Hostname: page-component-78c5997874-j824f Total loading time: 0 Render date: 2024-11-19T08:22:30.387Z Has data issue: false hasContentIssue false
  • English
  • Français

Myocardial stress perfusion magnetic resonance in children with hypertrophic cardiomyopathy

Published online by Cambridge University Press:  22 February 2018

Lazaro E. Hernandez
Lazaro E. Hernandez*
Affiliation:
Joe DiMaggio Children’s Hospital at Memorial, Hollywood, FL, USA
*
Author for correspondence: Lazaro E. Hernandez, MD, Joe DiMaggio Children’s Hospital at Memorial, 1150N 35th Ave, Hollywood, FL 33021, USA. Tel: 954 265 3437; Fax: 954 983 5052; E-mail: [email protected]
Get access Rights & Permissions [Opens in a new window]

Abstract

Background

Microvascular dysfunction in hypertrophic cardiomyopathy has been associated with poor clinical outcome. Several studies have demonstrated a reduced perfusion reserve proportional to the magnitude of the hypertrophy. We investigated the utility of stress perfusion cardiac MRI to detect microvascular dysfunction in children with hypertrophic cardiomyopathy.

Methods

From January 2016 to January 2017, 13 patients, with a mean age of 15.3 years, with hypertrophic cardiomyopathy underwent regadenoson stress perfusion cardiac MRI (1.5-T Siemens Aera). A single-shot, T1-weighted saturation recovery gradient echo was used for first-pass perfusion in a multiple-slices group, including three short-axis slices and one four-chamber slice. Coronary vasodilatory stress was achieved using bolus injection of regadenoson (lexiscan 0.4 mg/5 ml) and dynamic perfusion during rest and stress performed by administering 0.05 mmol/kg of gadolinium contrast agent (gadoteridol) injected at a rate of 3.5 ml/s, followed by assessment of viability using two-dimensional phase-sensitive inversion recovery of the entire myocardium.

Results

All patients completed protocols with no interruptions. In all, seven patients developed perfusion defects after the administration of regadenoson. Asymmetric septal hypertrophy was the most common pattern of hypertrophic cardiomyopathy (n=4) in those with abnormal perfusion. A total of four patients with perfusion defects had a maximum wall thickness <30 mm. The finding of perfusion defects in areas without late gadolinium enhancement in some of our patients indicates that gadolinium enhancement by itself could underestimate the true extension of microvascular disease. Out of seven patients, five patients with positive stress cardiac MRI have undergone implantable cardioverter defibrillator placement based on current guidelines.

Conclusions

Regadenoson stress cardiac MRI is feasible and clinically valuable in paediatric patients. It is particularly effective in unmasking abnormal myocardial perfusion in the presence of microvascular dysfunction in children with hypertrophic cardiomyopathy.

Keywords

Hypertrophic cardiomyopathystress perfusion cardiac MRIchildren
Type
Original Articles
Information
Cardiology in the Young , Volume 28 , Issue 5 , May 2018 , pp. 702 - 708
Check for updates
Copyright
© Cambridge University Press 2018 

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

1. Villa, AD, Sammut, E, Zarinabad, N, et al. Microvascular ischemia in hypertrophic cardiomyopathy: new insights from high-resolution combined quantification of perfusion and late gadolinium enhancement. J Cardiovasc Magn Reson 2016; 18: 4.Google Scholar
2. Maron, BJ. Hypertrophic cardiomyopathy: a systematic review. JAMA 2002; 287: 1308–1320.CrossRefGoogle ScholarPubMed
3. Ismail, TF, Hsu, LY, Greve, AM, et al. Coronary microvascular ischemia in hypertrophic cardiomyopathy – a pixel-wise quantitative cardiovascular magnetic resonance perfusion study. J Cardiovasc Magn Reson 2014; 16: 49.CrossRefGoogle ScholarPubMed
4. Cecchi, F, Olivotto, I, Gistri, R, Lorenzoni, R, Chiriatti, G, Camici, PG. Coronary microvascular dysfunction and prognosis in hypertrophic cardiomyopathy. N Engl J Med 2003; 349: 1027–1035.CrossRefGoogle ScholarPubMed
5. Guclu, A, Happe, C, Eren, S, et al. Left ventricular outflow tract gradient is associated with reduced capillary density in hypertrophic cardiomyopathy irrespective of genotype. Eur J Clin Invest 2015; 45: 1252–1259.CrossRefGoogle ScholarPubMed
6. Hittinger, L, Mirsky, I, Shen, YT, Patrick, TA, Bishop, SP, Vatner, SF. Hemodynamic mechanisms responsible for reduced subendocardial coronary reserve in dogs with severe left ventricular hypertrophy. Circulation 1995; 92: 978–986.Google Scholar
7. Petersen, SE, Jerosch-Herold, M, Hudsmith, LE, et al. Evidence for microvascular dysfunction in hypertrophic cardiomyopathy: new insights from multiparametric magnetic resonance imaging. Circulation 2007; 115: 2418–2425.Google Scholar
8. Noel, CV, Krishnamurthy, R, Moffett, B, Krishnamurthy, R. Myocardial stress perfusion magnetic resonance: initial experience in a pediatric and young adult population using regadenoson. Pediatr Radiol 2017; 47: 280–289.Google Scholar
9. Noureldin, RA, Liu, S, Nacif, MS, et al. The diagnosis of hypertrophic cardiomyopathy by cardiovascular magnetic resonance. J Cardiovasc Magn Reson 2012; 14: 17.Google Scholar
10. Maron, BJ. Risk stratification and role of implantable defibrillators for prevention of sudden death in patients with hypertrophic cardiomyopathy. Circ J. 2010; 74: 2271–2282.CrossRefGoogle ScholarPubMed
11. Al Jaroudi, W, Iskandrian, AE. Regadenoson: a new myocardial stress agent. J Am Coll Cardiol 2009; 54: 1123–1130.Google Scholar
12. Bhave, NM, Freed, BH, Yodwut, C, et al. Considerations when measuring myocardial perfusion reserve by cardiovascular magnetic resonance using regadenoson. J Cardiovasc Magn Reson 2012; 14: 89.Google Scholar
13. Vasu, S, Bandettini, WP, Hsu, LY, et al. Regadenoson and adenosine are equivalent vasodilators and are superior than dipyridamole- a study of first pass quantitative perfusion cardiovascular magnetic resonance. J Cardiovasc Magn Reson 2013; 15: 85.CrossRefGoogle ScholarPubMed
14. Timmer, SA, Knaapen, P. Coronary microvascular function, myocardial metabolism, and energetics in hypertrophic cardiomyopathy: insights from positron emission tomography. Eur Heart J Cardiovasc Imaging 2013; 14: 95–101.CrossRefGoogle ScholarPubMed
15. Cianciulli, TF, Saccheri, MC, Masoli, OH, et al. Myocardial perfusion SPECT in the diagnosis of apical hypertrophic cardiomyopathy. J Nucl Cardiol 2009; 16: 391–395.CrossRefGoogle ScholarPubMed
16. Litmathe, J, Stosch, D, Klues, HG, Boeken, U, Korbmacher, B, Gams, E. Determination of the coronary flow reserve of the LAD in patients with HOCM using the intracoronary Doppler catheter. Thorac Cardiovasc Surg 2004; 52: 287–292.CrossRefGoogle ScholarPubMed
17. Kutty, S, Olson, J, Danford, CJ, et al. Ultrasound contrast and real-time perfusion in conjunction with supine bicycle stress echocardiography for comprehensive evaluation of surgically corrected congenital heart disease. Eur Heart J Cardiovasc Imaging 2012; 13: 500–509.Google Scholar
18. Jablonowski, R, Fernlund, E, Aletras, AH, et al. Regional stress-induced ischemia in non-fibrotic hypertrophied myocardium in young HCM patients. Pediatr Cardiol 2015; 36: 1662–1669.Google Scholar
19. Davies, MJ, McKenna, WJ. Hypertrophic cardiomyopathy – pathology and pathogenesis. Histopathology 1995; 26: 493–500.CrossRefGoogle ScholarPubMed
20. Friehs, I, Moran, AM, Stamm, C, et al. Promoting angiogenesis protects severely hypertrophied hearts from ischemic injury. Ann Thorac Surg 2004; 77: 2004–2010; discussion 2011.CrossRefGoogle ScholarPubMed
21. Maestri, R, Milia, AF, Salis, MB, et al. Cardiac hypertrophy and microvascular deficit in kinin B2 receptor knockout mice. Hypertension 2003; 41: 1151–1155.CrossRefGoogle ScholarPubMed
22. Chiribiri, A, Leuzzi, S, Conte, MR, et al. Rest perfusion abnormalities in hypertrophic cardiomyopathy: correlation with myocardial fibrosis and risk factors for sudden cardiac death. Clin Radiol 2015; 70: 495–501.Google Scholar
23. Soler, R, Rodriguez, E, Monserrat, L, Mendez, C, Martinez, C. Magnetic resonance imaging of delayed enhancement in hypertrophic cardiomyopathy: relationship with left ventricular perfusion and contractile function. J Comput Assist Tomogr 2006; 30: 412–420.Google Scholar