Pulmonary arterial hypertension (PAH) is a major cause of death in patients with scleroderma. Right heart catheterization (RHC) still remains the gold-standard for diagnosing or ruling out PAH. However, lung function testing (PFT), echocardiography, cardiopulmonary exercise testing (CPET), biomarkers, and 6-minute walking distance (6MWD) are routinely performed as non-invasive tests for screening purposes, as well as for initial workup when suspecting PAH. Here, we analyzed the correlation of these non-invasive tests with the presence of PAH which was confirmed or ruled out by RHC.
We prospectively recruited 31 consecutive patients with scleroderma from the Dermatology Clinic of the Cologne University Hospital. None of the patients had previously been diagnosed with PAH. Each patient underwent PFT, echocardiography, CPET as well as measurements of the 6MWD and NT-proBNP serum levels. Whenever suspicion of PAH was raised, a RHC was performed. The diagnosis of PAH was made according to the current definition (mean pulmonary artery pressure > 25 mmHg, pulmonary capillary wedge pressure ≤ 15 mmHg). In the subgroup of patients who underwent RHC, all measured non-invasive parameters were analyzed with respect to their ability of detecting the presence or absence of the disease.
In 17 of the 31 patients, RHC was performed due to abnormalities in the non-invasive diagnostic workup. In this subgroup, 10 patients were found to have PAH, and in 7 patients the disease was ruled out. The correlation of the non-invasively obtained key measurements with the definite diagnosis of PAH in these 17 pts are shown in table 1. The most relevant parameters in this cohort were ventilation/perfusion mismatch during exercise, the 6-MWD, the estimated pulmonary artery pressure, and NTpro BNP levels.
Non-invasive diagnostic tests are important for the initial workup of scleroderma patients suspected to have PAH, as well as for PAH screening procedures.
A larger data set with serial measurements over time are needed to demonstrate that non-invasive diagnostic procedures are equally reliable for confirming or ruling-out the diagnosis of scleroderma-related PAH.
Daniel Dumitrescu, No Financial Disclosure Information; No Product/Research Disclosure Information