Danon Disease Presenting as Eosinophilic Myocarditis: Key Role of Endomyocardial Biopsy

Basilio Angulo-Lara; Ramón Garrido-González; Clara Salas Antón; Juan Francisco Oteo-Domínguez; Javier Segovia-Cubero; Fernando Domínguez

doi:10.1016/j.jaccas.2023.101765

Danon Disease Presenting as Eosinophilic Myocarditis: Key Role of Endomyocardial Biopsy

JACC Case Rep. 2023 Feb 22:11:101765. doi: 10.1016/j.jaccas.2023.101765. eCollection 2023 Apr 5.

Authors

Basilio Angulo-Lara¹, Ramón Garrido-González¹, Clara Salas Antón², Juan Francisco Oteo-Domínguez¹, Javier Segovia-Cubero^{1

3}, Fernando Domínguez^{1

3}

Affiliations

¹ Cardiology Department, Hospital Universitario Puerta de Hierro, Madrid, Spain.
² Pathology Department, Hospital Universitario Puerta de Hierro, Madrid, Spain.
³ Centro de Investigación Biomédica en Red de Enfermedades Cardiovasculares, Madrid, Spain.

Abstract

A 21-year-old woman with a history of atopy, peripheral eosinophilia, Wolf-Parkinson-White syndrome, and 5 episodes of myocarditis was diagnosed with eosinophilic myocarditis. Despite adequate immunosuppressive treatment and resolution of the myocarditis episode, the patient developed dilated cardiomyopathy and presented with worsening of her functional class. Finally, genetic testing unveiled an additional diagnosis: Danon disease. (Level of Difficulty: Advanced.).

Keywords: Danon disease; endomyocardial biopsy (EMB); eosinophilic myocarditis; genetic testing; inherited cardiomyopathies.

Publication types

Case Reports