Mustafa YILMAZ, Şafak ALPAT, İlker ERTUĞRUL, Tevfik KARAGÖZ, Metin DEMİRCİN

Noonan Sendromlu Bir Hastada Nadir Olarak Saptanan Kardiyak Anomalilerin Tedavisi

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.

Anahtar Kelimeler:

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

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

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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ISSN: 2149-2972
Yayın Aralığı: Yılda 3 Sayı
Başlangıç: 1990
Yayıncı: Sağlık Bilimleri Üniversitesi, Kartal Koşuyolu Yüksek İhtisas Eğitim ve Araştırma Hastanesi