Abstract
Background: Pulmonary arterial hypertension (PAH) in systemic sclerosis (SSc) leads to substantial morbidity and mortality despite modern targeted therapy. Therefore early detection is essential and a reliable non-invasive screening method is needed.
Methods: 76 consecutive SSc patients were assessed in this cohort study by lung function tests, electrocardiogram at rest, lab tests and biomarkers, echocardiography at rest, stress Doppler echocardiography (SDE) and by right heart catheter at rest and during exercise by two different experienced investigators.
Transthoracic echocardiography at rest and SDE were compared with hemodynamic measurement to assess the value and reliability of SDE in the noninvasive detection of PAH in SSc.
Results: 22 of the 76 SSc patients had a manifest pulmonary hypertension, 4 had elevated pulmonary arterial wedge pressures >15mmHg, therefore 24% had an associated pulmonary arterial hypertension (aPAH SSc). 28% of patients had an exercise induced pulmonary hypertension, 43% of patients had normal PA-pressures at rest and during exercise. The sensitivity of echo at rest in diagnosing PAH was 75% with a specificity of 82%. This was improved by using SDE with a sPAP cut-off value of 40mmHg under exercise. SDE could improve sensitivity from 75 to 90% by worsening specificity from 82 to 69%.
Conclusion:Stress Doppler echocardiography is a safe and reliable procedure and might be a useful non-invasive tool for the screening for manifest aPAH in SSc patients. SDE could improve the sensitivity by a slight worsening of specificity, mostly due to concomitant left heart diseases with elevated downstream pressures under exercise.
- © 2014 ERS
