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Korean J Crit Care Med > Volume 28(3); 2013 > Article
Korean Journal of Critical Care Medicine 2013;28(3): 204-209. doi: https://doi.org/10.4266/kjccm.2013.28.3.204
패혈성 쇼크로 중환자실 입실한 성인 환자에서 처음 진단된 다운증후군과 모야모야병이 동반된 아이젠멩거 증후군
이석정ㆍ강혜원ㆍ허정원*ㆍ김서우ㆍ조형원ㆍ류연주ㆍ이진화ㆍ장중현
이화여자대학교 의학전문대학원 내과학교실, *진단검사의학교실
A Case of Patient with Eisenmenger Syndrome Admitted to Intensive Care Unit Due to Septic Shock Complicated with Newlydiagnosed Down Syndrome and Moyamoya Disease
Seok Jeong Lee, Hye Won Kang, Jung Won Huh, Seo Woo Kim, Hyoung Won Cho, Yon Ju Ryu, Jin Hwa Lee, Jung Hyun Chang
1Department of Internal Medicine, Ewha Womans University School of Medicine, Seoul, Korea. jinhwalee@ewha.ac.kr
2Department of Laboratory Medicine, Ewha Womans University School of Medicine, Seoul, Korea.
ABSTRACT
Eisenmenger syndrome is a severe form of pulmonary arterial hypertension related to congenital cardiac defects. Many patients die at a young age from such complications. The treatment of primary pulmonary hypertension is being applied to Eisenmenger syndrome such as endothelin receptor antagonists, phosphodiesterase-5 blockers, and prostacyclin. We experienced a case of 29-year female with ventricular septal defect-related Eisenmenger syndrome complicated with Down syndrome and Moyamoya disease, who was admitted to intensive care unit due to enteritis-associated septic shock. After the combination treatment with iloprost and sildenafil within the intensive care unit, the patient was able to wean mechanical ventilation without further applications of invasive rescue therapy such as extracorporeal membrane oxygenator. She was later discharged with bosentan. She maintained bosentan therapy for 34 months continuously without aggravations of symptom but eventually died with intracranial hemorrhage, a complication of Moyamoya disease. To our knowledge, this is the first case report of Eisenmenger syndrome accompanied by mosaic Down syndrome and Moyamoya disease.
Key Words: bosentan; Down syndrome; Eisenmenger syndrome; iloprost; Moyamoya disease; sildenafil
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