Borderline Mean Pulmonary Artery Pressure in Patients With Systemic Sclerosis: Transpulmonary Gradient Predicts Risk of Developing Pulmonary Hypertension

Authors

  • Christopher J. Valerio,

    1. Royal Free Hospital, London, UK
    Search for more papers by this author
    • Dr. Valerio has received speaking fees and honoraria from Actelion (less than $10,000) and educational grants from Pfizer and Actelion.

  • Benjamin E. Schreiber,

    1. Royal Free Hospital, London, UK
    Search for more papers by this author
    • Dr. Schreiber has received consulting fees, speaking fees, and/or honoraria from Pfizer, GlaxoSmithKline, Eli Lilly, and Actelion (less than $10,000 each).

  • Clive E. Handler,

    1. Royal Free Hospital, London, UK
    Search for more papers by this author
    • Dr. Handler has received consulting fees, speaking fees, and/or honoraria from Actelion, GlaxoSmithKline, and Pfizer (less than $10,000 each).

  • Christopher P. Denton,

    1. Royal Free Hospital, London, UK
    Search for more papers by this author
    • Dr. Denton has received consulting fees, speaking fees, and/or honoraria from Actelion, GlaxoSmithKline, Pfizer, Roche, and Novartis (less than $10,000 each).

  • John G. Coghlan

    Corresponding author
    1. Royal Free Hospital, London, UK
    • National Pulmonary Hypertension Unit, Royal Free Hospital, Pond Street, London NW3 2QG, UK

    Search for more papers by this author
    • Dr. Coghlan has received consulting and speaking fees from Actelion, GlaxoSmithKline, United Therapeutics, and Pfizer (less than $10,000 each).


Abstract

Objective

To determine whether patients with systemic sclerosis (SSc) and borderline mean pulmonary artery pressure (PAP) at cardiac catheterization are more likely to develop pulmonary hypertension (PH) than those in whom pulmonary pressure is normal.

Methods

Patients with SSc in whom PH and significant interstitial lung disease had been excluded at baseline were enrolled in our prospective cohort. Analysis of followup data identified patients who met prespecified criteria prompting repeat catheterization to reassess for possible PH. Using Kaplan-Meier, receiver operating characteristic, and Cox regression methods, we studied the development of PH and death.

Results

Of 228 patients in this study, 86 had borderline mean PAP (21–24 mm Hg) at baseline. Following prespecified criteria, 76 patients underwent repeat catheterization, and 29 of these developed PH. Two cases were related to disease of the left side of the heart. The average mean PAP increased from baseline (20.2 mm Hg) to followup (24.3 mm Hg) (P < 0.05 by Student's t-test). Patients with borderline mean PAP were more likely to develop PH than patients with mean PAP ≤20 mm Hg (P < 0.001 by log rank test, hazard ratio [HR] 3.7). A transpulmonary gradient (TPG) ≥11 mm Hg at baseline also predicted PH (P < 0.001 by log rank test, HR 7.9). Incident development of pulmonary arterial hypertension (PAH) was not benign, with a mortality of 18% within 3 years.

Conclusion

Our findings indicate that borderline mean PAP and an elevated TPG in patients with SSc predict progression to PH. These patients should be monitored closely for the development of PH. Our findings indicate that catheterization data are useful in patients considered at risk of PAH.

Ancillary