一期手术纠治主-肺动脉窗及伴发畸形

目的总结一期手术纠治主－肺动脉窗及伴发畸形的临床经验，以提高手术疗效。方法我科共收治26例主－肺动脉窗患者．男14例．女12例；年龄1．4±1．6岁；体重7．8±3．8kg。其中单纯主－肺动脉窗8例，合并主动脉弓中断、右肺动脉异常起源于主动脉、法洛四联症、房间隔缺损、室间隔缺损、二尖瓣反流和气管狭窄等18例。25例患者一期手术经主动脉切口补片关闭瘘口纠治主－肺动脉窗，同时纠治伴发的畸形；1例放弃治疗。结果全组无手术死亡。2例伴主动脉弓中断、动脉导管未闭和右肺动脉起源于升主动脉的新生儿术后3d延迟关胸；1例术后出血，3h后再次开胸止血。术后随访22例．随访时间1个月～4年。所有患者无明显的主动脉瓣上狭窄和肺动脉分支狭窄，2例残留轻度二尖瓣反流，1例残留轻至中度二尖瓣反流。结论主－肺动脉窗患者早期易发生肺动脉高压，一经诊断应立即手术。手术方式首选修补主－肺动脉窗及一期纠治伴发畸形。尽管主－肺动脉窗可合并各种心内外畸形，但早期手术纠治可获得较好的中长期疗效。     

一期手术矫治主动脉弓中断及伴发畸形

目的总结主动脉弓中断(IAA)及伴发畸形的手术治疗经验。方法自2000年1月至2005年12月我科共收治48例IAA及伴发畸形患者(平均年龄1.1岁、平均体重7.0kg),合并畸形有室间隔缺损23例,完全性大动脉错位2例,主-肺动脉窗伴右肺动脉起源于主动脉3例,第5主动脉弓残存狭窄2例,右心室双出口2例,永存动脉干2例,迷走右锁骨下动脉伴降主动脉发育不良1例。48例中35例接受手术纠治,其中34例IAA及伴发畸形一期解剖纠治,1例伴右心室双出口患者行主动脉弓吻合及肺动脉环缩术纠治。结果手术死亡4例。术后26例随访3个月~4年,死亡2例。术后发生并发症7例,Ⅲ°房室传导阻滞和膈神经麻痹各1例,轻度吻合口梗阻5例,其余患者无残余解剖问题,心功能良好。结论在新生儿期施行IAA一期矫治手术成功率高,围术期合适的干预是治疗成功的关键;由于伴发畸形复杂,远期生存率不容乐观。     

右肺动脉异常起源于升主动脉的外科治疗

右肺动脉异常起源于升主动脉是一种少见畸形，未经外科治疗的患者病死率较高，外科治疗的目的在于将异常起源的右肺动脉连接重建到主肺动脉，北京阜外心血管病医院从1998年11月至2005年8月对8例右肺动脉异常起源于升主动脉的患儿进行了外科治疗，随访11—92个月，平均（47±33）个月，现报道如下。[第一段]     

婴幼儿一侧肺动脉起源异常的外科治疗

目的总结婴幼儿一侧肺动脉起源异常的外科治疗经验。方法回顾性分析2005年3月至2010年5月第四军医大学西京医院11例一侧肺动脉起源异常合并心血管畸形患者行外科手术治疗的临床资料,手术时平均年龄11.5(2～36)个月;平均体重7.1(4～13)kg。右肺动脉起源于升主动脉7例,左肺动脉起源于升主动脉4例。11例患者均合并其它心血管畸形及中度以上肺动脉高压。全组患者均在低温体外循环下经胸骨正中切口径路行一期手术矫治。结果手术时间169～293(231±55)min,体外循环时间87～210(138±47)min,主动脉阻断时间45～133(86±28)min。围术期死亡1例,病死率9.1%,为法洛四联症合并右肺动脉起源异常患者术后死于低心排血量。生存患者术后超声心动图提示:左、右肺动脉均经右心室肺动脉发出,无狭窄、残余漏,畸形矫治满意。随访10例,随访率100%,平均随访13.5(3～32)个月。超声心动图提示:肺动脉连接均通畅,9例患者的肺动脉压均明显下降。结论一侧肺动脉起源异常应尽早外科手术治疗,婴幼儿一侧肺动脉起源异常行外科手术治疗可获得良好的近期疗效,年龄较大肺动脉压力不可逆增高者外科治疗应谨慎;术中充分游离异常起源的肺动脉及升主动脉,并视具体情况横断主动脉可获得较好的手术显露;自体肺动脉补片加宽修补主动脉缺损可有效避免术后动脉瘤的发生。     

成人口径人工血管治疗右肺动脉起自升主动脉的临床分析

目的总结成人口径人工血管治疗右肺动脉起自升主动脉的经验,探讨手术适应证、手术设计、手术方法以及治疗效果。方法回顾性分析2008年5月至2013年12月哈尔滨医科大学附属第二院11例成人口径人工血管一期矫治右肺动脉起自升主动脉的临床资料,患儿4~25个月,体重4~15 kg,病种包括永存动脉干4例、主-肺动脉间隔缺损7例,全部患者合并中度以上肺动脉高压。全部患儿在体外循环下心脏停搏进行一期矫治,术中将右肺动脉和16~18 mm口径人工血管端侧吻合,将人工血管从主动脉上方连接到主肺动脉或右室流出道切口上。结果患儿平均手术时间224±68(179~325)min,平均体外循环时间117±54(81~208)min,平均主动脉阻断时间42±21(29~63)min,平均ICU停留时间109±42(71~197)h,平均呼吸机辅助91±26(59~191)h,平均住院时间28±11(21~39)d。术后最多发生的并发症是肺动脉高压所致的低心排血量综合征,共有5例,其中死亡2例,其余3例经降低肺动脉压力和腹膜透析治疗恢复良好。9例患儿长期生存良好,术后超声心动图显示人工血管通畅,无扭曲,各吻合口均无狭窄,畸形矫治满意,9例患儿随访3~60个月,心脏彩色超声心动图未发现吻合口及人工血管狭窄,肺动脉压均明显下降。结论右肺动脉起自升主动脉患儿应尽早手术治疗,成人口径人工血管治疗一期矫治右肺动脉起自升主动脉和其它手术方法相比较,临床短期和长期效果均较好,术后低心排血量综合征是常见并发症。     

法洛四联症伴冠状动脉畸形的外科治疗

目的：总结法洛四联症伴冠状动脉畸形手术治疗经验,探讨冠状动脉畸形在法洛四联症纠治手术中的意义。方法：自1994年1月至1999年12月,手术治疗法洛四联症伴冠状动脉畸形15例,年龄43d-8岁,其中右冠状动脉起源于左冠状动脉或左前降支6例,单支左冠状动脉和左前降支起源于右冠状动脉各3例。双前降支,单支右冠状动脉和右冠状动脉肺动脉瘘各1例。一期根治手术13例,姑息手术2例。结果：姑息手术无死亡,根治手术早期死亡1例;无冠状动脉手术意外损伤;随访结果满意。结论：根据肺血管条件和畸形冠状动脉走行特点,选择适当的手术方式和右室流出道重建方法可提高手术效果;冠状动脉畸形不再是法洛四联症纠治手术中的风险因素。     

肺动脉异常起源于主动脉的外科治疗

肺动脉异常起源于升主动脉少见 ,包括右肺动脉起源于升主动脉 (AORPA)和左肺动脉起源于升主动脉 (AOLPA)。病人非手术治疗预后极差 ,多于 1岁以内死亡 ,早期行根治术可治愈。我们共收治病人 13例 ,现总结报道如下。临床资料　 13例病人中AORPA 9例 ,AOLPA 4例 ;年龄5 5d～ 30岁。活动后胸闷、气短 9例 ,发绀 9例 ,咯血 3例。听诊多于胸骨左缘第 2～ 4肋间闻及收缩期或连续性杂音。X线胸片多显示肺部纹理重 ,有时两侧肺纹理不对称 ,主动脉结增宽 ,部分AORPA病人右肺动脉影显示不清或右肺下动脉干起始位置偏高 ;平均心胸比率 0 6 …     

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。     

左冠状动脉异常起源于肺动脉的临床治疗

目的总结左冠状动脉异常起源于肺动脉的手术治疗经验。方法回顾性研究总结近年9例左冠状动脉异常起源于肺动脉的患者,对4例于肺动脉起始部结扎左冠状动脉,5例于体外循环下行肺动脉内隧道成形术(Takeuchi术),合并的心脏畸形同时予以纠治。结果全组9例术后无心肌缺血或梗死、残余分流和晚期死亡,心功能恢复到I级。结论左冠状动脉异常起源于肺动脉畸形一旦确诊,主张手术治疗。     

主动脉与人工血管侧侧吻合技术在体肺动脉分流术中的应用

目的 探讨主动脉与人工血管侧侧吻合技术在体肺动脉分流术中的应用.方法 2010年4月至2011年6月,44例紫绀型先天性心脏病患儿行主动脉与人工血管侧侧吻合的体肺动脉分流术,其中升主动脉-主肺动脉38例,升主动脉-左肺动脉4例,升主动脉-右肺动脉2例.男、女各22例;＜1岁23例,1～3岁14例,＞3岁7例;体质量(9.2±3.6) kg.肺动脉闭锁32例(3例室间隔完整,29例伴室间隔缺损),法洛四联症10例,右室双出口合并肺动脉狭窄和矫正性大动脉转位并肺动脉狭窄各1例.术后定期随访.结果 全组无手术死亡.2例出现早期狭窄,1例因早期分流堵塞再次行中心分流术,1例分流过多.术后经皮血氧饱和度由0.67±0.10增加至0.81±0.07.术后动脉压增加0～ 25mm Hg(1 mm Hg =0.133 kPa),中位值6.5 mm Hg.所有患儿随访12 ～ 23个月.2例出院后第1、3个月因误吸死亡;2例人工血管狭窄;2例人工血管闭塞,其中1例术后14个月再次行体肺动脉分流术;7例肺动脉发育良好者行进一步手术治疗,其中4例行根治手术,3例行Glenn手术.余患儿经皮血氧饱和度0.82±0.05,超声心动图示分流血管通畅,血流束与人工血管口径一致.结论 主动脉与人工血管侧侧吻合技术的体肺动脉分流术操作简单,安全性高,人工血管堵塞率低,可以推广应用.     

Aortopulmonary window

Aortopulmonary window is a rare defect caused by failure of fusion of the two opposing conotruncal ridges that are responsible for separating the truncus arteriosus into the aorta and pulmonary artery. Aortopulmonary window may occur as an isolated lesion or it can be associated with other cardiac abnormalities in one third to one half of cases. The most common associated lesions are arch abnormalities, specifically interrupted aortic arch and coarctation of the aorta. Antenatal diagnosis is rare. In the current era, early mortality following repair of simple aortopulmonary window approaches zero and depends on the presence of associated lesions, especially interrupted aortic arch. Long-term outcome should be excellent. Early morbidity includes pulmonary artery stenosis and residual aortopulmonary septal defects. Long-term follow-up is indicated to look for recurrent lesions such as the development of branch pulmonary artery stenosis and arch obstruction.     

Surgical management of aortopulmonary window and associated lesions

BACKGROUND: Aortopulmonary window is a rare congenital heart defect commonly associated with other cardiac anomalies. Although single institutional experience is low, several surgical techniques have been reported. The purpose of this study is to describe our approach to the management of aortopulmonary window and its associated lesions. METHODS: Between February 1996 and November 2002, 13 patients underwent repair of aortopulmonary window. The age range went from 4 days to 5.5 months (mean 42 +/- 52 days), with 9 patients younger than 1 month old. The weight range was from 1.9 to 6.7 kg (mean 3.5 +/- 1.2 kg). Concomitant cardiac anomalies were present in 11 patients. The major additional anomalies were interruption of aortic arch in 4 patients and tracheal stenosis in 1 patient. Initial diagnoses were made using two-dimensional echocardiography only. RESULTS: There was one postoperative death. In general, patients with aortopulmonary window and additional major defects had a prolonged intensive care unit and hospital stay when compared with the other patients. Follow-up time ranged from 2 months to 6.8 years (mean of 2.5 +/- 2.2 years). There were no reoperations and no late deaths. Transcatheter balloon dilatation of the repaired aortic arch was required in 1 patient and of the right pulmonary artery in another. All other patients had good flow to both pulmonary arteries. No residual shunts were detected at the aortopulmonary window site, and pulmonary pressures were normal. CONCLUSIONS: Aortopulmonary window may be effectively diagnosed with echocardiography. Early surgical treatment (neonatal period, if possible) is safe and associated with the best long-term results, even in the presence of other cardiac anomalies. Complete separation and reconstruction of both aorta and pulmonary arteries under direct vision may prevent recurrence and distortion of adjacent structures.     

Surgical management of aortopulmonary window.

OBJECTIVE: Aortopulmonary window is a rare congenital malformation involving a window-like communication between the ascending aorta and the pulmonary artery. Here, we present our experience regarding the surgical repair of an aortopulmonary window, and also assess the long-term outcome. METHODS: Thirteen children with an aortopulmonary window associated with various congenital lesions underwent a repair of the defect. The age at operation ranged from 3 days to 1 year (median age, 19 days). The patient's weight ranged from 2.1 to 7.0 kg (mean weight, 3.6 kg). The associated lesions included an interrupted aortic arch (5 patients), a ventricular septal defect (2), an atrial septal defect (1), mitral valve regurgitation (1), and tricuspid atresia [Ic] with mitral valve regurgitation (1). The aortopulmonary window was repaired with a cardiopulmonary bypass in 11 patients, and 2 patients were ligated without a cardiopulmonary bypass. RESULTS: One patient associated with tricuspid atresia died (mortality rate of 7.7%). There has been no late death during a mean follow-up of 7 years and 3 months. CONCLUSIONS: The surgical results for an aortopulmonary window are encouraging, even if such patients are associated with major cardiac anomalies and an interrupted aortic arch. Most have shown a good long-term outcome.     

Anomalous origin of left coronary artery from the right pulmonary artery in association with type III aortopulmonary window and interrupted aortic arch

Anomalous origin of the left coronary artery from the pulmonary artery, also known as Garland-Bland-White syndrome, usually occurs as an isolated condition. We report an infant with caudal regression sequence diagnosed with interrupted aortic arch type B and type III aortopulmonary window, who was found to have anomalous origin of the left coronary artery from the right pulmonary artery at surgical repair. Successful repair of the aortopulmonary window and interruption was performed with reimplantation of the left coronary artery into the ascending aorta. This report highlights the importance of closely assessing the coronary ostia in patients undergoing complex aortopulmonary window repair.     

Primary repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals: a useful approach

BACKGROUND: The ultimate goal of surgical therapy for pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries is to create unobstructed and separate in series pulmonary and systemic circuits. Our preference has been a 1-stage complete unifocalization technique, avoiding collateral anastomosis with either the native pulmonary arteries or other aortopulmonary collateral vessels. METHODS AND RESULTS: Since 1998, 5 patients (median age 29.6 months) with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries have undergone surgical correction, consisting of (1) exclusion of a descending thoracic aortic segment from which all major aortopulmonary collateral arteries originate, and (2) connection of this aortic segment to the native pulmonary artery using an interposition polytetrafluoroethylene conduit. The ventricular septal defect was closed in all patients, and the right ventricle was connected to the unifocalized pulmonary artery with a valved conduit. All patients survived the operation. Two patients required reexploration for postoperative bleeding. One patient remained on mechanical ventilation for 17 days due to a pulmonary infection. During follow-up (12-21 months), no patient required additional interventions. The postoperative right ventricular/left ventricular pressure ratio was 0.55 median. No significant stenosis within the reconstructed pulmonary circuit was identified. All patients remain free of symptoms, requiring no medications. CONCLUSION: Intracardiac repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries can be accomplished by a midline 1-stage repair including complete unifocalization of all pulmonary blood supply without individual collateral anastomosis in selected patients. This approach offers a convenient and satisfactory surgical option.     

Surgical treatment of interrupted aortic arch in infants under three months of age

From March, 1982, through March, 1988, 8 infants less than 3 months of age underwent surgical treatment of interrupted aortic arch. Five patients had IAA type A and 3 patients had type B. Seven patients with associated VSD underwent staged operations and 1 infant with aortopulmonary window underwent primary repair off cardiopulmonary bypass. Aortic arch reconstruction was by subclavian aortic anastomosis (6) or large tube graft (8 mm) interposition (2) combined with pulmonary artery banding (5). All seven patients with associated VSD survived the first-stage operation. One patient subsequently underwent pulmonary artery banding, two underwent patch aortoplasty and four patients underwent intracardiac repair and removal of a pulmonary artery band, with six survivors (86%). The operative death occurred in an infant in whom modified Damus-Kaye-Stansel operation was carried out for severe subaortic stenosis, which rerouting all left ventricular blood through VSD and the main pulmonary artery into the ascending aorta and reconstructing right ventricular-distal pulmonary artery connection by a valved external conduit. All six surviving patients are doing well at present (mean follow up of 3 years) without any significant pressure gradient between the ascending and thoracic aorta. Based on these data we conclude: (1) Aortic arch reconstruction and pulmonary artery banding can be reliably performed even in critically ill infants with IAA and isolated VSD. (2) The primary repair will provide better chance of survival in infants with IAA associated with significant LVOTO, truncus arteriosus or aortopulmonary window that do not readily lend themselves to pulmonary artery banding.     

Anomalous origin of the left coronary artery. A twenty-year review of surgical management.

Children with anomalous origin of the left coronary artery from the pulmonary artery are at risk for myocardial infarction and death. Surgical management of this condition in children has evolved significantly during the past 20 years. Between 1970 and 1990, a total of 20 of these patients underwent surgical intervention at two institutions. Age at operation ranged from 3 weeks to 11 years (mean, 26 months). Twelve patients had congestive heart failure, three were in cardiogenic shock, and two had cardiac murmurs. Operative techniques included ligation (n = 9), subclavian artery anastomosis (n = 5), aortic implantation (n = 3), internal mammary artery anastomosis (n = 1), intrapulmonary tunnel from aortopulmonary window to coronary artery (n = 1), and cardiac transplantation (n = 1). The three deaths in the series occurred at 3 weeks, at 2 months, and at 9 years after ligation. There have been no deaths after establishment of a two coronary artery system or after transplantation. Two of the five patients who had subclavian artery anastomosis to the anomalous coronary artery have severe anastomotic stenosis and collateralization. For patients with anomalous origin of the left coronary artery from the pulmonary artery, we recommend direct aortic implantation of the anomalous coronary artery at the time of diagnosis. Intrapulmonary tunnel from aortopulmonary window to coronary artery, or aorta-coronary bypass with internal mammary artery are recommended for children in whom aortic implantation is not anatomically feasible. Left coronary artery ligation is not indicated for these patients; those who have survived ligation should be considered for elective establishment of a two coronary artery system because of the risk of late death.     

Translocation of Anomalous Right Coronary Artery to Aortic Side of the Aortopulmonary Window: A Different Approach for a Rare Combination

Abstract   A case of aortopulmonary window associated with anomalous origin of the right coronary artery from the pulmonary artery in a four-month-old boy is reported in this paper with a different method of repair. In this patient, surgical repair was done by transferring the right coronary artery from the pulmonary artery to the aorta at the aortopulmonary window side. The defect at the pulmonary artery was repaired by a pericardial patch, so no other secondary opening was used for the aorta in order to reimplant the coronary artery .     

Aortopulmonary window and anomalous coronary artery: an exceptional association

Aortopulmonary window is rarely associated with other cardiac anomalies, such as anomalous origin of one coronary artery. This exceptional association has been reported in 15 patients, with different surgical solutions. We describe 2 patients with aortopulmonary window with anomalous origin of the right coronary artery, one of which presented with tetralogy of Fallot-pulmonary atresia. A neonatal modified Blalock-Taussig shunt and ligature of the distal pulmonary artery at the level of pulmonary bifurcation was performed in the first patient, and the second patient underwent aortopulmonary window division with anomalous coronary artery reimplantation. Both patients had an uneventful postoperative course.