Key result
Anti-HMGCR myopathy can present with acute systolic heart failure, as demonstrated by a 72-year-old woman who developed severe cardiomyopathy and died from multiorgan failure despite immunotherapy.
Why the study?
Cardiac muscle involvement is considered very uncommon in anti-HMGCR myopathy, leading to early cardiac evaluation not being prioritized.
Case Report (n=1)
No
Heart failure in anti-HMGCR myopathy may not be as rare as previously thought, suggesting early cardiac evaluation should be considered in these patients.
Cardiac involvement may occur in anti-HMGCR myopathy; leaves open whether routine screening alters outcomes.
Involvement of cardiac muscle is felt to be very uncommon in anti-HMGCR myopathy, and therefore early cardiac evaluation is not considered a high priority for this condition. We herein present the case of a 72 year-old woman admitted due to dyspnea and orthopnea, who, in retrospect, suffered from proximal more than distal muscle weakness for 3 months prior to admission. She was found to have acute systolic heart failure. Serologic testing showed positive 3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) IgG antibodies, and muscle biopsy showed necrotizing myopathy. No alternative explanation for heart failure was found. Despite immunotherapy and symptomatic treatment, she died from multiorgan failure. Our study suggests that heart failure in anti HMGCR myopathy may not be as rare as previously thought, and therefore early cardiac evaluation should be considered in patients with this diagnosis, to minimize morbidity and mortality.
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Ghannam et al. (2020) conducted a case report in Anti-HMGCR myopathy with acute systolic heart failure (n=1). Anti-HMGCR myopathy was evaluated. Anti-HMGCR myopathy can present with acute systolic heart failure, as demonstrated by a 72-year-old woman who developed severe cardiomyopathy and died from multiorgan failure despite immunotherapy.
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