Pulmonary hypertension in patients with a subaortic right ventricle

Prevalence, impact and management

Alexander Van De Bruaene, Norihisa Toh, Edward J. Hickey, Lee Benson, Eric Horlick, John T. Granton, William G. Williams, S. Lucy Roche

Research output: Contribution to journalArticle

Abstract

Objective: This study sought to determine the prevalence, predictors, prognostic relevance and evolution of pulmonary hypertension (PH) (mean pulmonary artery pressure ≥25 mm Hg) in adult patients with a subaortic right ventricle (RV) in a biventricular circulation (2V-RV). Methods: We analysed retrospective data from patients with 2V-RV undergoing cardiac catheterisation in our centre between 2000 and 2018. Echocardiographic assessment of subpulmonary ventricular pressures (left ventricular systolic pressure (LVSP)), age and B-type natriuretic peptide (BNP) were assessed as PH screening tools. Kaplan-Meier curves examined time to a composite outcome of death, transplant or ventricular assist device (VAD). Data from repeat catheterisations were analysed to evaluate PH changes over time, including the effects of therapy. Results: A total of 141 patients (median age 39 (IQR 33-45) years, 68% men) underwent 191 cardiac catheterisations. At baseline, 55% had PH (isolated postcapillary 24%, combined precapillary and postcapillary 26% and precapillary 5%). BNP (area under the curve 0.80; 95% CI 0.72 to 0.88; p<0.0001), but not age at catheterisation or echocardiographic estimates of LVSP were associated with the presence of PH. The absence of PH and BNP <100 pg/mL discriminated a subgroup at very low risk during short-term (2.5 (1.3-3.9) years) follow-up (p<0.0001). Diuretics, milrinone and VAD improved haemodynamics over time. Conclusion: PH is prevalent in patients with 2V-RV even when asymptomatic. It is difficult to identify by echocardiography and most importantly, is strongly associated with adverse outcomes. PH affects prognosis and transplant options for this patient group and yet is often amenable to treatment. Awareness of these results ought to lower the threshold for invasive haemodynamic assessment and may change the management of failing patients with 2V-RV.

Original languageEnglish
JournalHeart
DOIs
Publication statusPublished - Jan 1 2019
Externally publishedYes

Fingerprint

Pulmonary Hypertension
Heart Ventricles
Brain Natriuretic Peptide
Ventricular Pressure
Heart-Assist Devices
Cardiac Catheterization
Catheterization
Hemodynamics
Milrinone
Blood Pressure
Transplants
Diuretics
Pulmonary Artery
Area Under Curve
Echocardiography
Pressure
Therapeutics

Keywords

  • heart failure
  • pulmonary hypertension
  • systemic right ventricle
  • transposition of the great arteries
  • VAD

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

Cite this

Pulmonary hypertension in patients with a subaortic right ventricle : Prevalence, impact and management. / Van De Bruaene, Alexander; Toh, Norihisa; Hickey, Edward J.; Benson, Lee; Horlick, Eric; Granton, John T.; Williams, William G.; Roche, S. Lucy.

In: Heart, 01.01.2019.

Research output: Contribution to journalArticle

Van De Bruaene, Alexander ; Toh, Norihisa ; Hickey, Edward J. ; Benson, Lee ; Horlick, Eric ; Granton, John T. ; Williams, William G. ; Roche, S. Lucy. / Pulmonary hypertension in patients with a subaortic right ventricle : Prevalence, impact and management. In: Heart. 2019.
@article{3dc4f0ea48d142cfbd81289f46a82b1c,
title = "Pulmonary hypertension in patients with a subaortic right ventricle: Prevalence, impact and management",
abstract = "Objective: This study sought to determine the prevalence, predictors, prognostic relevance and evolution of pulmonary hypertension (PH) (mean pulmonary artery pressure ≥25 mm Hg) in adult patients with a subaortic right ventricle (RV) in a biventricular circulation (2V-RV). Methods: We analysed retrospective data from patients with 2V-RV undergoing cardiac catheterisation in our centre between 2000 and 2018. Echocardiographic assessment of subpulmonary ventricular pressures (left ventricular systolic pressure (LVSP)), age and B-type natriuretic peptide (BNP) were assessed as PH screening tools. Kaplan-Meier curves examined time to a composite outcome of death, transplant or ventricular assist device (VAD). Data from repeat catheterisations were analysed to evaluate PH changes over time, including the effects of therapy. Results: A total of 141 patients (median age 39 (IQR 33-45) years, 68{\%} men) underwent 191 cardiac catheterisations. At baseline, 55{\%} had PH (isolated postcapillary 24{\%}, combined precapillary and postcapillary 26{\%} and precapillary 5{\%}). BNP (area under the curve 0.80; 95{\%} CI 0.72 to 0.88; p<0.0001), but not age at catheterisation or echocardiographic estimates of LVSP were associated with the presence of PH. The absence of PH and BNP <100 pg/mL discriminated a subgroup at very low risk during short-term (2.5 (1.3-3.9) years) follow-up (p<0.0001). Diuretics, milrinone and VAD improved haemodynamics over time. Conclusion: PH is prevalent in patients with 2V-RV even when asymptomatic. It is difficult to identify by echocardiography and most importantly, is strongly associated with adverse outcomes. PH affects prognosis and transplant options for this patient group and yet is often amenable to treatment. Awareness of these results ought to lower the threshold for invasive haemodynamic assessment and may change the management of failing patients with 2V-RV.",
keywords = "heart failure, pulmonary hypertension, systemic right ventricle, transposition of the great arteries, VAD",
author = "{Van De Bruaene}, Alexander and Norihisa Toh and Hickey, {Edward J.} and Lee Benson and Eric Horlick and Granton, {John T.} and Williams, {William G.} and Roche, {S. Lucy}",
year = "2019",
month = "1",
day = "1",
doi = "10.1136/heartjnl-2019-314756",
language = "English",
journal = "Heart",
issn = "1355-6037",
publisher = "BMJ Publishing Group",

}

TY - JOUR

T1 - Pulmonary hypertension in patients with a subaortic right ventricle

T2 - Prevalence, impact and management

AU - Van De Bruaene, Alexander

AU - Toh, Norihisa

AU - Hickey, Edward J.

AU - Benson, Lee

AU - Horlick, Eric

AU - Granton, John T.

AU - Williams, William G.

AU - Roche, S. Lucy

PY - 2019/1/1

Y1 - 2019/1/1

N2 - Objective: This study sought to determine the prevalence, predictors, prognostic relevance and evolution of pulmonary hypertension (PH) (mean pulmonary artery pressure ≥25 mm Hg) in adult patients with a subaortic right ventricle (RV) in a biventricular circulation (2V-RV). Methods: We analysed retrospective data from patients with 2V-RV undergoing cardiac catheterisation in our centre between 2000 and 2018. Echocardiographic assessment of subpulmonary ventricular pressures (left ventricular systolic pressure (LVSP)), age and B-type natriuretic peptide (BNP) were assessed as PH screening tools. Kaplan-Meier curves examined time to a composite outcome of death, transplant or ventricular assist device (VAD). Data from repeat catheterisations were analysed to evaluate PH changes over time, including the effects of therapy. Results: A total of 141 patients (median age 39 (IQR 33-45) years, 68% men) underwent 191 cardiac catheterisations. At baseline, 55% had PH (isolated postcapillary 24%, combined precapillary and postcapillary 26% and precapillary 5%). BNP (area under the curve 0.80; 95% CI 0.72 to 0.88; p<0.0001), but not age at catheterisation or echocardiographic estimates of LVSP were associated with the presence of PH. The absence of PH and BNP <100 pg/mL discriminated a subgroup at very low risk during short-term (2.5 (1.3-3.9) years) follow-up (p<0.0001). Diuretics, milrinone and VAD improved haemodynamics over time. Conclusion: PH is prevalent in patients with 2V-RV even when asymptomatic. It is difficult to identify by echocardiography and most importantly, is strongly associated with adverse outcomes. PH affects prognosis and transplant options for this patient group and yet is often amenable to treatment. Awareness of these results ought to lower the threshold for invasive haemodynamic assessment and may change the management of failing patients with 2V-RV.

AB - Objective: This study sought to determine the prevalence, predictors, prognostic relevance and evolution of pulmonary hypertension (PH) (mean pulmonary artery pressure ≥25 mm Hg) in adult patients with a subaortic right ventricle (RV) in a biventricular circulation (2V-RV). Methods: We analysed retrospective data from patients with 2V-RV undergoing cardiac catheterisation in our centre between 2000 and 2018. Echocardiographic assessment of subpulmonary ventricular pressures (left ventricular systolic pressure (LVSP)), age and B-type natriuretic peptide (BNP) were assessed as PH screening tools. Kaplan-Meier curves examined time to a composite outcome of death, transplant or ventricular assist device (VAD). Data from repeat catheterisations were analysed to evaluate PH changes over time, including the effects of therapy. Results: A total of 141 patients (median age 39 (IQR 33-45) years, 68% men) underwent 191 cardiac catheterisations. At baseline, 55% had PH (isolated postcapillary 24%, combined precapillary and postcapillary 26% and precapillary 5%). BNP (area under the curve 0.80; 95% CI 0.72 to 0.88; p<0.0001), but not age at catheterisation or echocardiographic estimates of LVSP were associated with the presence of PH. The absence of PH and BNP <100 pg/mL discriminated a subgroup at very low risk during short-term (2.5 (1.3-3.9) years) follow-up (p<0.0001). Diuretics, milrinone and VAD improved haemodynamics over time. Conclusion: PH is prevalent in patients with 2V-RV even when asymptomatic. It is difficult to identify by echocardiography and most importantly, is strongly associated with adverse outcomes. PH affects prognosis and transplant options for this patient group and yet is often amenable to treatment. Awareness of these results ought to lower the threshold for invasive haemodynamic assessment and may change the management of failing patients with 2V-RV.

KW - heart failure

KW - pulmonary hypertension

KW - systemic right ventricle

KW - transposition of the great arteries

KW - VAD

UR - http://www.scopus.com/inward/record.url?scp=85065239497&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85065239497&partnerID=8YFLogxK

U2 - 10.1136/heartjnl-2019-314756

DO - 10.1136/heartjnl-2019-314756

M3 - Article

JO - Heart

JF - Heart

SN - 1355-6037

ER -