Noonan Sendromlu Bir Hastada Nadir Olarak Saptanan Kardiyak Anomalilerin Tedavisi

Year 2015, Volume: 18 Issue: 1, 45 - 46, 06.04.2015

Mustafa Yılmaz Şafak Alpat İlker Ertuğrul Tevfik Karagöz Metin Demircin

Abstract

Noonan sendromu farklı kardiyak
anomalilerin de eşlik ettiği nadir görülen bir hastalıktır. Sunulan olguda
Noonan sendromlu bir hastada kardiyak anomalilerin daha önce bildirilmemiş bir
kombinasyonu bildirilmiştir. Eşlik eden kardiyak anomaliler atriyal septal
defekt, atriyal fibrilasyon ve ventrikül miyokardında noncompaction idi. Atriyal
septal defekt cerrahi olarak fenestre biçimde kapatılırken maze prosedürü
uygulandı. Sendromlu hastalarda saptanan konjenital kalp defektlerinin cerrahi
tedavisinde sendromu olmayan hastalara göre farklı güçlükler ile
karşılaşılmaktadır. Bu nedenle sendromlu vakalarda özellikli bir yaklaşım
ihtiyacı vardır.

Keywords

Noonan sendromu, atriyal fibrilasyon, atriyal septal defekt, noncompaction, hasta sinüs sendromu

Management of Rare Combination of Cardiac Anomalies in A Patient with Noonan Syndrome

Abstract

Noonan syndrome is a rare
disorder in which different cardiac abnormalities can exist. This report
describes the combination of cardiac abnormalities in a patient with Noonan
syndrome, which has not been reported previously. Coexisting cardiac
abnormalities were: atrial septal defect, atrial fibrillation and noncompaction
of the ventricular myocardium. Surgical fenestrated closure of atrial septal
defect and concomitant maze procedure were performed. Surgery of congenital
heart defects in syndromic patients has different difficulties than those in
nonsyndromic patients. Thus, specific approach should be taken in these
patients.

Keywords

Noonan syndrome, atrial fibrillation, atrial septal defect, noncompaction, sick sinus syndrome

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