Isolated Exercise-Induced Pulmonary Hypertension Associates with Higher Cardiovascular Risk in Scleroderma Patients

Background and aim: Isolated exercise-induced pulmonary hypertension (ExPH) associates with cardiovascular (CV) events in patients with left heart disease. We investigated its prognostic significance in scleroderma patients at risk for pulmonary arterial hypertension (PAH). Methods: In 26 consecutive scleroderma female patients with either low (n = 13) or intermediate probability (n = 13) of pulmonary hypertension (PH) at rest, we evaluated, both at time 0 and 1 year, prognostic determinants of CV risk: onset or progression of heart failure/syncope; worsening of functional class; functional performance at the 6-minute walking test and at cardiopulmonary exercise test; right atrial area; and pericardial effusion. We assigned a severity score 1-3 to each prognostic determinant, derived an overall CV risk score, and its 0-1 year change. Isolated ExPH during the cardiopulmonary exercise test (CPET) was defined as absence of PH at rest, reduced peak VO2, VE/VCO2 >30 at anaerobic threshold, reduced O2 pulse, and ΔVO2/ΔW <9 mL/min/W. We then correlated ExPH at time 0 with clinical worsening (risk score increase >20% after 1 year). Results: ExPH was strongly associated with clinical worsening compared to patients without ExPH (p = 0.005). In patients without ExPH, none had > 20% increased CV risk score after 1 year. Conversely, about 50% of patients with ExPH had such an increase, suggesting a worsening of prognosis. Conclusions: Isolated ExPH associates with higher cardiovascular risk and thus clinical worsening in scleroderma patients. The assessment of ExPH by CPET can thus contribute to a better risk stratification and the planning of a more adequate follow-up.