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Myocardial Crypts

2012; Lippincott Williams & Wilkins; Volume: 5; Issue: 4 Linguagem: Inglês

10.1161/circimaging.112.975888

1942-0080

James Moon, William J. McKenna,

Congenital Heart Disease Studies

HomeCirculation: Cardiovascular ImagingVol. 5, No. 4Myocardial Crypts Free AccessEditorialPDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissions ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toFree AccessEditorialPDF/EPUBMyocardial CryptsA Prephenotypic Marker of Hypertrophic Cardiomyopathy? James C. Moon and William J. McKenna James C. MoonJames C. Moon From the Inherited Cardiovascular Diseases Unit (W.J.M.) and Heart Hospital Imaging Centre (J.C.M.), Heart Hospital; and the Institute of Cardiovascular Science, (J.C.M., W.J.M.), University College London, London, United Kingdom. and William J. McKennaWilliam J. McKenna From the Inherited Cardiovascular Diseases Unit (W.J.M.) and Heart Hospital Imaging Centre (J.C.M.), Heart Hospital; and the Institute of Cardiovascular Science, (J.C.M., W.J.M.), University College London, London, United Kingdom. Originally published1 Jul 2012https://doi.org/10.1161/CIRCIMAGING.112.975888Circulation: Cardiovascular Imaging. 2012;5:431–432It is more than 50 years since the first modern description of hypertrophic cardiomyopathy (HCM).1 Understanding has been driven by technology, with the first descriptions focusing on clinical examination, ECG changes, and histological changes from autopsy study. As technology evolved from ventriculography, M-mode, then 2-dimensional echocardiography, and cardiovascular magnetic resonance (CMR), understanding has evolved and the known phenotype of the disease has extended. Recent notable examples of this have been hypertrophy missed by echo, particularly but not exclusively at the apex, mitral valve abnormalities, apical aneurysms, and proposed familial criteria, where even subtle abnormalities may be adjudged significant in the context of a pretest probability of 50% of genetic mutation carriage. In this issue of Circulation: Cardiovascular Imaging, Maron et al2 have explored the potential significance of myocardial crypts. These "architectural abnormalities" of the left ventricle (LV) occur particularly in the septum and inferior (posterior) right ventricular (RV) insertion point and had been observed at increased frequency in HCM. Such abnormalities may be important because they may represent a "prephenotypic" marker of HCM—that is, a sign of abnormal fetal cardiomorphogenesis triggered by the in utero consequences of the underlying sarcomeric mutation.Article see p 441In the report, using conventional CMR cine imaging, HCM patients, gene-positive phenotype-negative (meaning in this instance the absence of left ventricular hypertrophy [LVH]), and normal control subjects had respective crypt prevalences of 4%, 61%, and 0%, respectively. The study confirms the proposal of others3 that crypts may help define gene carriage before hypertrophy, selecting patients for closer scrutiny for the detection of the development of hypertrophy and risk, a promising result, and that the crypts presumably become less apparent when hypertrophy develops.The results support an extended role for sarcomeric proteins beyond simply contraction and relaxation on command—additional known roles are in signaling, stretch detection, energy utilization, growth, and now embryonic cardiomorphogenesis. How such mutations could cause clefts remains unknown, but there may be analogy with left ventricular noncompaction (LVNC), which is an arrest of the compaction process in utero after ventricular septation and before birth (around E14.5 development stage to birth in murine models).4 HCM may demonstrate features similar to LVNC in an overlap appearance. It may be that rather than mutations creating these clefts, the pre-LVH HCM heart is architecturally neotonal and arrested in a premature developmental phase, where such clefts are persistent and visible to our current technology.How may we use these data clinically? First, a note of caution about the ascertainment and the significance of single rather than multiple crypts. Although the authors found no crypts in normal subjects, others have found single basal inferior clefts or septal clefts in 6% and 5% of normal volunteers, respectively, and higher rates in certain congenital heart diseases.5 These rates may get higher if a modified 2-chamber view is used through the inferior RV insertion point rather than the inferior wall. It is worth noting that the definition of these crypts did not include their disappearance in systole; that the slice thickness was 7 mm rather than 10 mm; and that the control cohort in the Maron et al report consisted of patients referred for CMR who were found to have no CMR abnormalities rather than being healthy normal volunteers. Second, ethnicity may confound interpretation: the results should be treated with caution in, for example, Afro-Caribbean individuals, in whom crypt prevalence is unknown but trabeculae are known to be more prominent.6 Third, if overt HCM has reduced cleft prevalence, it may be that other diseases may confound their ascertainment—one could speculate that mild hypertrophy from hypertension could reduce their prominence or that they may become less apparent with growth and age.Currently, known prephenotypic markers in the familial context, where the pretest probability is 50%, include ECG changes, biomarkers for diffuse fibrosis, and advanced echocardiographic techniques. In the future, other "prephenotypic markers" of genetic carriage may become useful. Focal fibrosis via the CMR late gadolinium enhancement technique appears relatively uncommon in HCM before hypertrophy, which may reflect its usual position later in the myocardial phenotype development. Diffuse fibrosis quantification techniques using T1 mapping may be useful. Diffusion tensor imaging for fiber architecture7 may also hold promise of disarray detection in vivo, but the biology of when disarray develops in HCM and its specificity are not yet known. As next-generation sequencing becomes more penetrant into clinical practice, one might consider that the need for these markers would diminish. However, it may be that their utility may be enhanced, with the roles of more genetic modifiers being identified as a deeper understanding of cardiac morphogenesis, gene-to-phenotype pathways from cradle to grave develop, and we refine our global understanding of heart muscle in health and disease.DisclosuresNone.FootnotesThe opinions expressed in this article are not necessarily those of the editors or of the American Heart Association.Correspondence to James C. Moon, MD, MRCP, Heart Hospital, 16-18 Westmoreland St, London W1G 8PH, UK. E-mail [email protected]References1. Teare D. Asymmetrical hypertrophy of the heart in young adults.Br Heart J. 1958; 20:1–8.CrossrefMedlineGoogle Scholar2. Maron MS, Rowin EJ, Lin D, Appelbaum E, Chan RH, Gibson CM, Lesser JR, Lindberg J, Haas TS, Udelson JE, Manning WJ, Maron BJ. Prevalence and clinical profile of myocardial crypts in hypertrophic cardiomyopathy.Circ Cardiovasc Imaging. 2012; 5:441–447.LinkGoogle Scholar3. Germans T, Wilde AA, Dijkmans PA, Chai W, Kamp O, Pinto YM, van Rossum AC. Structural abnormalities of the inferoseptal left ventricular wall detected by cardiac magnetic resonance imaging in carriers of hypertrophic cardiomyopathy mutations.J Am Coll Cardiol. 2006; 48:2518–2523.CrossrefMedlineGoogle Scholar4. Chen H, Zhang W, Li D, Cordes TM, Mark Payne R, Shou W. Analysis of ventricular hypertrabeculation and noncompaction using genetically engineered mouse models.Pediatr Cardiol. 2009; 30:626–634.CrossrefMedlineGoogle Scholar5. Johansson B, Maceira AM, Babu-Narayan SV, Moon JC, Pennell DJ, Kilner PJ. Clefts can be seen in the basal inferior wall of the left ventricle and the interventricular septum in healthy volunteers as well as patients by cardiovascular magnetic resonance.J Am Coll Cardiol. 2007; 50:1294–1295.CrossrefMedlineGoogle Scholar6. Kawel N, Nacif M, Arai AE, Gomes AS, Hundley W, Johnson C, Prince MR, Stacey B, Lima JA, Bluemke DA. Trabeculated (non-compacted) and compact myocardium in adults: the multi-ethnic study of atherosclerosis.J Cardiovasc Magn Reson. 2012; 14(Suppl 1):O86.CrossrefMedlineGoogle Scholar7. Healy LJ, Jiang Y, Hsu EW. Quantitative comparison of myocardial fiber structure between mice, rabbit, and sheep using diffusion tensor cardiovascular magnetic resonance.J Cardiovasc Magn Reson. 2011; 13:74.Google Scholar Previous Back to top Next FiguresReferencesRelatedDetailsCited By Sanna G, Piga A, Parodi G, Sinagra G, Papadakis M, Pantazis A, Sharma S, Gati S and Finocchiaro G (2022) The Electrocardiogram in the Diagnosis and Management of Patients With Left Ventricular Non-Compaction, Current Heart Failure Reports, 10.1007/s11897-022-00580-z, 19:6, (476-490), Online publication date: 1-Dec-2022. Ozden Tok Ö, Ikonomidis I, Papadopoulos K, Göktekin Ö, Bingöl G and Di Salvo G (2021) High take off left main coronary artery accompanied by multicryptic left ventricle myocardium detected by cardiac computerized tomography in a young male: case report, BMC Cardiovascular Disorders, 10.1186/s12872-021-02341-7, 21:1, Online publication date: 1-Dec-2021. 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Hoey E, Teoh J, Pakala V and Ganeshan A (2013) Depiction of myocardial crypts in hypertrophic cardiomyopathy by cardiovascular MRI, Postgraduate Medical Journal, 10.1136/postgradmedj-2013-132072, 89:1056, (610-611), Online publication date: 1-Oct-2013. July 2012Vol 5, Issue 4 Advertisement Article InformationMetrics © 2012 American Heart Association, Inc.https://doi.org/10.1161/CIRCIMAGING.112.975888PMID: 22811415 Originally publishedJuly 1, 2012 Keywordshypertrophic cardiomyopathystructureEditorialsmagnetic resonance imagingPDF download Advertisement SubjectsCardiomyopathyComputerized Tomography (CT)Developmental BiologyMyocardial Biology