Anomalous origin of the left coronary artery from the pulmonary artery syndrome in an adult and his eight-year follow-up results

Abstract

Bland-White-Garland sendromu olarak da bilinen sol koroner arterin pulmoner arterden cikis anomalisi (ALCAPA), cok nadir gorulen bir dogustan anomalidir. Tedavi edilmedigi takdirde, ALCAPA sendromu cogunlukla miyokard iskemisi ve kalp yetmezligi nedeniyle yasamin ilk yilinda yuksek mortalite ile seyreder. Eriskin hastalarin bircogu asemptomatik olabilir ya da bu hastalarda anjina, nefes darligi, bayginlik, miyokard enfarktusu, aritmi veya ani kardiyak olum gorulebilir. Malign ventrikuler aritmilere sekonder ani kardiyak olum, bu hasta grubunda en sik gorulen durumdur. Bu yazida, aktif bir sporcu olan ve atipik yakinmalari olan 36 yasinda bir erkek olgu sunuldu. Transtorasik ekokardiyografide pulmoner arterde tanimlanamayan turbulans tespit edilmesi uzerine yapilan koroner anjiyografide ALCAPA sendromu saptandi. Anah tar soz cuk ler: Eriskin; koroner anjiyografi; koroner arter anomalisi. Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) which is also known as Bland-White-Garland syndrome, is a rare congenital anomaly. If it is left untreated, ALCAPA syndrome has a high mortality in the first year of life due to mostly myocardial ischemia and heart failure. Most adult patients may be asymptomatic or present with angina, dyspnea, syncope, myocardial infarction, arrhythmia or sudden cardiac death. Sudden cardiac death secondary to malign ventricular arrhythmias is the most common presentation in this patient population. In this article, we report a 36-yearold male case who was an active sportsman and presented with atypical complaints. Transthoracic echocardiography revealed an indefinite turbulence in pulmonary artery and coronary angiography showed ALCAPA syndrome.