Correlation of pulmonary function variables with hemodynamic measurements in patients with pulmonary arterial hypertension

Background: Pulmonary arterial hypertension is a life-limiting complication in patients with systemic sclerosis. Current recommendations suggest a minimum screening of an annual transthoracic echocardiogram and pulmonary function test. We hypothesize that modifiable provider-level hurdles contribute to inadequate screening and can be alleviated. Methods and Results: We performed a longitudinal study over 23 months of all systemic sclerosis patients seen in pulmonary and/or rheumatology clinics. Pulmonary arterial hypertension screening was measured monthly as percentage of patients with up-to-date transthoracic echocardiogram and pulmonary function test out of the total systemic sclerosis patients with an outpatient encounter that month. Tests were considered “up-to-date” if they were performed 12 months prior or within the next 6 months post-encounter. Baseline adherence to PAH screening was assessed using a 9-month preproject period. Physicians in rheumatology and pulmonary clinics were surveyed for knowledge and perceived as barriers to screening. Interventions focused on provider-level hurdles to improve the screening rate to >90%. Adherence to systemic sclerosis–associated pulmonary arterial hypertension was compared between baseline and post-intervention phases as a continuous variable using nonparametric Mann–Whitney–Wilcoxon test and as categorical variable using chi-square test for overall adherence rate. A total of 18 providers were surveyed, of which 67% knew minimal screening recommendations and 44% identified “difficulty ordering tests” as a barrier. Our interventions were as follows: (1) didactic education and literature dissemination and (2) best practice alert in the electronic medical record to facilitate just-in-time ordering of screening. Adherence was measured for 23 months, comprising 166 systemic sclerosis patients. Monthly adherence to pulmonary arterial hypertension screening increased by 10.4% (69.2%–79.6%, p = 0.01 with chi-square and p = 0.09 with Mann–Whitney–Wilcoxon test), with an increase in transthoracic echocardiogram by 7.5% and pulmonary function test by 4.9%. Conclusion: Provider-level hurdles contribute to sub-optimal systemic sclerosis–associated pulmonary arterial hypertension screening. Lack of knowledge and difficulty in ordering tests were major barriers. Structured education and decision-support aids improve screening. These interventions, however, were insufficient to improve screening to a goal target of >90%.

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Combination of Echocardiographic and Pulmonary Function Test Measures Improves Sensitivity for Diagnosis of Systemic Sclerosis-associated Pulmonary Arterial Hypertension: Analysis of 2 Cohorts

The Journal of Rheumatology ◽

10.3899/jrheum.130400 ◽

2013 ◽

Vol 40 (10) ◽

pp. 1706-1711 ◽

Cited By ~ 16

Author(s):

Heather Gladue ◽

Virginia Steen ◽

Yannick Allanore ◽

Rajeev Saggar ◽

Rajan Saggar ◽

...

Keyword(s):

Systemic Sclerosis ◽

Pulmonary Arterial Hypertension ◽

Pulmonary Function ◽

Arterial Hypertension ◽

Pulmonary Function Test ◽

Predictive Value ◽

Systolic Pressure ◽

Predictive Values ◽

Function Test ◽

Pulmonary Arterial

Objective.To evaluate routinely collected non-invasive tests from 2 systemic sclerosis (SSc) cohorts to determine their predictive value alone and in combination versus right heart catheterization (RHC)-confirmed pulmonary arterial hypertension (PAH).Methods.We evaluated 2 cohorts of patients who were at risk or with incident PAH: (1) The Pulmonary Hypertension Assessment and Recognition Outcomes in Scleroderma (PHAROS) cohort and (2) an inception SSc cohort at Cochin Hospital, Paris, France. Estimated right ventricular systolic pressure (eRVSP) as determined by transthoracic echocardiogram (TTE) and pulmonary function test (PFT) measures was evaluated, and the predictive values determined. We then evaluated patients with PAH missed on TTE cutoffs that were subsequently identified by a PFT measure.Results.In the PHAROS cohort (n = 206), 59 (29%) had RHC-defined PAH. An eRVSP threshold of 35–50 mm Hg failed to diagnose PAH in 7% to 31% of patients, 50% to 70% of which (n = 2–13) were captured by PFT measures. In the Cochin cohort (n = 141), 10 (7%) patients had RHC confirmed PAH. An eRVSP threshold of 35–50 mm Hg missed 0% to 70% (n = 0–7) of patients, of which 0% to 68% (n = 0–6) were met by PFT measures. The combination of TTE and PFT improved the negative predictive value for diagnosing PAH.Conclusion.In 2 large SSc cohorts, screening with TTE and PFT captured a majority of patients with PAH. TTE and PFT complement each other for the diagnosis of PAH.

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