HCM is commonly caused by sarcomere mutations, resulting in excess myosin-actin cross bridging and hypercontractility of the heart muscle1,5,6
Obstructive HCM is a chronic, progressive and potentially debilitating disease that can prevent patients from living with
HCM is characterised by LV hypertrophy (≥15 mm) that cannot be explained by another cardiac or systemic disease3,7–9
The estimated global prevalence of HCM is 1 in every 500 people, but the number of diagnosed cases is less than 1 in 3,0003
The majority of HCM cases are obstructive3,9
Obstructive HCM occurs when the blockage caused by the thickened heart muscle substantially reduces blood flow out of the left ventricle, which is referred to as LVOT obstruction.3,9
LVOT obstruction (defined as a peak instantaneous LVOT pressure gradient ⩾30 mmHg) occurs in ~25–30% of patients under resting conditions, but in up to 70% of patients either under resting conditions or with provocation with physiologic manoeuvres (such as Valsalva or exercise).10
Up to 70%
of patients with HCM have LVOT obstruction, either with
provocation or at rest10
Variable disease presentation and non-specific symptoms can make diagnosing obstructive HCM difficult3,11
Patients with obstructive HCM may experience a wide range of debilitating symptoms:3
Fatigue
Chest pain
Breathlessness
Palpitations
Dizziness
Syncope
Obstructive HCM can increase the risk of experiencing serious complications:1,12,13
Heart failure
Atrial fibrillation
Stroke
Sudden cardiac death
(<1% of patients)
- Current pharmacological therapies (such as beta blockers and calcium channel blockers) used for treating obstructive HCM offer a variable degree of symptomatic relief and do not address the underlying cause of the disease5,14
- Septal reduction therapies are complex invasive procedures that may carry important risks to patients and may result in irreversible
poor outcomes5,14
Patients with symptomatic obstructive HCM suffer from reduced quality of life compared with the general population4,15
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HCM, hypertrophic cardiomyopathy; LV, left ventricular; LVOT, left ventricular outflow tract.
References
- Ho CY et al. Circulation. 2018;138(14):1387–1398.
- Olivotto I et al. Circ Heart Fail. 2012;5(4):535–546.
- Zaiser E et al. J Patient Rep Outcomes. 2020;4(1):102.
- Capota R et al. Health Qual Life Outcomes. 2020;18(1):351.
- Olivotto I et al. Lancet. 2020;396(10253):759–769.
- Marian AJ, Braunwald E. Circ Res. 2017;121(7):749–770.
- Arbelo E et al. Eur Heart J. 2023;44(37):3503–3626.
- Pantazis A et al. Echo Res Pract. 2015;2(1):R45–R53.
- Ommen SR et al. Circulation. 2020;142(25):e558–e631.
- Mathai S, Williams L. Ther Adv Chronic Dis. 2022;13:20406223221136074.
- Argulian E et al. Am J Med. 2016;129(2):148–152.
- Maron BJ et al. J Am Coll Cardiol. 2002;39(2):301–307.
- O’Mahony C et al. Circ Arrhythm Electrophysiol. 2013;6(2):443–451.
- Desai N et al. Clin Ther. 2022;44(1):52–66.
- Magnusson P et al. Health Qual Life Outcomes. 2016;14:62.