Key result
Rare phenocopies of hypertrophic cardiomyopathy were associated with a higher prevalence of systolic dysfunction (26% vs 3%; p<0.0001) and increased all-cause mortality compared to idiopathic disease.
Why the study?
Does the presence of rare phenocopies compared to idiopathic or sarcomeric disease increase the prevalence of systolic dysfunction and worsen prognosis in patients with hypertrophic cardiomyopathy?
Observational (n=1,697)
Yes
Does the presence of rare phenocopies compared to idiopathic or sarcomeric disease increase the prevalence of systolic dysfunction and worsen prognosis in patients with hypertrophic cardiomyopathy?
p-value: p=<0.0001
In adults with HCM, LV systolic dysfunction is more frequent in those with rare phenocopies, which serves as a marker for specific aetiologies and is associated with poorer long-term survival.
No takes yet. Share an insight, caveat, or question.
Rare phenocopies warrant consideration in HCM with systolic dysfunction; observational data leave open whether etiology-specific therapies improve survival.
Rosmini et al. (2016) conducted an observational in Hypertrophic cardiomyopathy (n=1,697). Rare phenocopies of HCM vs. Idiopathic or sarcomeric HCM was evaluated on All-cause mortality or heart transplantation (HTx) for end-stage heart failure (HF) (p=<0.0001). Rare phenocopies of hypertrophic cardiomyopathy were associated with a higher prevalence of systolic dysfunction (26% vs 3%; p<0.0001) and increased all-cause mortality compared to idiopathic disease.
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