Ersin EREK, Selim AYDIN, Muazzez ÇEVİK, Dilek SUZAN, Ender ÖDEMİŞ

Pediatrik bir olguda tek ventriküllü kalp ve Cantrell sendromu

Cantrell sendromu, çeşitli derecelerde orta hat duvar defektleri ve doğuştan kardiyak anomalilerin eşlik ettiği nadir bir sendromdur. Bu yazıda, tek ventrikül, pulmoner darlık, ventrikül divertikülü, iki taraflı superior vena kava, sternum alt uç ve supraumbilikal batın ön duvar defekti nedeniyle Cantrell sendromu tanısı konan altı yaşında bir kız olgu sunuldu. Ciddi siyanozu olmasına rağmen, ailenin kararı nedeniyle daha önce girişim yapılmayan hasta ameliyata alındı. Çocuk cerrahisi iş birliği ile iki taraflı Glenn ameliyatı, ventrikül divertikül rezeksiyonu ve batın ön duvar tamiri uygulandı. Hasta %80-85 SpO2 ile sorunsuz taburcu edildi. Ameliyat sonrası altıncı ay kontrolünde herhangi bir sorun saptanmadı

Univentricular heart and Cantrell syndrome in a pediatric case

Cantrell syndrome is a rare syndrome associated with varying degrees of midline wall defects and congenital cardiac anomalies. In this article, we report a six-year-old girl with Cantrell syndrome associated with single ventricle, pulmonary stenosis, ventricular diverticulum, bilateral superior vena cava, lower sternal, and supra-umbilical abdominal anterior wall defect. The patient, who had no previous intervention despite severe cyanosis (SpO2: 65 to 70%) due to the parent decision, underwent operation. Bilateral Glenn procedure, resection of the ventricular diverticulum, and anterior abdominal wall repair were performed in collaboration with pediatric surgery. The patient was discharged uneventfully with a SpO2 of 80 to 85%. No problem was observed in the postoperative sixth month visit

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