Lee Andrew C, Foster Elyse, Yeghiazarians Yerem
Division of Cardiology, University of California, San Francisco, CA 94143, USA.
Congenit Heart Dis. 2006 May;1(3):111-5. doi: 10.1111/j.1747-0803.2006.00017.x.
Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital cardiovascular defect that occurs in approximately 1/300 000 live births or 0.5% of children with congenital heart disease. The mortality of untreated ALCAPA has been estimated to range from 35% to greater than 85% in the first year of life. However, in some cases patients can survive past infancy and into adulthood and do not present with symptoms until later in life. These older patients often manifest their anomalies as congestive heart failure, malignant arrhythmias, or even sudden death. We report a series of 3 cases from our institution illustrating the various late presentations of this unusual diagnosis. We review the pathophysiology of this rare congenital anomaly and discuss some of the signs, symptoms, and diagnostic tests that can help diagnose this unusual condition in adults.
左冠状动脉起源于肺动脉(ALCAPA)是一种罕见的先天性心血管缺陷,发生率约为每30万活产儿中有1例,或占先天性心脏病患儿的0.5%。未经治疗的ALCAPA在出生后第一年的死亡率估计在35%至85%以上。然而,在某些情况下,患者可以存活至婴儿期以后并进入成年期,直到晚年才出现症状。这些老年患者常表现为充血性心力衰竭、恶性心律失常,甚至猝死。我们报告了本院的3例病例,展示了这种罕见诊断的各种晚期表现。我们回顾了这种罕见先天性异常的病理生理学,并讨论了一些有助于诊断成人这种罕见疾病的体征、症状和诊断检查。