Abstract

BackgroundMicrovascular dysfunction in HCM has been associated with adverse clinical outcomes. Advances in quantitative cardiovascular magnetic resonance (CMR) perfusion imaging now allow myocardial blood flow to be quantified at the pixel level. We applied these techniques to investigate the spectrum of microvascular dysfunction in hypertrophic cardiomyopathy (HCM) and to explore its relationship with fibrosis and wall thickness.MethodsCMR perfusion imaging was undertaken during adenosine-induced hyperemia and again at rest in 35 patients together with late gadolinium enhancement (LGE) imaging. Myocardial blood flow (MBF) was quantified on a pixel-by-pixel basis from CMR perfusion images using a Fermi-constrained deconvolution algorithm. Regions-of-interest (ROI) in hypoperfused and hyperemic myocardium were identified from the MBF pixel maps. The myocardium was also divided into 16 AHA segments.ResultsResting MBF was significantly higher in the endocardium than in the epicardium (mean ± SD: 1.25 ± 0.35 ml/g/min versus 1.20 ± 0.35 ml/g/min, P < 0.001), a pattern that reversed with stress (2.00 ± 0.76 ml/g/min versus 2.36 ± 0.83 ml/g/min, P < 0.001). ROI analysis revealed 11 (31%) patients with stress MBF lower than resting values (1.05 ± 0.39 ml/g/min versus 1.22 ± 0.36 ml/g/min, P = 0.021). There was a significant negative association between hyperemic MBF and wall thickness (β = −0.047 ml/g/min per mm, 95% CI: −0.057 to −0.038, P < 0.001) and a significantly lower probability of fibrosis in a segment with increasing hyperemic MBF (odds ratio per ml/g/min: 0.086, 95% CI: 0.078 to 0.095, P = 0.003).ConclusionsPixel-wise quantitative CMR perfusion imaging identifies a subgroup of patients with HCM that have localised severe microvascular dysfunction which may give rise to myocardial ischemia.

Highlights

  • Microvascular dysfunction in hypertrophic cardiomyopathy (HCM) has been associated with adverse clinical outcomes

  • There were no significant differences between the severe and non-severe groups with respect to baseline clinical and demographic features, there was a trend towards a higher incidence of left ventricular outflow tract obstruction amongst those with severe microvascular dysfunction (Table 1)

  • In a subset of patients, pixelwise analysis revealed regions with a blunted or inadequate hyperemic response to vasodilator stress, but evidence of severe microvascular dysfunction that is likely to result in ischemia, with stress myocardial blood flow (MBF) levels below those of rest perfusion

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Summary

Introduction

Advances in quantitative cardiovascular magnetic resonance (CMR) perfusion imaging allow myocardial blood flow to be quantified at the pixel level We applied these techniques to investigate the spectrum of microvascular dysfunction in hypertrophic cardiomyopathy (HCM) and to explore its relationship with fibrosis and wall thickness. The use of a sector-based or global approach alone whilst improving signal-to-noise, may mask or significantly underestimate the local severity of perfusion defects due to coronary microvascular dysfunction [23] This is of particular importance as the burden and severity of ischemia may be of prognostic importance as a mechanism leading to myocardial fibrosis, and independently as a trigger for ventricular arrhythmia [19,24]

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