A Case of Cardiogenic Shock due to Propionic Acidemia-Associated Cardiomyopathy Successfully Treated with a Combination of Mechanical Circulatory Support and Medical Therapy
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- Ishikawa Yusuke
- Department of Cardiovascular Medicine, Graduate School of Medical Sciences, Kyushu University
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- Fujino Takeo
- Department of Cardiovascular Medicine, Graduate School of Medical Sciences, Kyushu University Department of Advanced Cardiopulmonary Failure, Graduate School of Medical Sciences, Kyushu University
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- Hashimoto Toru
- Department of Cardiovascular Medicine, Graduate School of Medical Sciences, Kyushu University
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- Shinohara Keisuke
- Department of Cardiovascular Medicine, Graduate School of Medical Sciences, Kyushu University
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- Matsushima Shouji
- Department of Cardiovascular Medicine, Graduate School of Medical Sciences, Kyushu University
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- Fuke Yoshifumi
- Department of Cardiovascular Surgery, Graduate School of Medical Sciences, Kyushu University
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- Ushijima Tomoki
- Department of Cardiovascular Surgery, Graduate School of Medical Sciences, Kyushu University
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- Sonoda Hiromichi
- Department of Cardiovascular Surgery, Graduate School of Medical Sciences, Kyushu University
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- Nakashima Yu
- Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University
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- Mushimoto Yuichi
- Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University
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- Ishii Kanako
- Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University
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- Ide Tomomi
- Department of Cardiovascular Medicine, Graduate School of Medical Sciences, Kyushu University
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- Tsutsui Hiroyuki
- Department of Cardiovascular Medicine, Graduate School of Medical Sciences, Kyushu University School of Medicine and Graduate School, International University of Health and Welfare
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- Kinugawa Shintaro
- Department of Cardiovascular Medicine, Graduate School of Medical Sciences, Kyushu University
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- Shiose Akira
- Department of Cardiovascular Surgery, Graduate School of Medical Sciences, Kyushu University
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- Abe Kohtaro
- Department of Cardiovascular Medicine, Graduate School of Medical Sciences, Kyushu University
Description
<p>An 18-year-old man experienced refractory cardiogenic shock due to dilated cardiomyopathy of unknown aetiology, and was referred to our hospital to undergo a paracorporeal left ventricular assist device (LVAD) implantation. After surgery, the patient lost consciousness, with subsequent head computed tomography revealing low-density areas in the bilateral basal ganglia. Metabolic acidosis and hyperammonaemia also appeared. Metabolic evaluation and genetic tests led to a diagnosis of propionic acidemia. Following appropriate management of heart failure and propionic acidemia, his neurological and cardiac functions gradually recovered, and the paracorporeal LVAD was successfully explanted. Dilated cardiomyopathy and heart failure are rare manifestations of adult-onset propionic acidemia. This is the first reported case of cardiogenic shock due to propionic acidemia-associated cardiomyopathy successfully treated with a combination of mechanical circulatory support and medical therapy for heart failure and propionic acidemia.</p>
Journal
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- International Heart Journal
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International Heart Journal 65 (6), 1172-1176, 2024-11-30
International Heart Journal Association