冠状动脉异常起源于肺动脉的外科治疗

目的总结冠状动脉异常起源于肺动脉的外科治疗经验及疗效随访分析。方法从1999年4月至2005年8月共对12例冠状动脉异常起源于肺动脉的患者进行了外科治疗,其中左冠状动脉起源异常8例,右冠状动脉起源异常4例。异常起源的冠状动脉直接吻合到主动脉9例,应用Takeuchi技术,即肺动脉内隧道重建冠状动脉3例。有7例左冠状动脉起源异常的患者,因中、重度二尖瓣关闭不全同期行二尖瓣成形;1例合并房间隔缺损的右冠状动脉起源异常的患者,同期在非体外循环下行房间隔缺损封堵术。结果无死亡及术后并发症,随访7~83个月,平均(32±24)个月,左冠状动脉起源异常的患者,左室舒张末径从术前的(45±7)mm减小至术后的(39±6)mm(P=0.011),左室短轴缩短率从术前的0.21±0.09增加至术后的0.35±0.06(P=0.006),7例二尖瓣成形的患者术后二尖瓣反流程度均减轻;所有患者活动能力正常,均无症状。结论外科治疗冠状动脉异常起源于肺动脉,需恢复2个冠状动脉系统供血;左冠状动脉起源异常的患者,术后左室功能明显改善,对中、重度二尖瓣关闭不全的患者,需同期行二尖瓣成形;外科治疗早、中期效果良好,远期结果需进一步随访。     

儿童冠状动脉异常起源于肺总动脉的外科治疗

目的　总结小儿冠状动脉异常起源于肺总动脉进行重建双冠状动脉系统的外科治疗经验。方法　 4例中 3例施行了改良肺动脉内隧道加人造主 肺动脉窗术 ,另 1例行冠状动脉移植术。其中 1例术中同时行房间隔缺损修补术和二尖瓣成形术。手术时平均年龄为 10岁。结果　平均体外循环 91min ,主动脉阻断 5 6min。无住院和远期并发症死亡病例。随访 1～ 3年 ,冠状动脉血流通畅 ,心功能正常 [射血分数 (EF) (6 6± 8) % ,心室缩短分数 (FS) (40± 5 ) % ,NYHAI级 ]。结论　尽早根据冠状动脉异常起源于肺总动脉不同解剖类型 ,采用不同方法重建双冠状动脉系统矫治术的治疗效果佳 ;适宜的心肌保护是手术成败关键之一。     

成人左冠状动脉起源于肺动脉外科治疗

目的探索成人左冠状动脉起源于肺动脉的外科治疗效果。方法 1991年11月至2017年11月,手术治疗36例年龄18岁左冠状动脉起源于肺动脉患者,其中男9例、女27例,年龄(36.6±13.3)岁,体重(60.0±9.4)kg。术前超声心动图显示左心室射血分数(LVEF)为57%±6%,左心室舒张期末内径(LVEDD)为(52.3±6.3)mm;二尖瓣反流(MR)重度1例,中度5例。其中行冠状动脉再植17例,肺动脉内隧道修补术(Takeuchi术)16例,异常起源左冠状动脉结扎+冠状动脉旁路移植术3例,同期行二尖瓣成形术6例。结果体外循环时间为(152.5±72.9)min,主动脉阻断时间(101.9±43.6)min,无院内死亡,呼吸机辅助时间(17.3±16.3)h,ICU滞留时间(43.1±30.7)h,术后LVEF为59%±6%,较术前无明显改善(P=0.10),术后LVEDD为(46.9±5.9)mm较术前显著缩小(P=0.02),6例二尖瓣成形患者中,1例重度变为轻度,5例中度变为微量。所有患者顺利出院。随访35例,平均5.5年,最长26年,随访期间无死亡,2例行肺动脉内隧道修补术患者发生肺动脉内隧道瘘,分别成功行介入封堵术和外科修补术;所有患者心功能分级(NYHA)Ⅰ或Ⅱ级;最后一次随访LVEF为69%±7%,较术前显著改善,LVEDD为(48.7±5.9)mm;MR中度2例,轻度10例。结论成人左冠状动脉起源于肺动脉外科治疗近期和远期效果满意,肺动脉内隧道修补术远期可能出现内隧道瘘需要再次干预。     

40岁以上继发孔型房间隔缺损手术治疗经验

目的　总结 40岁以上继发孔型房间隔缺损 (房缺 )手术治疗的经验。方法　 46例 40岁以上继发孔型房间隔缺损病人 ,年龄 40～ 6 2岁 ,平均 (45 7± 4 4)岁。合并三尖瓣关闭不全 35例 ,其中中度以上关闭不全 2 2例 ;合并二尖瓣关闭不全 10例 ;房颤 6例 ,房扑 2例 ;术前肺动脉测压 2 7例 ,肺动脉平均压为 (35 7± 16 7)mmHg,肺动脉平均压最高为 6 3mmHg。房缺修补采用直接连续缝合和心包片或涤纶补片修补 ,同期行三尖瓣成形术 2 2例 ,其中 10例行Kay二叶瓣成形术 ,12例行DeVega成形术 ;二尖瓣成形术 4例 ,行二尖瓣置换术 2例 ,3例合并有房颤病人同期行右房迷宫术。结果　 46例病人术后全部生存 ,心功能均有明显改善。结论　年龄因素不是房缺手术的禁忌证 ,合并症的同期手术处理是手术预后的关键     

左心室收缩功能低下冠心病病人冠状动脉旁路移植术的中远期效果

目的　总结并分析左心室收缩功能低下冠心病病人行冠状动脉旁路移植手术(CABG)的中、远期效果。方法　34例左心室射血分数(LVEF)低于0 30且不伴左心室室壁瘤的冠状动脉粥样硬化心脏病病人行CABG ,平均年龄(5 8 0±9 4 )岁。冠状动脉造影显示LVEF为0 15～0 30 ,平均0 2 7±0 0 4 ,其中2支病变3例,3支病变31例(包括左主干病变4例)。超声心动显示左心室舒张末直径(LVDD)平均为(6 1 5±8 9)mm ,LVEF平均0 2 8±0 0 7。心功能分级平均为2 9±0 7。体外循环下手术2 6例,非体外循环常温手术(OPCAB) 8例。每例旁路移植2～6支,平均(3 9±0 9)支。随访率94 1% ,随访时间平均(3 5±1 9)年。结果　无手术死亡。早期主要并发症为心功能不全。所有病人心绞痛症状明显减轻,左心室舒张末直径平均(5 5 2±7 1)mm ,LVEF平均0 4 7±0 11。心功能分级平均1 9±0 3。以上指标与术前进行统计学比较,差异均具统计学意义。随访3年生存率为91 9% ,5年生存率为85 7%。5年免除心绞痛为81 3% ,心功能分级为1～3级,平均1 4±0 6。结论　伴左心室收缩功能低下的CABG病人的中、远期疗效满意,充分的术前准备是手术成功关键。     

改良迷宫手术治疗二尖瓣病变伴慢性心房颤动的疗效

迷宫手术〔1〕的技术复杂、心脏停搏时间较长 ,为此 ,一些学者对其进行了改进〔2 -5〕。 1998年 3月我们也采用改良迷宫手术 (ｍｏｄｉｆｉｅｄｍａｚｅｐｒｏｃｅｄｕｒｅ)治疗二尖瓣病变伴慢性心房颤动 (房颤 )的病人 ,现报道如下。资料和方法　 2 2例病人中男 5例 ,女 17例 ;平均 (39 7±10 9)岁。房颤平均持续 (8 8± 6 7)年。其中二尖瓣狭窄 10例 ,二尖瓣关闭不全 8例 ,二尖瓣狭窄伴关闭不全 4例。左房径 (6 0 8± 16 7)ｍｍ ,射血分数 0 5 3± 0 12 ,缩短分数 0 2 8±0 0 7。常规建立体外循环 ,冷晶体心停跳液保护心肌 ,血流…     

风湿性瓣膜病二尖瓣与主动脉瓣置换术1154例长期效果分析

目的　评价风湿性联合瓣膜病二尖瓣与主动脉瓣双瓣置换术的近期与远期疗效 ,分析影响手术疗效的因素。　方法　回顾性分析 1981年 5月～ 2 0 0 1年 5月 2 0年间 ,115 4例风湿性心脏病患者行双瓣膜置换术的临床资料和长期随访结果 ,其中二尖瓣与主动脉瓣均为狭窄病变者 2 5 3例 ,二尖瓣狭窄合并主动脉瓣关闭不全者 345例 ,二尖瓣关闭不全合并主动脉瓣狭窄者 119例 ,二尖瓣与主动脉瓣均为关闭不全者 437例 ;合并三尖瓣病变的占 5 4 0 0 %( 75 7例 ) ,其中器质性病变 7 2 7%( 84例 ) ,功能性关闭不全 5 8 31%( 6 73例 ) ;合并中度以上肺动脉高压者 339例 ;术前NYHA心功能分级Ⅲ级与Ⅳ级者分别为 873例和 186例。应用侧倾碟瓣或双叶机械瓣施行瓣膜置换术 ,合并三尖瓣功能或器质性病变者 ,同期行瓣膜成形手术。　结果　本组患者术后住院病死率为 6 5 0 %( 75 / 115 4)。早期死亡的主要原因为低心排出量综合征、顽固性心律失常、肾功能或呼吸功能衰竭 ,以及抗凝有关的出血等。长期生存 10 79例 ,随访时间为 8个月～ 2 0年 ,平均随访时间为 4 5 %病人·年。晚期死亡 6 6例 ( 0 39%病人·年 ) ;5、10与 15年累计生存率分别为 ( 89 46± 1 35 ) %、( 86 5 0± 1 91) %与 ( 6 7 86±6 16 ) %。生存的 92 9例患     

前降支弥漫病变27例的外科处理

20 0 1年我们完成的冠状动脉旁路移植术 (ＣＡＢＧ) 2 5 1例中前降支弥漫病变 2 7例 ,现总结处理方法和经验如下。临床资料　 2 7例中男 16例 ,女 11例。平均年龄 ( 6 5 3±12 6 )岁 ,占同期ＣＡＢＧ术的 10 8%。其中合并糖尿病 16例 ,高血压 10例 ,前壁心肌梗死 6例 ,前壁心尖部室壁瘤形成 2例 ,合并二尖瓣关闭不全 2例。术前射血分数 (ＥＦ)平均0 5 6± 0 2 2。术前冠状动脉造影显示前降支管径明显细小 ,多处狭窄、钙化者共 2 1例。手术方法　全组均在全麻体外循环下进行 ,平均搭桥( 3 8± 3 2 )支 ,行室壁瘤切除 2例 ,二尖瓣成形 2…     

电视胸腔镜辅助心内直视手术初探

20 0 1年 9月始我们将电视胸腔镜技术与体外循环直视心内手术结合应用于临床 ,现将初步结果总结如下。临床资料　本组共 2 0例 ,男 5例 ,女 15例 ;年龄 13～ 4 5岁 ,平均 (2 4 7± 9 7)岁。其中先天性心脏病房间隔缺损 (房缺 )继发孔型 17例 (其中 1例合并重度三尖瓣关闭不全 ) ,室间隔缺损 (室缺 ) 2例 ,冠状动脉 -肺动脉瘘 1例。术前二维超声心动图 (2D UCG)测得平均室缺直径 (1 0 0± 0 0 5 )cm ,房缺直径 (2 36± 0 6 3)cm。冠状动脉 -肺动脉瘘经造影确诊。同期常规手术 10例 ,作为对照。手术采用全麻、双腔气管插管、左肺…     

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。     

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.     

Experience with an alternative technique for the management of anomalous left coronary artery from the pulmonary artery

BACKGROUND: Several operative approaches are utilized for the management of anomalous origin of the left coronary artery from the pulmonary artery, each with some limitation. The long-term results of a technique that facilitates direct and tension-free implantation of the anomalous artery to the aorta in all patients are described. METHODS: From January 1, 1992 through August 30, 2000, 10 consecutive patients with anomalous left coronary artery underwent operation using this technique. It consists of isolating an anterior and posterior transverse segment of pulmonary artery in continuity with the origin of the anomalous coronary artery. The two segments are folded with the orifice of the coronary as its fulcrum, and the edges sutured together to form an extension tube of pulmonary artery tissue. This lengthens the coronary artery and allows direct aortic implantation (posterior to the pulmonary artery) without tension. The pulmonary artery is reconstructed with autologous pericardium. RESULTS: Patient age ranged from 3 weeks to 3 years old (median 8 weeks), with 80% of patients less than 11 weeks old. Median weight was 4.6 kg (3.7 to 23 kg). The left ventricle was dilated with an end-diastolic diameter z-value of +1 to +3, and the shortening fraction was markedly reduced to 16% +/- 6% (7% to 28%), with 8 of 10 patients having a shortening fraction less than 20%. Mitral regurgitation was severe in 5 patients, moderate in 2 patients, and all patients were in congestive heart failure. After repair there were no hospital deaths. Inotropic support was needed in all patients, but none required mechanical assistance. At a follow-up of 4.3 +/- 2.5 years (0.5 to 8.5 years), 9 patients are asymptomatic and 1 patient has intermittent chest pain. All patients (10/10) have echocardiographic documented patency of the reimplanted coronary artery, as well as marked improvement in the left ventricular shortening fraction (37% +/- 5%; p > 0.05 versus preoperative) and decrease in the end-diastolic diameter z-value (-1 to +1; p > 0.05 versus preoperative). Mitral regurgitation was absent in 4 patients, mild in 4 patients, and moderate in 2 patients. severe in 1 patient. Four patients have evidence of mild supravalvar pulmonary stenosis (15 to 32 mm Hg), 1992. CONCLUSIONS: This technique allows a tension-free direct aortic connection in all cases, has a low rate of coronary artery occlusion, and avoids significant pulmonary artery distortion or stenosis, making it an excellent alternative for the surgical management of anomalous origin of the coronary artery.     

Anomalous origin of left coronary artery from pulmonary artery. Ligation versus establishment of a two coronary artery system

Between 1959 and 1985, 24 patients (mean age 38 months, range 15 days to 13 years) with anomalous origin of the left coronary artery from the pulmonary artery as an isolated lesion were treated surgically at Children's Hospital, Boston. In 11 cases a left coronary-to-aortic tunnel was created with a pulmonary artery baffle (Takeuchi) with no deaths either early or late over a mean follow-up period of 18 1/2 months. Late complications of this procedure include moderate aortic regurgitation (one), supravalvular pulmonary stenosis (two-one required a second operation), obstructed baffle (one-asymptomatic). In 11 cases of coronary ligation or ostial closure there was a 27% early mortality and a 25% late mortality over a mean follow-up period of 10 1/2 years. Late complications include residual shunt (three-two required a second operation), severe mitral regurgitation (one), and recurrence of angina (one). Two patients had other procedures. Both early and late deaths occurred in the group who had congestive heart failure and who had simple ligation. Five infants who had profoundly depressed ventricular function and moderate to severe mitral regurgitation, together with widespread Q waves on electrocardiogram, showed a dramatic improvement in ventricular function after the Takeuchi procedure. The Takeuchi procedure is a simple and effective means of establishing a two coronary artery system in the child with anomalous origin of the left coronary artery from the pulmonary artery. This procedure is particularly recommended over coronary ligation in patients in congestive heart failure.     

Anomalous coronary artery origin from the pulmonary artery: correlation between surgical timing and left ventricular function recovery

BACKGROUND: This study investigates the correlation between surgical timing and 15-year longitudinal left ventricular and mitral valve function, after repair of anomalous coronary artery origin from the pulmonary artery. METHODS: Between 1987 and 2002, 31 patients (median age, 7.1 months) underwent repair for anomalous origin of the left (n = 28), right (n = 2), or both (n = 1) coronary arteries from the pulmonary artery. Repair was accomplished by subclavian interposition in 5 patients, intrapulmonary tunnel in 12, and direct aortic reimplantation in 14. Primary mitral valve repair was never associated with coronary revascularization. Total follow-up was 186.4 patient-years (mean, 77.2 months). RESULTS: Fifteen-year actuarial survival was 92.9% +/- 4.9% for coronary transfer, 40.0% +/- 21.9% for subclavian interposition, and 89.9% +/- 7.5% for intrapulmonary tunnel (p = 0.019). Five patients required further intervention for supravalvular pulmonary stenosis (n = 3), baffle leak (n = 1), and mitral valve replacement (n = 1). Coronary transfer allowed best freedom from long-term reoperation (92.3% +/- 7.4%). Left ventricular shortening fraction increased from 17.3% +/- 6.3% before operation to 34.1% +/- 4.6% at last follow-up (p < 0.01). Regression analysis demonstrated a linear relationship between age at repair and shortening fraction recovery (r(2) = 0.573, p < 0.01). Patients younger than 6 months of age showed worse preoperative shortening fraction (15.9% +/- 5.2%) and best longitudinal shortening fraction recovery (36.4% +/- 5.1%; p < 0.001). Major improvement in mitral valve function was observed within 1 year from surgery in 90.4% of survivors. CONCLUSIONS: Repair of anomalous coronary artery origin from the pulmonary artery in younger symptomatic infants offers the best potential for recovery of left ventricular function, despite a worse initial presentation. Coronary transfer is associated with superior long-term survival and freedom from reoperation. Most patients with patent two-coronary repair will recover normal mitral valve function; therefore, simultaneous mitral valve surgery seems unwarranted.     

Anomalous origin of the left coronary artery from the pulmonary artery: results of surgical correction in five infants

BACKGROUND: Five infants operated on for anomalous origin of the left coronary artery from the pulmonary artery were retrospectively analyzed. The mean age at operation was 12 +/- 6.7 weeks (95% confidence interval, 3.5 to 20 weeks), and mean weight at operation was 4.43 +/- 0.68 kg (95% confidence interval, 3.7 to 5.27 kg). All babies presented in infancy with left ventricular failure. Three had evidence of ischemia with left ventricular strain, and two had Q waves in anterolateral leads on electrocardiograph. Cross-sectional echocardiography showed dilated left ventricles with poor contractility in all babies with fractional shortening of 15.8% +/- 4.02% (95% confidence interval, 12% to 20%); moderate mitral regurgitation was seen in all babies. METHODS: All babies underwent operation as soon as the diagnosis was made. Four babies had direct reimplantation of left coronary artery into the aorta, and 1 had tunnel repair. Intraaortic balloon counterpulsation was used in 1 baby for hemodynamic instability and as prophylaxis in the remaining 4 babies postoperatively for 115 +/- 26.2 hours (95% confidence interval, 72 to 144 hours). RESULTS: All babies had delayed closure of the chest. There was no operative mortality. One baby was reoperated on for tunnel stenosis as well as pulmonary stenosis 4 months after primary repair. All babies were followed for 192 patient-months and show an improved fractional shortening. CONCLUSIONS: Early operation, early institution of intraaortic balloon counter pulsation for left ventricular support, and delayed sternal closure are the key to good results.     

Anatomic repair of anomalous left coronary artery from the pulmonary artery by aortic reimplantation: early survival,patterns of ventricular recovery and late outcome

BACKGROUND: To determine the early and late outcomes of patients presenting with anomalous left coronary artery from the pulmonary artery who had repair by aortic reimplantation. METHODS: From January 1952 to July 2000, 67 patients presented with anomalous coronary artery from the pulmonary artery. Forty-seven patients who had repairs performed by aortic reimplantation are the subject of this study. The median age at repair was 7.7 months. Before repair, 10 infants (21%) presented in extremis requiring ventilatory and inotropic support, and 38 infants (80%) presented in heart failure. Autologous pericardial hood coronary arterioplasty was used in 4 patients, and concomitant mitral valve repair was used in 1 patient. RESULTS: Hospital survival was 92%. Five children required postoperative extracorporeal membrane oxygenation for a median of 4 days (range, 2 to 8 days). Patients who had extracorporeal membrane oxygenation were significantly more likely to have presented in critical condition (40% vs 3% if no extracorporeal membrane oxygenation; p = 0.006) or with ventricular arrhythmias (67% vs 7%; p = 0.027), to have presented with significantly lower preoperative repair median ejection fraction (10%, n = 5 vs 40%, n = 38; p = 0.01) or to have presented with more severe left ventricular dilatation (p = 0.03). Within a 15-year or less follow-up (mean, 4.7 years) there were no late deaths. Kaplan-Meier survival was 91% at 5 years, and freedom from reoperation was 93% at 10 years. At late follow-up, echocardiography demonstrated significant improvements in mean ejection fraction (64% +/- 9% vs 33% +/- 21% preoperatively, p < 0.0001); moderate mitral regurgitation (9% vs 38% preoperatively, p < 0.02); and wall motion abnormalities (15% vs 81% preoperatively, p < 0.002). The ratio of measured left ventricular end-diastolic dimension to the 95th percentile of normal declined from 1.4 +/- 0.3 to 1.0 +/- 0.1 (p < 0.0006). Children who had extracorporeal membrane oxygenation had normal ejection fractions and ventricular dimensions at follow-up (n = 3). Repeated measures of mixed linear regression analysis demonstrated that normalization of ejection fraction and left ventricular function occurred within 1 year of repair. Improvements in mitral regurgitation lagged behind normalization of ejection fraction and left ventricular dilatation. CONCLUSIONS: Anatomic repair of anomalous left coronary artery from the pulmonary artery by aortic reimplantation yields excellent early survival and late functional outcomes even in critically ill infants.     

Surgical intervention for anomalous origin of the left coronary artery from the pulmonary artery: the Tokyo experience

BACKGROUND: Few studies after surgical repair of the anomalous origin of the left coronary artery have reported the importance of the mitral annuloplasty or the long-term results. METHODS: Between January 1982 and March 2000, 29 patients with anomalous origin underwent surgical intervention at our institution (direct aortic reimplantation in 19 and Takeuchi procedure in 10). Age at the time of operation ranged from 2 months to 24 years (median, 29.3 months), and 9 patients were infants. Twenty-four patients had varying degrees of mitral incompetence. Simultaneous mitral annuloplasty at the anterolateral commissure was performed in all 24 patients with incompetence. RESULTS: There were 2 hospital deaths among the infants, and no late deaths. Mean follow-up was 100 +/- 57 months, and the actuarial survival was 93.1% at 10 years (70% confidence limits, 87-99). Cardiothoracic ratio at discharge was not decreasing significantly (P =.35); however, this value 5 years after the operation showed the significant decrease (P =.003) versus preoperative value. Preoperative mitral incompetence decreased in all but one of the operative survivors with mitral annuloplasty at the last follow-up. The left ventricular fractional shortening z-score was not normalized at discharge but was normalized in the late period. CONCLUSION: These data demonstrate that impaired left ventricular function normalized in the long term (even if it was below normal immediately after operation) after 2-coronary repair. We recommend that the simultaneous mitral annuloplasty should be performed at the time of operation for patients who have mitral incompetence with anomalous origin of the left coronary artery.     

Primary repair minimizing the use of conduits in neonates and infants with tetralogy or double-outlet right ventricle and anomalous coronary arteries

OBJECTIVE: The purpose of this study was to review our results with an approach of early primary repair for tetralogy of Fallot or double-outlet right ventricle with anomalous coronary arteries, using several techniques to minimize the use of a conduit. METHODS: Twenty consecutive neonates and infants with anomalous coronary arteries crossing an obstructed right ventricular outflow tract underwent primary repair. Median age was 5.5 months and mean weight 6.22 kg. The anomalous coronary arteries included the left anterior descending from the right coronary artery (n = 10), the right coronary artery from the left anterior descending (n = 1), the left anterior descending from the right sinus (n = 1), and a significant conal branch from the right coronary artery (n = 7) or left anterior descending (n = 1). Two neonates had pulmonary atresia. The right ventricular outflow tract was reconstructed without a conduit in 18 patients, including those with pulmonary atresia. Surgical techniques included main pulmonary artery translocation in 4 patients, transannular repair under a mobilized left anterior descending coronary artery in 2 patients, and displaced ventriculotomy with subcoronary suture lines in 8 patients. In 4 patients the right ventricular outflow tract was repaired via the ventriculotomy and/or pulmonary arteriotomy. A homograft was used as the sole right ventricle-pulmonary artery connection in 1 patient and in another a homograft was added to a hypoplastic native pathway. RESULTS: There have been no early or late deaths. The right ventricular/left ventricular pressure ratio within 48 hours of the operation was 0.47 +/- 0.10. There were 2 reoperations at 8 and 11 years after the operation, during a mean follow-up of 5.2 years (1-11.3 years). CONCLUSIONS: Primary repair of tetralogy of Fallot or double-outlet right ventricle with anomalous coronary arteries can be done in neonates and infants with excellent results. Alternative surgical techniques for right ventricular outflow tract reconstruction, such as main pulmonary artery translocation, can avoid the use of a conduit in most patients.     

Anomalous left coronary artery from the pulmonary artery: results of isolated aortic reimplantation.

Thirty-one consecutive children with anomalous left coronary artery underwent direct aortic reimplantation of the anomalous artery without an associated procedure. There were five deaths (16%; 70% confidence limits, 9% to 26%), three in the hospital and two early (within 3 months). The severity of preoperative left ventricular dysfunction was the only incremental risk factor for mortality: 31% mortality rate among patients with left ventricular shortening fraction of less than 0.20 versus 0% among patients with a left ventricular shortening fraction of 0.20 or more (p = 0.03). There were no late deaths up to 6 years, a survival rate of 84% +/- 7%. Late results were studied in 23 survivors having a follow-up of longer than 12 months. Ninety-six percent were free of symptoms; left ventricular function recovered to normal in all patients; moderate to severe mitral regurgitation decreased to minimal or no regurgitation in most patients (5/7); and the reimplanted anomalous left coronary artery was patent in each patient. Based on this study, we reached five conclusions. (1) Direct aortic reimplantation is technically feasible in most patients with anomalous left coronary artery and yields a high rate of late patency. (2) Left ventricular resection is unnecessary. (3) The mitral valve should not be interfered with at the initial operation, but mitral regurgitation may persist in a few patients and necessitate later operation. (4) In patients with moderate left ventricular dysfunction, the operative risk is low and early operation indicated. (5) In patients with severe left ventricular dysfunction, the operative risk is high; heart transplantation may be suggested, but our current approach favors an immediate corrective procedure.