Frequency of acute vasodilator response (AVR) in incident and prevalent patients with pulmonary arterial hypertension: Results from the pulmonary vascular disease phenomics study

Mario Naranjo; Erika B Rosenzweig; Anna R Hemnes; Miriam Jacob; Ankit Desai; Nicholas S Hill; A Brett Larive; J Emanuel Finet; Jane Leopold; Evelyn Horn; Robert Frantz; Franz Rischard; Serpil Erzurum; Gerald Beck; Stephen C Mathai; Paul M Hassoun; PVDOMICS Study Group

doi:10.1002/pul2.12281

Frequency of acute vasodilator response (AVR) in incident and prevalent patients with pulmonary arterial hypertension: Results from the pulmonary vascular disease phenomics study

Pulm Circ. 2023 Aug 21;13(3):e12281. doi: 10.1002/pul2.12281. eCollection 2023 Jul.

Authors

Mario Naranjo¹, Erika B Rosenzweig², Anna R Hemnes³, Miriam Jacob⁴, Ankit Desai⁵, Nicholas S Hill⁶, A Brett Larive⁷, J Emanuel Finet⁴, Jane Leopold⁸, Evelyn Horn⁹, Robert Frantz¹⁰, Franz Rischard⁵, Serpil Erzurum¹¹, Gerald Beck⁷, Stephen C Mathai¹, Paul M Hassoun¹; PVDOMICS Study Group

Affiliations

¹ Division of Pulmonary and Critical Care Medicine, Department of Medicine Johns Hopkins University Baltimore Maryland USA.
² Department of Pediatrics and Medicine Columbia University New York New York USA.
³ Division of Allergy, Pulmonary and Critical Care Medicine Vanderbilt University Medical Center Nashville Tennessee USA.
⁴ Department of Cardiovascular Medicine Cleveland Clinic Cleveland Ohio USA.
⁵ Department of Medicine, College of Medicine The University of Arizona Tucson Arizona USA.
⁶ Division of Pulmonary, Critical Care, and Sleep Medicine Tufts Medical Center Boston Massachusetts USA.
⁷ Department of Quantitative Health Sciences Cleveland Clinic Cleveland Ohio USA.
⁸ Department of Cardiovascular Medicine, Brigham and Women's Hospital Harvard University Boston Massachusetts USA.
⁹ Division of Cardiology Weill Cornell University New York New York USA.
¹⁰ Department of Cardiovascular Medicine Mayo Clinic Rochester Minnesota USA.
¹¹ Department of Inflammation and Immunity Cleveland Clinic Cleveland Ohio USA.

Abstract

The prevalence of acute vasodilator response (AVR) to inhaled nitric oxide (iNO) during right heart catheterization (RHC) is 12% in idiopathic pulmonary arterial hypertension (IPAH). AVR, however, is reportedly lower in other disease-associated pulmonary arterial hypertension (PAH), such as connective tissue disease (CTD). The prevalence of AVR in patients on PAH therapy (prevalent cases) is unknown. We sought to determine AVR prevalence in Group 1 PH in the PVDOMICS cohort of incident and prevalent patients undergoing RHC. AVR was measured in response to 100% O₂ and O₂ plus iNO, with positivity defined as (1) decrease in mean pulmonary artery pressure (mPAP) by ≥10 mmHg to a value ≤40 mmHg, with no change or an increase in cardiac output (definition 1); or (2) decrease in mPAP by ≥12% and pulmonary vascular resistance by ≥30% (definition 2). AVR rates and cumulative survival were compared between incident and prevalent patients. In 338 mainly prevalent (86%) patients, positive AVR to O₂-only was <2%, and 5.1% to 16.9%, based on definition 1 and 2 criteria, respectively; following O₂ + iNO. IPAH AVR prevalence (4.1%-18.7%) was similar to prior reports. AVR positivity was 7.7% to 15.4% in mostly CTD-PAH prevalent cases, and 2.6% to 11.8% in other PAH groups. Survival was 89% in AVR responders versus 77% in nonresponders from PAH diagnosis, and 91% versus 86% from PVDOMICS enrollment (log-rank test p = 0.04 and p = 0.05, respectively). In conclusion, AVR in IPAH patients is similar to prior studies. AVR in non-IPAH patients was higher than previously reported. The relationship between PAH therapy, AVR response, and survival warrants further investigation.

Keywords: pulmonary arterial hypertension; survival; vasoreactivity.