Type:
Poster Presentation
Keywords:
Cardiac, MR, CT, Echocardiography, Diagnostic procedure, Hyperplasia / Hypertrophy, Education and training, Image verification
Authors:
E. Pershina, O. Larina, E. A. Mershina, V. Sinitsyn; Moscow/RU
Purpose
Hypertrophic cardiomyopathy (HCM) is one of the most common genetic disorders of the heart.
HCM is characterized by a wide range of clinical features,
ranging from asymptomatic mutation in genes encoding sarcomeric proteins to sudden cardiac death as the first manifestation of the disease. Сardiovascular magnetic resonance (CMR) is increasingly used to characterize morphologic,
functional and tissue abnormalities associated with HCM because of its precise determination of myocardial anatomy and the depiction of myocardial fibrosis.
Several morphological variants of HCM that can be identified by cardiac CMR have been described: asymmetric HCM with sigmoid septal contour ("septal HCM"),
asymmetric HCM with reversed septal contour, HCM with mid-ventricular obstruction (with or without a LV apical diverticulum),
apical HCM,
symmetric (concentric) HCM and focal HCM [1,2] - Fig.1.
Hystological markers include myocyte and myofibrillar disarray,
myocyte hypertrophy,
fibrosis replacement,
dysplasia of small arteries,
seen as medial and intimal smooth muscle cell proliferation with luminal narrowing,
reduced arteriolar density wich lead to small-vessel intramural coronary artery disease (SICAD) as an early manifestation of HCM [2].
Late gadolinium enhancement at CMR is present in approximately 60% of HCM patients with left ventricular hypertrophy and may provide information regarding risk stratification in HCM.
LGE in HCM appears as patchy mid-wall hyperenhancement in the most thickened segments and at the junction of the septum to the anterior and posterior walls of LV (Fig.2).
Our routine practice revealed that atypical patterns of LGE could be also visualized in patients with HCM.
The purpose of our study was to provide physicians with a review of atypical pattern of LGE in HCM that could mimic ischemical,
inflammatory systemic or metabolic diseases.