Education and training, Education, Diagnostic procedure, MR, Cardiovascular system, Cardiac
M. Rodríguez Álvarez, C. Saborido Avila, L. Trillo, P. pazos, J. M. Vieito Fuentes; Vigo/ES
Methods and Materials
Hypertrophic cardiomyopathy (HCM) is the most common inherited cardiomyopathy, affecting one of every 500 adults. Represents the most common cause of Sudden cardiac death (SCD) in young athletes due to malignant ventricular arrhythmias. HCM is caused by mutations in sarcomeric genes transmitted following an autosomal dominant pattern. There is a great variability of phenotypes.
The pathologic hallmarks of HCM are myofibrillary disarray, interstitial fibrosis and abnormal dysplasia of intramural coronary arterioles (microvascular dysfunction).
The diagnosis requires exclusion of disease entities that may lead to inappropriate myocardial wall thickening of other etiologies (phenocopies).
Because the clinical manifestations and electrocardiographic findings are nonspecific noninvasive imaging modalities play a main role in diagnosis, management and understanding pathophysiology of HCM.
The use of CMRI combined with genetic tests reveal a new overview on the frequency, management, and prognosis of HCM. We review CMR estudies performed in HCM patients at our institution and resume and discuss in an schematic view, the paper of CMRI in HCM, its various phenotypes and phisyology, risk stratification, follow-up and evaluation of family members.
We also searched PubMed for clinical trials that investigated the prognostic utility of LGE and T1mapping in risk stratification of HCM patients and correlation it with our experience.