无肺动脉狭窄右心室双出口的外科治疗

目的总结32例无肺动脉狭窄(PS)右心室双出口(DORV)患者的外科治疗经验.方法所有患者均行双心室修复,建立左心室至主动脉的心内隧道.结果全组早期死亡5例(15.6%),其中2例死于肺动脉高压危象,3例死于低心排血量综合征;晚期死亡1例(3.1%),术后95天死于残余漏导致的右心衰竭.24例随访2个月～22年,患者自觉症状消失,能从事正常学习或工作.结论无PS的DORV手术治疗效果良好,一旦诊断明确,应尽早手术治疗,以避免出现不可逆的肺血管病变,影响治疗效果.     

房室连接不一致的心室双出口外科治疗

目的　报道房室连接不一致的心室双出口外科治疗经验。　方法　从 1985年 1月至 2 0 0 2年 9月 ,共进行房室连接不一致的心室双出口外科治疗 11例 (年龄 3～ 2 5岁 ) ,其中右心室双出口 (DORV) 10例 ,左心室双出口(DOL V) 1例 ,行双心室修复术 9例 ,采用心内补片、心外带瓣管道或冷冻同种带瓣管道修复 ;行 Fontan类手术 2例。结果　双心室修复术后早期死亡 2例 ,死亡原因为严重低心排血量综合征 ;Fontan类手术修复后血氧饱和度和活动耐力明显改善 ,无早期死亡 ;双心室修复术后长期随访 4例 ,随访时间 1～ 11.5年 ,NYHA心功能 级或 级 3例 , 级 1例。　结论　房室连接不一致的心室双出口根据心室、肺动脉发育和合并心内畸形决定手术方式 ,完整修复室间隔缺损 ,保证心室大动脉流出道通畅 ,修复房室瓣和防止损伤传导束是传统心内修复术的关键。     

Nikaidoh手术的临床应用

目的 总结Nikaidoh手术治疗右心室双出口(DORV)和大动脉转位(TGA)患者的临床经验,以提高手术疗效. 方法 3例先天性心脏病患者中DORV 1例,TGA 2例(平均年龄11.6岁),均行Nikaidoh手术,术中行Lecompte操作,Gore-tex人工血管补片修补室间隔缺损并重建左心室流出道,18＃带单瓣牛心包片补片重建肺动脉及右室流出道. 结果 术后无早期死亡,平均住院时间13.6d.发生低心排血量综合征1例,肝功能损害2例,二次开胸止血1例,均经相应的处理治愈.随访3例,随访时间3～5个月,复查超声心动图未发现左、右心室流出道梗阻;其中2例发现轻度至中度主动脉瓣关闭不全,但定期随访未发现继续加重. 结论 采用Nikaidoh手术治疗DORV和TGA患者,术后可获得良好的血流动力学效果,早期临床结果满意.     

双向上腔静脉肺动脉吻合术治疗左心室发育不良的右心室双出口

目的总结双向上腔静脉肺动脉吻合术治疗合并左心室发育不良的右心室双出口(DORV)的临床经验。方法2000年1月至2004年12月手术治疗7例患者,均伴有肺动脉狭窄和左心室发育不良,左心室舒张期末容积指数均<30m l/m2。5例在体外循环下完成手术,2例在非体外循环下完成手术。结果全组无手术死亡。术后机械通气时间为9.0±7.9 h,无严重术后并发症发生,活动能力改善。随访6个月~4年,效果满意。结论对左心室发育不良的DORV患者,双向上腔静脉肺动脉吻合术可以获得满意的治疗效果。     

房室连接不一致右心室双出口根治手术的选择与适应证优化

目的　回顾性分析房室连接不一致右心室双出口(doubleoutlet right ventricle,DORV)的病理解剖特点、手术方法选择和治疗结果,探讨手术适应证和手术方法的优化。　方法　1990年9月至2 0 0 4年8月对7例房室连接不一致的DORV患者施行根治手术治疗,年龄3～7岁,其中形态学左心室与肺动脉心外管道连接和室间隔缺损修补术3例,double- switch(双调转)手术2例,改良Fontan手术2例。　结果　手术早期死亡1例(14 .3% ,1/ 7) ,为手术后2个月并发Hom ograft感染。 度房室传导阻滞1例,安装永久心外膜起搏器;1例改良Fontan手术后并发上腔静脉回流梗阻和心包积液,症状改善后出院。Double- switch手术后平均监护时间最短为术后6 d。　结论　房室连接不一致DORV根治手术应首选double- switch手术,尤其对形态学右心室功能不全和三尖瓣关闭不全者,如果合并多种复杂畸形解剖无法矫治,可选择单个心室的修补方案。     

儿童心脏瓣膜置换手术及其疗效

目的探讨儿童心脏瓣膜置换手术及治疗效果。方法1990年1月至2002年12月，45例14岁以下儿童施行了心脏瓣膜置换手术。其中男26例，女19例。年龄3—14岁，平均10．8岁；≤10岁15例，10～14岁30例。包括先天性心脏瓣膜病32例，风湿性瓣膜病6例，心内膜炎3例，部分型房室管畸形修补术后二尖瓣关闭不全3例，室间隔缺损修补术后主动脉关闭不全1例。行二尖瓣置换23例，其中2例为矫正型大动脉转位行解剖位三尖瓣置换，主动脉瓣置换9例；二尖瓣与主动脉瓣双瓣置换4例；三尖瓣置换9例。5例使用生物瓣膜或同种主动脉瓣，余40例均采用机械瓣膜，包括进口双叶瓣34枚，进口单叶瓣3枚，国产单叶瓣7枚。结果本组手术死亡(术后30d内)2例，死亡率4．4％，均死于手术当日，1例因顽固性心律失常，1例术后低心输出量综合征。生存者随访8个月-12年，平均4．9年。4例晚期死亡，晚期死亡率9．3％。置入机械瓣膜者均采用华法林抗凝治疗，未发生血栓栓塞及抗凝相关并发症，病儿术后心功能均Ⅰ-Ⅱ级。结论儿童瓣膜病病人，大多数可以置入合适的成人型号人工瓣膜，保证其术后生长发育，减少二次手术。采用华法林进行抗凝治疗，经过平均4．5年随访，无血栓栓塞或抗凝有关的出血并发症发生，治疗效果较为满意。     

阜外医院71例Rastelli手术的近中期结果

目的总结分析阜外医院Rastelli手术的近中期结果。方法2010年5月至2017年3月阜外医院完成71例Rastelli手术治疗大动脉转位(TGA)或者右心室双出口(DORV)合并室间隔缺损(VSD)和肺动脉狭窄患儿,男48例,女23例;手术时年龄(4.7±2.7)岁,其中10例完全型大动脉转位;27例DORV,34例矫正型TGA(CTGA)。30例有手术史,其中Blalock-Taussig分流13例,双向Glenn手术17例。31例同时扩大室间隔缺损。外管道使用同种带瓣管道9例,牛颈静脉管道56例,自制Gore-Tex外管道6例;管道直径(17.9±3.3)mm。结果本组主动脉阻断(132.0±71.1)min,体外循环(209.0±83.4)min。机械通气时间(102.6±81.7)h。术后住院(13.6±12.8)天。早期死亡1例(1.4%)。早期并发症12例(16.9%),其中因Ⅲ度房室传导阻滞安装永久起搏器4例,心包积液开窗3例,延迟关胸3例,二次开胸2例。术后随访4个月~6.8年。1、5年生存率分别为97.2%,97.2%;1、5年免除右心室流出道狭窄(RVOTO)率分别为98.6%,84.1%;1、5年免除干预率分别为98.6%,90.0%。随访期间置换外管道1例,介入球囊扩张7例10次。所有患儿最后一次随访均未见左心室流出道狭窄,左心室到主动脉压差(10.5±8.8)mmHg。结论Rastelli手术适用于部分DORV、CTGA、TGA合并VSD和严重的肺动脉狭窄或肺动脉闭锁患儿,早期死亡比例低,中期效果好,但是远期常需要再手术,尤其是置换外管道。     

303例右室双出口手术后早期转归

目的　总结右室双出口 (DORV)病例的术后处理及不同外科术式的早期转归。方法　 1988年 1月至 2 0 0 3年 12月期间收治 30 3例DORV病儿 ,按室间隔缺损 (VSD)的部位分为主动脉下VSD ,肺动脉下VSD ,双动脉下VSD和远离大动脉下VSD 4组。总结比较不同外科术式术后的并发症和死亡率及其原因。结果　再手术 18例 ,死亡 2 9例 ,死亡率为 9 6 % ,其中肺动脉下VSD死亡率最高 ,为 2 4 . 4 % (χ2 =14 . 12 ,P <0 . 0 1) ;根治术和姑息术的死亡率分别为 10 . 6 %和 6 . 6 % (χ2 =1. 0 3,P >0 .0 5 )。死亡原因主要是低心排血量综合征 (LCOS) ,占 82 . 6 % ;其也是术后主要并发症 ,占 31 .0 %。结论　LCOS是术后主要并发症和死亡的高危因素。有效预防和控制LCOS是取得治疗成功的关键。     

法洛四联症合并完全性房室间隔缺损的手术治疗

目的 总结并探讨法洛四联症合并完全性房室间隔缺损(TOF-CAVC)的外科治疗方式及时机.方法 2007年6月至2012年4月,11例TOF-CAVC患儿行双心室或部分双心室修补,男6例,女5例;年龄5 ～32个月.手术经右心房或右心室流出道纵切口,采用“双片法”修补房室通道,其中8例通过单一的右心房切口修补室间隔缺损.所有患儿均行右心室流出道-肺动脉扩大补片修补手术,其中7例由于肺动脉瓣发育欠佳需跨瓣修补,1例用带单瓣的心包补片扩大右室流出道,3例未跨瓣修补.结果 手术死亡1例,术后第2天死于严重低心排出量综合征.1例术后6个月死于肺炎、心力衰竭.生存9例术后均有效随访,随访3～60个月.根据Kaplan-Meier生存曲线,5年生存率为79.5％.术后3个月,2例患儿分别出现右心室流出道及肺动脉分支梗阻,2例残余房间隔缺损0.2 ～0.3 cm,1例心室水平残余细丝分流,患儿均无明显临床症状.随访期间所有生存患儿心功能Ⅰ～Ⅱ级.结论 法洛四联症合并完全性房室间隔缺损近期疗效肯定,采用双片法通过单一的右心房切口修补房室通道时,术中的精细操作是手术成功与否的关键.     

心室双出口手术治疗临床研究

目的：探讨心室双出口的手术治疗效果。方法：回顾性分析72例心室双出口病人手术治疗方法及治疗效果。右室双出口71例中SDD型64例，ILL型3例，SDL型3例，IDD型1例；左室双出口1例（ILD型）。采用左心室-主动脉心内隧道连接61例，全腔静脉-肺动脉连接2例，左心室-主动脉心内隧道并右心室-肺动脉心外管道连接3例，Glenn分流6例。结果：死亡2例。1例术后3个月发现残余室间隔缺损，予再次手术缝合。余者效果良好，无远期死亡及相关并发症发生。结论：根据心室双出口的不同类型，选择最佳手术方式及掌握手术时机是成功的重要因素，重建通畅良好的左或右室流出道是提高手术成功率的关键因素。     

Biventricular repair in double outlet right ventricle: surgical results based on the STS-EACTS International Nomenclature classification.

BACKGROUND: The STS-EACTS International Nomenclature for Congenital Heart Surgery (CHS) defines four anatomic subtypes of double outlet right ventricle (DORV) based on the relationship of the ventricular septal defect (VSD) with the great vessels and the presence of right ventricular outflow tract obstruction (RVOTO). We reviewed our experience with DORV patients and two ventricles that underwent repair, applying this nomenclature. METHODS: Between January 2000 and January 2005, 50 patients with DORV and two viable ventricles underwent surgical intervention: 44 patients had biventricular repair, 3 had 1.5 ventricular repair, 2 underwent a Fontan, and 1 died prior to corrective surgery. Median age at repair was 9.1 months (range: 4D-4Y). Eighteen patients (36%) were DORV-Fallot (including 5 with AVSD and heterotaxy), 9 (18%) were DORV-TGA (Taussig-Bing), 12 (24%) were DORV-VSD, and 11 (22%) were DORV non-committed VSD. Corrective surgery included 35 repairs with a VSD-aorta baffle+/-RVOTO procedure and 12 arterial switches with a VSD-PA baffle (9 Taussig-Bing and 3 DORV-ncVSD). Associated procedures included 13 VSD enlargements, 8 subaortic resections, 9 arch repairs, 5 AVSD repairs, and 7 others. RESULTS: There were three deaths in the 50 patients studied (overall mortality of 6%). Excluding one patient that died prior to corrective surgery and the two patients palliated with a Fontan procedure, the actual surgical mortality for a corrective repair was 4.3% (2/47 patients). Two surgical deaths occurred following, respectively, one repair of a Taussig-Bing with an interrupted arch and a Swiss cheese VSD and one repair of ncVSD-type with pulmonary atresia that had undergone a previous cavo-pulmonary anastamosis. No late deaths occurred. Two late reoperations included a heart transplant in a DORV-Fallot patient with Swiss cheese VSD and subaortic resection in a DORV-ncVSD patient. Angioplasties were needed for PA stenosis (n=2) and aortic arch obstruction (n=2). Four patients had LV to aorta baffle gradients between 10 and 20 mmHg. All patients were of NYHA class I/II. CONCLUSIONS: The STS-EACTS International Nomenclature provides more uniform analysis of outcomes with respect to acceptable surgical risk and mortality. Biventricular repair can be safely achieved on selected DORV, including DORV-ncVSD and DORV with AVSD and heterotaxy lesions traditionally indicated for a single ventricle palliative approach.     

完全性心内膜垫缺损合并右室双出口的双心室修复

目的 总结完全性心内膜垫缺损合并右室双出口双心室修复的经验.方法 2006年7月至209年11月对6例完全性心内膜垫缺损合并右室双出口病人采用双片法修补完全性心内膜垫缺损,人工血管补片作右心室内隧道,连接室间隔缺损与主动脉口,引导左心室血经内隧道进入主动脉;应用牛心包补片扩大右室流出道;肺动脉瓣环小者用带瓣补片跨环修补右室流出道;心室内隧道有可能阻塞右室流出道造成重度梗阻者,右室流出道与主肺动脉之间安置带瓣管道(Rastelli手术).结果 术后死亡1例,占16.6%,其余病例恢复良好.结论 完全性心内膜垫缺损合并右室双出口一期双心室修复可获得良好效果.

Abstract：

Objective Double-outlet right ventricle,which often associated with total anomalous pulmonary venous connection and complete endocardial cushion defects,has been considered a risk factor for biventricular repair procedure.To reviewed cases treated by biventricular repair for endocardial cushion defects with double outlet right ventricle.Methods From July to November of 2009,6 patients (3 males and 3 females) aged from 7 to 24 (16.17±5.98) years and with endocardial cushion defects and double outlet right ventricle underwent operation of biventricular repair The duration of follow-up ranged from 10 days to 2 years(median,16 months).Endocardial cushion defects were repaired with a 2-patch technique.The artificial vascular patch was implanted to connect the ventricular septal defects and the aorta for draining the blood stream from the left ventricle to the aorta.The other patch was used to repair the ostium primum atrial septal defects.Right ventricular outflow tract obstructions was released and reconstructed by transplanting a bovine pericardium patch.If the size of pulmonary valve annulus was far more below the normal,a transannular pericardial patch was used.Rastelli procedure with a valved conduit between the right ventricle and the pulmonary artery would be performed if the obstruction in the right ventricular outfolw tract was severe.Results One death occurred 2 days after the operation,resulting in a mortality rate of 16.6%.The case,a boy of 7 years old,had a mirror-image dextrocardia,complete endocardial cushion defect ( C type),anomalous pulmonary venous drainage and single atrium.In this case,the operation lasted for 8 hours,acute renal failure happened next day to the operation,the effect of CRRT was unsatisfied,and eventually cardiac arrest occurred as a result of hyperkalemia.The remaining cases had survived.Follow-up examinations showed that the systolic pressure gradients across the pulmonary valves decreased,with a range of 14 to 40 mm Hg,(23.9 ± 11.03) mm Hg.Mild mitral and tricuspid regurgiration were identified in 4 cases and moderate mitral regurgitation was identified in one case.The cardiac function in all patients was classified as NYHA class Ⅰ/Ⅱ,Conclusion Endocardial cushion defects with double outlet right ventricle can be corrected by means of biventricular repair procedure.The procedure was associated with a low mortality,The interim life quality of patients may be improved.The longterm outcomes should be further studied.     

Surgical results in patients with double outlet right ventricle: a 20-year experience.

BACKGROUND: The objective of this study was to review our surgical strategy in children with double outlet right ventricle and to assess risk factors for early and late mortality and reoperation. METHODS: Patients (n = 124; June 1980 to January 2000; age range, 7 days to 16 years; mean, 2.8 years) who underwent repair of double outlet right ventricle. The patients were divided into three groups. Group 1 (n = 47) had noncomplex patients with atrioventricular concordance, a single ventricular septal defect, balanced ventricles, no straddling atrioventricular valves, and no major pulmonary artery anomalies. Group 2 (n = 39) included patients with double outlet right ventricle and a subpulmonary ventricular septal defect (Taussig-Bing). Group 3 (n = 38) had patients with complex anomalies including straddling atrioventricular valves, atrioventricular septal defects or a hypoplastic valve or ventricle, or a combination of atrioventricular septal defects and hypoplastic valve or ventricle. RESULTS: Four types of definitive repairs were performed: (1) intraventricular tunnel repair with a baffle from the left ventricle to the aorta (n = 53); (2) use of a valved or nonvalved conduit (n = 20); (3) arterial switch operation with a patch committing the left ventricle to the neo-aorta (n = 16); and (4) cavopulmonary shunt and Fontan procedures (n = 33). Two patients with late postoperative cardiomyopathy had heart transplantation. Potential risk factors included location of the largest ventricular septal defect, presence of additional ventricular septal defects, ventricular outflow obstruction or hypoplasia, or both ventricular outflow obstruction and hypoplasia, previous palliation, and type of definitive operation. There were six early deaths (4.8%) and four late deaths (3.2%), and two heart transplants (1.6%). Overall 15-year survival was 95.8%, 89.7%, and 89.5% for groups 1, 2, and 3, respectively (p = 0.08). Thirteen patients (11.4%) have required 15 reoperations. Mean follow-up for survivors was 76.6 +/- 52.8 months. Up-to-date follow-ups are available on 114 surviving patients. Ninety-five of these patients (83.3%) were in New York Heart Association class I, and the remaining 19 patients (16.7%) were in New York Heart Association class II. Freedom from reoperation was 87%, 72%, and 100% at 15 years for groups 1, 2, and 3, respectively (p = 0.11). CONCLUSIONS: Survival was high for all patients with double outlet right ventricle undergoing intraventricular tunnel repair, arterial switch operation, and repair with a conduit or a modified Fontan procedure. Careful attention to preoperative anatomy dictates the best surgical approach and will enhance outcomes.     

Double-outlet right ventricle with non-committed ventricular septal defect

Objective: The term non-committed was used to define hearts in which the VSD was anatomically related to, or was close to, neither great vessel, being separated from both by considerable muscle. We report our experience of the surgical management of this subset, considered being of particular surgical relevance. Methods: Between January 1987 and December 1997, 23 patients having double-outlet right ventricle (DORV) with non-committed VSD underwent biventricular repair. Nine (39%) had undergone previous palliation. The median age was 20 months and the median weight was 8.5 kg. Two main types of repair were used: intraventricular baffle repair (n=21) and arterial switch operation with VSD to pulmonary artery baffle (n=2). At repair, 12 (52%) patients required concomitant VSD enlargement. In two other patients presenting with restrictive inlet VSD associated with tricuspid attachments, crossing the subaortic pathway biventricular repair was abandoned at operation. Results: There were two hospital deaths (9%, 70% CL: 3–19%). Eight patients (35%, 70% CL: 23–48%) underwent nine reoperations, six for subaortic stenosis. No late death occurred. At last visit, all patients were asymptomatic and only two had cardiac medication. Conclusions: The biventricular repair of DORV with non-committed VSD is feasible in the vast majority of cases with comparable results to other subsets of DORV. After repair, the subaortic region is at risk for development of subaortic stenosis.     

Atrioventricular discordance: results of repair in 127 patients.

OBJECTIVE: The conventional management of patients with atrioventricular discordance is directed at associated lesions, taking advantage of physiologic "correction"; however, the morphologic right ventricle and tricuspid valve support the systemic circulation. Questions surrounding survival using this approach led us to analyze our institutional results. METHODS: All patients with atrioventricular discordance undergoing biventricular repair were analyzed (n = 127, 1959-1997), excluding those with functionally univentricular hearts. The ventriculoarterial connection associated with atrioventricular discordance varied and was most commonly discordant (87%), but occasionally concordant (6%), double-outlet right ventricle (6%), or double-outlet left ventricle (1%). At initial presentation, the most common lesions associated with atrioventricular discordance were ventricular septal defect (86%), pulmonary stenosis (64%), tricuspid regurgitation (28%), and atrioventricular block (12%). Nine patients underwent a double switch procedure to create ventriculoarterial concordance and the remainder were managed conventionally without correcting discordant connections. RESULTS: Operative mortality was 6% and did not vary by associated lesion. Twenty years after repair, survival was 48%. Within 20 years, 56% of patients required reoperation, usually for atrioventricular valve incompetence (n = 16), pulmonary stenosis (n = 16), or both (n = 3). Pacemakers were required in 50 patients, 4 before repair, 40 within 2 months of repair, and 6 remotely after repair. In early follow-up, the double switch procedure (n = 9) had equivalent mortality and a high pacemaker requirement for atrioventricular block. CONCLUSIONS: Analysis of conventional management of atrioventricular discordance revealed cumulative increases in mortality, systemic atrioventricular valve (tricuspid) replacement, complete atrioventricular block, and incidence of reoperation. Alternative management should be examined.     

Double disposition of the major vessels from the right ventricle of the Fallot tetralogy type

Forty-six patients with double disposition of the major vessels from the right ventricle in combination with stenosis of the pulmonary artery, in sub-aortal and bi-committed defects of the interventricular septum have undergone biventricular intraventricular correction. The most specific and accurate anatomic characteristics were: 1) position of the major vessels (particularly side-to-side); 2) location of two and a part of the third aortal sinuses above the right ventricle; 3) bilateral cone; 4) absence of aorto-mitral contact; 5) location of valves of aorta and pulmonary artery on the same level. One (2,2%) patient died during surgery due to progressed cardiac insufficiency. One (2,5%) patient died 2 years after radical correction of the defect due to progressive dysfunction of the right ventricle.     

Staged biventricular repair of pulmonary atresia or stenosis with intact ventricular septum

BACKGROUND: Since 1991 we have performed a multistage palliative approach to biventricular repair of pulmonary atresia or critical pulmonary stenosis with intact ventricular septum in infants with a detectable right ventricular infundibulum. METHODS: A total of 25 patients (19 pulmonary atresia and 6 critical pulmonary stenosis) underwent initial palliation consisting of a transarterial pulmonary valvotomy and a polytetrafluoroethylene shunt between the left subclavian artery and pulmonary trunk. Among the 23 survivors, 15 underwent balloon valvotomy. Six of these patients later required additional palliative surgery that consisted of repeat pulmonary valvotomy, adjustment of an atrial communication, and resection of the hypertrophied muscles in the right ventricle. RESULTS: Of the 25 patients, 23 (92%) survived. In all, 20 patients underwent definitive operations: 18 (90%) biventricular repair (12 pulmonary atresia, and 6 critical pulmonary stenosis), one bidirectional Glenn, and one Fontan procedure. The actuarial probability of achieving a biventricular repair at 36 months of age was 69%. In 18 patients right ventricular end-diastolic volume significantly increased but tricuspid valve diameter did not change. CONCLUSIONS: The multistage palliation procedure to promote right ventricular growth makes a definitive biventricular repair of pulmonary atresia or critical pulmonary stenosis with intact ventricular septum possible in the majority of infants with a patent infundibulum.     

Right heart bypass operation of complex cardiac anomalies in 4 patients with viscero-atrial heterotaxic syndrome

Four patients with viscero-atrial heterotaxic syndrome underwent a right heart bypass operation. 2 patients had right isomerism, and two had left isomerism. 3 patients had common atrio-ventricular canal, three had double outlet right ventricle, one had mitral valve hypoplasia, and one had double inlet right ventricle. All patients had anomalous systemic or pulmonary venous drainage. Redirection of systemic venous drainage was accomplished by total cavo-pulmonary shunt (TCPS) (2 patients), atrial baffle (1 patient), and intraatrial conduit (1 patient). In one patient underwent TCPS, regurgitant common atrioventricular valve was replaced with a prosthetic valve. One who had total anomalous pulmonary venous drainage (type Ib) died after cardiac repair. The three survivors are in improved condition. The right heart bypass operation, like total cavopulmonary shunt or connection, simplifies the redirection of systemic venous drainage and offers improved results in the surgical treatment of complex cardiovascular anomalies with viscero-atrial heterotaxic syndrome.     

Biventricular conversion for Fontan failure

Certain cardiac malformations may pose difficulties in achieving a biventricular repair, and univentricular repair may be selected for them if the Fontan criteria are satisfied. However, univentricular palliation is not without its own drawbacks. We present a patient with double outlet of the right ventricle with pulmonic stenosis where the Fontan operation was conducted but where there was early Fontan failure in the short term and she needed conversion to a biventricular physiology.     

One-stage total repair of aortic arch anomaly using regional perfusion.

OBJECTIVE: Primary repair of aortic arch obstructions and associated cardiac anomalies is a surgical challenge in neonates and infants. Deep hypothermic circulatory arrest prolongs myocardial ischemia and might induce cerebral and myocardial dysfunction. METHODS: From March 2000 to December 2005, 69 neonates or infants with aortic arch anomaly underwent one-stage biventricular repair with continuous cerebral perfusion in the presence of a nonworking beating heart using the dual perfusion technique on the innominate artery and aortic root. Preoperative diagnoses of arch anomaly comprised aortic coarctation (n=54) or an interrupted aortic arch (n=15). Combined anomalies were ventricular septal defect (n=52), anomalous origin of the right pulmonary artery from ascending aorta (n=3), hypoplastic left heart syndrome (n=2), truncus arteriosus (n=2), atrioventricular septal defect (n=2), double outlet right ventricle (n=1), total anomalous pulmonary venous return (n=1), partial anomalous pulmonary venous return (n=1), and aortic stenosis (n=1). RESULTS: The mean regional perfusion time was 27.8+/-9.8 min. There was no operative mortality. Postoperative low cardiac output was present in four patients (5.8%). A neurologic complication was noted in one patient (1.5%) who developed transient chorea, but recovered completely. During 32.8+/-17.5 months of follow-up, one late death (1.5%) occurred. There was neither reoperation associated with arch anomaly nor recoarctation except in one patient. One patient developed left main bronchial compression necessitating aortopexy. CONCLUSIONS: One-stage total arch repair using our regional perfusion technique is an excellent method that may minimize neurologic and myocardial complications without mortality. Our surgical strategy for arch anomaly has a low rate of residual and recurrent coarctation when performed in neonates and infants.