromas: a five-year follow-up study

BACKGROUND: Mobile atheromas of the thoracic aorta have been identified as a major cause of stroke after coronary artery bypass grafting (CABG). This prospective study was undertaken to identify mobile atheromas and to determine the incidence of immediate postoperative embolic events after suitable surgical modifications. Late clinical events attributable to embolization were also studied. METHODS: Between January 1993 and July 1997, 3,660 patients scheduled for CABG underwent intraoperative transesophageal echocardiography to identify aortic atheromatous disease. The disease was graded as follows: grade I, plaques extending less than 5 mm into the aortic lumen; grade II, plaques extending more than 5 mm into the aortic lumen; and grade III, plaques with a mobile element. Only patients with grade III atheromas were included in the study. Various surgical modifications were done depending on the location of the lesion, eg, aortic arch atherectomy, CABG combined with transmyocardial laser revascularization, off-pump CABG by median sternotomy, and minimally invasive direct coronary artery bypass. Measured outcomes were death, stroke, and other vascular events, both early (within 1 week) and late (1 to 5 years) after operation. RESULTS: Of the 3,660 patients, 104 (2.84%) had mobile atheromas. The perioperative stroke rate was 0.96%, and the incidence of other vascular events was 1.92% at 1 week. There was no embolic event in the group of 88 patients who underwent off-pump CABG. Of the study group, 98.07% are in regular follow-up. At 5 years, 1 patient had had a nonfatal stroke, and 2 patients had died of causes unrelated to atheromatous disease. CONCLUSIONS: The stroke rate was very low in patients with mobile aortic atheromas who underwent CABG after modification in surgical technique, especially off-pump CABG. A follow-up of 5 years showed that patients with mobile atheromas have a very low incidence of spontaneous embolization.     

Aortic root replacement by cryopreserved homogrft for prosthetic valve detachment case due to aortitis

We present a surgical case of a 35-year-old man with aortitis. He had been performed the reconstruction of the right common carotid artery with a saphenous vein graft at 23 years old for his ruptured aneurysm by aortitis. The aortic valve replacement and CABG (LITA to LAD, SVG to D1 and SVG to RCA) were performed for aortic regurgitation and aneurysms of coronary arteries two years ago. The diastolic murmur was first heard at 18 months after the operation. The echocardiography on admission showed an abnormal movement of the prosthetic valve with perivalvular leakage. At the second operation, the valve dehiscence was observed. Although the tissues around the dehiscence was friable and edematous, there wre no signs of vegetation nor abscess formation. His aortic root was replaced with a cryopreserved aortic allograft conduit. His postoperative course was uneventful and aortography revealed neither aortic regurgitation nor stenosis of the coronary artery or SVGs. We think the softness of the allograft valve ring is favorable in valve detachment cases due to not only infection but also aortitis, to prevent redetachment.     

Individualized surgical management of complete atrioventricular canal

In a 5 year interval 46 children with complete atrioventricular canal (CAVC) required 51 operations. Thirty-two children underwent correction; nine received surgical palliation. The ages of the children undergoing correction ranged from 4 months to 14.6 years (mean 4.2 years); eight were less than 1 year old. Weights ranged from 4.1 kg to 39 kg (mean 13.5 kg); 15 weighted less than 10 kg. Ten had undergone previous palliation (seven by pulmonary artery band; three by shunt). There were no early deaths and two late deaths. One infant required mitral replacement at correction; two required subsequent mitral replacement. The ages of the children undergoing palliation ranged from 8 days to 1.34 years (mean 5.8 months); 16 were less than 1 year old. Weights ranged from 2.5 kg to 8.5 kg (mean 4.4 kg); 14 weighted less than 5 kg. Operations included pulmonary artery banding in 14, shunt creation in four, and pericardial enlargement of the right ventricular outflow tract in one. One death occurred 5 days after pulmonary artery banding in an infant with unrecognized coarctation. One late death occurred several months after the creation of a second shunt in a child with severe tetralogy of Fallot and hypoplastic pulmonary arteries. Forty-two (91%) of these children were alive at the time of this review. The outcome in these 46 patients supports individualized choice of initial operation (palliation versus correction) based upon clinical condition, weight, and associated anomalies. The pulmonary artery is banded in infants less than 4 to 5 kg; larger infants and children undergo correction primarily.     

Renal artery stenosis in infants: long-term medical treatment before surgery

This report describes five infants (3 male, 2 female) with renal artery stenosis diagnosed in their 1st year of life. The age at initial presentation was 5 days to 10 months. All had symptoms of congestive heart failure, cardiomegaly on chest X-ray, and left ventricular hypertrophy by electrocardiogram or echocardiogram. Renograms were abnormal in four of the five infants. An intravenous pyelogram was obtained in three infants and was abnormal in two. Renal ultrasounds were obtained in two infants and were normal in both. Patients were treated for 4.4±0.9 years with antihypertensive drug therapy until surgical correction of the renal artery stenosis. Blood pressure was persistently elevated above the 95th percentile in four of the infants during the course of antihypertensive therapy prior to surgery. Patients have been followed for 9.4±2 years since surgery. The blood pressure of four patients is normal, and the blood pressure of the oldest patient (age 23 years) is borderline hypertensive. These data show that infants with renal artery stenosis can be cared for successfully with long-term antihypertensive drug therapy to preserve renal mass with minimal chronic adverse effects.     

小儿主-肺动脉间隔缺损的外科治疗

目的 总结小儿主-肺动脉间隔缺损(APSD)的外科治疗经验。方法 15例APSD，年龄5个月～11岁，体重4.5～21.0kg。按Richardson分型，Ⅰ型7例，Ⅱ型3例，Ⅲ型5例。8例合并其他心脏畸形，其中4例合并复杂畸形(26.7％)。8例Ⅰ及Ⅱ型用补片修补缺损；3例Ⅲ型将人工血管剪成半圆柱形，在主动脉内作内隧道，连接右肺动脉(RPA)和APSD；1例Ⅲ型行人工血管RPA-肺动脉干连接术；1例Ⅲ型用切下一片主动脉壁延伸RPA至APSD；2例直接切开缝合。合并心脏畸形同时予以纠治。结果 手术死亡1例(6.7％)。11例随访3个月～13年，恢复良好。结论 APSD合并复杂心脏畸形较易漏诊，二维多普勒超声检查，结合心导管和心血管造影以及磁共振检查可明确诊断。一经诊断应及早手术，手术年龄以婴儿早期为佳，以免产生肺血管疾病。Ⅲ型APSD及APSD合并复杂心脏畸形是手术死亡的主要原因，术前明确诊断，充分认识病理生理是纠治术成功的关键。