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(Circulation. 2007;115:e610-e611.)
© 2007 American Heart Association, Inc.

Images in Cardiovascular Medicine

Prominent Crypt Formation in the Inferoseptum of a Hypertrophic Cardiomyopathy Mutation Carrier Mimics Noncompaction Cardiomyopathy

Tjeerd Germans, MD; Pieter A. Dijkmans, MD; Arthur A.M. Wilde, MD, PhD; Otto Kamp, MD, PhD; Albert C. van Rossum, MD, PhD

From the Department of Cardiology, VU University Medical Center (T.G., P.A.D., O.K., A.C.v.R.), Amsterdam; Department of Cardiology (A.A.M.W.), Academic Medical Center, Amsterdam; and Interuniversity Cardiology Institute of the Netherlands (T.G., A.A.M.W., O.K., A.C.V.R.), Utrecht, the Netherlands.

Correspondence to Tjeerd Germans, MD, Department of Cardiology, VU University Medical Center, De Boelelaan 1117, 1081 HV Amsterdam, The Netherlands. E-mail T.Germans{at}vumc.nl

Recently, we reported that crypts can be identified in the inferior part of the septum by cardiac magnetic resonance (CMR) imaging in most carriers of hypertrophic cardiomyopathy mutations (HCM) who have not yet developed hypertrophy.¹ The crypts may be an early sign of maladaptive myocardial alterations that ultimately lead to the development of the characteristic hypertrophy in HCM patients.

In this patient, a 30-year-old asymptomatic female HCM carrier with a maximal septal wall thickness of 6 mm, the crypts were very prominent in the inferoseptum (Figure 1) and consequently were visible with echocardiography (Figure 2A and 2B). On both the CMR and echocardiographic images, the crypts were identified within the basal inferoseptum (Figure 2A and 2B and Movies I and II); flow in the crypts also was observed (Figure 2C and Movie III). No contrast enhancement was visible within the myocardium on delayed contrast-enhanced CMR images.

View larger version (126K): [in this window] [in a new window]   Figure 1. End-diastolic short-axis CMR image showing a hypertrophic cardiomyopathy mutation carrier. Note the triangular blood-containing bright spot in the inferoseptum through which a dedicated 2-chamber view is planned (dotted line).

View larger version (62K): [in this window] [in a new window]   Figure 2. CMR cine showing a dedicated 2-chamber view of a hypertrophic cardiomyopathy mutation carrier (A). Black arrowheads indicate crypts in the inferoseptum. These crypts also can be observed with 2-dimensional echocardiography, as indicated by the white arrowheads (B). Blood flow in the crypts (blue) can be visualized with color Doppler echocardiography (white arrowheads in C).

When crypt formation is very prominent in an HCM carrier, as in this patient, the cardiac image mimics the image of noncompaction cardiomyopathy on echocardiography.² However, crypt formation as part of the development of HCM can be distinguished from noncompaction cardiomyopathy on the basis of several criteria, First, noncompaction generally is located in the apical and/or lateral segments of the left ventricle and very rarely in the basal inferoseptum. Second, the crypts penetrate compact myocardium sometimes up to the epicardial myocardial layer, in contrast to noncompaction cardiomyopathy, in which a noncompacted layer of myocardium parallels a compact layer alongside the ventricular cavity. Third, the crypt formation does not seem to affect global left ventricular contractile function, whereas in noncompaction cardiomyopathy, patients may have a dilated and poorly contracting left ventricle.³

Distinguishing between these 2 cardiomyopathies has important clinical therapeutic implications because HCM carriers may remain asymptomatic for decades and patients with noncompaction cardiomyopathy are at increased risk of thromboembolic events and supraventricular arrhythmias when heart failure is present.³ However, the very recent discovery of the crypts in HCM carriers probably means that HCM carriers have been misdiagnosed in the past as having some form of noncompaction cardiomyopathy.

In conclusion, if crypts are very prominent in HCM carriers, they also can be observed with echocardiography on a dedicated apical 2-chamber view through the inferoseptum but must be carefully distinguished from noncompaction cardiomyopathy.

	Acknowledgments

 
We gratefully acknowledge Milan van Tuin for acquiring the CMR images and Yolanda de Groot for making the echocardiography images.

Sources of Funding

Dr Germans (grant 2006B213), Dr Wilde (grant 2003T302), and Dr van Rossum (2006B213) have received research grants from the Netherlands Heart Foundation.

Disclosures

None.

	Footnotes

 
The online-only Data Supplement, consisting of Movies I-III, is available with this article at http://circ.ahajournals.org/cgi/content/full/115/24/e610/DC1.

	References

Top References

 

1. 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.[Abstract/Free Full Text]
   
2. Jenni R, Oechslin E, Schneider J, Jost CA, Kaufmann PA. Echocardiographic and pathoanatomical characteristics of isolated left ventricular non-compaction: a step towards classification as a distinct cardiomyopathy. Heart. 2001; 86: 666–671.[Abstract/Free Full Text]
   
3. Stollberger C, Finsterer J. Left ventricular hypertrabeculation/noncompaction and stroke or embolism. Cardiology. 2005; 103: 68–72.[CrossRef][Medline] [Order article via Infotrieve]
   

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