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Background  Pulmonary hypertension (PH) is a known complication of HCM and is a strong predictor of mortality. We aim to investigate the relationship between microvascular dysfunction measured by quantitative PET and PH in HCM patients.    Methods  Eighty-nine symptomatic HCM patients were included in the study. Each patient underwent two 20-min  13 N-NH 3  dynamic PET scans for rest and stress conditions, respectively. A 2-tissue irreversible compartmental model was used to fit the segments time activity curves for estimating segmental and global myocardial blood flow (MBF) and myocardial flow reserve (MFR). Echocardiographic derived PASP was utilized to estimate PH.    Results  Patients were categorized into two groups across PASP: PH (PASP > 36 mmHg) and no-PH (PASP ≤ 36 mmHg). patients with PH had larger left atrium, ratio of higher inflow early diastole (E) and atrial contraction (A) waves, E/A, and ratio of inflow and peak early diastolic waves, E/e', significantly reduced global stress MBF (1.85 ± 0.52 vs. 2.13 ± 0.56 ml/min/g;  p  = 0.024) and MFR (2.21 ± 0.57 vs. 2.62 ± 0.75;  p  = 0.005), while the MBFs at rest between the two groups were similar. There were significant negative correlations between global stress MBF/MFR and PASP (stress MBF: r = -0.23,  p  = 0.03; MFR: r = -0.32,  p  = 0.002); for regional MBF and MFR measurements, the highest linear correlation coefficients were observed in the septal wall (stress MBF: r = -0.27,  p  = 0.01; MFR: r = -0.31,  p  = 0.003). Global MFR was identified to be independent predictor for PH in multivariate regression analysis.    Conclusion  Echocardiography-derived PASP is negatively correlated with global MFR measured by  13 N-NH 3  dynamic PET. Global MFR is suggested to be an index of PH in HCM patients.

plos one

Association of PET-measured myocardial flow reserve with echocardiography-estimated pulmonary artery systolic pressure in patients with hypertrophic cardiomyopathy