Patterns of late gadolinium enhancement (LGE) in hypertrophic cardiomyopathy (HCM): spectrum of findings

Transcription

1 Patterns of late gadolinium enhancement (LGE) in hypertrophic cardiomyopathy (HCM): spectrum of findings Poster No.: 482 Congress: ESCR 2013 Type: Authors: Keywords: Poster Presentation E. Pershina, O. Larina, E. A. Mershina, V. Sinitsyn; Moscow/RU Cardiac, MR, CT, Echocardiography, Diagnostic procedure, Hyperplasia / Hypertrophy, Education and training, Image verification Any information contained in this pdf file is automatically generated from digital material submitted to EPOS by third parties in the form of scientific presentations. References to any names, marks, products, or services of third parties or hypertext links to thirdparty sites or information are provided solely as a convenience to you and do not in any way constitute or imply ECR's endorsement, sponsorship or recommendation of the third party, information, product or service. ECR is not responsible for the content of these pages and does not make any representations regarding the content or accuracy of material in this file. As per copyright regulations, any unauthorised use of the material or parts thereof as well as commercial reproduction or multiple distribution by any traditional or electronically based reproduction/publication method ist strictly prohibited. You agree to defend, indemnify, and hold ECR harmless from and against any and all claims, damages, costs, and expenses, including attorneys' fees, arising from or related to your use of these pages. Please note: Links to movies, ppt slideshows and any other multimedia files are not available in the pdf version of presentations. Page 1 of 17

2 Purpose Hypertrophic cardiomyopathy (HCM) is one of the most common genetic disorders of the heart. HCM is characterized by a wide range of clinical features, ranging from asymptomatic mutation in genes encoding sarcomeric proteins to sudden cardiac death as the first manifestation of the disease. #ardiovascular magnetic resonance (CMR) is increasingly used to characterize morphologic, functional and tissue abnormalities associated with HCM because of its precise determination of myocardial anatomy and the depiction of myocardial fibrosis. Several morphological variants of HCM that can be identified by cardiac CMR have been described: asymmetric HCM with sigmoid septal contour ("septal HCM"), asymmetric HCM with reversed septal contour, HCM with mid-ventricular obstruction (with or without a LV apical diverticulum), apical HCM, symmetric (concentric) HCM and focal HCM [1,2] - Fig.1. Hystological markers include myocyte and myofibrillar disarray, myocyte hypertrophy, fibrosis replacement, dysplasia of small arteries, seen as medial and intimal smooth muscle cell proliferation with luminal narrowing, reduced arteriolar density wich lead to small-vessel intramural coronary artery disease (SICAD) as an early manifestation of HCM [2]. Late gadolinium enhancement at CMR is present in approximately 60% of HCM patients with left ventricular hypertrophy and may provide information regarding risk stratification in HCM. LGE in HCM appears as patchy mid-wall hyperenhancement in the most thickened segments and at the junction of the septum to the anterior and posterior walls of LV (Fig.2). Our routine practice revealed that atypical patterns of LGE could be also visualized in patients with HCM. The purpose of our study was to provide physicians with a review of atypical pattern of LGE in HCM that could mimic ischemical, inflammatory systemic or metabolic diseases. Images for this section: Page 2 of 17

3 Fig. 1: Left ventricular variants HCM: (A, a) normal LV, (B, b) sigmoid septum showing SAM of mitral valve (white arrow), (C, c) reversed septal contour, note that there is no signs of LVOT, (D, d) mid ventricular hypertrophy, (E, e) Apical HCM, (F, f) symmetric HCM. Page 3 of 17

4 Fig. 2: Patchy mid-wall hyperenhancement in the most thickened segments Page 4 of 17

5 Methods and Materials 21 patients (m/f-11/10, age yrs, mean age ±15,9 yrs) were diagnosed with HCM in our department in 2012: 8 - asymmetric septal hypertrophy (ASH) with sigmoid septal contour and outflow tract obstruction, 6 patients - ASH with reversed septal contour without outflow tract obstruction, 4 - apical form, 2 - midventricular form and 1 - focal HCM. CMR protocol was performed at 1.5T scanner (Magnetom Avanto, Siemens AG) using multichannel surface coil with cardiac synchronization. Breath-hold cine images were acquired with fast imaging using steady-state precession technique (TrueFISP). Late gadolinium enhanced (LGE) images were obtained minutes after intravenous injection of contrast medium. Segmented inversion-recovery breath-hold T1-sequence was used. Time of inversion was adjusted for each patient between 200 and 350 (more often ) to achieve the optimal suppression of normal myocardium. Left ventricle was examined in vertical long, horizontal long and short axis positions. The location, extent and enhancement pattern of hyperenhanced myocardium were analyzed in a 17-segment AHA model. Cine MR-images were quantitatively evaluated using Siemens cardiac image analysis software (ARGUS). Echocardiogram was made in 3 cases when structural abnormalities were detected with CMR to quantify mid-ventricular and subaortic gradient of obstruction. MDCT coronary angiography was performed at 64-slice scanner (Discovery CT750 HD, GE) in 2 pts with ischemical patterns of LGE to exclude coronary stenoses. Chest CT was performed in cases with LGE in non-hypertrophied segment to exclude systemic sarcoidosis. Results Focuses of LGE were determined in 13 of 21 patients (61,9%). 9 patients had small punctuate, patchy mid-wall hyper-enhancement - typical patterns of LGE corresponded to the most hypertrophied part of the myocardium and at the junction of the septum to the anterior and posterior walls of LV (Fig.2) 4 of 13 patients with LGE focuses (30%) had atypical pattern of contrast-enhancement. 2 patients had subendocardial type of enhancement mimicked ischemical disorders. No significant coronary stenoses were found in both cases. Page 5 of 17

6 - 1 st patient was diagnosed with mid-ventricular HCM (myocardial mass g, thickness of anterobasal septum - 21 mm and mid-vetricular septum - 23 mm) - Fig. 4 on page 7. This patient had transmural ischemic pattern with thinning of the apex wall, apical aneurism of LV and small apical thrombus without enhancement in the most hypertrophied middle segments of myocardium (Fig. 5 on page 8, Fig. 6 on page 9). "Freeze" ECG-feature of apical aneurism (Fig. 3 on page 7) and midventricular systolic gradient measured 20mmHg by Echo was found. - 2 nd patient was diagnosed with apical HCM (myocardial mass - 215g, thickness of anterobasal septum - 20 mm and mid-ventricular septum - 26 mm). This patient had circular subendocardial (10-15% of myocardial thickness) hyperenhancement in the most hypertrophied apical segments. Corresponded perfusion deficit was found by CT (Fig. 7 on page 10. Fig. 8 on page 11). The ischemical pattern of hyperenhancement in these cases may be explained by dysplasia of small arteries, reduced arteriolar density, small vessel intramural coronary artery disease (SICAD). The apical chamber is a subject to greater and sustained systolic stress due to the high mid-ventricular gradient [4]. The apical aneurysm («burned-out apex») observed in this patient could appear due to microvascular dysfunction and mid-ventricular obstruction. 3 rd patient with ASH had curved septal contour and no outflow tract obstruction (myocardial mass - 301g, the anteromiddle septum measured 17 mm and mid-vetricular septum mesuard 24 mm). A patchy mid-wall hyperenhancement in the non-most-hypertrophied left ventricular arterial segments was observed and absence of hyperenhancement in the most hypertrophied mid-ventricular segments was noted (Fig. 9 on page 12, Fig. 10 on page 13) Late enhancement of the non-hypertrophied segments in HCM should not be considered exceptional, since the myopathic process (i.e. disarray, fibrosis or necrosis) affects the entire left ventricle irrespectively of the wall thickness [6]. 4 th pateint with ASH had curved septal contour and no outflow tract obstruction (myocardial mass - 199g, basal mid-vetricular segment mesuared 16 mm, middle - 18 mm, apical -14 mm). This patient had subepicardial hyperenchancement in apical and posterobasal segments. The ejection fraction was about 42%. Despite of myocardium mass increase eventually it was considered as inflammatory disorder and patient was diagnosed with myocarditis (Fig. 11 on page 14). This example of Page 6 of 17

7 atypical hyperenhancement should be consider in the framework of HCM pathogenesis too. Images for this section: Fig. 3: "Freeze" ECG-feature of apical aneurism localisation, invrsed (T) in V4-V6 Page 7 of 17

8 Fig. 4: Mid-ventricular HCM: anterobasal septum 21 mm, mid-vetricular septum 23 mm Page 8 of 17

9 Fig. 5: "Burned-out apex": transmural ischemic pattern with thinning of the apex wall, apical aneurism of LV and small apical thrombus Page 9 of 17

10 Fig. 6: The apical aneurysm («burned-out apex») with thrombus. Page 10 of 17

11 Fig. 7: Circular subendocardial hyperenhancement in the most hypertrophied apical segments Page 11 of 17

12 Fig. 8: Subendocardial hyperenhancement in the most hypertrophied apical segments Page 12 of 17

13 Fig. 9: Hyperenhancement in the non-most-hypertrophied left ventricular anteromiddle segment and absence of hyperenhancement in the most hypertrophied mid-ventricular segments Page 13 of 17

14 Fig. 10: Hyperenhancement in the non-most hypertrophied left ventricular arterial segments and absence of hyperenhancement in the most hypertrophied mid-ventricular segments Page 14 of 17

15 Fig. 11: Inflammatory pattern of hyperenhancement: epicardial hyperenhancement in midventricular septum Page 15 of 17

16 Conclusion It is known that increased LV mass and focuses of late gadolinium enhancement could be associated with increased possibility of future adverse cardiovascular events in patients with HCM. CMR appears to be highly relevant diagnostic tool in clinical and preclinical HCM. Our overview observed several atypical patterns of LGE in HCM, which could be confused with ischemical, inflammatory systemic or metabolic disorders, but should be assessed in the pathogenesis of HCM. References 1. Radwa A Noureldin, Songtao Liu, Marcelo S Nacif, Daniel P Judge, Marc K Halushka, Theodore P Abraham, Carolyn Ho, David A Bluemke. The diagnosis of cardiomyopathy by cardiovascular magnetic resonance. Journal of Cardiovascular Magnetic Resonance Mark W. Hansen, Naeem Merchant. MRI of Hypertrophic Cardiomyopathy: Part 2, Differential Diagnosis, Risk Stratification, and Posttreatment MRI Appearances. American Journal of Roentgenology (2007)189:6, doi/suppl/ /ajr Mark W. Hansen, Naeem Merchant. MRI of Hypertrophic Cardiomyopathy: Part 2, Differential Diagnosis, Risk Stratification, and Posttreatment MRI Appearances. American Journal of Roentgenology (2007)189:6, Francesco De Cobelli, Antonio Esposito, Gianluca Perseghin, Claudio Sallemi, Elena Belloni, Silvia Ravelli, Chiara Lanzani, Alessandro Del Maschio. Intraindividual Comparison of Gadobutrol and Gadopentetate Dimeglumine for Detection of Myocardial Late Enhancement in Cardiac MRI. American Journal of Roentgenology (2012) 198:4, Tomás F Cianciulli, María C Saccheri, Isabel V Konopka, Dora F Serans,Rafael S Acunzo, Alejandro MG Escudero, Osvaldo H Masoli and Horacio A Prezioso. Subaortic Page 16 of 17

17 and mid-ventricular obstructive hypertrophic cardiomyopathy with an apical Aneurysm: a case report 5. Jan Bogaert1, Marcelo Goldstein, Fadi Tannouri, Jafar Golzarian and Steven Dymarkowski Late Myocardial Enhancement in Hypertrophic Cardiomyopathy with Contrast-Enhanced MR Imaging 6. K. Efyhimidis, P. Spanos,Giannakoulas.Hypertrophic Cardiomyopathy with Late Enhancement of the Non-Hypertrophied Left Ventricular Segments.Hellenic J Cardiol 49: , 2008 Personal Information Page 17 of 17