Publication
Apical hypertrophic cardiomyopathy : a review
| Summary: | Hypertrophic cardiomyopathy (HCM) is a relatively common, complex and heterogeneous disease that does not seem to have sexual, racial or geographical preference. It is also the leading cause of sudden cardiac death among young people. Although it has a very strong genetic and hereditary component, with more than 1400 different mutations already identified, the reason why different subjects with the same mutation or mutations on the same gene have different HCM phenotypes is still not understood.The present work focuses on one of these phenotypes, classically characterized by asymmetrical apical hypertrophy of the myocardium (AHCM), giant negative T-waves on ECG, ace-of-spade shape on left ventriculography and a better prognosis compared to the usual HCM phenotype (asymmetrical septal hypertrophy). But is this particular phenotype just an apical “variant” of HCM or could it be a different entity with some similar phenotypic manifestations, sharing clinical, diagnostic and genetic features? And is its prognosis still as good as it was when first described? AHCM often represents a dilemma for clinicians and cardiovascular specialists, either due to the absence of randomized trials on the subject, or due to the relative rarity of the disease. Diagnostic criteria, clinical course and treatment management are matters for which there are no assertive answers. Consequently, an up-to-date review with the most recent available information on AHCM was considered necessary.The aim of this work is to review the particular aspects of AHCM and to compare its features, including management and prognosis, with the most common phenotype of HCM. Methods of the Review: Information for this review was retrieved from PUBMED and MEDLINE searches (1976-2014) by using the key-words below through a systematic analysis. |
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| Main Authors: | Cardoso, André Torres |
| Subject: | Hypertrophic cardiomyopathy Apical hypertrophic cardiomyopathy Electrocardiography Echocardiography Prognosis Treatment |
| Year: | 2014 |
| Country: | Portugal |
| Document type: | master thesis |
| Access type: | restricted access |
| Associated institution: | Universidade de Lisboa |
| Language: | English |
| Origin: | Repositório da Universidade de Lisboa |
| Summary: | Hypertrophic cardiomyopathy (HCM) is a relatively common, complex and heterogeneous disease that does not seem to have sexual, racial or geographical preference. It is also the leading cause of sudden cardiac death among young people. Although it has a very strong genetic and hereditary component, with more than 1400 different mutations already identified, the reason why different subjects with the same mutation or mutations on the same gene have different HCM phenotypes is still not understood.The present work focuses on one of these phenotypes, classically characterized by asymmetrical apical hypertrophy of the myocardium (AHCM), giant negative T-waves on ECG, ace-of-spade shape on left ventriculography and a better prognosis compared to the usual HCM phenotype (asymmetrical septal hypertrophy). But is this particular phenotype just an apical “variant” of HCM or could it be a different entity with some similar phenotypic manifestations, sharing clinical, diagnostic and genetic features? And is its prognosis still as good as it was when first described? AHCM often represents a dilemma for clinicians and cardiovascular specialists, either due to the absence of randomized trials on the subject, or due to the relative rarity of the disease. Diagnostic criteria, clinical course and treatment management are matters for which there are no assertive answers. Consequently, an up-to-date review with the most recent available information on AHCM was considered necessary.The aim of this work is to review the particular aspects of AHCM and to compare its features, including management and prognosis, with the most common phenotype of HCM. Methods of the Review: Information for this review was retrieved from PUBMED and MEDLINE searches (1976-2014) by using the key-words below through a systematic analysis. |
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