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Background: Apical hypertrophic cardiomyopathy (HCM) is commonly associated with drug-refractory chest pain. We sought to determine whether, in apical HCM, coronary perfusion time is abbreviated by the diastolic persistence of apical contraction, resulting in impaired myocardial perfusion and chest pain.Methods: 62 apical HCM patients had cardiac magnetic resonance (CMR) scans assessed for stress perfusion (myocardial perfusion reserve index (MPRi)), late gadolinium enhancement (LGE; % of myocardial volume) left ventricular (LV) volumes and LV contractile persistence (% total cardiac cycle) at the LV apex and base. Radial and circumferential strain were assessed. Patients were divided into three groups on the basis of severity of contractile persistence. The interval between earliest and latest systolic peaks was measured from strain data from each of the apical segments.Results: Compared to subjects with the least contractile persistence (C1), those with the most (C3) were more likely to have chest pain (94% vs 63%, p 130ms as a threshold, circumferential dyssynchrony was present in 25% of C1 and 81% of C3 patients (p<0.001) and radial dyssynchrony in 65% of C1 and 95% of C3 patients (p<0.05). In patients with radial dyssynchrony, the earliest peak was most often in the inferior or anterior segments (60%) and the latest in the lateral segment (33%). In patients with circumferential dyssynchrony, the earliest peak was most often in the inferior or anterior segments (59%) and the latest in the lateral segment (41%).Conclusion: In apical HCM, regional persistence of contractility into diastole causes myocardial ischaemia and chest pain. This is the first description of contractile persistence and dyssynchrony as a mechanism for myocardial perfusion abnormalities and presents novel therapeutic opportunities for drug-refractory chest pain in apical HCM.

european heart journal

Apical hypertrophic cardiomyopathy: chest pain and myocardial perfusion defects result from regional diastolic persistence of hyperdynamic cardiac contractility