Cardiac Multislice Computed Tomography in the Detection of Phenotypic Polymorphism of Hypertrophic Cardiomyopathy
Background. Hypertrophic cardiomyopathy (HCM) is an autosomal dominant disease with a prevalence of 1 case per 500 people and is the most common cause of sudden cardiac death in young patients. As clinical manifestations and electrocardiographic data are nonspecific and diverse, noninvasive imaging techniques play a key role in the detection of HCM and the understanding of its pathophysiology.
The aim. To evaluate the possibilities of ECG-synchronized cardiac multislice computed tomography (MSCT) as a highly informative diagnostic tool for assessing the morpho-functional state of the heart in patients with HCM.
Materials and methods. This was a retrospective analysis conducted at the National Amosov Institute of Cardiovascular Surgery from January 2020 to December 2021. We examined 221 cardiac MSCT scans of patients who underwent the examination to assess the spread of myocardial hypertrophy. Particular attention was paid to the presence of crypts at different levels of the left ventricle (LV), anatomical features of the mitral valve and subvalvular apparatus. The presence of systolic pulling of the anterior mitral valve to the interventricular septum, myocardial mass, LV end-diastolic, LV end-systolic volumes and the corresponding indices of body surface area, ejection fraction were determined and calculated during the functional analysis. Additionally, the anatomy and patency of the coronary arte ries were assessed. The studies were performed on a 640-slice Canon Aquilion One CT scanner with retrospective ECG gating and subsequent image processing. The studies were transferred to a workstation for review and evaluation by a team of radiologists.
Results. The mean patient age was 46 ± 23 years, 48% were male. Mean maximal LV wall thickness was 19 mm (range 16–34). In 159 patients (71.9%), there was an asymmetric form of HCM with a predominant thickening of the anterior and anteroseptal segments of the left ventricle at the basal and midventricular levels. Fifty-four (24.4%) patients had symmetric form of HCM. The midventricular form of HCM was detected in 4 patients (1.8%). Apical form of HCM was detected in 3 patients (1.3%). One patient was diagnosed with a tumor-like variant of HCM (0.5%). In 198 patients (89.6%), systolic anterior motion of the mitral valve to the interventricular septum was found. In 95 cases (42.9%), morphological abnormality, abnormality of the number or attachment of the papillary muscles were detected. Forty-eight myocardial crypts were detected in 44 patients (21.7%). In 194 patients (87.7%), patent coronary arteries without signs of stenosis were found, 68 patients (30.7%) had 74 myocardial bridges (33.4%).
Conclusions. HCM is a genetic heart disease with enormous phenotypic diversity. Due to its high spatial resolution, cardiac MSCT is an accurate diagnostic tool, which allows to assess the morphofunctional state of the LV, mitral valve, subvalvular apparatus, as well as to analyze the anatomy and narrowing of coronary arteries in patients with HCM.
2. Chun EJ, Choi SI, Jin KN, Kwag HJ, Kim YJ, Choi BW, et al. Hypertrophiccardiomyopathy:assessmentwithMRimaging and multidetector CT. Radiographics. 2010;30(5):1309-28. https://doi.org/10.1148/rg.305095074
3. Marian AJ, Roberts R. Recent advances in the molecular genetics of hypertrophic cardiomyopathy. Circulation. 1995;92(5):1336-47. https://doi.org/10.1161/01.cir.92.5.1336
4. Rickers C, Wilke NM, Jerosch-Herold M, Casey SA, Panse P, Panse N, et al. Utility of cardiac magnetic resonance imaging in the diagnosis of hypertrophic cardiomyopathy. Circulation. 2005;112(6):855-61. https://doi.org/10.1161/CIRCULATIONAHA.104.507723
5. Elliott PM, Anastasakis A, Borger MA, Borggrefe M, Cecchi F, Charron P, et al. 2014 ESC Guidelines on diagnosis and management of hypertrophic cardiomyopathy: the Task Force for the Diagnosis and Management of Hypertrophic Cardiomyopathy of the European Society of Cardiology (ESC). Eur Heart J. 2014;35(39):2733-79. https://doi.org/10.1093/eurheartj/ehu284
6. Yingchoncharoen T, Tang WW. Recent advances in hypertrophic cardiomyopathy. F1000Prime Rep. 2014;6:12. https://doi.org/10.12703/P6-12
7. Sara L, Szarf G, Tachibana A, Shiozaki AA, Villa AV, de Oliveira AC, et al. Guidelines on Cardiovascular Magnetic Resonance and Computed Tomography of the Brazilian Society of Cardiology and the Brazilian College of Radiology. Arq Bras Cardiol. 2014;103(6 Suppl 3):1-86. Portuguese. https://doi.org/10.5935/abc.2014S006
8. Gersh BJ, Maron BJ, Bonow RO, Dearani JA, Fifer MA, Link MS, et al.; American College of Cardiology Foundation/ American Heart Association Task Force on Practice Guidelines. 2011 ACCF/AHA Guideline for the Diagnosis and Treatment of Hypertrophic Cardiomyopathy: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines. J Am Coll Cardiol. 2011;58(25):e212-60. https://doi.org/10.1016/j.jacc.2011.06.011
9. Maron BJ, Ommen SR, Semsarian C, Spirito P, Olivotto I, Maron MS. Hypertrophic cardiomyopathy: present and future, with translation into contemporary cardiovascular medicine. J Am Coll Cardiol. 2014;64(1):83-99. https://doi.org/10.1016/j.jacc.2014.05.003
10. Hoey ET, Teoh JK, Das I, Ganeshan A, Simpson H, Watkin RW. The emerging role of cardiovascular MRI for risk stratification in hypertrophic cardiomyopathy. Clin Radiol. 2014;69(3):221-30. https://doi.org/10.1016/j.crad.2013.11.012
11. Oda S, Kidoh M, Nagayama Y, Takashio S, Usuku H, Ueda M, et al. Trends in Diagnostic Imaging of Cardiac Amyloidosis: Emerging Knowledge and Concepts. Radiographics. 2020;40(4):961-81. https://doi.org/10.1148/rg.2020190069
12. Bogaert J, Olivotto I. MR Imaging in Hypertrophic Cardiomyopathy: From Magnet to Bedside. Radiology. 2014;273(2):329-48. https://doi.org/10.1148/radiol.14131626
13. Maron MS, Rowin EJ, Lin D, Appelbaum E, Chan RH, Gibson CM, et al. Prevalence and clinical profile of myocardial crypts in hypertrophic cardiomyopathy. Circ Cardiovasc ypts in hypertrophic cardiomyopathy Imaging. 2012;5(4):441-7. https://doi.org/10.1161/CIRCIMAGING.112.972760
14. Maron MS, Olivotto I, Zenovich AG, Link MS, Pandian NG, Kuvin JT, et al. Hypertrophic cardiomyopathy is predominantly a disease of left ventricular outflow tract obstruction. Circulation. 2006;114(21):2232-9. https://doi.org/10.1161/CIRCULATIONAHA.106.644682
15. Braunwald E, Lambrew CT, Rockoff SD, Ross J Jr, Morrow AG. Idiopathic hypertrophic subaortic stenosis. I. A description of the disease based upon an analysis of 64 patients. Circulation. 1964;30(Suppl 4):3-119. https://doi.org/10.1161/01.cir.29.5s4.iv-3
16. Brock R. Functional obstruction of the left ventricle; acquired aortic subvalvar stenosis. Guys Hosp Rep. 1957;106(4):221-38.
17. Shah PM, Gramiak R, Kramer DH. Ultrasound localization of left ventricular outflow obstruction in hypertrophic obstructive cardiomyopathy. Circulation. 1969;40(1):3-11. https://doi.org/10.1161/01.cir.40.1.3
18. Ommen SR, Mital S, Burke MA, Day SM, Deswal A, Elliott P, et al. 2020 AHA/ACC Guideline for the Diagnosis and TreatmentofPatientsWithHypertrophicCardiomyopathy: A Report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines. Circulation. 2020;142(25):e558-e631. https://doi.org/10.1161/CIR.0000000000000937
19. Maron MS, Hauser TH, Dubrow E, Horst TA, Kissinger KV, Udelson JE, et al. Right ventricular involvement in hypertrophic cardiomyopathy. Am J Cardiol.j.amjcard.2007.05.06