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10 , 11 , 12 , 17 A number of studies have suggested that the presence of a BMPR2 mutation in heritable PAH is associated with cardiomyopathic changes independent of the load stress imposed by pulmonary vascular remodeling, and thus the BMPR2 mutant mouse model in our study is still applicable to patients with heritable PAH or idiopathic PAH associated with decreases in BMPR2 expression
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