Clinical implications of determining BMPR2 mutation status in a large cohort of children and adults with pulmonary arterial hypertension.

@article{Rosenzweig2008ClinicalIO,
  title={Clinical implications of determining BMPR2 mutation status in a large cohort of children and adults with pulmonary arterial hypertension.},
  author={Erika S Berman Rosenzweig and Jane H. Morse and James A Knowles and Kiran K Chada and Amar M Khan and Kari E. Roberts and Jude J. McElroy and Nicole K Juskiw and Nicole C Mallory and Stuart Rich and Beverly Diamond and Robyn J. Barst},
  journal={The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation},
  year={2008},
  volume={27 6},
  pages={668-74}
}
BACKGROUND Bone morphogenetic protein receptor type 2 (BMPR2) mutations occur in idiopathic and familial pulmonary arterial hypertension (IPAH, FPAH); however, the impact of these mutations on clinical assessment and disease severity remains unclear. We investigated the role of BMPR2 mutations on acute vasoreactivity and disease severity in IPAH/FPAH children and adults. METHODS BMPR2 mutation types were determined in 147 IPAH/FPAH patients. Hemodynamics were obtained at baseline and with… CONTINUE READING

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