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Review
. 2020 Nov;115(5):927-935.
doi: 10.36660/abc.20190802.

Hypertrophic Cardiomyopathy: A Review

[Article in English, Portuguese]
Silméia Garcia Zanati Bazan  1 , Gilberto Ornellas de Oliveira  1 , Caroline Ferreira da Silva Mazeto Pupo da Silveira  1 , Fabrício Moreira Reis  1 , Karina Nogueira Dias Secco Malagutte  1 , Lucas Santos Nielsen Tinasi  1 , Rodrigo Bazan  1 , João Carlos Hueb  1 , Katashi Okoshi  1
Affiliations
Review

Hypertrophic Cardiomyopathy: A Review

[Article in English, Portuguese]
Silméia Garcia Zanati Bazan et al. Arq Bras Cardiol. 2020 Nov.

Abstract
in English, Portuguese

Hypertrophic cardiomyopathy (HCM) is the most common heart disease with a genetic origin, and its main characteristic is left ventricular hypertrophy that occurs in the absence of other conditions that trigger this change. HCM may present from asymptomatic forms to manifestations of sudden cardiac death and severe heart failure. Contemporary high-resolution imaging methods and more accurate clinical scores have been used and developed to provide a prognostic assessment and adequate functional assessments, as well as to allow for the stratification of clinical severity. These aspects will be addressed in this review, along with other classic topics inherent to the study of this disease.

A cardiomiopatia hipertrófica (CMH) é a doença cardíaca de origem genética mais comum, cuja principal característica consiste na hipertrofia ventricular esquerda que acontece na ausência de outras patologias que desencadeiam tal alteração. A CMH pode se apresentar desde formas assintomáticas até manifestações de morte cardíaca súbita e de insuficiência cardíaca refratária. Métodos de imagem contemporâneos de alta resolução e escores clínicos mais acurados vêm sendo utilizados e desenvolvidos no sentido de propiciar uma avaliação prognóstica e funcional mais adequada, bem como possibilitar a estratificação dos casos de maior gravidade. Nesta revisão, serão abordados esses aspectos, entre outros tópicos clássicos inerentes ao estudo dessa doença.

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Conflict of interest statement

Potencial conflito de interesses

Declaro não haver conflitos de interesse pertinentes.

Figures

Figura 1
Figura 1. Esquemas de um coração normal (painel esquerdo) e um coração com CMH (painel direito).
Figura 2
Figura 2. Distribuição de mutação dos genes na CMH (Adaptado de Maron BJ et al.8)
Figura 3
Figura 3. Desarranjo de miócitos do tecido miocárdico de um paciente com CMH.
Figura 4
Figura 4. Ecorcardiograma transtorácico revelando hipertrofia assimétrica do septo interventricular. SIV: septo interventricular; VE: ventrículo esquerdo; AE: átrio esquerdo; Ao: raiz aórtica. (Serviço de Ecocardiografia do HC - Faculdade de Medicina de Botucatu - UNESP).
Figura 5
Figura 5. RMC de um paciente com CMH e apresentação septal assimétrica não obstrutiva. AE: átrio esquerdo; AD: átrio direito; SIV: septo intraventricular; VE: ventrículo esquerdo; VD: ventrículo direito; VSVE: via de saída do ventrículo esquerdo (Cortesia do Departamento de Radiologia do HC - Faculdade de Medicina de Botucatu - UNESP).
Figura 6
Figura 6. Fluxograma para determinar a medicação apropriada para os pacientes com CMH.
Figure 1
Figure 1. Schematic of a normal heart (left panel) and a heart with HCM (right panel).
Figure 2
Figure 2. Distribution of gene mutations in HCM (Adapted from Maron BJ et al.8)
Figure 3
Figure 3. Myocyte disarray in the myocardial tissue of a patient with HCM.
Figure 4
Figure 4. Transthoracic echocardiogram showing asymmetrical hypertrophy of the interventricular septum. IVS: interventricular septum; LV: left ventricle; LA: left atrium; AO: aortic root. (Serviço de Ecocardiografia do HC – Faculdade de Medicina de Botucatu – UNESP).
Figure 5
Figure 5. CMR of a patient with HCM and the nonobstructive asymmetric septal presentation. LA: left atrium; RA: right atrium; IVS: interventricular septum; LV: left ventricle; RV: right ventricle; LVOT: left ventricular outflow tract. (Courtesy of the Department of Radiology of the HC – Faculdade de Medicina de Botucatu – UNESP).
Figure 6
Figure 6. Flow chart to determine the appropriate medication for patients with HCM.
See this image and copyright information in PMC

References

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