Inline perfusion mapping provides insights into the disease mechanism in hypertrophic cardiomyopathy

Claudia Camaioni1, Kristopher D Knott1,2, Joao B Augusto1,2

  • 1Advanced Cardiac Imaging, Barts Health NHS Trust, London, UK.

Insights

Microvascular dysfunction is common in hypertrophic cardiomyopathy (HCM), affecting myocardial blood flow (MBF) even in normal-appearing segments. This dysfunction is linked to hypertrophy and late gadolinium enhancement (LGE), suggesting it may be an early disease marker.

Area of Science:

  • Cardiology
  • Cardiovascular Imaging
  • Medical Research

Background:

  • Hypertrophic cardiomyopathy (HCM) is a complex condition where small vessel disease and myocardial perfusion are not fully understood.
  • Quantitative data on absolute myocardial blood flow (MBF) in HCM patients are limited.
  • Cardiovascular magnetic resonance (CMR) offers advanced imaging capabilities for assessing cardiac function and structure.

Purpose of the Study:

  • To investigate the relationship between myocardial perfusion, cardiac hypertrophy, and late gadolinium enhancement (LGE) in patients with HCM.
  • To quantify absolute myocardial blood flow (MBF) using CMR-based fully quantitative perfusion mapping.
  • To determine the extent of microvascular dysfunction in HCM.

Main Methods:

  • 101 HCM patients and 30 controls underwent CMR perfusion mapping during adenosine stress and rest.
  • Pixel-wise analysis calculated stress MBF, rest MBF, and myocardial perfusion reserve (MPR).
  • MBF and MPR were correlated with segmental wall thickness and LGE findings.

Main Results:

  • Reduced stress MBF and MPR were observed in HCM patients compared to controls.
  • Globally, stress MBF decreased with increasing left ventricle mass.
  • Segmentally, reduced MBF and MPR correlated with increased wall thickness and LGE.
  • In 21% of HCM patients, stress MBF was lower than rest MBF (MPR <1), predominantly in subendocardial segments.
  • Even apparently normal HCM segments showed reduced stress MBF and MPR compared to controls.

Conclusions:

  • Microvascular dysfunction is prevalent in HCM and associated with hypertrophy and LGE.
  • Myocardial perfusion abnormalities, including reduced MBF and MPR, are present even in segments without hypertrophy or LGE.
  • These findings suggest that impaired myocardial perfusion may serve as an early indicator of disease progression in HCM.
Abstract