Öz
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
Anahtar Kelimeler
Protein C deficiency cardiopulmonary bypass thrombosis complex congenital heart defects
Kaynakça
- Ranucci M, Ballotta A, Frigiola A, Boncilli A, Brozzi S, Costa E, Mehta RH. Pre-operative homocysteine levels and morbidity and mortality following cardiac surgery. Eur Heart J 2009;30:995–1004. https://doi. org/10.1093/eurheartj/ehp015
- Dahlbäck B. Advances in understanding pathogenic mechanisms ofthrombophilic disorders. Blood 2008;112:19–27. https://doi. org/10.1182/blood-2008-01-077909
- Marlar RA, Mastovich S. Hereditary protein C deficiency: a review of the genetics, clinical presentation, diagnosis and treatment. Blood Coagul Fibrinolysis 1990;1:319–30.
- Simsic JM, Uber WE, Lazarchick J, Bradley SM. Systemic-to-pulmonary artery shunt thrombosis in a neonate with factor V Leiden mutation. Ann Thorac Surg 2002;74:2179–81.
- Saileela R, Shanthi C, Agarwal R, Subramanyan R, Cherian K. Factor V Leiden mutation: An added risk in single ventricle palliation. Ann Pediatr Cardiol 2012;5:205–6. https://doi. org/10.4103/0974-2069.99630
- Alioglu B, Avci Z, Tokel K, Atac FB, Ozbek N. Thrombosis in children with cardiac pathology: analysis of acquired and inherited risk factors. Blood Coagul Fibrinolysis 2008;19:294–304. https://doi. org/10.1097/MBC.0b013e3282fe73b1
Kompleks Kalp Anomalisi ile Birlikte Protein C Eksikliği Bulunan Çocukta Aşamalı Tam Düzeltme Operasyonu
Öz
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
Anahtar Kelimeler
Protein C eksikliği kardiyopulmoner bypass tromboz kompleks konjenital kalp anomalisi
Kaynakça
- Ranucci M, Ballotta A, Frigiola A, Boncilli A, Brozzi S, Costa E, Mehta RH. Pre-operative homocysteine levels and morbidity and mortality following cardiac surgery. Eur Heart J 2009;30:995–1004. https://doi. org/10.1093/eurheartj/ehp015
- Dahlbäck B. Advances in understanding pathogenic mechanisms ofthrombophilic disorders. Blood 2008;112:19–27. https://doi. org/10.1182/blood-2008-01-077909
- Marlar RA, Mastovich S. Hereditary protein C deficiency: a review of the genetics, clinical presentation, diagnosis and treatment. Blood Coagul Fibrinolysis 1990;1:319–30.
- Simsic JM, Uber WE, Lazarchick J, Bradley SM. Systemic-to-pulmonary artery shunt thrombosis in a neonate with factor V Leiden mutation. Ann Thorac Surg 2002;74:2179–81.
- Saileela R, Shanthi C, Agarwal R, Subramanyan R, Cherian K. Factor V Leiden mutation: An added risk in single ventricle palliation. Ann Pediatr Cardiol 2012;5:205–6. https://doi. org/10.4103/0974-2069.99630
- Alioglu B, Avci Z, Tokel K, Atac FB, Ozbek N. Thrombosis in children with cardiac pathology: analysis of acquired and inherited risk factors. Blood Coagul Fibrinolysis 2008;19:294–304. https://doi. org/10.1097/MBC.0b013e3282fe73b1
Ayrıntılar
| Birincil Dil | Türkçe |
|---|---|
| Bölüm | Case Report |
| Yazarlar | |
| Yayımlanma Tarihi | 1 Mart 2018 |
| Yayımlandığı Sayı | Yıl 2018Sayı: 1 |