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The outcome of surgical repair of anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) in infants
The Cardiothoracic Surgeon ( IF 0.5 ) Pub Date : 2019-07-15 , DOI: 10.1186/s43057-019-0003-y
Leena Moshref , Rana Moshref , Mazen Faden , Osman Al-Radi

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital malformation in infants presenting with angina, dyspnea, and excessive perspiration invoked by crying and feeding. The aim of this study was to evaluate the factors affecting morbidity and the use of extracorporeal membrane oxygenation (ECMO) and to assess the quality of life post-repair. In this retrospective study, information about five infants who underwent ALCAPA repair was collected from the databases of two tertiary referral cardiac centers (King Abdulaziz University Hospital and King Faisal Specialist Hospital & Research Center, Jeddah, Saudi Arabia) from 2011 to 2018. The patients were diagnosed using echocardiography, and data including mitral insufficiency and ejection fraction were assessed preoperatively and postoperatively. Quality of life was assessed using a questionnaire-based interview. The median (range) age at the time of repair was 95 (34–144) days, and the median weight was 4.9 (3–5.7) kg. Two patients underwent the Takeuchi procedure, and three patients underwent left coronary artery reimplantation and translocation to the aorta. The median preoperative ejection fraction was 25 (12.5–45)%, and at the last follow-up, the median EF of the three survivors was 59 (50–70)%. There was no significant change in mitral insufficiency grade postoperatively. Two patients had ECMO support and died postoperatively. The infants who died were younger (< 75 days) and had lower weight (< 4.5 kg) at the time of intervention. Patients who survived showed good quality of life were asymptomatic and had heart function within the normal range. Echocardiography and CT angiogram are effective tools for ALCAPA diagnosis in pediatric patients. Low weight at time or repair was associated with increased morbidity and ECMO did not increase survival.

中文翻译:

婴儿左冠状动脉异常起源于肺动脉的手术修复结果

左冠状动脉异常起源于肺动脉(ALCAPA)是罕见的先天性畸形,在婴儿中出现心绞痛,呼吸困难和因哭泣和进食而引起的出汗过多。这项研究的目的是评估影响发病率和使用体外膜氧合(ECMO)的因素,并评估修复后的生活质量。在这项回顾性研究中,从2011年至2018年从两个三级转诊心脏中心(阿卜杜勒阿齐兹国王大学医院和沙特阿拉伯吉达的费萨尔国王专科医院和研究中心)的数据库中收集了有关五名接受ALCAPA修复的婴儿的信息。经超声心动图检查诊断,并在术前和术后评估包括二尖瓣关闭不全和射血分数在内的数据。使用基于问卷的访谈评估生活质量。修复时的中位年龄为95(34–144)天,中位体重为4.9(3–5.7)kg。两名患者接受了Takeuchi手术,三名患者接受了左冠状动脉再植入并转移至主动脉。术前射血分数中位数为25(12.5-45)%,在最后一次随访中,三名幸存者的中位射血分数为59(50-70)%。术后二尖瓣关闭不全等级无明显变化。两名患者在ECMO支持下死于术后。干预时死亡的婴儿年龄较小(<75天),体重较轻(<4.5 kg)。存活的患者表现出良好的生活质量,无症状且心功能在正常范围内。超声心动图和CT血管造影是诊断小儿患者ALCAPA的有效工具。体重减轻或修复时间短与发病率增加有关,ECMO并未增加生存率。
更新日期:2019-07-15
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