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Published on: November 2, 2020
[Hypertrophic miocardiopathy. An historical and anatomopathological review]
Manlio F Márquez1, Teresita de Jesús Ruíz-Siller1, Rosario Méndez-Ramos1
1Departamento de Electrofisiología, Instituto Nacional de Cardiología Ignacio Chávez, Ciudad de México, México.
Insights
Hypertrophic cardiomyopathy (HCM) is a heart condition causing abnormal left ventricle thickening. This review details its characteristics, symptoms, and sudden cardiac death risks, highlighting Iberoamerican research contributions.
Area of Science:
- Cardiology
- Pathology
Background:
- Hypertrophic cardiomyopathy (HCM) is defined by unexplained left ventricular hypertrophy.
- It can present with or without left ventricular outflow tract obstruction.
- Key concerns include symptom development and sudden cardiac death risk due to arrhythmias.
Purpose of the Study:
- To review the historical contributions of Iberoamerican researchers to understanding HCM.
- To summarize current knowledge on HCM's anatomopathological features.
- To discuss myofiber disarray as a primary histopathologic characteristic.
Main Methods:
- Literature review focusing on Iberoamerican contributions to HCM research.
- Analysis of historical and contemporary anatomopathological findings in HCM.
- Synthesis of information on clinical manifestations and histopathology.
Main Results:
- HCM involves abnormal left ventricular thickening without other causes.
- Outflow tract obstruction and ventricular arrhythmias are significant clinical aspects.
- Myofiber disarray is identified as a key histopathologic feature.
Conclusions:
- HCM is a primary cardiac condition with distinct pathological features.
- Understanding its history and pathology is crucial for diagnosis and management.
- Iberoamerican research has significantly advanced HCM knowledge.
Abstract:
Hypertrophic cardiomyopathy (HCM) is characterized by the presence of an abnormal hypertrophy of the left ventricle (LV), without dilation, and in the absence of any condition or another cardiac or systemic disease capable of inducing such hypertrophy. This primary or idiopathic hypertrophy can occur with or without dynamic obstruction (induced by exercise) of the LV outflow tract, so in its natural history two fundamental aspects are highlighted: the production of symptoms by blocking the LV outflow tract and the occurrence of sudden cardiac death secondary to ventricular arrhythmias. This revision includes the work of different Iberoamerican investigators, who contributed in an important way to lay the groundwork of what we know nowadays as HCM. It also includes the main anatomopathological characteristics, from its initial description to the new perspective we have concerning the myofiber disarray as the main histopathologic feature.
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