Abstract
Aims
Pulmonary arterial hypertension (PAH) has been described in patients with hypertrophic cardiomyopathy (HCM) and it was associated with a worse prognosis. Nevertheless in most HCM patients, despite normal pulmonary pressures at rest, congestive symptoms are elicited by exercise. In the present study, combining cardiopulmonary exercise test (CPET) with echocardiography, we aimed to evaluate the presence of exercise-induced pulmonary hypertension (EiPAH) its role in functional limitation and its prognostic significance in a cohort of patients with obstructive and non-obstructive HCM.
Methods and results
182 HCM patients (35% females, mean age 47.5 ± 15.9) undergoing CPET. During CPET, LVOT velocities and trans-tricuspid gradient were measured. Thirty-seven patients (20%) developed sPAP > 40 mmHg at peak exercise (EiPAH). EiPAH was associated with an lower exercise performance, larger left atrial volumes, higher left ventricular gradient and higher VE/VCO2 slope .At multivariable model baseline sPAP (P < 0.0001) and baseline left ventricular obstruction (LVOT) (P = 0.028) were significantly associated with EiPAH .Kaplan-Meier curve analysis showed EiPAH was a significant predictor of HCM –related morbidity (hazards ratio: 6.21, 95% CI: 1.47–26.19; P = 0.05; 4.21, 95% CI: 1.94–9.12; P < 0.001) for the primary and the secondary endpoint respectively.
Conclusions
EiPAH was present in about one fifth of HCM patients without evidence of elevated pulmonary pressures at rest, and was associated with adverse clinical outcome. Diagnosing EiPAH by exercise echo/CPET may help physicians to detect early stage of PAH requiring a closer clinical monitoring and individualized treatment strategies.
Objective Nutritional Index is Associated with Worse Cardiopulmonary Exercise Test Performance in Advanced Heart Failure Patients
