Right ventricular dysfunction in systemic sclerosis-associated pulmonary arterial hypertension

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

    Research output: Contribution to journalArticle

    104 Citations (Scopus)

    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

    Fingerprint

    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

    Tedford, R. J., Mudd, J., Girgis, R. E., Mathai, S. C., Zaiman, A. L., Housten-Harris, T., ... Kass, D. A. (2013). Right ventricular dysfunction in systemic sclerosis-associated pulmonary arterial hypertension. Circulation: Heart Failure, 6(5), 953-963. https://doi.org/10.1161/CIRCHEARTFAILURE.112.000008

    Right ventricular dysfunction in systemic sclerosis-associated pulmonary arterial hypertension. / Tedford, Ryan J.; Mudd, James; Girgis, Reda E.; Mathai, Stephen C.; Zaiman, Ari L.; Housten-Harris, Traci; Boyce, Danielle; Kelemen, Benjamin W.; Bacher, Anita C.; Shah, Ami A.; Hummers, Laura K.; Wigley, Fredrick M.; Russell, Stuart D.; Saggar, Rajeev; Saggar, Rajan; Maughan, W. Lowell; 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, J, Girgis, RE, Mathai, SC, Zaiman, AL, Housten-Harris, T, Boyce, D, Kelemen, BW, Bacher, AC, Shah, AA, Hummers, LK, Wigley, FM, 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 ; Girgis, Reda E. ; Mathai, Stephen C. ; Zaiman, Ari L. ; Housten-Harris, Traci ; Boyce, Danielle ; Kelemen, Benjamin W. ; Bacher, Anita C. ; Shah, Ami A. ; Hummers, Laura K. ; Wigley, Fredrick M. ; Russell, Stuart D. ; Saggar, Rajeev ; Saggar, Rajan ; Maughan, W. Lowell ; 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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    title = "Right ventricular dysfunction in systemic sclerosis-associated pulmonary arterial hypertension",
    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.",
    keywords = "Hypertension, pulmonary, Scleroderma, systemic, Vascular capacitance, Vascular resistance, Ventricular function, right",
    author = "Tedford, {Ryan J.} and James Mudd and Girgis, {Reda E.} and Mathai, {Stephen C.} and Zaiman, {Ari L.} and Traci Housten-Harris and Danielle Boyce and Kelemen, {Benjamin W.} and Bacher, {Anita C.} and Shah, {Ami A.} and Hummers, {Laura K.} and Wigley, {Fredrick M.} and Russell, {Stuart D.} and Rajeev Saggar and Rajan Saggar and Maughan, {W. Lowell} and Hassoun, {Paul M.} and Kass, {David A.}",
    year = "2013",
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    doi = "10.1161/CIRCHEARTFAILURE.112.000008",
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    T1 - Right ventricular dysfunction in systemic sclerosis-associated pulmonary arterial hypertension

    AU - Tedford, Ryan J.

    AU - Mudd, James

    AU - Girgis, Reda E.

    AU - Mathai, Stephen C.

    AU - Zaiman, Ari L.

    AU - Housten-Harris, Traci

    AU - Boyce, Danielle

    AU - Kelemen, Benjamin W.

    AU - Bacher, Anita C.

    AU - Shah, Ami A.

    AU - Hummers, Laura K.

    AU - Wigley, Fredrick M.

    AU - Russell, Stuart D.

    AU - Saggar, Rajeev

    AU - Saggar, Rajan

    AU - Maughan, W. Lowell

    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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