BACKGROUND: 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. METHODS AND RESULTS: 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. CONCLUSIONS: 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.
BACKGROUND: 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. METHODS AND RESULTS: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. CONCLUSIONS: 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.
Authors: Christoph Langer; M Both; H Harders; M Lutz; M Eden; C Kühl; B Sattler; O Jansen; P Schaefer; N Frey Journal: Eur Radiol Date: 2014-10-15 Impact factor: 5.315
Authors: Jolanda van der Velden; Carolyn Y Ho; Jil C Tardiff; Iacopo Olivotto; Bjorn C Knollmann; Lucie Carrier Journal: Cardiovasc Res Date: 2015-01-28 Impact factor: 10.787
Authors: Carolyn Y Ho; Sharlene M Day; Steven D Colan; Mark W Russell; Jeffrey A Towbin; Mark V Sherrid; Charles E Canter; John L Jefferies; Anne M Murphy; Allison L Cirino; Theodore P Abraham; Matthew Taylor; Luisa Mestroni; David A Bluemke; Petr Jarolim; Ling Shi; Lynn A Sleeper; Christine E Seidman; E John Orav Journal: JAMA Cardiol Date: 2017-04-01 Impact factor: 14.676