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Cardiomyopathy III: Hypertrophic Cardiomyopathy01:29

Cardiomyopathy III: Hypertrophic Cardiomyopathy

Hypertrophic cardiomyopathy, or HCM, is an autosomal dominant genetic disorder characterized by asymmetric left ventricular hypertrophy without ventricular dilation. It is more common in men and is typically diagnosed in young, athletic adults.EtiologyHCM is primarily genetic and is caused by mutations in genes encoding sarcomeric proteins. Researchers have identified over 1400 mutations across at least 11 different genes. Among these, the most frequently occurring mutations are found in the...
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Cardiomyopathy IV: Restrictive Cardiomyopathy

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Morphological and Functional Assessment of the Right Ventricle Using 3D Echocardiography
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Right ventricular function in AL amyloidosis: characteristics and prognostic implication.

Francesco Cappelli1, Maria Cristina Porciani, Franco Bergesio

  • 1Intensive Cardiac Care Unit, Heart and Vessel Department, Azienda Ospedaliero Universitaria Careggi, University of Florence, Largo Brambilla 3, 50127 Florence, Italy. cappellifrancesco@inwind.it

European Heart Journal. Cardiovascular Imaging
|December 20, 2011
PubMed
Summary

Right ventricle (RV) dysfunction significantly worsens prognosis in AL amyloidosis patients with cardiac involvement. RV longitudinal strain is the strongest predictor of death, highlighting the need for routine RV function assessment.

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Area of Science:

  • Cardiology
  • Cardiovascular Imaging
  • Amyloidosis Research

Background:

  • Right ventricle (RV) dysfunction's role in AL amyloidosis is often underestimated.
  • Cardiac involvement in AL amyloidosis significantly impacts patient prognosis.

Purpose of the Study:

  • To comprehensively evaluate RV function in AL amyloidosis patients.
  • To determine the prognostic role of RV function in AL amyloidosis with and without cardiac involvement.

Main Methods:

  • Echocardiographic evaluation (traditional, TDI, speckle-tracking) of 52 AL amyloidosis patients (27 with cardiac involvement [CA] and 25 without [NCA]) and controls.
  • Analysis of RV diastolic diameter, wall thickness, longitudinal systolic function, and diastolic function.
  • 19-month median follow-up for mortality, with Cox multivariate analysis.

Main Results:

  • CA patients showed increased RV free wall thickness and significantly depressed RV longitudinal systolic function compared to controls and NCA patients.
  • No significant differences in Doppler diastolic evaluation, but CA patients had impaired tricuspidal annulus TDI parameters.
  • N-terminal pro-Brain natriuretic peptide and RV longitudinal strain were the strongest predictors of death.

Conclusions:

  • RV involvement in AL amyloidosis develops later than LV deposition but portends a worse prognosis.
  • RV longitudinal strain is the sole echocardiographic predictor of prognosis in these patients.
  • Routine RV function analysis is recommended in echocardiographic evaluations for AL amyloidosis.