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Sarcoidosis, pulmonary hypetension, hemodynamics, echocardiography
Introduction: Echocardiographic measurement of the right ventricular systolic pressure (RVSP) is commonly used for estimating systolic pulmonary artery pressure (PASP) measured during right heart catheterization (RHC) in patients suspected for pulmonary hypertension (PH). Generally, there is a strong correlation. However, this has been reported as less robust in sarcoidosis. We analyzed factors influencing the relationship between RVSP and PASP in sarcoidosis.
Methods & Results: Data of patients with and without sarcoidosis associated PH who had both a measurable echocardiographic RVSP and invasive PASP were collected from the RESAPH registry, PULSAR study and Cincinnati Sarcoidosis Clinic database (n=173, 60.1% female, mean age 56.0±9.5years). Among them, 124 had PH confirmed by RHC. There was a strong correlation between PASP and RVSP (r=0.640). This correlation was significant in both male and female, white or non-white, (forced vital capacity) FVC >60%, and presence of fibrosis (p<0.001). However, it was less robust in patients with FVC of 50% or less. RVSP was considered inaccurate if the difference with PASP was more than 10mmHg. Inaccurate echocardiographic estimation of the invasive PASP occurred in 50.8%, with overestimation mostly in patients without PH, and underestimation in patients with severe PH. An RVSP>50mmHg was associated with worse survival.
Conclusions: In this multicenter cohort of sarcoidosis patients, we found a significant correlation between RVSP as determined by echocardiography and PASP measured by RHC. Over- or underestimation of PASP occurred frequently. Therefore, echocardiography alone to screen for pulmonary hypertension in sarcoidosis should be used with caution.
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