Rationale: Germline mutations in the gene encoding for bone morphogenetic protein receptor II (BMPR2) are a cause of pulmonary arterial hypertension. Objectives: We conducted a study to determine the influence, if any, of a BMPR2 mutation on clinical outcome. Methods: The French Network of Pulmonary Hypertension obtained data of 223 consecutive patients displaying idiopathic or familial pulmonary arterial hypertension in whom point mutation and large size rearrangements of BMPR2 were screened for. Clinical, functional, and hemodynamic characteristics, as well as outcomes were compared in BMPR2 mutation carriers and noncarriers. Meaurements and Main Results: Sixty-eight BMPR2 mutation carriers (28 familial and 40 idiopathic pulmonary arterial hypertension) were compared to 155 noncarriers (all displaying idiopathic pulmonary arterial hypertension). As compared with noncarriers, BMPR2 mutation carriers were younger at diagnosis of pulmonary arterial hypertension (36.5±14.5 versus 46.0±16.1 years, P<0.0001), had higher mean pulmonary artery pressure (64±13 versus 56±13 mmHg, P<0.0001), lower cardiac index (2.13±0.68 versus 2.50±0.73 L/min/m², P=0.0005), higher pulmonary vascular resistance (17.4±6.1 versus 12.7±6.6 mmHg/L/min/m², P<0.0001), lower mixed venous oxygen saturation (59±9 versus 63±9 percent, P=0.02), shorter time to death or lung transplantation (P=0.044), younger age at death (P=0.002), but similar overall survival (P=0.51). Conclusions: BMPR2 mutation carriers with pulmonary arterial hypertension present approximately 10 years earlier than noncarriers with a more severe hemodynamic compromise at diagnosis.