PURPOSE: Pulmonary arterial hypertension (PAH) is the most common cause of death in systemic sclerosis (SSc). Given the paucity of robust data, , the Dana Point guidelines have recommended that the exercise criterion should be eliminated. However, exercise hemodynamics may be useful in assessing “early” pulmonary arterial vasculopathy or pulmonary venous hypertension particularly in SSc. The present study examines the findings of exercise right heart catheterizations (ExRHC) in the PHAROS registry.
METHODS: PHAROS is a multicenter cohort including SSc patients at risk for developing PH or established resting PH. ExRHCs were performed according to local protocols. The following PH definitions were used: PAH = mPAP≥25mmHg, wedge ≤15mmHg; PVH = mPAP≥25mmHg, wedge>15mmHg; ePH = mPAP> 30mmHg, wedge ≤18mmHg; ePVH = mPAP>30mmHg, wedge>18mmHg. Clinical features of resting RHC (rRHC) and ExRHC were compared in these patients.
RESULTS: There are 390 patients in PHAROS, 87 patients had ExRHC. In 47 patients (54%) the rRHC was not different than the ExRHC, including 19 patients whose rRHC and ExRHC were both normal (NL). However, in 40 patients (46%) the ExRHC led to a different diagnosis than the rRHC: 22 patients (55%) had a NL rRHC but with exercise had ePH; 9 (22%) had a NL rRHC but with exercise had ePVH. There were 6 patients who had rPAH (mPAP 28mmHg) but with exercise had ePVH (mean wedge 24mmHg). In patients with a NL rRHC, PVR > 150 was strongly associated with ePH, (83% vs 28%, p<0.005). They also were more likely to have a mPAP>17mmHg, (74% vs 28%, p<0.002). A wedge pressure > 10mmHg was strongly associated with ePVH, (89% vs 5%, p<0.0001). The ePVH patients were more likely to have a ‘borderline’ mPAP (>20mmHg) (78% vs 11%, p<0.03).
CONCLUSIONS: In SSc patients an ExRHC can add assist in clarifying the type of pulmonary hypertension that is present. Patients with a ‘normal’ resting RHC who have a PVR > 150 and a mPAP> 17 may have ePH. Those who have a wedge > 10 and particularly with a mPAP>20 may have ePVH.
CLINICAL IMPLICATIONS: Exercise RHC should be considered when symptomatic SSc patients have normal resting hemodynamics.
DISCLOSURE: The following authors have nothing to disclose: Virginia Steen, Rajeev Saggar, Francisco Soto, Tunay Kuru, Dinesh Khanna, Jerry Molitor, Vivien Hsu, Hunter Champion
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