Right ventricular remodeling in idiopathic and scleroderma-associated pulmonary arterial hypertension: Two distinct phenotypes

Benjamin W. Kelemen, Stephen Mathai, Ryan J. Tedford, Rachel L Damico, Celia Corona Villalobos, Todd Matthew Kolb, Neal F. Chaisson, Traci Housten Harris, Stefan Zimmerman, Ihab R Kamel, David A Kass, Paul M Hassoun

Research output: Contribution to journalArticle

Abstract

Patients with scleroderma (SSc)–associated pulmonary arterial hypertension (PAH) have worse survival than patients with idiopathic PAH (IPAH). We hypothesized that the right ventricle (RV) adapts differently in SSc-PAH versus IPAH. We used cardiac magnetic resonance imaging (cMRI) and hemodynamic characteristics to assess the relationship between RV morphology and RV load in patients with SSc-PAH and IPAH. In 53 patients with PAH (35 with SSc-PAH and 18 with IPAH) diagnosed by right heart catheterization (RHC), we examined cMRIs obtained within 48 hours of RHC and compared RV morphology between groups. Regression analysis was used to assess the association between diagnosis (IPAH vs. SSc-PAH) and RV measurements after adjusting for age, sex, race, body mass index (BMI), left ventricular (LV) mass, and RV load. There were no significant differences in unadjusted comparisons of cMRI measurements between the two groups. Univariable regression showed RV mass index (RVMI) was linearly associated with measures of RV load in both the overall cohort and within each group. Multivariable linear regression models revealed a significant interaction between disease type and RVMI adjusting for pulmonary vascular resistance (PVR), age, sex, race, BMI, and LV mass. This model showed a decreased slope in the relationship between RVMI and PVR in the SSc-PAH group compared with the IPAH group. RVMI varies linearly with measures of RV load. After adjusting for multiple potential confounders, patients with SSc-PAH demonstrated significantly less RV hypertrophy with increasing PVR than patients with IPAH. This difference in adaptive hypertrophy may in part explain previously observed decreased contractility and poorer survival in SSc-PAH.

Original languageEnglish (US)
Pages (from-to)327-334
Number of pages8
JournalPulmonary Circulation
Volume5
Issue number2
DOIs
StatePublished - Jun 1 2015

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Ventricular Remodeling
Pulmonary Hypertension
Heart Ventricles
Phenotype
Vascular Resistance
Cardiac Catheterization
Hypertrophy
Linear Models
Body Mass Index
Magnetic Resonance Imaging
Survival
Hemodynamics
Regression Analysis

Keywords

  • Cardiac magnetic resonance imaging
  • Pulmonary arterial hypertension
  • Right ventricle
  • Scleroderma

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine

Cite this

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title = "Right ventricular remodeling in idiopathic and scleroderma-associated pulmonary arterial hypertension: Two distinct phenotypes",
abstract = "Patients with scleroderma (SSc)–associated pulmonary arterial hypertension (PAH) have worse survival than patients with idiopathic PAH (IPAH). We hypothesized that the right ventricle (RV) adapts differently in SSc-PAH versus IPAH. We used cardiac magnetic resonance imaging (cMRI) and hemodynamic characteristics to assess the relationship between RV morphology and RV load in patients with SSc-PAH and IPAH. In 53 patients with PAH (35 with SSc-PAH and 18 with IPAH) diagnosed by right heart catheterization (RHC), we examined cMRIs obtained within 48 hours of RHC and compared RV morphology between groups. Regression analysis was used to assess the association between diagnosis (IPAH vs. SSc-PAH) and RV measurements after adjusting for age, sex, race, body mass index (BMI), left ventricular (LV) mass, and RV load. There were no significant differences in unadjusted comparisons of cMRI measurements between the two groups. Univariable regression showed RV mass index (RVMI) was linearly associated with measures of RV load in both the overall cohort and within each group. Multivariable linear regression models revealed a significant interaction between disease type and RVMI adjusting for pulmonary vascular resistance (PVR), age, sex, race, BMI, and LV mass. This model showed a decreased slope in the relationship between RVMI and PVR in the SSc-PAH group compared with the IPAH group. RVMI varies linearly with measures of RV load. After adjusting for multiple potential confounders, patients with SSc-PAH demonstrated significantly less RV hypertrophy with increasing PVR than patients with IPAH. This difference in adaptive hypertrophy may in part explain previously observed decreased contractility and poorer survival in SSc-PAH.",
keywords = "Cardiac magnetic resonance imaging, Pulmonary arterial hypertension, Right ventricle, Scleroderma",
author = "Kelemen, {Benjamin W.} and Stephen Mathai and Tedford, {Ryan J.} and Damico, {Rachel L} and {Corona Villalobos}, Celia and Kolb, {Todd Matthew} and Chaisson, {Neal F.} and Harris, {Traci Housten} and Stefan Zimmerman and Kamel, {Ihab R} and Kass, {David A} and Hassoun, {Paul M}",
year = "2015",
month = "6",
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doi = "10.1086/680356",
language = "English (US)",
volume = "5",
pages = "327--334",
journal = "Pulmonary Circulation",
issn = "2045-8932",
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TY - JOUR

T1 - Right ventricular remodeling in idiopathic and scleroderma-associated pulmonary arterial hypertension

T2 - Two distinct phenotypes

AU - Kelemen, Benjamin W.

AU - Mathai, Stephen

AU - Tedford, Ryan J.

AU - Damico, Rachel L

AU - Corona Villalobos, Celia

AU - Kolb, Todd Matthew

AU - Chaisson, Neal F.

AU - Harris, Traci Housten

AU - Zimmerman, Stefan

AU - Kamel, Ihab R

AU - Kass, David A

AU - Hassoun, Paul M

PY - 2015/6/1

Y1 - 2015/6/1

N2 - Patients with scleroderma (SSc)–associated pulmonary arterial hypertension (PAH) have worse survival than patients with idiopathic PAH (IPAH). We hypothesized that the right ventricle (RV) adapts differently in SSc-PAH versus IPAH. We used cardiac magnetic resonance imaging (cMRI) and hemodynamic characteristics to assess the relationship between RV morphology and RV load in patients with SSc-PAH and IPAH. In 53 patients with PAH (35 with SSc-PAH and 18 with IPAH) diagnosed by right heart catheterization (RHC), we examined cMRIs obtained within 48 hours of RHC and compared RV morphology between groups. Regression analysis was used to assess the association between diagnosis (IPAH vs. SSc-PAH) and RV measurements after adjusting for age, sex, race, body mass index (BMI), left ventricular (LV) mass, and RV load. There were no significant differences in unadjusted comparisons of cMRI measurements between the two groups. Univariable regression showed RV mass index (RVMI) was linearly associated with measures of RV load in both the overall cohort and within each group. Multivariable linear regression models revealed a significant interaction between disease type and RVMI adjusting for pulmonary vascular resistance (PVR), age, sex, race, BMI, and LV mass. This model showed a decreased slope in the relationship between RVMI and PVR in the SSc-PAH group compared with the IPAH group. RVMI varies linearly with measures of RV load. After adjusting for multiple potential confounders, patients with SSc-PAH demonstrated significantly less RV hypertrophy with increasing PVR than patients with IPAH. This difference in adaptive hypertrophy may in part explain previously observed decreased contractility and poorer survival in SSc-PAH.

AB - Patients with scleroderma (SSc)–associated pulmonary arterial hypertension (PAH) have worse survival than patients with idiopathic PAH (IPAH). We hypothesized that the right ventricle (RV) adapts differently in SSc-PAH versus IPAH. We used cardiac magnetic resonance imaging (cMRI) and hemodynamic characteristics to assess the relationship between RV morphology and RV load in patients with SSc-PAH and IPAH. In 53 patients with PAH (35 with SSc-PAH and 18 with IPAH) diagnosed by right heart catheterization (RHC), we examined cMRIs obtained within 48 hours of RHC and compared RV morphology between groups. Regression analysis was used to assess the association between diagnosis (IPAH vs. SSc-PAH) and RV measurements after adjusting for age, sex, race, body mass index (BMI), left ventricular (LV) mass, and RV load. There were no significant differences in unadjusted comparisons of cMRI measurements between the two groups. Univariable regression showed RV mass index (RVMI) was linearly associated with measures of RV load in both the overall cohort and within each group. Multivariable linear regression models revealed a significant interaction between disease type and RVMI adjusting for pulmonary vascular resistance (PVR), age, sex, race, BMI, and LV mass. This model showed a decreased slope in the relationship between RVMI and PVR in the SSc-PAH group compared with the IPAH group. RVMI varies linearly with measures of RV load. After adjusting for multiple potential confounders, patients with SSc-PAH demonstrated significantly less RV hypertrophy with increasing PVR than patients with IPAH. This difference in adaptive hypertrophy may in part explain previously observed decreased contractility and poorer survival in SSc-PAH.

KW - Cardiac magnetic resonance imaging

KW - Pulmonary arterial hypertension

KW - Right ventricle

KW - Scleroderma

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