HEMODYNAMIC PHENOTYPES OF PULMONARY HYPERTENSION IN PVDOMICS

Abstract

SESSION TITLE: Pulmonary Vascular Disease Posters SESSION TYPE: Original Investigation Posters PRESENTED ON: October 18-21, 2020 PURPOSE: PVDOMICS (Redefining Pulmonary Hypertension through Pulmonary Vascular Disease Phenomics) is a NIH-funded initiative that aims to re-examine the current pulmonary hypertension (PH) classification based on shared biological features. The study enrolled 1198 subjects comprised of patients with PH and comparators with heart, lung and sleep comorbidities without PH and healthy controls from six centers in the United States. We sought to describe clinical phenotypes of enrollees based on hemodynamic profiles of isolated pre-capillary PH (PrePH), isolated post-capillary PH (PostPH), and combined pre- and post-capillary PH (CpcPH) defined by the 2013 WSPH statement used at the time of protocol development. METHODS: Enrollees including incident or prevalent diagnoses, underwent deep phenotyping including history and physical examination, cardiac imaging, pulmonary function testing, six-minute walk testing and right heart catheterization. Enrollees were assigned to WSPH categories by the center investigator with a secondary diagnosis allowed based on clinical context including imaging and provocative testing. PrePH (mean PA pressure (mPAP) >25mmHg, wedge pressure <15mmHg), PostPH (mPAP>25mmHg, wedge pressure >15mmHg, diastolic pressure gradient (DPG) <7mmHg) and CpcPH (mPAP >25mmHg, wedge pressure >15mmHg, DPG≥7mmHg). p<0.05 was considered significant. RESULTS: In the 709 enrollees whose hemodynamics were in one of the above categories, 474 (66%) had PrePH, 116 (16%) had CpcPH and 119 (17%) had PostPH. CpcPH hemodynamics were found in 43 (13%) of Group 1, 44 (33%) Group 2, 16 (11%) Group 3, and 7 (13%) of Group 4 enrollees. In the 338 patients with Group 1, Group 2 and combined 1 and 2 (Group 1,2) PH, there were 220 enrollees with PrePH, 52 with CpcPH, and 66 with PostPH. CpcPH included 23 Group 1, 14 Group 1,2, 14 Group 2 PH and 1 Group 2 comparator patients. CpcPH patients had higher BMI than PrePH, but similar age. Six minute walk distance was lower in CpcPH compared to PrePH. On cardiac MRI, there were no differences in RV ejection fraction between PrePH and CpcPH, and both were lower than PostPH. Compared with PrePH, CpcPH patients had higher resting right atrial pressure, higher mPAP though PVR was similar. Wedge pressure was intermediate between PrePH and PostPH. CONCLUSIONS: Resting CpcPH hemodynamics are present in all WSPH Groups and most common in Group 2 PH. There was modest agreement between physician’s judgement of mixed group 1,2 PH and CpcPH hemodynamics, suggesting that resting hemodynamics alone cannot fully account for clinically assigned phenotype. CpcPH patients had similar degree of RV dysfunction by cardiac MRI and echocardiogram compared with PrePH, however they had evidence of more significant right heart failure on right heart catheterization. CLINICAL IMPLICATIONS: CpcPH hemodynamics are prevalent in all WSPH groups of PH. How this impacts outcomes and treatment is unknown. DISCLOSURES: No relevant relationships by Erika Berman Rosenzweig, source=Web Response Advisory Committee Member relationship with Liquidia Please note: $1001 - $5000 Added 03/19/2020 by Robert Frantz, source=Web Response, value=Consulting fee Advisory Committee Member relationship with Altavant Please note: $1001 - $5000 Added 03/19/2020 by Robert Frantz, source=Web Response, value=Consulting fee Advisory Committee Member relationship with Actelion Please note: $5001 - $20000 Added 07/16/2020 by Robert Frantz, source=Web Response, value=Consulting fee No relevant relationships by Paul Hassoun, source=Web Response Advisory Committee Member relationship with actelion Please note: $1001 - $5000 Added 06/01/2020 by Anna Hemnes, source=Web Response, value=Consulting fee Advisory Committee Member relationship with bayer Please note: $1001 - $5000 Added 06/01/2020 by Anna Hemnes, source=Web Response, value=Consulting fee Advisory Committee Member relationship with Complexa Please note: $1-$1000 Added 06/01/2020 by Anna Hemnes, source=Web Response, value=Consulting fee Advisory Committee Member relationship with United Therapeutics Please note: $1001 - $5000 Added 06/01/2020 by Anna Hemnes, source=Web Response, value=Consulting fee Scientific Medical Advisor relationship with PHPrecisionMed Please note: $1-$1000 Added 06/01/2020 by Anna Hemnes, source=Web Response, value=Ownership interest no disclosure on file for Evelyn Horn; No relevant relationships by Miriam Jacob, source=Web Response My spouse/partner as a Consultant relationship with Cellectis Please note: >$100000 Added 05/28/2020 by Jane Leopold, source=Web Response, value=Consulting fee No relevant relationships by Milena Radeva, source=Web Response no disclosure on file for Franz Rischard