Didem ARMAN ÇAKTIR, ELİF ÖZALKAYA, Taner YAVUZ, Hande Özgün KARATEPE, Tuğba GÜRSOY, Hüsnü Fahri OVALI, Güner KARATEKİN

Yenidoğan Döneminde Hidrops Fetalis ve Mobitz Tip 2 ile Prezente Olan Biventriküler Noncompaction Kardiyomyopati Olgusu

Miyokardiyal nonkompaction endokard ve myokardın normal embriyogenezisin duraklaması sonucu oluşan genetik primer kardiyomyopatidir. Otuzbeş haftalık erkek olgunun intrauterin AV blok ve bradikardi tanısı vardı. 1. dakikada APGAR skoru 4tü. Doğum odasında bradikardisi nedeni ile resusite edildi. Geliş EKGsinde mobitz tip 2 av blok saptandı. Çekilen EKOda nonkompaction kardiyomyopati saptandı. İzleminde 02 ihtiyacı gerileyen, inotrop desteği olmayan, aritmisi aralıklı devam eden hasta kardiyoloji poliklini- ğine bağlanarak taburcu edildi. Biz bu olgu sunumunda hidrosp fetalis ve mobitz tip 2 atrioventriküler blok ile prezente olan biventriküler nonkompaction tanısı alan hastayı sunmayı amaçladık.

Myocardial nonkompaction is a genetic primary cardiomyopathy whıch develops as a result of the arrest of normal embroyogenesis of the endocardium and myocardium. A thirty-five-week old male newborn has the intrauterine diagnosis of AV block and bradycardia. The APGAR score was 4 points at 1 minute after birth. He was resuscitated at the delivery room because of bradycardia. The ECG on admission showed mobitz type 2 AV block. An echocardiography showed biventricular noncompaction cardiomyopathy. At the follow-up the patient without O2 need and inotropic support with ongoing intermittent arrhythmia, connected to the cardiology outpatient clinic. In this case report we aimed to present the patient with a dignosis of bi-ventricular noncompaction cardiomyopathy presenting with mobitz type 2 atrioventricular block and hidrosp fetalis.

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