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İstanbul Üniversitesi İstanbul Tıp Fakültesi ¹Pediatri Uzmanı, ²Pediatri Profesörü, ³Kalp ve Damar Cerrahisi Doçenti, ⁴Pediatri Araştırma Görevlisi, ⁵Pediatri Doçenti, ⁶Kalp ve Damar Cerrahisi Profesörü Angiography was planned to confirm the diagnosis in a 13-month-old boy with the presumptive diagnosis of ALCAPA syndrome (anomalous origin of the left coronary artery from the pulmonary artery). In laboratory tests before the procedure, aPTT test was measured extremely long, and severe hemophilia A was diagnosed. With the factor VIII support, coronary angiography was done, and ALCAPA syndrome was confirmed. After the procedure, a thrombosis developed in the femoral artery but it resolved the next day following IV heparin infusion. With the Factor VIII infusion, "Takeuchi operation (intrapulmonary re-roting)" was applied, and Factor VIII was used for the next 23 days after the operation. No complication developed during the postoperative period. In the 11 months since the operation, clinical and echocardiographical findings of the patient improved successfully. The rare association of ALCAPA syndrome and hemophilia A is emphasized. Anahtar Kelimeler : ALCAPA sendromu, hemofili, kardiyomiyopati, kanama diyatezi, koroner, ALCAPA, hemophilia, cardiomyopathy, bleeding diathesis, coronary