Ayşe İnci YILDIRIM, Babürhan ÖZBEK, Kenan ÖZTÜRKER, Ergin ARSLANOĞLU, Ömer Faruk ŞAVLUK, Deniz ÇEVİRME, Eylem TUNÇER, Nihat ÇİNE, Hakan CEYRAN

Holt-Oram Sendromunda Triküspid Kapak Tamiri

Holt-Oram sendromu kardiyak defektler ve üst ekstremite iskelet anormallikleri ile karakterize otozomal dominant geçişli kalıtsal bir hastalıktır. İlk olarak 1960 yılında tariflenen bu sendromun sıklığı 100.000 doğumda 1 olarak bildirilmektedir. Burada, kalp-el sendromu olarak da adlandırılan Holt-Oram sendromuna sahip 13 yaşındaki kız olgu sunulmuştur. Beraberinde triküspid kapak yetmezliği de mevcut olan hastaya konjenital kardiyak defekt tamiri yanında triküspid kapak tamiri de yapılmıştır. Hasta başarılı bir kardiyak cerrahi düzeltme geçirmiştir.

Tricuspid Valve Repair in Holt-Oram Syndrome

Holt-Oram syndrome is a disease with autosomal dominant inheritance characterized with cardiac defects and upper extremity skeletal anomalies. The prevalence of this syndrome is reported as 1 in 100.000 births. A 13-year-old female patient with Holt-Oram syndrome also called as Heart and Hand syndrome was presented. Tricuspid valve repairment was made in addition to the congenital cardiac defect repairment in the patient who also had tricuspid valve deficiency. The patient underwent a successful surgery.

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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