Physicians' Academy for Cardiovascular Education

20-year prognosis of hypertrophic cardiomyopathy: low mortality but high morbidity

Very long-term prognosis in patients with hypertrophic cardiomyopathy: a longitudinal study with a period of 20 years

Literature - Sugiura K, Kubo T, Ochi Y, et al. - ESC Heart Fail. 2022 Jun 2, doi: 10.1002/ehf2.13983

Introduction and methods

Background

Hypertrophic cardiomyopathy (HCM) is a slowly progressive disorder in which clinical features can develop throughout life [1,2]. Recent studies have suggested that the prognosis has improved and HCM is generally associated with mild disability and normal life expectancy, if sudden death can be prevented [3-6].However, the follow-up period of most studies on clinical outcomes of HCM was less than 10 years [7,8].

Aim of the study

The authors aimed to determine the clinical course of HCM over a follow-up period of approximately 20 years.

Methods

In this retrospective, single-center, longitudinal study, complete and detailed clinical records of 93 consecutive patients were analyzed who had been diagnosed with HCM at the Kochi Medical School Hospital in Kochi, Japan by 31 December 2000.

Based on morphologic and hemodynamic assessments by echocardiography, patients were divided into 5 groups:

1. hypertrophic obstructive cardiomyopathy (HOCM), defined as presence of basal LV outflow tract obstruction (gradient: >30 mmHg);

2. mid-ventricular obstruction (MVO), defined as presence of systolic LV cavity obliteration at mid ventricle, creating MVO with peak systolic gradient >30 mmHg;

3. end-stage HCM, defined as LV systolic dysfunction of global EF <50%;

4. apical HCM, defined as hypertrophy confined to LV apex below papillary muscle level;

5. hypertrophic non-obstructive cardiomyopathy (HNCM) (i.e., non-obstructive HCM other than end-stage HCM or apical HCM).

Outcomes

The occurrence of HCM-related death and HCM-related adverse events were evaluated.

Main results

Baseline patient characteristics

Hypertrophic cardiomyopathy-related deaths

Hypertrophic cardiomyopathy-related adverse events

Atrial fibrillation and left ventricular remodeling

Conclusion

In this community-based cohort of Japanese patients, annual HCM-related mortality was relatively low (1.1%). However, about half of the patients suffered from one or more HCM-related adverse events during the 20-year follow-up period. As in some patients the first HCM-related adverse event occurred in the third decade since the initial evaluation, the authors believe their results indicate HCM patients should be carefully followed up at all ages.

References

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Find this article online at ESC Heart Fail.

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