Online ISSN: 2187-2988 Print ISSN: 0911-1794
特定非営利活動法人日本小児循環器学会 Japanese Society of Pediatric Cardiology and Cardiac Surgery
Pediatric Cardiology and Cardiac Surgery 37(1): 51-56 (2021)

症例報告Case Report

短腸症候群に合併したカルニチン欠乏性心筋症Secondary Cardiomyopathy Caused by Carnitine Deficiency with Short Bowel Syndrome

兵庫県立こども病院 循環器内科Department of Cardiology, Kobe Children’s Hospital ◇ Hyogo, Japan

受付日:2020年7月23日Received: July 23, 2020
受理日:2020年9月18日Accepted: September 18, 2020
発行日:2021年4月1日Published: April 1, 2021

特発性心筋症の診断には,全身疾患と関連がある二次性心筋症の除外が必要である.栄養素欠乏はその一つである.今回我々は,短腸症候群に合併したカルニチン欠乏性心筋症を経験した.6歳女児が入院5日前から感冒症状があり,起坐呼吸を認めたため入院となった.短腸症候群のため経静脈栄養を行っていた.腸管からの慢性出血による貧血を認めていた.入院時胸部レントゲンでは心胸郭比60%,心エコーで左室内径短縮率17%であった.Hb 6.7 g/dLと貧血を認め赤血球輸血を行い,ミルリノンを投与するも心不全症状は改善しなかった.既往からL-カルニチンを補充し,3日で心収縮は改善した.治療後に遊離カルニチン17.8 µmol/Lと判明し,カルニチン欠乏性心筋症と診断した.カルニチン欠乏は治療可能な二次性心筋症の鑑別の一つとして重要であり,リスクを有する児では定期的な血中濃度測定が望まれる.貧血や感染など心負荷増大時には注意を要する.心機能改善には早期のL-カルニチン補充が重要である.

Secondary cardiomyopathy related to a systemic disorder should be ruled out when diagnosing idiopathic cardiomyopathy. Nutrient deficiency is a known cause of secondary cardiomyopathy. Herein, we report a case of secondary cardiomyopathy caused by carnitine deficiency with short bowel syndrome. A 6-year-old girl receiving total parenteral nutrition for short bowel syndrome manifested cold symptoms for 5 days that resulted in orthopnea, thereby prompting hospitalization. Chest radiography showed a cardiothoracic ratio of 60%, and echocardiography revealed fraction shortening of 17%. Her operated bowel was bleeding, causing chronic anemia, obtaining a hemoglobin level of 6.7 g/dL. Cardiac function was not improved by blood transfusion and milrinone therapy but improved in merely 3 days by L-carnitine administration. The free carnitine level was found to be low after treatment (17.8 µmol/L); therefore, she was diagnosed with secondary cardiomyopathy caused by carnitine deficiency. In conclusion, carnitine deficiency can cause secondary cardiomyopathy; hence, the serum carnitine level of patients at risk should be examined periodically. Furthermore, the risk of cardiomyopathy must be considered in patients with increased cardiac load, including anemia and infection. Thus, early administration of L-carnitine is necessary for secondary cardiomyopathy caused by carnitine deficiency.

Key words: secondary cardiomyopathy; carnitine deficiency; short bowel syndrome

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