Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2024 Oct;43(10):1668-1676.
doi: 10.1016/j.healun.2024.06.003. Epub 2024 Jun 26.

Right ventricular phenotyping in incident patients with idiopathic pulmonary arterial hypertension

Stefano Ghio  1 , Roberto Badagliacca  2 , Michele D'Alto  3 , Laura Scelsi  4 , Paola Argiento  3 , Natale D Brunetti  5 , Gavino Casu  6 , Nadia Cedrone  7 , Marco Confalonieri  8 , Marco Corda  9 , Michele Correale  10 , Carlo D'Agostino  11 , Elisabetta De Tommasi  11 , Domenico Filomena  2 , Giuseppe Galgano  12 , Alessandra Greco  4 , Massimo Grimaldi  12 , Carlo Lombardi  13 , Rosalinda Madonna  14 , Giovanna Manzi  2 , Valentina Mercurio  15 , Alexandra Mihai  2 , Massimiliano Mulè  16 , Giuseppe Paciocco  17 , Silvia Papa  2 , Tommaso Recchioni  2 , Antonella Romaniello  18 , Emanuele Romeo  3 , Davide Stolfo  19 , Patrizio Vitulo  20 , Raymond L Benza  21 , Carmine D Vizza  2 ; Italian Pulmonary Hpertension NETwork (iPHNET)
Affiliations

Right ventricular phenotyping in incident patients with idiopathic pulmonary arterial hypertension

Stefano Ghio et al. J Heart Lung Transplant. 2024 Oct.

Abstract

Background: Right ventricular (RV) imaging has not a definite role in risk stratification of pulmonary arterial hypertension (PAH) patients. We tested the hypothesis that echocardiography-derived phenotypes, depicting different degrees of RV remodeling and dysfunction, may provide additional prognostic information to current risk stratification tools.

Methods: Consecutive incident PAH patients aged ≥18 years, diagnosed between January 2005 and December 2021, underwent clinical assessment, right heart catheterization, standard echocardiography. Simple echocardiographic variables were combined in order to define a priori four phenotypes representing different degrees of RV dilatation and RV-pulmonary arterial (PA) coupling: Phenotype 1 with mildy dilated right ventricle and preserved RV-PA coupling (n = 152 patients); phenotype 2 with mildly dilated right ventricle and poor RV-PA coupling (n = 143 patients); phenotype 3 with severely dilated right ventricle and preserved RV-PA coupling (n = 201 patients); phenotype 4 with severely dilated right ventricle and poor RV-PA coupling, with or without severe tricuspid regurgitation (n = 519 patients). Risk stratification was based on the European Society of Cardiology/European Respiratory Society (ESC/ERS) 3-strata model and Registry to Evaluate Early and Long-Term PAH disease Management (REVEAL) 2.0 score.

Results: These phenotypes were present in all risk groups. Notably, regardless of the ESC/ERS risk stratum assigned to the patient, phenotype 4 was associated with a 2-fold increase of the odds of death (HR 2.1, 95% CI 1.6-2.8, p < 0.001), while phenotype 1 was associated with a 71% reduction in the odds of dying (HR 0.29, 95% CI 0.18-0.47, p < 0.001).

Conclusions: Echocardiography-derived phenotypes describing RV remodeling and dysfunction may provide prognostic information which is independent of and additional to the clinically defined risk in incident PAH patients.

Keywords: echocardiography; outcome; phenotypes; pulmonary arterial hypertension; right heart remodeling; transplantation.

PubMed Disclaimer

MeSH terms