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...
Methods and Materials
21 patients (m/f-11/10,
age 17-62 yrs,
mean age - 35.8±15,9 yrs) were diagnosed with HCM in our department in 2012: 8 – asymmetric septal hypertrophy (ASH) with sigmoid septal contour and outflow tract obstruction,
6 patients - ASH with reversed septal contour without outflow tract obstruction,
4 - apical form,
2 – midventricular form and 1 - focal HCM.
CMR protocol was performed at 1.5T scanner (MagnetomAvanto,Siemens AG) using multichannel surface coil with cardiac synchronization.
Breath-hold cine images were acquired with fast imaging using steady-state...
Results
Focuses of LGE were determined in 13 of 21 patients (61,9%).
9 patients had small punctuate,
patchy mid-wall hyper-enhancement– typical patterns of LGE corresponded to the most hypertrophied part of the myocardium and at the junction of the septum to the anterior and posterior walls of LV (Fig.2)
4 of 13 patients with LGE focuses (30%) had atypical pattern of contrast-enhancement.
2 patients had subendocardial type of enhancement mimicked ischemical disorders.
No significant coronary stenoses were found in both cases.
- 1st patient was diagnosed...
Conclusion
It is known that increased LV mass and focuses of late gadolinium
enhancement could be associated with increasedpossibility of future adverse cardiovascular events in patients with HCM.
CMR appears to be highly relevant diagnostic tool in clinical and preclinical HCM.
Our overview observed several atypical patterns of LGE in HCM,
which could be confused with ischemical,
inflammatory systemic or metabolic disorders,
but should be assessed in the pathogenesis of HCM.
References
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Radwa A Noureldin,Songtao Liu,Marcelo S Nacif,Daniel P Judge,Marc K Halushka,Theodore P Abraham,Carolyn Ho,David A Bluemke.
The diagnosis of cardiomyopathy by cardiovascular magnetic resonance.
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MRI of Hypertrophic Cardiomyopathy: Part 2,
Differential Diagnosis,
Risk Stratification,
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American Journal of Roentgenology (2007)189:6,1344-1352 http://www.ajronline.org/doi/suppl/10.2214/AJR.07.2286
2.Mark W.
Hansen,
Naeem Merchant.
MRI of Hypertrophic Cardiomyopathy: Part 2,
Differential Diagnosis,
Risk Stratification,
and Posttreatment MRI Appearances.
American Journal of Roentgenology (2007)189:6,1344-1352
http://www.ajronline.org/doi/suppl/10.2214/AJR.07.2286
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Francesco De Cobelli,
Antonio Esposito,...