Myocardial Crypts Research Articles

Myocardial Crypts: An incidental finding in a symptomatic patient with raised troponin

Myocardial Crypts: An incidental finding in a symptomatic patient with raised troponin

Hypertrophic cardiomyopathy: still connecting the dots between genotype and phenotype.

The current era of cardiac magnetic resonance (CMR) imaging has resulted in a resurgence of interest in hypertrophic cardiomyopathy (HCM). We are now much more aware of the various phenotypic expressions of this disease, which can manifest with typical septal thickening or other variants of hypertrophy, such as apical, midwall or concentric. Additionally, CMR has enabled us to appreciate the wide variety of abnormalities in papillary muscle morphology, which in some cases can contribute to left ventricular outflow tract or mid-cavitary level obstruction (1). In some subjects, mitral valve elongation, myocardial crypts, prominent apical trabeculation, papillary muscle thickening (with or without apical displacement) and abnormal chordal attachments may be subtle phenotypic HCM variants, despite normal left ventricular wall thickness. These abnormalities may be symptomatic to varying degrees or remain subclinical and be noted incidentally or on HCM familial screening programs. Whether these subjects will go on to develop progressive left ventricular hypertrophy (LVH) or whether they are manifesting incomplete phenotypic penetrance of the disease process remains unclear.

Prevalence and characteristics of myocardial crypts in Japanese patients referred for cardiovascular magnetic resonance

Background Myocardial crypts are narrow, blood-filled invaginations within the left ventricle (LV) wall and detected by cardiovascular magnetic resonance (CMR). Recent studies show that a higher prevalence of crypts in patients with hypertrophic cardiomyopathy (HCM) and genotype positive but phenotype negative relatives. Other studies show that myocardial crypts are relatively common in the normal population and incidental variants of local myocardial structures. However, these studies were performed in the western countries and the prevalence and kinds of HCM are different between Japanese and Western people. We aimed to investigate the prevalence and characteristics of myocardial crypts in Japanese people by using CMR. Methods We examined retrospectively 266 consecutive patients (mean age 63.0±15.4 years, 65.8% male) referred for CMR. Crypts were defined as >50% invagination into normal myocardium. We performed 1.5T cardiac MRI including conventional cine imaging and late gadolinium enhancement (LGE) imaging. The location and the number of crypts were evaluated by using a 17-segment model. The prevalence of crypts was compared between patients groups. We also investigated the location of LGE, family history and the first recognition of abnormal ECG in the patients with crypts. Results Crypts were identified in 12 patients (4.5%). Among them, ten patients were with HCM (83%) and 1 patient with congenital heart disease and 1 patient with arrhythmia but with normal structural heart. The prevalence of myocardial crypt is significantly higher in patients with HCM (10 of 82: 12.2%) than in patients without HCM (2 of 184: 1.1%) (P=0.0002). In 10 patients with HCM and crypt, 3 patients had apical hypertrophy. There was no significant difference in prevalence of crypts between HCM patients with apical hypertrophy and those without apical hypertrophy (3 of 23:13.0%, 7 of 59: 11.8%, respectively). Eight patients had single crypt and 4 patients had multiple crypts. All patients had at least one crypt at LV basal level and most popular location was LV basal inferior wall (10 patients). LGE was found in 6 patients and all were with HCM. Two patients had LGE in the same segment with crypt. There were 5 patients with HCM with family histories of sudden death or LV hypertrophy or dilated cardiomyopathy, but no patient without HCM had family history. Abnormal ECG findings were detected under age of 20 in four patients with HCM but in no patients without HCM. Conclusions Myocardial crypts were more frequently seen in the patients with HCM and also seen in the patients with other cardiac diseases in Japan. Although genetic tests were not performed in these patients, genetic factors were suggested in the patients with myocardial crypts and HCM. Funding N/A.

Myocardial Crypt in an Asymptomatic Young Athlete: How to Interpret?

Myocardial crypts are extensions of blood signal penetrating the compact myocardium and are considered in literature as either a distinctive cardiac magnetic resonance (CMR) imaging marker for hypertrophic cardiomyopathy or as benign congenital malformations. What if CMR reveals a myocardial crypt in the presence of an altered ECG in an asymptomatic, enlarged young athlete's heart? The illustrated case demonstrates that new insights in CMR can also require further diagnostic interventions, which might have deleterious consequences for the individual athlete because of the uncertain interpretation of some findings in the demanding new world of a rapidly developing diagnostic imaging technique.

Abnormal septal convexity into the left ventricle occurs in subclinical hypertrophic cardiomyopathy.

BackgroundSarcomeric gene mutations cause hypertrophic cardiomyopathy (HCM). In gene mutation carriers without left ventricular (LV) hypertrophy (G + LVH-), subclinical imaging biomarkers are recognized as predictors of overt HCM, consisting of anterior mitral valve leaflet elongation, myocardial crypts, hyperdynamic LV ejection fraction, and abnormal apical trabeculation. Reverse curvature of the interventricular septum (into the LV) is characteristic of overt HCM. We aimed to assess LV septal convexity in subclinical HCM.MethodsCardiovascular magnetic resonance was performed on 36 G + LVH- individuals (31 ± 14 years, 33 % males) with a pathogenic sarcomere mutation, and 36 sex and age-matched healthy controls (33 ± 12 years, 33 % males). Septal convexity (SCx) was measured in the apical four chamber view perpendicular to a reference line connecting the mid-septal wall at tricuspid valve insertion level and the apical right ventricular insertion point.ResultsSeptal convexity was increased in G + LVH- compared to controls (maximal distance of endocardium to reference line: 5.0 ± 2.5 mm vs. 1.6 ± 2.4 mm, p ≤ 0.0001). Expected findings occurred in G + LVH- individuals: longer anterior mitral valve leaflet (23.5 ± 3.0 mm vs. 19.9 ± 3.1 mm, p ≤ 0.0001), higher relative wall thickness (0.31 ± 0.05 vs. 0.29 ± 0.04, p ≤ 0.05), higher LV ejection fraction (70.8 ± 4.3 % vs. 68.3 ± 4.4 %, p ≤ 0.05), and smaller LV end-systolic volume index (21.4 ± 4.4 ml/m2vs. 23.7 ± 5.8 ml/m2, p ≤ 0.05). Other morphologic measurements (LV angles, sphericity index, and eccentricity index) were not different between G + LVH- and controls.ConclusionsSeptal convexity is an additional previously undescribed feature of subclinical HCM.

Prediction of sarcomere mutations in subclinical hypertrophic cardiomyopathy.

Sarcomere protein mutations in hypertrophic cardiomyopathy induce subtle cardiac structural changes before the development of left ventricular hypertrophy (LVH). We have proposed that myocardial crypts are part of this phenotype and independently associated with the presence of sarcomere gene mutations. We tested this hypothesis in genetic hypertrophic cardiomyopathy pre-LVH (genotype positive, LVH negative [G+LVH-]). A multicenter case-control study investigated crypts and 22 other cardiovascular magnetic resonance parameters in subclinical hypertrophic cardiomyopathy to determine their strength of association with sarcomere gene mutation carriage. The G+LVH- sample (n=73) was 29 ± 13 years old and 51% were men. Crypts were related to the presence of sarcomere mutations (for ≥1 crypt, β=2.5; 95% confidence interval [CI], 0.5-4.4; P=0.014 and for ≥2 crypts, β=3.0; 95% CI, 0.8-7.9; P=0.004). In combination with 3 other parameters: anterior mitral valve leaflet elongation (β=2.1; 95% CI, 1.7-3.1; P<0.001), abnormal LV apical trabeculae (β=1.6; 95% CI, 0.8-2.5; P<0.001), and smaller LV end-systolic volumes (β=1.4; 95% CI, 0.5-2.3; P=0.001), multiple crypts indicated the presence of sarcomere gene mutations with 80% accuracy and an area under the curve of 0.85 (95% CI, 0.8-0.9). In this G+LVH- population, cardiac myosin-binding protein C mutation carriers had twice the prevalence of crypts when compared with the other combined mutations (47 versus 23%; odds ratio, 2.9; 95% CI, 1.1-7.9; P=0.045). The subclinical hypertrophic cardiomyopathy phenotype measured by cardiovascular magnetic resonance in a multicenter environment and consisting of crypts (particularly multiple), anterior mitral valve leaflet elongation, abnormal trabeculae, and smaller LV systolic cavity is indicative of the presence of sarcomere gene mutations and highlights the need for further study.

Prevalence of myocardial crypts in a large retrospective cohort study by cardiovascular magnetic resonance.

BackgroundMyocardial crypts are discrete clefts or fissures in otherwise compacted myocardium of the left ventricle (LV). Recent reports suggest a higher prevalence of crypts in patients with hypertrophic cardiomyopathy (HCM) and also within small samples of genotype positive but phenotype negative relatives. The presence of a crypt has been suggested to be a predictor of gene carrier status. However, the prevalence and clinical significance of crypts in the general population is unclear. We aimed to determine the prevalence of myocardial crypts in a large cohort of subjects using clinical cardiovascular magnetic resonance (CMR).MethodsConsecutive subjects referred for clinical CMR during a 12-month period (n = 1020, age 52.6 ± 17, males: 61%) were included. Crypts were defined as >50% invagination into normal myocardium and their overall prevalence, location and shape was investigated and compared between different patient groups.ResultsThe overall prevalence of crypts was 64/1020 (6.3%). In a predefined ‘normal’ control group the prevalence was lower (11/306, 3.6%, p = 0.031), but were equally prevalent in ischemic heart disease (12/236, 5.1%, p = n/s) and the combined non-ischemic cardiomyopathy (NICM) groups (24/373; 6.4%, p = n/s). Within the NICM group, crypts were significantly more common in HCM (9/76, 11.7%, p = 0.04) and hypertensive CM subjects (3/11, 27%, p = 0.03). In patients referred for CMR for family screening of inherited forms of CM, crypts were significantly more prevalent (10/41, 23%, p < 0.001), including a smaller group with a first degree relative with HCM (3/9, 33%, p = 0.01).ConclusionMyocardial crypts are relatively common in the normal population, and increasingly common in HCM and hypertensive cardiomyopathy. Crypts are also more frequently seen in normal phenotype subjects referred because of a family history of an inherited cardiomyopathy and HCM specifically. It is uncertain what the significance of crypts are in this group, and because of variability in the imaging protocols used and their relative frequency within the normal population, should not be used to clinically stratify these patients. Prospective studies are required to confirm the clinical significance of myocardial crypts, as their significance remains unclear.Electronic supplementary materialThe online version of this article (doi:10.1186/s12968-014-0066-0) contains supplementary material, which is available to authorized users.

Myocardial Clefts, Crypts, or Crevices

With the increasing accuracy of cardiac imaging techniques, new findings, not or rarely observed before, come to light. In particular, the improvement in high spatial resolution of a multiplanar imaging technique such as cardiac magnetic resonance (CMR), associated with its increased contrast between blood and endocardial border surface of current cine images, led the appreciation of subtle left ventricular (LV) wall structural features, not seen or neglected by 2-dimensional echocardiography. This is the case of myocardial clefts, described both in hypertrophic cardiomyopathy (HCM) and in healthy volunteers, thus providing another hot potato to the hands of the clinical cardiologist. Article see p 259 These myocardial invaginations have been variably called clefts, crypts, fissures, crevices, and atypical LV noncompaction. Although the etiopathogenesis remains a matter of debate, it is a common belief that they represent a failure to resorb the trabeculated part of ventricular wall during normal embryological development, as seen in isolated LV noncompaction.1,2 Before the development of coronary circulation, the myocardium consists of a spongy meshwork of interwoven myocardial fibers organized into trabeculae with deep recesses, the latter communicating with the LV cavity and enabling a direct blood supply to the myocardium. Myocardial crypts have been described within the LV wall of patients with HCM since early postmortem studies.3–5 In particular, to the best of our knowledge, the first pathological description of a myocardial cleft dates back to 1958, in the historical publication of Donald Teare.3 Noteworthy, Teare …

Prevalence of Inferobasal Myocardial Crypts Among Patients Referred for Cardiovascular Magnetic Resonance

Crypts or clefts in the left ventricular inferobasal myocardium have been detected by cardiovascular magnetic resonance (CMR), but the extent to which they represent prephenotypic markers of hypertrophic cardiomyopathy (HCM) or incidental structural variants remains controversial. We examined retrospectively the routine vertical long-axis cines in 686 consecutive patients (48±20 years, 55% men) referred for CMR. Crypts were identified in 46 (6.7%), 17 being among patients (8.7% of 196) with otherwise normal CMR findings and without a known family history of HCM. Higher percentages were found in patients with HCM (16%), myocarditis (15%), and hypertension (14%) but without reaching statistical significance (P=0.12). Only 1 (5%) of 20 phenotype-negative HCM family members had a visible crypt. Relative to those without, patients with crypts had lower indexed left ventricular end-systolic volumes (P=0.042) and higher indexed left and right ventricular stroke volumes (P=0.007 and P=0.015) and ejection fractions (P=0.003 and P=0.021). Crypts tended to narrow in systole, varying slightly in size, shape- and number, without obvious group-related features. Single or paired inferobasal myocardial crypts were an occasional and by no means rare finding among patients referred for CMR without a pretest suspicion of HCM. This, together with similar previous findings in a cohort of healthy volunteers, supports their being regarded, in such individuals, as incidental variants of local myocardial structure, unlikely to require further investigation. However, a larger registry-type study may be justified to investigate the clinical implications of multiple crypts, especially if associated with HCM family history.

An unusual presentation of a myocardial crypt in hypertrophic cardiomyopathy.

Hypertrophic cardiomyopathy (HCM) is a common inherited cardiovascular disease with prevalence of 0.2% in the population. More than 1000 mutations in more than 10 genes encoding for proteins of the cardiac sarcomere have been identified. Cardiac magnetic resonance imaging (CMR) is used to characterize left ventricular morphology with great precision in patients with HCM and it identifies unique structural abnormalities in patients with HCM. We present a case of a 56-year-old man who had positive family history of HCM who was a carrier of the genetic MYH-7 2770 G > C, exon 23 mutation. Transthoracic echocardiography showed thickening of the interventricular septum (16 mm) and in particular the basal septum. CMR confirmed the diagnosis of HCM in the anteroseptal myocardium with a thickness of 23 mm and also revealed large and deep myocardial crypts in the anterior wall. These myocardial crypts are rarely found in the so-called genotype positive and phenotype positive patients, as in our case. Also the crypts in this case are deeper and wider than those reported in other cases. So in conclusion, this case reveals an uncommon finding of a myocardial crypt at an unusual myocardial site with the unusual morphology in a patient with genotypic and phenotypic expression of hypertrophic cardiomyopathy.

Circulation: Cardiovascular Imaging Editors’ Picks

<i>Circulation: Cardiovascular Imaging</i> Editors’ Picks

Prevalence of myocardial crypts in a cardiac magnetic resonance population - a large cohort study

Background Myocardial crypts are discrete clefts or fissures in otherwise compacted myocardium of the left ventricle (LV) commonly located in basal inferior or inferoseptal segments. Few small studies reported higher prevalence of crypts in patients with hypertrophic cardiomyopathy (HCM) or their unexpressed relatives, and linked crypts to a marker of genotype positive phenotype negative carrier status. The exact clinical significance of crypts is unclear. We assessed the prevalence of myocardial crypts seen in consecutive subjects referred for cardiac magnetic resonance (CMR). Methods We analyzed a cohort of consecutive subjects referred for clinical CMR during a period of 9 months (n=730, males: 443). Based on pre-test probability and CMR findings (low pretest probability and normal findings vs. high pretest probability and ischemic or non-ischemic cardiomyopathy), 3 groups were formed and examined for the presence of myocardial crypts. The non-ischemic cardiomyopathy group was further stratified into subgroups of identified etiology. Myocardial crypts were described according to their shape (i-, u- and v-form), depth (30-50%, 50-70%, >70% of myocardial diameter) and the segment location. Results Overall, myocardial crypts were identified in 136 subjects (19%) with no gender difference (males: n=81, 18%; females: 55, 19%, p=0.78). Crypts were similarly proportioned in normal group (n=44,17%) and those with ischemic (n=37,22%) or non-ischemic cardiomyopathy (n=55,18%) (p=0.40). In the latter group, crypts were most common in subjects with HCM (n=19/62, 31%) again p - value against the other groups. In relatives to the patients with confirmed genetic cardiomyopathy only 11% crypts were found. Crypts most commonly presented by the u-shape (45%), followed by the v(40%) and i - shape (15%). Sixty-percent of crypts were 30-50% deep, followed by 50-70% (35%) and 5 % > 70% myocardial diameter. Most of the myocardial crypts could be identified in the basal inferior segment (49%). In twenty percent, crypts were multiple, with no difference in representation in health or disease (p=0.22-0.89). Conclusions In a large study cohort we demonstrate that myocardial crypts are similarly prevalent in subjects with and without cardiomyopathy. A subanalysis of non-ischaemic group reveals disproportionate representation with bias towards HCM. Contrasting previous reports, crypts were

Response to Letter Regarding Article, “Prevalence and Clinical Profile of Myocardial Crypts in Hypertrophic Cardiomyopathy”

We appreciate the comments by Puntmann et al on our manuscript.1 The authors suggest that myocardial crypts are a common morphologic finding in patients studied with cardiovascular magnetic resonance (CMR) including those with congenital heart disease and even in normal volunteers. On this point, we agree that myocardial crypts do not appear to be restricted to hypertrophic cardiomyopathy (HCM),2 although as we have suggested, additional systematic CMR-based studies in larger populations are necessary to precisely define the true prevalence of myocardial crypts in other forms of cardiovascular disease. In addition, apparent discrepancies noted with regard to the prevalence of crypts in these other populations may also simply be due …

Myocardial Crypts

It 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. …

Prevalence and Clinical Profile of Myocardial Crypts in Hypertrophic Cardiomyopathy

In hypertrophic cardiomyopathy (HCM), cardiovascular MR can detect morphological abnormalities of the left ventricle (LV) not visualized with echocardiography. Although myocardial crypts (ie, narrow, blood-filled invaginations within the LV wall) have been recognized in HCM, all clinical implications of these structural abnormalities within the broad clinical HCM spectrum are not completely resolved. Therefore, we sought to characterize the prevalence and diagnostic significance of myocardial crypts in HCM patients. Cine and late gadolinium enhancement cardiovascular MR and 2-dimensional echocardiography were obtained in 292 consecutive patients with HCM including 31 genotype-positive/phenotype-negative family members without LV hypertrophy (28 ± 16 years; 51% male) and 261 patients with LV hypertrophy (46 ± 18 years; 60% male). Ninety-eight subjects without cardiovascular disease were controls. Myocardial crypts (1-6/patient) were identified only by cardiovascular MR in 19 of 31 genotype-positive/phenotype-negative patients (61%) compared with only 10 of 261 (4%) patients with HCM with LV hypertrophy (P<0.001) and were absent in control subjects. Twelve-lead electrocardiograms were normal in 10 (53%) of the genotype-positive/phenotype-negative patients with crypts. Crypts were confined to the basal LV, most commonly in the ventricular septum (n=21) or posterior LV free wall (n=4), and associated with normal LV contractility and absence of late gadolinium enhancement in all but one patient. LV myocardial crypts represent a distinctive morphological expression of HCM, occurring with different frequency in HCM patients with or without LV hypertrophy. Crypts are a novel cardiovascular MR imaging marker, which may identify individual HCM family members who should also be considered for diagnostic genetic testing. These data support an expanded role for cardiovascular MR in early evaluation of HCM families.

Myocardial crypts in hypertrophic cardiomyopathy: the new gang in town

This editorial refers to ‘Multiple myocardial crypts on modified long-axis view are a specific finding in pre-hypertrophic HCM mutation carriers’ by W.P. Brouwer et al ., on pages 292–297. Since the initial description of hypertrophic cardiomyopathy (HCM) over 50 years ago, the majority of our understanding of this complex and heterogeneous genetic heart disease has been the result of insights gained through advances in cardiovascular imaging techniques.1–5 In the pre-echocardiographic era, the haemodynamic laboratory provided the sole method of diagnosis for HCM by demonstrating a pressure gradient between the left ventricular cavity and the aorta.1,2 The introduction of echocardiographic imaging in the early 1970s led to a greatly expanded appreciation for the vast heterogeneity of phenotypic expression in this disease, including the first non-invasive demonstration of the characteristic asymmetric pattern of LV hypertrophy, as well as defining the non-obstructive form as an important subset of the HCM disease spectrum.1,2 More recently, advanced imaging techniques such as cardiovascular magnetic resonance (CMR) have emerged, ushering in a new era of cardiac imaging.3–5 CMR provides three-dimensional tomographic images with high spatial resolution, with sharp contrast between the blood pool and the myocardium. These unique imaging strengths make it particularly well suited to characterize the diverse morphological expression of HCM.3 Indeed, CMR has now been applied to large cohorts of HCM patients resulting in a number of clinically relevant observations, further expanding our appreciation and understanding of HCM disease expression beyond traditional imaging modalities.3–7 CMR improves diagnosis of HCM, by detecting LV hypertrophy unrecognized (or not well seen) by echocardiography, particularly when confined to the anterolateral free wall (or apex).3–5 Furthermore, in some HCM patients, the magnitude of wall thickening may be underestimated by echocardiography, which …

Multiple myocardial crypts on modified long-axis view are a specific finding in pre-hypertrophic HCM mutation carriers

Crypts can be found with cardiovascular magnetic resonance imaging (CMR) in hypertrophic cardiomyopathy (HCM) mutation carriers without hypertrophy (carriers) using a modified two-chamber view through the inferoseptum, but also in other patients and healthy individuals with standard long-axis views. Since it is currently unknown if carriers display a specific crypt morphology, we compared crypts in carriers with other cardiac pathologies (controls). Besides, we aimed to determine the optimal imaging plane for the detection of crypts by comparing modified two-chamber views with standard long-axis views. Finally, we evaluated the accuracy of crypts to identify carriers in HCM family screening. Standard CMR long-axis views with additional modified two-chamber views were prospectively performed in carriers (n= 43), consecutive CMR control patients (n= 252), and mutation-negative family members (n= 15). Crypts were found in 70% (30/43) of carriers and in 12% (31/252) of controls (P< 0.001). Crypts in carriers showed deeper penetrance into the myocardium compared with controls (74 ± 21% vs. 59 ± 22%, P< 0.01). Detection of two or more crypts had a sensitivity of 51% and specificity of 94% for carriership. Modified two-chamber views doubled the sensitivity to detect crypts compared with standard long-axis views. In family screening, ≥2 crypts had a 100% positive predictive value to identify carriers. Multiple crypts in the absence of left ventricular hypertrophy are highly specific for HCM mutation carriership and warrant clinical follow-up. A modified two-chamber view has a superior sensitivity compared with standard long-axis views for crypt detection. CMR may be of additional value to identify carriers in family screening.

Clinical utility of cardiovascular magnetic resonance in hypertrophic cardiomyopathy.

Hypertrophic cardiomyopathy (HCM) is characterized by substantial genetic and phenotypic heterogeneity, leading to considerable diversity in clinical course including the most common cause of sudden death in young people and a determinant of heart failure symptoms in patients of any age. Traditionally, two-dimensional echocardiography has been the most reliable method for establishing a clinical diagnosis of HCM. However, cardiovascular magnetic resonance (CMR), with its high spatial resolution and tomographic imaging capability, has emerged as a technique particularly well suited to characterize the diverse phenotypic expression of this complex disease. For example, CMR is often superior to echocardiography for HCM diagnosis, by identifying areas of segmental hypertrophy (ie., anterolateral wall or apex) not reliably visualized by echocardiography (or underestimated in terms of extent). High-risk HCM patient subgroups identified with CMR include those with thin-walled scarred LV apical aneurysms (which prior to CMR imaging in HCM remained largely undetected), end-stage systolic dysfunction, and massive LV hypertrophy. CMR observations also suggest that the cardiomyopathic process in HCM is more diffuse than previously regarded, extending beyond the LV myocardium to include thickening of the right ventricular wall as well as substantial morphologic diversity with regard to papillary muscles and mitral valve. These findings have implications for management strategies in patients undergoing invasive septal reduction therapy. Among HCM family members, CMR has identified unique phenotypic markers of affected genetic status in the absence of LV hypertrophy including: myocardial crypts, elongated mitral valve leaflets and late gadolinium enhancement.The unique capability of contrast-enhanced CMR with late gadolinium enhancement to identify myocardial fibrosis has raised the expectation that this may represent a novel marker, which may enhance risk stratification. At this time, late gadolinium enhancement appears to be an important determinant of adverse LV remodeling associated with systolic dysfunction. However, the predictive significance of LGE for sudden death is incompletely resolved and ultimately future large prospective studies may provide greater insights into this issue. These observations underscore an important role for CMR in the contemporary assessment of patients with HCM, providing important information impacting diagnosis and clinical management strategies.

Multiple myocardial crypts on cardiac CT angiography in a symptomatic patient with troponin elevation

A 57-year-old Middle Eastern woman presented to the emergencydepartment(ED)withleftarmandchestdiscomfortwhichdevelopedwhile driving her car. The discomfort lasted for a few minutes andresolved spontaneously. Past medical history was remarkable forknown LVH by ECG, severe concentric LVH by echocardiography andno coronary heart disease risk factors. ECG in the ED showed LVH andST segment depression in leads V4 through V6 and was unchangedfrom prior ECG done 1 year before. Serum troponin I was elevated at0.16 ng/ml (normal b0.05 ng/ml). Based on the clinical history,abnormal ECG and elevated troponin levels, the ED physicianconsidered the diagnosis of an acute coronary syndrome, adminis-teredaspirinandclopidogrelandrecommendedadmissionforfurtherobservation and management. Cardiology consultation was thensought in the ED, and in view of previously known diagnosis ofhypertrophic cardiomyopathy, immediate coronary CT angiography(CCTA) was recommended to rule out obstructive coronary arterydisease.Low dose CCTA (4.9 mSv) was performed using a 64 slice dual-source CT scanner (Somatom, Siemens Medical Solutions, Forchheim,Germany).CCTArevealednoevidenceforcoronaryplaqueorstenosis.Despite the positive troponin and the abnormal ECG, a diagnosis ofacute coronary syndrome was excluded, and the patient wasdischarged from the ED.Non-coronary assessment of the CCTA confirmed severe asym-metric septal hypertrophy and demonstrated an unusual finding ofthree thin crypts in the left ventricle. The largest crypt was 12 mm inlength in the superior mid septum at the junction of the left and rightventricles with a 2 mm separation from the right ventricle (Fig. 1 a

Hypertrophic Cardiomyopathy With Restrictive Phenotype and Myocardial Crypts

This report describes a 22-year-old woman who has clinical and physiologic features of a restrictive cardiomyopathy. Magnetic resonance imaging showed myocardial delayed enhancement and interventricular septal crypts characteristic of hypertrophic cardiomyopathy (HCM). Transcatheter biopsy confirmed the diagnosis, revealing marked myocyte hypertrophy, interstitial fibrosis, and fiber disarray, which are findings consistent with HCM. A review of the literature suggests that this is the first case of HCM reported with a restrictive pattern and myocardial crypts.