Highlights
- •There are several morphological features besides left ventricular hypertrophy which can suggest a diagnosis of Hypertrophic Cardiomyopathy (HCM)
- •A spade-shaped appearance of the left ventricle is characteristic of apical HCM
- •In patients with HCM, there may apical displacement of the papillary muscles, papillary muscle hypertrophy, and elongation of the mitral leaflets which may lead to outflow tract obstruction
- •Myocardial crypts and left ventricular bands are variants associated with HCM
- •In HCM, dystrophic calcifications may result from decreased microvascular perfusion pressure and ischemia.
Abstract
Hypertrophic cardiomyopathy (HCM) is characterized by left ventricular hypertrophy
(LVH) in the absence of another causal disease. Several morphologic and histologic
changes have been described. Given the morbidity and mortality associated with HCM,
understanding these anatomic variations is key to interpreting imaging. This is especially
important since many patients exhibit these associated findings in the absence of
LVH and prompt early detection of these variations may lead to early diagnosis and
treatment. This article describes the appearance of morphologic variations seen in
HCM beyond myocardial thickening including: papillary muscle and mitral valve variants,
myocardial crypts, left ventricular myocardial bands, and dystrophic calcification
related to increased wall tension.
Keywords
To read this article in full you will need to make a payment
Purchase one-time access:
Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online accessOne-time access price info
- For academic or personal research use, select 'Academic and Personal'
- For corporate R&D use, select 'Corporate R&D Professionals'
Subscribe:
Subscribe to Clinical ImagingAlready a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
References
- Hypertrophic cardiomyopathy from a to Z: genetics, pathophysiology, imaging, and management.Radiographics. 2016; 36: 335-354
- American College of Cardiology/European Society of Cardiology clinical expert consensus document on hypertrophic cardiomyopathy. A report of the American College of Cardiology Foundation task force on clinical expert consensus documents and the european Society of Cardiology Committee for practice guidelines.J Am Coll Cardiol. 2003; 42: 1687-1713
- Familial muscular subaortic stenosis: an unrecognized form of "idiopathic heart diseases," with clinical and autopsy observations.Circulation. 1960; 21: 167-180
- Asymmetrical hypertrophy of the heart in young adults.Br Heart J. 1958; 20: 1-8
- Myocardial fibrosis in hypertrophic cardiomyopathy: accurate reflection of histopathological findings by CMR.JACC Cardiovasc Imaging. 2013; 6: 587-596
- Systolic anterior motion begins at low left ventricular outflow tract velocity in obstructive hypertrophic cardiomyopathy.J Am Coll Cardiol. 2000; 36: 1344-1354
- Hypertrophic cardiomyopathy: assessment with MR imaging and multidetector CT.Radiographics. 2010; 30: 1309-1328
- Apical variant hypertrophic cardiomyopathy "multimodality imaging evaluation".Int J Cardiovasc Imaging. 2020; 36: 553-561
- Phenotypic markers for forensic purposes.in: Parra S.C.Z. Roberto C. Ubelaker Douglas H. Forensic science and humanitarian action: interacting with the dead and the living. 2020
- Abnormal septal convexity into the left ventricle occurs in subclinical hypertrophic cardiomyopathy.J Cardiovasc Magn Reson. 2015; 17: 64
- Relation between left ventricular outflow tract obstruction and left ventricular shape in patients with hypertrophic cardiomyopathy: a cardiac magnetic resonance imaging study.Arch Cardiovasc Dis. 2013; 106: 440-447
- Magnetic resonance imaging of abnormal ventricular septal motion in heart diseases: a pictorial review.Insights Imaging. 2011; 2: 483-492
- Usefulness of apical area index to predict left ventricular thrombus in patients with systolic dysfunction: a novel index from cardiac magnetic resonance.BMC Cardiovasc Disord. 2019; 19: 15
- Papillary muscles do not attach directly to the solid heart wall.Circulation. 2004; 109: 3145-3148
- CT and MR imaging of the mitral valve: radiologic-pathologic correlation.Radiographics. 2010; 30: 1603-1620
- Obstructive hypertrophic cardiomyopathy associated with minimal left ventricular hypertrophy.Am J Cardiol. 1984; 53: 377-379
- Abnormal papillary muscle morphology is independently associated with increased left ventricular outflow tract obstruction in hypertrophic cardiomyopathy.Heart. 2008; 94: 1295-1301
- Solitary papillary muscle hypertrophy as a possible form of hypertrophic cardiomyopathy.Jpn Circ J. 1998; 62: 811-816
- Papillary muscles of left ventricle-morphological variations and it's clinical relevance.Indian Heart J. 2018; 70: 894-900
- Solitary accessory and papillary muscle hypertrophy manifested as dynamic mid-wall obstruction and symptomatic heart failure: diagnostic feasibility by multi-modality imaging.BMC Cardiovasc Disord. 2014; 14: 34
- Isolated papillary muscle hypertrophy: a gap in our knowledge of hypertrophic cardiomyopathy?.Rev Port Cardiol. 2014; 33 (379.e1-5)
- Characteristics and surgical outcomes of symptomatic patients with hypertrophic cardiomyopathy with abnormal papillary muscle morphology undergoing papillary muscle reorientation.J Thorac Cardiovasc Surg. 2010; 140: 317-324
- Mitral valve abnormalities identified by cardiovascular magnetic resonance represent a primary phenotypic expression of hypertrophic cardiomyopathy.Circulation. 2011; 124: 40-47
- Diversity of structural mitral valve alterations in hypertrophic cardiomyopathy.Circulation. 1992; 85: 1651-1660
- Mechanism of mitral regurgitation in hypertrophic cardiomyopathy: mismatch of posterior to anterior leaflet length and mobility.Circulation. 1998; 98: 856-865
- Echocardiographic assessment of mitral valve size in obstructive hypertrophic cardiomyopathy. Anatomic validation from mitral valve specimen.Circulation. 1993; 88: 548-555
- Symptomatic exercise-induced left ventricular outflow tract obstruction without left ventricular hypertrophy.J Am Soc Echocardiogr. 2013; 26: 556-565
- Prognostic significance of anterior mitral valve leaflet length in individuals with a hypertrophic cardiomyopathy gene mutation without hypertrophic changes.J Ultrasound. 2018; 21: 217-224
- Myocardial strain evaluation with cardiovascular MRI: physics, principles, and clinical applications.Radiographics. 2022; 42: 968-990
- Cardiovascular magnetic resonance feature tracking strain analysis for discrimination between hypertensive heart disease and hypertrophic cardiomyopathy.PLoS One. 2019; 14e0221061
- Hypertensive heart disease versus hypertrophic cardiomyopathy: multi-parametric cardiovascular magnetic resonance discriminators when end-diastolic wall thickness ≥ 15 mm.Eur Radiol. 2017; 27: 1125-1135
- Cardiac outpouchings: definitions, differential diagnosis, and therapeutic approach.Cardiol Res Pract. 2021; 20216792643
- Multimodality imaging and clinical significance of congenital ventricular outpouchings: recesses, diverticula, aneurysms, clefts, and crypts.J Cardiovasc Echogr. 2018; 28: 9-17
- Overview of left ventricular outpouchings on cardiac magnetic resonance imaging.Cardiovasc Diagn Ther. 2015; 5: 464-470
- Cardiac outpouchings: a multimodality imaging review.J Thorac Imaging. 2020; 35: W18-w29
- Prevalence and clinical profile of myocardial crypts in hypertrophic cardiomyopathy.Circ Cardiovasc Imaging. 2012; 5: 441-447
- Prevalence of myocardial crypts in a large retrospective cohort study by cardiovascular magnetic resonance.J Cardiovasc Magn Reson. 2014; 16: 66
- Myocardial crypt, diverticulum, or aneurysm? CTA as an adjudicator.Int J Cardiovasc Imaging. 2020; 36: 2061-2062
- Significance of left ventricular apical-basal muscle bundle identified by cardiovascular magnetic resonance imaging in patients with hypertrophic cardiomyopathy.Eur Heart J. 2014; 35: 2706-2713
- Intramural ("small vessel") coronary artery disease in hypertrophic cardiomyopathy.J Am Coll Cardiol. 1986; 8: 545-557
- A pattern-based approach to assessment of delayed enhancement in nonischemic cardiomyopathy at MR imaging.Radiographics. 2009; 29: 89-103
- The diagnosis of hypertrophic cardiomyopathy by cardiovascular magnetic resonance.J Cardiovasc Magn Reson. 2012; 14: 17
- What is late gadolinium enhancement in hypertrophic cardiomyopathy?.Rev Esp Cardiol. 2007; 60: 1-4
- Prognostic value of LGE-CMR in HCM: a meta-analysis.JACC Cardiovasc Imaging. 2016; 9: 1392-1402
- Carriers of the hypertrophic cardiomyopathy MYBPC3 mutation are characterized by reduced myocardial efficiency in the absence of hypertrophy and microvascular dysfunction.Eur J Heart Fail. 2011; 13: 1283-1289
- Isolated microvascular obstruction of the papillary muscles: an unveiling by cardiac MRI.J Cardiovasc Imaging. 2022; 30: 229-230
- Cardiac MR assessment of microvascular obstruction.Br J Radiol. 2015; 8820140470
- Myocardial perfusion defects in hypertrophic cardiomyopathy mutation carriers.J Am Heart Assoc. 2021; 10e020227
- Inline perfusion mapping provides insights into the disease mechanism in hypertrophic cardiomyopathy.Heart. 2020; 106: 824-829
Article info
Publication history
Published online: January 12, 2023
Accepted:
January 2,
2023
Received in revised form:
December 23,
2022
Received:
July 5,
2022
Footnotes
☆Conflicts of interest and source of funding: The authors declare no conflicts of interest. This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.
Identification
Copyright
© 2023 Published by Elsevier Inc.
