Table 2. Performance of an optimal screening tool#
PopulationPrevalence %Accuracy %PPV %Screening tests/diagnosisRHC/100RHC/diagnosis
PAH-SSc12.582418.3302.5
PortoPH4811726.3236.1
PAH-SCD0.580771.42215.1
PAH-SLE0.58022002041
Idiopathic PAH8015000.2101
  • PPV: positive predictive value; RHC/100: number of right heart catheterisations required per 100 of population screened; RHC/diagnosis: number of right heart catheterisations performed for every patient diagnosed with pulmonary arterial hypertension (PAH); PAH-SSc: PAH associated with systemic sclerosis; PortoPH: portopulmonary pulmonary hypertension; PAH-SCD: PAH associated with sickle cell disease; PAH-SLE: PAH associated with systemic lupus erythematosus. #: assumptions: 96% sensitivity; 80% specificity. : assumed for each population based on available literature and may be underestimates.