학술논문
The cardiomyopathy of cystic fibrosis: a modern form of Keshan disease
Document Type
article
Author
Javier Segovia-Cubero; Lorena Ruiz-Bautista; Luis Maiz-Carro; Rosa M. Girón-Moreno; M. Concepción Prados-Sánchez; M. Teresa Martínez-Martínez; Montserrat González-Estecha; Susana Mingo-Santos; Manuel Gómez-Bueno; Clara Salas-Antón; Miguel A. Cavero-Gibanel; Miguel Pastrana-Ledesma; Pablo García-Pavía; Rosalía Laporta-Hernández; David Sánchez-Ortiz; Luis Alonso-Pulpón
Source
Frontiers in Cardiovascular Medicine, Vol 11 (2024)
Subject
Language
English
ISSN
2297-055X
Abstract
IntroductionWe conducted a study to determine the prevalence of structural heart disease in patients with CF, the characteristics of a cardiomyopathy not previously described in this population, and its possible relationship with nutritional deficiencies in CF.MethodsWe studied 3 CMP CF patients referred for heart-lung transplantation and a prospective series of 120 adult CF patients. All patients underwent a clinical examination, blood tests including levels of vitamins and trace elements, and echocardiography with evaluation of myocardial strain. Cardiac magnetic resonance imaging (CMR) was performed in patients with CMP and in a control group. Histopathological study was performed on hearts obtained in transplant or necropsy.ResultsWe found a prevalence of 10% (CI 4.6%–15.4%) of left ventricular (LV) dysfunction in the prospective cohort. Myocardial strain parameters were already altered in CF patients with otherwise normal hearts. Histopathological examination of 4 hearts from CF CMP patients showed a unique histological pattern of multifocal myocardial fibrosis similar to Keshan disease. Four of the five CF CMP patients undergoing CMR showed late gadolinium uptake, with a characteristic patchy pattern in 3 cases (p