Chest
Volume 123, Issue 2, February 2003, Pages 344-350
Journal home page for Chest

Clinical Investigations
PULMONARY CIRCULATION
Hemodynamics and Survival in Patients With Pulmonary Arterial Hypertension Related to Systemic Sclerosis*

https://doi.org/10.1378/chest.123.2.344Get rights and content

Study objectives:

The goal of this study was to determine whether the survival of patients with pulmonary hypertension related to systemic sclerosis (SScPH) was different from that of patients with other forms of pulmonary arterial hypertension.

Design:

Retrospective cohort study.

Setting:

Tertiary care medical center.

Patients:

Our cohort was composed of 33 patients with pulmonary hypertension that is sporadic, familial, or related to anorexigen use (PPH) and 22 patients with SScPH who underwent initial pulmonary artery catheterization and vasodilator study at our center between January 1997 and June 2001.

Measurements and results:

Patients with SScPH had somewhat lower percentage of predicted lung volumes than patients with PPH (total lung capacity, 80% vs 92%; p = 0.06) and had lower percentage of predicted diffusion capacity of the lung for carbon monoxide (42% vs 68%; p = 0.0002). Right atrial pressure, pulmonary artery pressure, and cardiac index were similar between the groups. Patients with SScPH and PPH were treated with usual medical therapies, such as digoxin, warfarin, and continuous IV epoprostenol. Despite these similarities, the risk of death in patients with SScPH was higher than in patients with PPH (unadjusted hazard ratio, 2.9; 95% confidence interval, 1.1 to 7.8; p = 0.03). This increased risk appeared to persist after adjustment for a variety of demographic, hemodynamic, or treatment variables.

Conclusions:

Despite having similar hemodynamics, patients with SScPH have a higher risk of death than patients with PPH. Future studies of the mechanism and therapy of pulmonary arterial hypertension should focus on the distinctions between the different forms of this disease.

Section snippets

Materials and Methods

We performed a retrospective cohort study of consecutive patients with SScPH or PPH who underwent initial pulmonary artery catheterization and vasodilator study from January 1997 to June 2001 at the University of Pennsylvania Pulmonary Vascular Disease Program. The study was approved by the Committee on Studies Involving Human Beings.

The cohort was assembled using the PICARD database, which contains diagnosis data from all inpatient and outpatient medical contacts in the University of

Patient Population

Our cohort was composed of 33 patients with PPH and 22 patients with SScPH. Of patients with PPH, 22 patients (67%) had sporadic pulmonary hypertension, 3 patients (9%) had familial pulmonary hypertension, and 8 patients (24%) had pulmonary hypertension related to anorexigen use. Of patients with SScPH, 16 patients (73%) had SSc with limited cutaneous scleroderma, 4 patients (18%) had SSc with diffuse cutaneous scleroderma, and 2 patients (9%) had SSc in overlap. Patients with missing data were

Discussion

We did not observe significant differences in demographics or hemodynamics between patients with SScPH and patients with PPH. The somewhat lower mean FVC% and TLC% in patients with SScPH are likely due to mild parenchymal lung disease, which may accompany SScPH.20 Of note, pulmonary function testing revealed mild restriction in many of our patients with PPH as well, which has been documented previously.2122 These differences have unclear clinical relevance.

We found a significantly lower Dlco%

Conclusion

Our results suggest that patients with SScPH have a risk of death that is greater than that of patients with PPH after initial evaluation for vasodilator therapy. Although we have not defined the physiologic factor that mediates this increased risk, it is likely that the fibrotic process affects cardiac and/or pulmonary vascular function in patients with SSc.

The worse outcomes in this patient group may have implications in future clinical trials, as this subgroup may not respond to medical

ACKNOWLEDGMENT

The authors thank Dr. Robert Kotloff, Dr. John Hansen-Flaschen, Dr. Yale Enson, and Dr. Robyn Barst for review of this article.

REFERENCES (37)

  • GG Pietra et al.

    Histopathology of primary pulmonary hypertension: a qualitative and quantitative study of pulmonary blood vessels from 58 patients in the National Heart, Lung, and Blood Institute, Primary Pulmonary Hypertension Registry

    Circulation

    (1989)
  • BH Brundage

    Pulmonary hypertension in collagen vascular disease

  • DB Badesch et al.

    Continuous intravenous epoprostenol for pulmonary hypertension due to the scleroderma spectrum of disease: a randomized, controlled trial

    Ann Intern Med

    (2000)
  • VV McLaughlin et al.

    Compassionate use of continuous prostacyclin in the management of secondary pulmonary hypertension: a case series

    Ann Intern Med

    (1999)
  • RJ Barst et al.

    A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension: The Primary Pulmonary Hypertension Study Group

    N Engl J Med

    (1996)
  • M Humbert et al.

    Short-term and long-term epoprostenol (prostacyclin) therapy in pulmonary hypertension secondary to connective tissue diseases: results of a pilot study

    Eur Respir J

    (1999)
  • R Narang et al.

    Mode of death in chronic heart failure: a request and proposition for more accurate classification

    Eur Heart J

    (1996)
  • DR Cox

    Regression models and life tables (with discussion)

    J R Stat Soc

    (1972)
  • Cited by (387)

    View all citing articles on Scopus

    Supported in part by grants HL07891, and HL04218 (Dr. Taichman) from the National Institutes of Health.

    View full text