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Journal Article Ahead of print 1 citation

PubMed: 42786594 DOI: 10.1161/JAHA.125.043647

Noninvasive Characterization of Myocardial Diseases With Hypertrophic Phenotypes Using Integrated ECG/Echocardiographic Indices.

J Am Heart Assoc

Authors: Valentina Allegro, Linda Pagura, Aldostefano Porcari, Giulia Barbati, Simone Longhi, Beatrice Musumeci, Alberto Cipriani, Marco Canepa, Cristina Chimenti, Rosa Lillo, Francesca Graziani, Giacomo Bonacchi, Alessia Argiro, Mattia Zampieri, Chiara Chiti, Irene Ruotolo, Giacomo Tini, Laura De Michieli, Giulio Sinigiani, Gianluca Di Bella, Marianna Eleonora Labate, Virginia Eustachi, Alessio Cianci, Aniello Sammartino, Iacopo Olivotto, Elena Biagini, Gianfranco Sinagra, Marco Merlo, Francesco Cappelli

Affiliations

  • Center for Diagnosis and Treatment of Cardiomyopathies, Cardiovascular Department Azienda Sanitaria Universitaria Giuliano-Isontina (ASUGI) and University of Trieste Trieste Italy.
  • European Reference Network for Rare, Low Prevalence and Complex Diseases of the Heart-ERN GUARD-Heart Trieste Italy.
  • National Amyloidosis Centre, Division of Medicine University College London, Royal Free Campus Rowland Hill Street London UK.
  • Biostatistics Unit, Department of Medical Sciences University of Trieste Trieste Italy.
  • Cardiology Unit, Cardiac Thoracic and Vascular Department IRCCS Azienda Ospedaliero-Universitaria di Bologna Bologna Italy.
  • European Reference Network for Rare, Low Prevalence and Complex Diseases of the Heart-ERN GUARD-Heart Bologna Italy.
  • Division of Cardiology, Department of Clinical and Molecular Medicine Sapienza University of Rome Rome Italy.
  • Department of Cardiac, Thoracic and Vascular Sciences and Public Health University of Padova Padova Italy.
  • Department of Internal Medicine University of Genoa Italy.
  • UOC Cardiologia IRCCS Ospedale Policlinico San Martino Genoa Italy.
  • Department of Clinical, Internal, Anesthesiologist and Cardiovascular Sciences La Sapienza University Rome Italy.
  • Dipartimento di Scienze Cardiovascolari-CUORE Fondazione Policlinico Universitario A. Gemelli IRCCS Rome Italy.
  • Cardiomyopathy Unit, Careggi University Hospital University of Florence Florence Italy.
  • Department of Cardiology University of Messina Messina Italy.

Abstract

BACKGROUND

Indices reflecting the discordance between QRS complex voltages on ECG and left ventricular (LV) wall thickness or LV mass index on echocardiogram (echo) have enabled identification of cardiac amyloidosis (CA) among patients with hypertrophic hearts. However, this evidence comes from studies that have excluded patients with established hypertrophic cardiomyopathy (HCM) and Anderson-Fabry disease (AFD). The aim of this study was to assess the performance of ECG/echo indices in characterizing adult patients with cardiomyopathies and hypertrophic phenotype.

METHODS

This was an Italian retrospective, cross-sectional, multicenter study. Data from ECGs and echo of consecutive adult patients with confirmed CA, HCM, and AFD, and an LV wall thickness >12 mm were analyzed to calculate different ECG/echo indices.

RESULTS

Of the 1181 patients included (median age, 65 years; 69% men), 35.5% (n=416) had CA, 46.5% (n=594) had HCM, and 14.5% (n=171) had AFD. All ECG/echo indices were able to distinguish CA from other HCM and AFD (P<0.001). The total QRS score/LV mass index was the best performing ECG/echo index for differentiating CA from other hypertrophic phenotypes (accuracy: 83%). The total QRS/maximum LV wall thickness was the best performing index in discriminating between the 3 cardiomyopathies (cutoff values of 6.21 versus 9.81 versus 11.47 in CA, HCM, AFD, respectively; P<0.001 for all comparisons).

CONCLUSIONS

ECG/echo indices, particularly total QRS/maximum LV wall thickness, may serve as easily accessible first-line tools to differentiate CA from HCM and AFD. These findings may enable prompt recognition of patients with different cardiomyopathies in everyday clinical practice, both in community-based centers and tertiary care facilities, and to orient subsequent diagnostic workup.

MeSH

Heart Diseases, Phenotype, Cardiomyopathy, Hypertrophic, Echocardiography, Cardiomyopathies, Fabry Disease