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Right Heart-Pulmonary Circulation Unit in Cardiomyopathies and Storage Diseases

Antonello D'Andrea1, Tiziana Formisano1, Andrè La Gerche2

  • 1Department of Cardiology, Luigi Vanvitelli University, Monaldi Hospital, AORN Ospedali dei Colli, Via Bianchi, Naples 80100, Italy.

Heart Failure Clinics
|July 4, 2018
PubMed

Insights

Cardiomyopathies are diverse heart muscle diseases. While some directly affect the right heart, others like hypertrophic or dilated forms show its prognostic significance, though its pathogenic role remains less understood.

Area of Science:

  • Cardiology
  • Cardiovascular Diseases
  • Pathology

Background:

  • Cardiomyopathies (CM) encompass a range of heart muscle diseases.
  • Key categories include dilated, hypertrophic, and restrictive cardiomyopathies.
  • Conditions like athlete's heart and hypertensive cardiopathy represent adaptive responses to increased cardiac workload.

Purpose of the Study:

  • To review the classification and characteristics of cardiomyopathies.
  • To examine the role and significance of right heart involvement in various cardiomyopathies.
  • To differentiate between direct right heart pathology and its prognostic impact.

Main Methods:

  • Literature review and synthesis of existing research on cardiomyopathies.
  • Analysis of the etiological and pathological features of different CM subtypes.
  • Evaluation of the clinical and prognostic implications of right heart abnormalities.

Main Results:

  • Cardiomyopathies are broadly classified into dilated, hypertrophic, and restrictive types.
  • Some CMs, such as arrhythmogenic CM, carcinoid syndrome, and endomyocardial fibrosis, clearly involve the right heart.
  • In hypertrophic and dilated CM, right heart involvement impacts prognosis but its direct pathogenic role is less defined.

Conclusions:

  • Cardiomyopathies represent a spectrum of myocardial diseases with varying degrees of right heart involvement.
  • The prognostic impact of the right heart in certain cardiomyopathies is established.
  • Further research is needed to elucidate the precise pathogenic mechanisms of right heart involvement in conditions like hypertrophic and dilated cardiomyopathy.

Keywords:
Athlete’s heartCardiac magnetic resonanceDilated cardiomyopathiesEchocardiographyHypertrophic cardiomyopathiesRestrictive cardiomyopathiesRight ventricleSpeckle tracking echocardiography

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