Clinical scenarios of hypertrophic cardiomyopathy-related mortality: Relevance of age and stage of disease at presentation. (1st March 2023)

• Record Type: Journal Article
• Authors: Zampieri, Mattia; Salvi, Samuele; Fumagalli, Carlo; Argirò, Alessia; Zocchi, Chiara; Del Franco, Annamaria; Iannaccone, Giulia; Giovani, Sara; Ferrantini, Cecilia; Palinkas, Eszter Dalma; Cappelli, Francesco; Olivotto, Iacopo
• Is Part Of: International journal of cardiology. Volume 374(2023)
• Page Start: 65
• Page End: 72
• Publication Date: 2023-03-01
• Subjects: Hypertrophic cardiomyopathy -- Stage -- Heart failure -- Sudden cardiac death -- Mortality -- Age; AF Atrial fibrillation -- CMR Cardiac magnetic resonance -- COPD Chronic obstructive pulmonary disease -- CV Cardiovascular -- EF Ejection fraction -- HCM Hypertrophic cardiomyopathy -- HF Heart failure -- ICD Implantable cardiac defibrillator -- LV Left ventricle -- LVOTO Left ventricular outflow tract obstruction -- SCD Sudden cardiac death -- TIA Transient ischemic attack; Cardiology -- Periodicals; Electronic journals; 616.12
• DOI: 10.1016/j.ijcard.2022.12.056
• Languages: English
• ISSNs: 0167-5273

Abstract

Abstract: The evolving epidemiology of hypertrophic cardiomyopathy (HCM) has progressively changed our perception of HCM-related mortality. However, recent studies detailing individual causes of death based on age and clinical setting are lacking. Thus, the present study aimed to describe the modes of death in a consecutive cohort of HCM patients based on presenting clinical features and stage of disease. Methods: By retrospective analysis of a large HCM cohort, we identified 161 patients with >1 year follow-up who died between 2000 and 2020 and thoroughly investigated their modes of death. HCM stage at presentation was defined as "classic", "adverse remodeling" or "overt dysfunction". Results: Of the 161 patients, 103 (64%) died of HCM-related causes, whereas 58 (36%) died of non-HCM-related causes. Patients who died of HCM-related causes were younger than those who died of non-HCM related causes. The most common cause of death was heart failure (HF). Sudden cardiac death (SCD) ranked third, after non cardiovascular death, and mostly occurred in young individuals. The proportion of HF related death and SCD per stage of disease was 14% and 27% in "classic", 38% and 21% in "adverse remodeling" and 74% and 10% in "overt dysfunction". Conclusions: Most HCM patients die due to complications of their own disease, mainly in the context of HF. While SCD tends to be juvenile, HF related deaths often occur in age groups no longer amenable to cardiac transplant. Modes of death vary with the stage of disease, with SCD becoming less prevalent in more advanced phases, when competitive risk of HF becomes overwhelming. Highlights: Most HCM patients die due to heart failure. Although these deaths are premature, often affect age groups no longer amenable to cardiac transplant. Heart failure-related death mainly involves those patients presenting with "adverse remodeling" or "overt dysfunction". Sudden cardiac death occurs at any age but mostly in younger patients. Sudden cardiac death was progressively less represented with increasing HCM stage severity.