Key result
Triglyceride deposit cardiomyovasculopathy carries ~72% 5-year overall survival in observational cohort.
Cohort (n=183)
Yes
This first survival report on TGCV reveals significant morbidity and mortality, with nearly 30% of patients dying within 5 years of diagnosis.
Triglyceride deposit cardiomyovasculopathy (TGCV) is an emerging, rare cardiovascular disorder (Orphanet ORPHA code: 565612) first reported in 2008 in patients requiring cardiac transplantation. 1 TGCV is caused by defective intracellular lipolysis of triglyceride (TG) leading to heart failure and diffuse narrowing atherosclerosis with TG deposition.Since 2009, the Japan TGCV study group has elucidated its pathophysiology and developed diagnostic methods and specific treatments as a part of a governmental rare disease project.
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Hirano et al. (2023) conducted a cohort in Triglyceride deposit cardiomyovasculopathy (n=183). Triglyceride deposit cardiomyovasculopathy was evaluated on Overall survival at 5 years (95% CI 62.6-79.0). Patients with triglyceride deposit cardiomyovasculopathy had a 5-year overall survival rate of 71.8% and a 5-year cardiovascular event-free survival rate of 54.0%.
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