成人冠状动脉异常起源于肺动脉的临床分析

The clinical analysis to anomalous origin of the coronary artery from the pulmonary artery in adult

目的：探讨成人冠状动脉异常起源于肺动脉的临床特点、诊断方法、治疗及意义.方法：北京安贞医院自2004年1月至2012年12月,共收治12例成人冠状动脉起源异常患者,对其临床资料、治疗情况及随访结果进行回顾性分析.结果：全组男性2例,女性10例,年龄16～66岁,平均年龄为（39.2±17.9）岁.其中10例为左冠状动脉起源于肺动脉,2例为右冠状动脉起源于肺动脉.患者临床表现缺乏特异性,在确诊前均误诊为其他疾病.全组均经冠状动脉造影,心脏超声心动图（UCG）,冠状动脉CTA等检查而确定诊断.其中9例患者行外科手术重建冠状动脉,2例患者拒绝手术,1例因严重心功能不全而失去手术时机.手术患者中2例患者因合并重度二尖瓣反流而同时行二尖瓣置换术,1例患者因合并冠心病同时行冠状动脉旁路移植术.无围手术期死亡病例及术后并发症.随访6 ～48个月,平均随访（27±13）个月,患者术后均症状消失或明显缓解,一般体力活动不受限.UCG提示：术后左心室舒张末径从[（47.4±7.2） mm减小到（41.8±6.5）mm,P＜0.01],左心室的短轴缩短率（FS）从[（24.3±11.3）％增至（36.1±11.6）％,P＜0.01）].未手术治疗患者1例死于心力衰竭.1例失访,1例仍时有活动后气短、心慌等症状.结论：成人冠状动脉异常起源于肺动脉无特征性临床表现,除了冠状动脉造影,UCG和冠状动脉CT等无创检查对确定诊断具有重要意义.手术治疗重建双冠状动脉系统是目前首选的治疗方案,可以改善心脏血供,从而防止猝死、心功能不全等严重心脏事件的潜在危险.

Objective：Anomalous origin of the coronary artery from the pulmonary artery （ACAPA） is a rare but serious congenital heart disease. We aimed to illustrate the clinical characteristics, diagnostic strategies, treatment outcomes of the adult ACAPA. Methods： Twelve adult ACAPA patients were admitted in Bei- jing Anzhen hospital from January 2004 to December 2012. The clinical findings and follow-up data of them （two males and ten females） were retrospectively analyzed. Results： The age ranged from 16 to 66 year old with an average of（39. 2 ± 17.9） years old. Ten of them suffered from anomalous origin of the left coronary ar tery from the pulmonary artery （ALCAPA）, and the other two suffered from anomalous origin of the right coro nary artery from the pulmonary artery （ARCAPA）. Due to the lack of specific clinical symptoms and its low incidence, they were all originally misdiagnosed. The diagnoses of ACAPA were confirmed through a serial of examinations including coronary angiography, echocardiography cardiogram and multi-slice spiral CT scan. Nine patients underwent surgical reconstruction of double-ostion eorrary system, while two ultrasound other patients refused operation and another patient lost the chance of operation because of severe heart failure. Two of the surgically treated patients with severe mitral regurgitation underwent additional mitral valve replacement. Another patient complieated with coronary heart disease underwent coronary artery bvlaass surgery. All surgically trea-ted patients had good early results. With a follow-up of six to forty-eight months, averagely （27 ± 13 ）months, the symptoms of the surgically treated patients disappeared or significantly alleviated without physical exercises restriction. According to the echocardiography eralclotion, the left ventricular end-diastolic diameter of the nine surgically treated patients reduced from（47. 4 ±7. 2）to（41.8 ± 6. 5）ram （P 〈0. 01 ）, and the left ventricu lar fractional shortening increased from （24. 3±11.3 ） % to （36. 1±11.6） % （ P 〈 0. 01 ）. One of three patients without operation died of cardiac insufficiency, one was lost to follow-up, and another still had such symptoms as shortness of breath, and palpitation during exercises. Conclusion ： The diagnosis of adult ACAPA is challenging due to the lack of specific symptoms. In addition to coronary angiography, echocardiography and multi-slice spiral CT scan are preferred as non-invasive diagnostic approaches of ACAPA. The re-establish of the double-ostium coronarv system bv surzical procedure may decrease the potential cardiac events.