Miocardiopatías III. Miocardiopatía restrictiva. Displasia arritmogénica del ventrículo derecho. Miocardiopatía no compactada

  1. Ortiz Bautista, C.
  2. Salguero Bodes, R.
  3. Delgado Jiménez, J.F.
Journal:
Medicine: Programa de Formación Médica Continuada Acreditado

ISSN: 0304-5412

Year of publication: 2017

Issue Title: Enfermedades cardiológicas (IX) Miocardiopatías

Series: 12

Issue: 43

Pages: 2573-2584

Type: Article

DOI: HTTPS://DOI.ORG/10.1016/J.MED.2017.10.013 DIALNET GOOGLE SCHOLAR

More publications in: Medicine: Programa de Formación Médica Continuada Acreditado

Abstract

Abstract Restrictive cardiomyopathy (RCM) is a rare entity characterised by increased left ventricular filling with normal systolic function and left ventricular thickness. These findings can appear in association with various inflammatory, infiltrative or deposition (secondary) diseases. However, recent results of molecular genetics research studies have demonstrated that a significant amount of RCM cases with no associated systemic disease are caused by sarcomeric gene mutations, previously associated with hypertrophic cardiomyopathy, dilated cardiomyopathy and non-compaction (primary) cardiomyopathy. In general, patients develop serious symptoms of heart failure over a short period of time, and most die within a few years after diagnosis unless they undergo heart transplantation. The objective of this update is to review the different clinical entities that present like RCM. We shall also review other cardiomyopathies that are not classified into the three classical types

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