Süt Çocuğunda Kalp Yetersizliği ile Başvuran Dilate Kardiyomiyopatinin Düzeltilebilir Bir Nedeni: ALCAPA Sendromu

Yıl 2017, Cilt: 15 Sayı: 1, 47 - 50, 01.04.2017

Osman Güvenç , Murat Saygı Erkut Öztürk Alper Güzeltaş

https://doi.org/10.4274/jcp.03371

Öz

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

Anahtar Kelimeler

ALCAPA sendromu, dilate kardiyomiyopati, ekokardiyografi

Correctable Cause of Dilated Cardiomyopathy in an Infant with Heart Failure: ALCAPA Syndrome

Öz

Anomalous origin of the left coronary artery arising from pulmonary artery ALCAPA syndrome is a rare congenital heart disease seen in children. If untreated, it may lead to congestive heart failure, dilated cardiomyopathy DCM , ischemic and arrhythmic complications may lead to patient’s death. ALCAPA is diagnosed with echocardiography; in the patients of suspected diagnosis, computerized tomography, magnetic resonance imaging and cardiac catheterization are used for further testing. Surgically correctable ALCAPA syndrome must be considered as etiology of DCM in children. In this report, we presented the case of an infant that was referred to our center with the diagnosis of DCM, who was echocardiographically diagnosed with ALCAPA syndrome and successfully treated with surgery, as well as a review of recent literature

Anahtar Kelimeler

ALCAPA syndrome, dilated cardiomyopathy, echocardiography

Kaynakça

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