Learning objectives
Resume in a practical guide for radiological use,
the paper of different Cardiac Magnetic Resonance Imaging (CMRI) techniques to demonstrate the typical morphological characteristics and phenotypes of Hypertrophic Cardiomyopathy (HCM).
Describe the interaction between different phenotypic patterns,
functional and hemodynamic consequences,
and potential adverse events
Revealthe different patterns of late gadolinium enhancement (LGE) and its correlation with fibrosis
Discuss the emerging role of CMRI in screening,
diagnosis,
and risk stratification.
Background
Hypertrophic cardiomyopathy (HCM) is the most common inherited cardiomyopathyandthe 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 agreat 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 amainrole indiagnosis,
management and understanding pathophysiology of HCM.
The use of CMRIcombined with genetic tests,reveal a...
Findings and procedure details
»CMRITECHNIQUES APPLIED TOHCM
CMRI tecniques let us a new perspective aboutthe frequency,
management,
and prognosis of HCM.
CINE SSFP MR IMAGING SEQUENCES
(Cine Steady-state free precession) are usedto evaluate:
-Cardiac function measurement
Biventricular volume quantification and function
Elevation of left ventricle ejection fraction(LVEF)
Sistolic disfunction often develops with end-stage HCM.
Assessment of global and segmental systolic thickening
-Morphological aspects:
Quantify the Myocardial thickness and mass accurately which are related to the diagnosis and prognosis of HCM:
Diagnostic criterion for HCM is a maximal LV wall...
Conclusion
MRI has proven to be an important tool for the evaluation of patients suspected of having HCM
Makingthe diagnosis and identifying the phenotypes of HCM
Can potentially identify the subset of patients at risk of sudden cardiac death
Differentiating between HCM and other myocardial diseases showing LVH (phenocopies).
playing an important role in screening of the family members of a patient with HCM.
Have a practical updated management guide by CMRI,
includingtechnical,
clinical and pathophysiological and risk startification aspects,
allows a faster and more reliable...
References
Ameya Jagdish Baxi,
MD Carlos S.
Restrepo,
MDDaniel Vargas,Hypertrophic Cardiomyopathy fromA to Z: Genetics,
Pathophysiology,
Imaging,
and Management.RadioGraphics 2016; 36:335–354.
Barry J.
Maron and Martin S.
Maron.
LGE Means Better Selection of HCM Patients for Primary Prevention Implantable Defibrillators.
JACC: Cardiovascular Imaging.Volume 9,
Issue 12,
December 2016
Jan Bogaert,
MDIacopo Olivotto,
MD.
MR Imaging in Hypertrophic Cardiomyopathy: From Magnet to Bedside.
Radiology:vo,
l273:N2—November 2014.
Christopher M.
Kramer,
MDa and Stefan Neubauer,
MDb.
Further Refining Risk in Hypertrophic Cardiomyopathy With Late Gadolinium Enhancement by CMR.
J...