Abstract
Background: World Symposium on Pulmonary Hypertension (WSPH) Group 3 (G3PH) is a heterogeneous group of lung disease related PH, which may share characteristics with Group 1 PAH or lung disease without PH. The PVDOMICS program deeply phenotypes patients with PH. We sought to examine WSPH mixed 1 AND 3 (G1,3), G3PH, and lung disease comparators.
Methods: This sub-analysis of PVDOMICS, a prospective cohort study, examines G1,3, G3PH and lung disease comparators referred for hemodynamic assessment. G1,3 patients met WSPH guidelines for Group 1 PAH and are classified with secondary group features by an adjudication panel based on lung disease severity. Single-beat analysis of right ventricular waveforms was performed on a subset to assess for contractility (Ees), RVPA coupling (Ees/Ea), and diastolic stiffness (β). Statistical testing is described in the Table.
Results: 77 and 79% of G1,3 COPD and ILD, respectively, and 29 and 42% of G3PH COPD and ILD patients were on active PAH therapy. Six-minute walk distance and DLCO were reduced in G1,3 and G3PH despite similar FEV1 and TLC as lung disease comparators. RVEF and RVPA coupling were reduced in G1,3 and G3PH and β increased in G3PH relative to comparators. Mortality was high in both PH groups (Table).
Conclusions: PVDOMICS G3PH and G1,3PH share similar RV dysfunction that appears disproportional to lung function relative to lung disease only comparators.
Footnotes
Cite this article as: European Respiratory Journal 2020; 56: Suppl. 64, 4044.
This abstract was presented at the 2020 ERS International Congress, in session “Respiratory viruses in the "pre COVID-19" era”.
This is an ERS International Congress abstract. No full-text version is available. Further material to accompany this abstract may be available at www.ers-education.org (ERS member access only).
- Copyright ©the authors 2020