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HYPERTROPHIC CARDIOMYOPATHY

Hypertrophic cardiomyopathy is a genetically determined cardiac disorder characterized by hypertrophy, often of the left ventricle (LV), with nondilated ventricular chambers and no other cardiac or systemic disease (eg aortic stenosis, thyroid disease or hypertension) capable of producing the extent of hypertrophy manifested by the patient.

Symptoms can range from patients being asymptomatic to mildly or significantly symptomatic with pre-syncopal or syncopal episodes, palpitations, chest pain, decreased exercise tolerance, symptoms of pulmonary congestion (eg fatigue, dyspnea, orthopnea and paroxysmal nocturnal dyspnea) and symptoms of end-stage heart failure.

Approach to treatment is based on clinical experience and observational data as large randomized trials regarding medical therapy in hypertrophic cardiomyopathy are lacking.

An empiric therapy is often necessary to identify the best possible drug for the patient, starting at a low dose and titrating until clinical improvement or occurrence of side effects.

Treat comorbidities such as hypertension, diabetes, obesity and hyperlipidemia according to existing guidelines due to the significant influence of concomitant coronary artery disease on the survival of patients with hypertrophic cardiomyopathy.

Hypertrophic%20cardiomyopathy Signs and Symptoms

Signs and Symptoms

Definition

A genetically determined cardiac disorder characterized by hypertrophy, often of the left ventricle (LV), with nondilated ventricular chambers and no other cardiac or systemic disease (eg aortic stenosis, thyroid disease or hypertension) capable of producing the extent of hypertrophy manifested by the patient
- Maximal LV wall thickness of ≥15 mm in adults or ≥2 standard deviations above the mean for age, sex or body size in children
- Results from mutations in one of the cardiac sarcomere protein genes that encode the parts of the apparatus for contraction: Actin, tropomyosin alpha-1 chain, myosin binding protein C, troponin I, troponin T, beta myosin heavy chain, essential and regulatory light chains

Signs and Symptoms

Symptoms can range from patients being asymptomatic to mildly or significantly symptomatic with pre-syncopal or syncopal episodes, palpitations, chest pain, decreased exercise tolerance, symptoms of pulmonary congestion (eg fatigue, dyspnea, orthopnea and paroxysmal nocturnal dyspnea) and symptoms of end-stage heart failure
Life expectancy is normal or near-normal in most patients with hypertrophic cardiomyopathy (HCM) though without possibility of remission; mortality is often due to embolic stroke, heart failure with sudden cardiac death

Epidemiology

The most common form of genetic cardiovascular disorder occurring in 1 out of 500 individuals regardless of gender or race
- Presents in all age groups
- An autosomal dominant inherited disorder, affected heterozygous individuals beget children with a 50% risk (equal risk for both males and females) of having the gene mutation
- Phenotype is abnormal, frequently focal, hypertrophy that leads to atrial and ventricular arrhythmia, diastolic dysfunction, mitral regurgitation, LV outflow tract obstruction (LVOTO), pulmonary hypertension, myocardial ischemia, coronary insufficiency and congestive heart failure

Dapagliflozin DELIVERs across full HF spectrum regardless of LVEF

Elvira Manzano, 04 Oct 2022

The selective, oral sodium–glucose cotransporter 2 (SGLT2) inhibitor dapagliflozin reduces the risk of cardiovascular (CV) death or worsening heart failure (HF) in patients with HF and mildly reduced or preserved ejection fraction (HFmrEF or HFpEF) in the phase III DELIVER* trial, touted as the largest and most inclusive thus far in this patient group.

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29 Sep 2021

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Empagliflozin not just another SGLT2i: Cardio-renal benefits extend to HF with or without diabetes

Roshini Claire Anthony, 13 Nov 2020

Diabetes is a key risk factor for heart failure (HF), which is the leading cause of hospitalization in patients with or without diabetes. SGLT-2* inhibitors (SGLT-2is) have been shown to reduce the risk of hospitalization for HF (HHF) regardless of the presence or absence of diabetes.