| 婴儿左冠状动脉异常起源于肺动脉的诊疗分析 |
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| 引用本文： | 邱家伟,李方,李轩,尹朝华,闫军,王强,李巅远. 婴儿左冠状动脉异常起源于肺动脉的诊疗分析[J]. 心脏杂志, 2015, 27(6): 669-672 |
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| 作者姓名： | 邱家伟 李方 李轩 尹朝华 闫军 王强 李巅远 |
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| 作者单位： | (中国医学科学院、北京协和医学院国家心血管病中心、阜外医院外科中心,北京 100037） |
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| 摘 要： | 目的 探讨左冠状动脉异常起源于肺动脉（anomalous origin of the left coronary artery from the pulmonary artery,ALCAPA）的临床特点、诊断方法、外科治疗及疗效分析。方法 2006年6月~2015年1月阜外医院诊断为ALCAPA并行手术治疗患儿29例,对这些患儿的临床资料、诊断与治疗情况及随访结果进行回顾性分析。随访结果根据包括复查结果及电话随访。结果 本组患者男性16例,女性13例,年龄2月~1（0.75±0.27）岁,体质量4.8~11.5（8.0±2.2）kg,左心室射血分数18~66(42±18)%。患儿均出现临床心功能不全症状,并通过心电图、心脏彩超及64排螺旋CT检查以明确诊断。并发二尖瓣轻度反流10例,中度反流5例,重度反流4例。手术中将左冠状动脉从肺动脉游离后,直接将左冠状动脉移植到升主动脉15例,用部分肺动脉干和主动脉壁或心包片作左冠状动脉延伸与升主动脉吻合13例,应用肺动脉内通道术（Takeuchi方法）1例。9例并发二尖瓣中度及以上反流者同期作二尖瓣成形术。术毕3例行体外膜肺氧合(extracorporeal membrane oxygenation,ECMO)辅助左心系统。无手术早期死亡。术后随访资料24例（83%,24/29）,随访1~80（22±20）个月,患儿术后症状均明显改善。结论 ALCAPA的患儿应早期诊断,及早手术治疗。冠状动脉直接移植术是目前重建双冠状动脉系统的首选方案, 外科治疗效果良好。
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| 关 键 词： | 婴儿 先天性冠状血管畸形 心脏手术 |
| 收稿时间： | 2015-03-13 |
Infant-type anomalous origin of left coronary artery from pulmonary artery: analysis of diagnosis and surgical treatment |
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| Abstract: | AIM To investigate the clinical features, diagnosis and treatment of anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) in infants. METHODS Initial and final diagnoses and surgery as well as follow-up results were reviewed in 29 ALCAPA patients who underwent surgical repair at Fuwai Hospital from June 2006 to January 2015. RESULTS Among the 29 infants with ALCAPA were 16 male patients and 13 female patients (mean age 0.75 years, range 2 months-1 year). Mean weight was 7.97 kg (range 4.8-11.5 kg) and mean left ventricle ejection fraction (EF) was 41.95% (range 18-66%). All patients had clinical symptoms of severe heart dysfunction. For confirmatory diagnoses, they underwent auxiliary examinations including electrocardiogram, echocardiogram (ECG) and ECG-gated 64-slice computed tomography. Mitral regurgitation was present in 19 patients: mild in ten patients, moderate in five patients and moderate to severe in four patients. Surgical methods were selected based on the locations of the anomalous left coronary artery origin from the pulmonary trunk and the distance between the left coronary artery and the aorta. Major surgical methods included the intrapulmonary tunnel (Takeuchi procedure) in one patient, direct reimplantation of the left coronary artery onto the aorta in 15 patients and reimplantation by pericardiac patch enlarge in 13 patients. Valvuloplasty was performed in nine patients with moderate and severe mitral regurgitation. Extracorporeal membrane oxygenation (ECMO) was used in three patients at the end of surgical repair. No hospital deaths and later deaths occurred. One to 80 months follow-up ［average (22±20) months］ in 24 patients (83%, 24/29) showed markedly alleviated symptoms in all 24 cases. CONCLUSION Early diagnosis and surgical treatment are important for ALCAPA patients. The establishment of a two-coronary system is the first choice of surgical therapy with satisfactory results of surgical repair of ALCAPA. |
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