GKD Anest Yoð Bak Dern Derg. 2017; 23(4): 153-156 | DOI: 10.5222/GKDAD.2017.153

Aortic Valve Replacement in a Patient with β-Thalassemia

Ali Sait Kavaklý¹, Nilgün Kavrut Öztürk¹, Raif Umut Ayoðlu², Muzaffer Yýlmaz², Kadir Saðdýç², Ömer Haldun Tekinalp², Mustafa Emmiler³, Kerem Ýnanoðlu^1
1University of Health Sciences Antalya Training and Research Hospital, Department of Anesthesiology and Reanimation, Antalya, Turkey
²University of Health Sciences Antalya Training and Research Hospital, Department of Cardiovascular Surgery, Antalya, Turkey
³Lara Anatolian Hospital, Department of Cardiovascular Surgery, Antalya, Turkey

Heart valve diseases can be seen as a consequence of elastic tissue defect in β-thalassemia patients. The possibility of pulmonary complications and rapid thrombosis of the prosthetic valve after valve replacement should be considered in these patients. In this case report, we aimed to present our experience of aortic valve replacement in a patient with β-thalassemia.
Transthoracic echocardiography of a 27-year-old male patient followed-up with ß-thalassemia major revealed severe aortic stenosis and regurgitation, left ventricular dysfunction (EF: 40%), left ventricular dilatation and maximum systolic pressure gradient of 80 mmHg across the aortic valve. Pulmonary artery pressure was 55 mmHg. According to his medical history, he had undergone splenectomy at age 17. Aortic valve replacement was scheduled for the patient and bileaflet mechanical valve replacement was performed. A total of 3 units of erythrocyte suspension were transfused within 24 hours postoperatively. Postoperative transthoracic echocardiography showed normal aortic valve function and a maximum systolic gradient of 15 mmHg. The patient was started on coumadin with a target INR of 3.0 to 3.5. He was discharged on the 5th postoperative day.
Close follow-up after valve replacement is required, considering the increased thrombosis tendency in patients with β-thalassemia. Appropriate anticoagulant therapy should be planned by evaluating the predisposing factors in patients.

Keywords: β, -thalassemia, aortic valve replacement, pulmonary hypertension, anticoagulant therapy

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β-talasemili Hastada Aort Kapak Replasmaný

Ali Sait Kavaklý¹, Nilgün Kavrut Öztürk¹, Raif Umut Ayoðlu², Muzaffer Yýlmaz², Kadir Saðdýç², Ömer Haldun Tekinalp², Mustafa Emmiler³, Kerem Ýnanoðlu^1
1Saðlýk Bilimleri Üniversitesi Antalya Eðitim ve Araþtýrma Hastanesi, Anesteziyoloji ve Reanimasyon Kliniði, Antalya
²Saðlýk Bilimleri Üniversitesi Antalya Eðitim ve Araþtýrma Hastanesi, Kalp Damar Cerrahisi Kliniði, Antalya
³Lara Anadolu Hastanesi, Kalp Damar Cerrahisi, Antalya

β-talasemi hastalarýnda elastik doku defektinin bir sonucu olarak kalp kapak hastalýklarý görülebilmektedir. Bu hastalarda kapak replasmaný sonrasý pulmoner komplikasyonlar ve protez kapakta hýzlý tromboz oluþmasý ihtimali gözönünde bulundurulmalýdýr. Bu olgu sunumu ile β-talasemi sebebiyle takip edilen hastada yapýlan aort kapak replasmaný deneyimimizi sunmayý amaçladýk.
β-talasemi major tanýsýyla takip edilen 27 yaþýnda erkek hastanýn ekokardiyografi incelemesinde ciddi aort darlýðý ve yetmezliði, sol ventrikül disfonksiyonu (EF: %40), sol ventrikül dilatasyonu ve aortta 80 mmHg sistolik gradient saptandý. Pulmoner arter basýncý 55 mmHg bulundu. Anamnezinde 10 yýl önce geçirilmiþ splenektomi öyküsü mevcuttu. Hastaya aortik kapak replasmaný planlandý ve bileaflet mekanik kapak replasmaný yapýldý. Hastaya postoperatif 24 saat içinde toplam 3 ünite eritrosit süspansiyonu replase edildi. Kontrol ekokardiyografisinde normal aort kapak fonksiyonlarý tespit edildi ve maksimum sistolik gradient 15 mmHg ölçüldü. INR deðeri 3-3,5 arasýnda olacak þekilde oral kumadin tedavisi baþlandý. Ameliyat sonrasý 5. gününde taburcu edildi.
β-talasemi hastalarýnda artmýþ tromboz eðilimi gözönünde bulundurularak kapak replasmaný sonrasý yakýn takip gerekmektedir. Uygun antikoagulan tedavi hastada mevcut predispozan faktörler deðerlendirilerek planlanmalýdýr.

Anahtar Kelimeler: β, -talasemi, aort kapak replasmaný, pulmoner hipertansiyon, antikoagulan tedavi

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