Type:
Educational Exhibit
Keywords:
Congenital, Outcomes analysis, Education, MR, Management, Cardiovascular system, Cardiac
Authors:
M. Rodríguez Álvarez, P. M. Dos Santos Reis Ferreira, C. Saborido Avila, L. Trillo, P. pazos, J. M. Vieito Fuentes; Vigo/ES
DOI:
10.26044/ecr2019/C-2305
Background
Hypertrophic cardiomyopathy (HCM) is the most common inherited cardiomyopathy and the most common cause of Sudden cardiac death (SCD) in young athletes. 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).
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,
resume and discuss the paper of CMRI in HCM.
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.