Dilek SUZAN, Selim AYDIN, Barış KIRAT, İbrahim Halil DEMİR, Ender ÖDEMİŞ, Ersin EREK

Kompleks Kalp Anomalisi ile Birlikte Protein C Eksikliği Bulunan Çocukta Aşamalı Tam Düzeltme Operasyonu

Kalp cerrahisi uygulanacak, koagulopati tanısı olan hastalar, yüksek oranda kanama ve tromboz riski ile karşı karşıya kalabilirler. Bu çalışmada kompleks kalp anomalisi ile birlikte Protein C eksikliği tanısı bulunan ve 4 yaşında, aşamalı Rastelli operasyonu uyguladığımız hastamız sunulmaktadır. Protein C eksikliği tanısı, hastamız 1.5 yaşında iken modifiye-BT şant uygulanmasının ardından erken dönem şant trombozu oluşumu nedeniyle yapılan tetkikler sonucunda konulmuştu. Şant ameliyatı sonrasında 2.5 yıl antikoagülan ve antiagregan tedaviyle şantı açık olarak izlenmiş ve 4 yaşında başarılı Rastelli operasyonu uygulanmıştır. Olgumuzu paylaşmaktaki amacımız kalp cerrahisi sonrası tromboembolik olaylara yol açabilen predispozan faktörlerin önemine dikkat çekmektir

Staged Total Correction In a Child With Complex Heart Anomaly Associated With Protein C Deficiency

Patients with pre-existing coagulopathies who undergo cardiac surgery are at increased risk for bleeding and thrombosis. In this study we report a staged Rastelli operation in a 4 year old child with complex congenital cardiac anomaly associated with protein C deficiency which was diagnosed due to early shunt thrombosis after modified BT shunt operation, when the patient was 1.5 years old. During 2.5 years follow-up shunt was open under anticoagulan and antiaggregant treatment. When the patient was 4 years old, Rasteli operation was performed successfully Our aim by sharing this case is to emphasize the importance of predispozing risk factors in patients with thromboembolic events after cardiac surgery

Keywords:

Protein C deficiency, cardiopulmonary bypass, thrombosis, complex congenital heart defects,

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