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Review
. 2020 Mar 25:27:100503.
doi: 10.1016/j.ijcha.2020.100503. eCollection 2020 Apr.

Hypertrophic cardiomyopathy

Murillo de Oliveira Antunes  1   2 , Thiago Luis Scudeler  1
Affiliations
Review

Hypertrophic cardiomyopathy

Murillo de Oliveira Antunes et al. Int J Cardiol Heart Vasc. .

Erratum in

Abstract

Hypertrophic cardiomyopathy (HCM) is the most common inherited cardiac disease. The disease is characterized by marked variability in morphological expression and natural history, ranging from asymptomatic to heart failure or sudden cardiac death. Left ventricular hypertrophy and abnormal ventricular configuration result in dynamic left ventricular outflow obstruction in most patients. The goal of pharmacological therapy in HCM is to alleviate the symptoms, and it includes pharmacotherapies and septal reduction therapies. In this review, we summarize the relevant clinical issues and treatment options of HCM.

Keywords: CMR, cardiac magnetic resonance; HCM, hypertrophic cardiomyopathy; HF, heart failure; ISH, interventricular septal hypertrophy; LVH, left ventricular hypertrophy; LVOT, left ventricular outflow tract; SAM, systolic anterior motion; SCD, sudden cardiac death.

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Figures

Fig. 1
Fig. 1
HCM phenotypes. Diagrams show focal basal septum HCM (A), diffuse septum (B), concentric and diffuse HCM (C), midventricular HCM (D) and apical HCM (E). Adapted from Baxi et al. .
Fig. 2
Fig. 2
(A) ECG showing LVH in a patient with HCM; (B) ECG of a patient with apical cardiac hypertrophy (Yamaguchi syndrome).
Fig. 3
Fig. 3
Two-dimensional echocardiogram. Left ventricular longitudinal long-axis 2-chamber in tele-diastole showing a mid-apical hypertrophy (arrow) and highlighting interventricular septal hypertrophy (ISH = 36 mm).
Fig. 4
Fig. 4
An asymptomatic 36-year-old man with HCM. CMR images demonstrate mild asymmetric septal hypertrophy (A and B) and late gadolinium enhancement (arrows in C) in mid-septal wall and located near the right ventricular insertion points.
Fig. 5
Fig. 5
A 17-year-old male patient with NYHA class II and massive LVH. (A) Electrocardiogram with LVH; (B) Transthoracic echocardiogram parasternal short-axis view shows severe asymmetric septal hypertrophy involving the anterior septum (wall thickness of 65 mm) and posterior LV (wall thickness of 24 mm); (C) M-mode echocardiogram recording of anterior systolic movement (ASM) and mitral leaflet septal contact (arrows); (D) CMR demonstrating the massive LVH.
Fig. 6
Fig. 6
Prognostic pathways and primary treatment strategies within the broad clinical spectrum of HCM. ICD = implantable cardioverter defibrillator; RF = radiofrequency.
Fig. 7
Fig. 7
Comparison between septal reduction therapies.
Fig. 8
Fig. 8
Follow-up of patients with HCM. SCD = sudden cardiac death; AF = atrial fibrillation; HCM = hypertrophic cardiomyopathy.
See this image and copyright information in PMC

Comment in

References

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