Table 2.

Comparison of Pathways and Variant Frequency in Vasodilator-Responsive Pulmonary Arterial Hypertension versus Vasodilator-Nonresponsive Pulmonary Arterial Hypertension

	VR-PAH (n = 17)		VN-PAH (n = 19)
	No. of Patients with Variant	No. of Affected Genes	No. of Patients with Variant	No. of Affected Genes
Wnt signaling	13	14	11	12
Vascular smooth muscle contraction	7	6*	1	1
Mutated variants with 2+ IPAH	12	33	10	10
Extracellular matrix organization	10	17	13	16
TGF-β signaling	5	7	3	3

Definition of abbreviations: IPAH = idiopathic pulmonary arterial hypertension; TGF = transforming growth factor; VN-PAH = vasodilator-nonresponsive pulmonary arterial hypertension; VR-PAH = vasodilator-responsive pulmonary arterial hypertension.

^*P = 0.0155.