Right ventricular dysfunction in systemic sclerosis-associated pulmonary arterial hypertension

Ryan J. Tedford, James O. Mudd, Reda E. Girgis, Stephen Mathai, Ari L. Zaiman, Traci Housten-Harris, Danielle Boyce, Benjamin W. Kelemen, Anita C. Bacher, Ami Shah, Laura Hummers, Fredrick Wigley, Stuart D. Russell, Rajeev Saggar, Rajan Saggar, William L Maughan, Paul M Hassoun, David A Kass

Research output: Contribution to journalArticle

Abstract

Background-Systemic sclerosis-associated pulmonary artery hypertension (SScPAH) has a worse prognosis compared with idiopathic pulmonary arterial hypertension (IPAH), with a median survival of 3 years after diagnosis often caused by right ventricular (RV) failure. We tested whether SScPAH or systemic sclerosis-related pulmonary hypertension with interstitial lung disease imposes a greater pulmonary vascular load than IPAH and leads to worse RV contractile function. Methods and Results-We analyzed pulmonary artery pressures and mean flow in 282 patients with pulmonary hypertension (166 SScPAH, 49 systemic sclerosis-related pulmonary hypertension with interstitial lung disease, and 67 IPAH). An inverse relation between pulmonary resistance and compliance was similar for all 3 groups, with a near constant resistance×compliance product. RV pressure-volume loops were measured in a subset, IPAH (n=5) and SScPAH (n=7), as well as SSc without PH (n=7) to derive contractile indexes (end-systolic elastance [Ees] and preload recruitable stroke work [Msw]), measures of RV load (arterial elastance [Ea]), and RV pulmonary artery coupling (Ees/Ea). RV afterload was similar in SScPAH and IPAH (pulmonary vascular resistance=7.0±4.5 versus 7.9±4.3 Wood units; Ea=0.9±0.4 versus 1.2±0.5 mm Hg/mL; pulmonary arterial compliance=2.4±1.5 versus 1.7±1.1 mL/mm Hg; P>0.3 for each). Although SScPAH did not have greater vascular stiffening compared with IPAH, RV contractility was more depressed (Ees=0. 8±0.3 versus 2.3±1.1, Pes/E a=1.0±0.5 versus 2.1±1.0; P=0.03). This ratio was higher in SSc without PH (Ees/Ea=2.3±1.2; P=0.02 versus SScPAH). Conclusions-RV dysfunction is worse in SScPAH compared with IPAH at similar afterload, and may be because of intrinsic systolic function rather than enhanced pulmonary vascular resistive and pulsatile loading.

Original languageEnglish (US)
Pages (from-to)953-963
Number of pages11
JournalCirculation: Heart Failure
Volume6
Issue number5
DOIs
StatePublished - Sep 2013

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Right Ventricular Dysfunction
Systemic Scleroderma
Pulmonary Hypertension
Pulmonary Artery
Blood Vessels
Lung Compliance
Interstitial Lung Diseases
Right Ventricular Function
Lung
Ventricular Pressure
Familial Primary Pulmonary Hypertension
Vascular Resistance
Stroke

Keywords

  • Hypertension, pulmonary
  • Scleroderma, systemic
  • Vascular capacitance
  • Vascular resistance
  • Ventricular function, right

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

Cite this

Right ventricular dysfunction in systemic sclerosis-associated pulmonary arterial hypertension. / Tedford, Ryan J.; Mudd, James O.; Girgis, Reda E.; Mathai, Stephen; Zaiman, Ari L.; Housten-Harris, Traci; Boyce, Danielle; Kelemen, Benjamin W.; Bacher, Anita C.; Shah, Ami; Hummers, Laura; Wigley, Fredrick; Russell, Stuart D.; Saggar, Rajeev; Saggar, Rajan; Maughan, William L; Hassoun, Paul M; Kass, David A.

In: Circulation: Heart Failure, Vol. 6, No. 5, 09.2013, p. 953-963.

Research output: Contribution to journalArticle

Tedford, RJ, Mudd, JO, Girgis, RE, Mathai, S, Zaiman, AL, Housten-Harris, T, Boyce, D, Kelemen, BW, Bacher, AC, Shah, A, Hummers, L, Wigley, F, Russell, SD, Saggar, R, Saggar, R, Maughan, WL, Hassoun, PM & Kass, DA 2013, 'Right ventricular dysfunction in systemic sclerosis-associated pulmonary arterial hypertension', Circulation: Heart Failure, vol. 6, no. 5, pp. 953-963. https://doi.org/10.1161/CIRCHEARTFAILURE.112.000008
Tedford, Ryan J. ; Mudd, James O. ; Girgis, Reda E. ; Mathai, Stephen ; Zaiman, Ari L. ; Housten-Harris, Traci ; Boyce, Danielle ; Kelemen, Benjamin W. ; Bacher, Anita C. ; Shah, Ami ; Hummers, Laura ; Wigley, Fredrick ; Russell, Stuart D. ; Saggar, Rajeev ; Saggar, Rajan ; Maughan, William L ; Hassoun, Paul M ; Kass, David A. / Right ventricular dysfunction in systemic sclerosis-associated pulmonary arterial hypertension. In: Circulation: Heart Failure. 2013 ; Vol. 6, No. 5. pp. 953-963.
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abstract = "Background-Systemic sclerosis-associated pulmonary artery hypertension (SScPAH) has a worse prognosis compared with idiopathic pulmonary arterial hypertension (IPAH), with a median survival of 3 years after diagnosis often caused by right ventricular (RV) failure. We tested whether SScPAH or systemic sclerosis-related pulmonary hypertension with interstitial lung disease imposes a greater pulmonary vascular load than IPAH and leads to worse RV contractile function. Methods and Results-We analyzed pulmonary artery pressures and mean flow in 282 patients with pulmonary hypertension (166 SScPAH, 49 systemic sclerosis-related pulmonary hypertension with interstitial lung disease, and 67 IPAH). An inverse relation between pulmonary resistance and compliance was similar for all 3 groups, with a near constant resistance×compliance product. RV pressure-volume loops were measured in a subset, IPAH (n=5) and SScPAH (n=7), as well as SSc without PH (n=7) to derive contractile indexes (end-systolic elastance [Ees] and preload recruitable stroke work [Msw]), measures of RV load (arterial elastance [Ea]), and RV pulmonary artery coupling (Ees/Ea). RV afterload was similar in SScPAH and IPAH (pulmonary vascular resistance=7.0±4.5 versus 7.9±4.3 Wood units; Ea=0.9±0.4 versus 1.2±0.5 mm Hg/mL; pulmonary arterial compliance=2.4±1.5 versus 1.7±1.1 mL/mm Hg; P>0.3 for each). Although SScPAH did not have greater vascular stiffening compared with IPAH, RV contractility was more depressed (Ees=0. 8±0.3 versus 2.3±1.1, Pes/E a=1.0±0.5 versus 2.1±1.0; P=0.03). This ratio was higher in SSc without PH (Ees/Ea=2.3±1.2; P=0.02 versus SScPAH). Conclusions-RV dysfunction is worse in SScPAH compared with IPAH at similar afterload, and may be because of intrinsic systolic function rather than enhanced pulmonary vascular resistive and pulsatile loading.",
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T1 - Right ventricular dysfunction in systemic sclerosis-associated pulmonary arterial hypertension

AU - Tedford, Ryan J.

AU - Mudd, James O.

AU - Girgis, Reda E.

AU - Mathai, Stephen

AU - Zaiman, Ari L.

AU - Housten-Harris, Traci

AU - Boyce, Danielle

AU - Kelemen, Benjamin W.

AU - Bacher, Anita C.

AU - Shah, Ami

AU - Hummers, Laura

AU - Wigley, Fredrick

AU - Russell, Stuart D.

AU - Saggar, Rajeev

AU - Saggar, Rajan

AU - Maughan, William L

AU - Hassoun, Paul M

AU - Kass, David A

PY - 2013/9

Y1 - 2013/9

N2 - Background-Systemic sclerosis-associated pulmonary artery hypertension (SScPAH) has a worse prognosis compared with idiopathic pulmonary arterial hypertension (IPAH), with a median survival of 3 years after diagnosis often caused by right ventricular (RV) failure. We tested whether SScPAH or systemic sclerosis-related pulmonary hypertension with interstitial lung disease imposes a greater pulmonary vascular load than IPAH and leads to worse RV contractile function. Methods and Results-We analyzed pulmonary artery pressures and mean flow in 282 patients with pulmonary hypertension (166 SScPAH, 49 systemic sclerosis-related pulmonary hypertension with interstitial lung disease, and 67 IPAH). An inverse relation between pulmonary resistance and compliance was similar for all 3 groups, with a near constant resistance×compliance product. RV pressure-volume loops were measured in a subset, IPAH (n=5) and SScPAH (n=7), as well as SSc without PH (n=7) to derive contractile indexes (end-systolic elastance [Ees] and preload recruitable stroke work [Msw]), measures of RV load (arterial elastance [Ea]), and RV pulmonary artery coupling (Ees/Ea). RV afterload was similar in SScPAH and IPAH (pulmonary vascular resistance=7.0±4.5 versus 7.9±4.3 Wood units; Ea=0.9±0.4 versus 1.2±0.5 mm Hg/mL; pulmonary arterial compliance=2.4±1.5 versus 1.7±1.1 mL/mm Hg; P>0.3 for each). Although SScPAH did not have greater vascular stiffening compared with IPAH, RV contractility was more depressed (Ees=0. 8±0.3 versus 2.3±1.1, Pes/E a=1.0±0.5 versus 2.1±1.0; P=0.03). This ratio was higher in SSc without PH (Ees/Ea=2.3±1.2; P=0.02 versus SScPAH). Conclusions-RV dysfunction is worse in SScPAH compared with IPAH at similar afterload, and may be because of intrinsic systolic function rather than enhanced pulmonary vascular resistive and pulsatile loading.

AB - Background-Systemic sclerosis-associated pulmonary artery hypertension (SScPAH) has a worse prognosis compared with idiopathic pulmonary arterial hypertension (IPAH), with a median survival of 3 years after diagnosis often caused by right ventricular (RV) failure. We tested whether SScPAH or systemic sclerosis-related pulmonary hypertension with interstitial lung disease imposes a greater pulmonary vascular load than IPAH and leads to worse RV contractile function. Methods and Results-We analyzed pulmonary artery pressures and mean flow in 282 patients with pulmonary hypertension (166 SScPAH, 49 systemic sclerosis-related pulmonary hypertension with interstitial lung disease, and 67 IPAH). An inverse relation between pulmonary resistance and compliance was similar for all 3 groups, with a near constant resistance×compliance product. RV pressure-volume loops were measured in a subset, IPAH (n=5) and SScPAH (n=7), as well as SSc without PH (n=7) to derive contractile indexes (end-systolic elastance [Ees] and preload recruitable stroke work [Msw]), measures of RV load (arterial elastance [Ea]), and RV pulmonary artery coupling (Ees/Ea). RV afterload was similar in SScPAH and IPAH (pulmonary vascular resistance=7.0±4.5 versus 7.9±4.3 Wood units; Ea=0.9±0.4 versus 1.2±0.5 mm Hg/mL; pulmonary arterial compliance=2.4±1.5 versus 1.7±1.1 mL/mm Hg; P>0.3 for each). Although SScPAH did not have greater vascular stiffening compared with IPAH, RV contractility was more depressed (Ees=0. 8±0.3 versus 2.3±1.1, Pes/E a=1.0±0.5 versus 2.1±1.0; P=0.03). This ratio was higher in SSc without PH (Ees/Ea=2.3±1.2; P=0.02 versus SScPAH). Conclusions-RV dysfunction is worse in SScPAH compared with IPAH at similar afterload, and may be because of intrinsic systolic function rather than enhanced pulmonary vascular resistive and pulsatile loading.

KW - Hypertension, pulmonary

KW - Scleroderma, systemic

KW - Vascular capacitance

KW - Vascular resistance

KW - Ventricular function, right

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