EKG – RVH and right axis deviation Echocardiogram – elevated PASP, TR Right heart catheterization – mean arterial pressure ≥25 mm Hg confirms diagnosis CT thorax – useful to exclude other secondary causes CTPA – useful to rule out chronic embolic disease as a cause Check anti-centromere, anti-Scl-70, anti-U1RNP (to rule out scleroderma and other overlap syndromes). The gene discussed is SNRNP70; the disease is overlapping connective tissue disease.