Süt Çocuğunda Kalp Yetersizliği ile Başvuran Dilate Kardiyomiyopatinin Düzeltilebilir Bir Nedeni: ALCAPA Sendromu
Sol koroner arterin pulmoner arterden çıkış anomalisi olarak tanımlanan ALCAPA sendromu, çocuklarda nadir görülen bir konjenital kalp hastalığıdır. Tedavi edilmediği zaman konjestif kalp yetmezliği, dilate kardiyomiyopati (DKM), iskemik ve aritmik komplikasyonlarla hasta kaybedilebilir. Tanı ekokardiyografi bulgularıyla koyulur, tanıdan şüphelenilen olgularda bilgisayarlı tomografi, manyetik rezonans görüntüleme ve kalp kateterizasyonu gibi ileri tetkiklerden faydalanılabilir. Çocukluk çağında DKM tanısı alan hastalarda etiyolojide, cerrahi olarak düzeltme şansı olan ALCAPA sendromu mutlaka düşünülmelidir. Bu yazıda, merkezimize DKM tanısıyla sevk edilen, ekokardiyografi ile ALCAPA sendromu tanısı konulup, başarılı cerrahi tamir yapılan hasta olgu sunumu yapıldı ve son literatür gözden geçirildi.
Correctable Cause of Dilated Cardiomyopathy in an Infant with Heart Failure: ALCAPA Syndrome
Anomalous origin of the left coronary artery arising from pulmonary arteryALCAPA syndrome is a rare congenital heart disease seen in children. If untreated,it may lead to congestive heart failure, dilated cardiomyopathy (DCM), ischemicand arrhythmic complications may lead to patient’s death. ALCAPA is diagnosedwith echocardiography; in the patients of suspected diagnosis, computerizedtomography, magnetic resonance imaging and cardiac catheterization are used forfurther testing. Surgically correctable ALCAPA syndrome must be considered asetiology of DCM in children. In this report, we presented the case of an infant that wasreferred to our center with the diagnosis of DCM, who was echocardiographicallydiagnosed with ALCAPA syndrome and successfully treated with surgery, as wellas a review of recent literature.
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