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. 2022 Jun 7;12(2):e12086.
doi: 10.1002/pul2.12086. eCollection 2022 Apr.

The impact of cardiovascular comorbidities associated with risk for left heart disease on idiopathic pulmonary arterial hypertension: Data from the Hellenic Pulmonary Hypertension Registry (HOPE)

Alexandra Arvanitaki  1 , Elena Vrana  1 , Maria Boutsikou  2 , Anastasia Anthi  3 , Sotiria Apostolopoulou  4 , Aikaterini Avgeropoulou  5 , Eftychia Demerouti  4 , Alexandros Patrianakos  6 , Panagiotis Karyofyllis  4 , Ioanna Mitrouska  6 , Sophia A Mouratoglou  1 , Katerina K Naka  7 , Stylianos E Orfanos  3 , Evangelia Panagiotidou  8 , Georgia Pitsiou  8 , Spyridon Rammos  4 , Ioannis Stanopoulos  8 , Adina Thomaidi  9 , Alexandra Frogoudaki  10 , Afroditi Boutou  8 , George Anastasiadis  11 , Styliani Brili  5 , Iraklis Tsangaris  10 , Dimitrios Tsiapras  4 , Vassilios Voudris  4 , Athanasios Manginas  2 , George Giannakoulas  1
Affiliations

The impact of cardiovascular comorbidities associated with risk for left heart disease on idiopathic pulmonary arterial hypertension: Data from the Hellenic Pulmonary Hypertension Registry (HOPE)

Alexandra Arvanitaki et al. Pulm Circ. .

Abstract

Whereas younger female patients were diagnosed with idiopathic pulmonary arterial hypertension (IPAH) in 1980s, it is now frequently encountered in elderly patients with cardiovascular comorbidities (CVCs) associated with increased risk for left heart disease. We present data until November 2019 regarding specific features and clinical outcomes of IPAH population from the Hellenic Pulmonary Hypertension Registry (HOPE). Patients were divided into two groups based on the presence of ≥ or <3 CVCs, arterial hypertension, diabetes mellitus, obesity, presence of coronary artery disease, or atrial fibrillation. Overall, 77 patients with IPAH (55.1 [interquartile range, IQR: 24.1] years, 62.8% women) have been recorded. Fifteen patients (19.2%) had ≥3 CVCs, while 25 (32%) were over 65 years old. Patients with ≥3 CVCs were older, presented an almost equal female to male ratio, walked less in 6-min walk test, and had lower mean arterial pulmonary pressure and pulmonary vascular resistance at baseline than patients with less CVCs. Fewer patients with ≥3 CVCs received PAH-specific treatment compared to patients with less comorbidities (n = 11 [73.3%] versus n = 58 [95.5%], p = 0.02). During a median follow-up period of 3.8 (IQR: 2.7) years, 18 patients died (all-cause mortality 24.3%). Male sex and older age were independent predictors of mortality and/or lung transplantation, while CVCs did not have a significant impact on clinical outcomes. In this nationwide, register-based study, the epidemiology of IPAH involves older patients with CVCs, who seem to have less hemodynamic compromise, but worse functional impairment and are treated less aggressively with PAH pharmacotherapy.

Keywords: cardiovascular comorbidities; elderly; idiopathic pulmonary arterial hypertension; survival; targeted therapy.

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Conflict of interest statement

Alexandra Arvanitaki has been the recipient of the International Training and Research Fellowship EMAH Stiftung Karla Voellm, Krefeld, Germany. Anastasia Anthi reports receiving honoraria and consultancy fees from Actelion Pharmaceuticals Hellas, Bayer, ELPEN, GSK, MSD, Lilly, and United Therapeutics. Eftychia Demerouti has been an advisory board member for Actelion Pharmaceuticals Hellas, MSD Hellas, and GlaxoSmithKline and an honorarium speaker for Actelion Pharmaceutical Hellas and MSD Hellas. Ioanna Mitrouska reports receiving honoraria and consultancy fees from Actelion Pharmaceuticals Hellas, Bayer, ELPEN, MSD, and GSK. Katerina K. Naka reports lecture fees from Novartis, Medtronic, and Abbott, while she serves as an investigator in randomized clinical trials and/or registries sponsored by Novartis, Merck, Amgen, BMS, Boehringer Ingelheim, Pfizer, and Actelion Pharmaceutical Hellas, outside the submitted work. Stylianos E. Orfanos reports has been receiving research grants and/or honoraria and/or consultancy fees from Actelion Pharmaceutical Hellas, Bayer, ELPEN, Galenica‐Ferrer, GSK, MSD, Pharmaserve Lilly, PharmaSwiss, Pfizer, and United Therapeutics. Georgia Pitsiou reports receiving honoraria and consultancy fees from Actelion Pharmaceutical Hellas, Bayer, ELPEN, MSD, and GSK. Ioannis Stanopoulos reports receiving honoraria and consultancy fees from Actelion Pharmaceuticals Hellas. Iraklis Tsangaris reports has been receiving honoraria and consultancy fees from Actelion Pharmaceuticals Hellas, Bayer, ELPEN, GSK, Pfizer, Lilly, and United Therapeutics. Athanasios Manginas reports consultation fees from Astra Zeneca, Bayer, ELPEN, Actelion Pharmaceuticals Hellas, and MSD. George Giannakoulas reports has been receiving honoraria and consultancy fees from Actelion Pharmaceuticals Hellas, Bayer, ELPEN, Galenica‐Ferrer, GSK, Pfizer, Lilly, MSD, and United Therapeutics. The remaining authors declare no conflicts of interest. The remaining authors declare no conflicts of interest.

Figures

Figure 1
Figure 1
Flow chart of the study participants. CHD‐PAH, pulmonary arterial hypertension related with congenital heart disease; CTD‐PAH, pulmonary arterial hypertension related with connective tissue disease; IPAH, idiopathic pulmonary arterial hypertension; PVOD, pulmonary vascular obstructive disease.
Figure 2
Figure 2
Survival in patients with idiopathic pulmonary arterial hypertension (IPAH) according to: (a) sex (male and female), (b) age (>65 years old and ≤65 years old), (c) risk score (low‐, intermediate‐, high‐risk for 1‐year mortality), and (d) the presence of cardiovascular comorbidities (CVCs) for left heart disease (LHD) (≥3 and <3). p < 0.05 indicates statistical significance between the groups.
Figure 3
Figure 3
Multivariable cox regression analysis predicting mortality and/or transplantation. *p < 0.05, AIC, Akaike information criterion; CI, confidence intervals; Comorbidities, cardiovascular comorbidities associated with left heart disease; HR, hazard ratio; risk stratification, an abbreviated version of the 2015 European Society of Cardiology (ESC)/European Respiratory Society (ERS) risk stratification strategy.
Figure 4
Figure 4
Special demographic, functional, hemodynamic, and treatment features of patients with idiopathic pulmonary arterial hypertension and ≥3 cardiovascular comorbidities (CVCs) associated with left heart disease (LHD) in the HOPE Registry. 6MWD, six‐minute walking distance; NYHA, New York Heart Association; PAH, pulmonary arterial hypertension.
See this image and copyright information in PMC

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