1	Relationship of BMPR2 Mutations to Vasoreactivity in Pulmonary Arterial Hypertension C. Greg Elliott, MD; Eric W. Glissmeyer, BS; Gregory T. Havlena, BS; John Carlquist, PhD; Jason T. McKinney, MS; Stuart Rich, MD; Michael D. McGoon, MD; Mary Beth Scholand, MD; Miryoung Kim, BS; Robert L. Jensen, PhD; Jon W. Schmidt; Kenneth Ward, MD LDS Hospital and the University of Utah, Salt Lake City, UT, Johns Hopkins University School of Medicine, University of Hawaii, Mayo Clinic College of Medicine, University of Chicago, Idaho Technology, Inc
2	Background
3	Question
4	Method – Utah PH Database
5	Method
6	Method
7	Results (n=67)
8	Results (n=67)
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10	Results
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12	Conclusion Nonsynonymous BMPR2 mutations identify IPAH or FPAH patients who are unlikely to demonstrate vasoreactivity
13	SUMMARY The presence of nonsynonymous BMPR2 DNA sequence variations (i.e. mutations) predict a lack of acute vasoreactivity in IPAH and FPAH patients
14	"5 IPAH patients had c.2324..." 5 IPAH patients had c.2324 G>A in exon 12 c.2324 G>A is a polymorphism with a reported genotype frequency of 5% and allelic frequency of 2.5% in the general population according to the International Hapmap project Polymorphisms are defined by frequency greater than 1%, not by whether or not they cause PAH
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18	PH Centers Where Vasoreactivity Was Tested
19	Vasodilators for 67 subjects
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22	Screen for BMPR2 sequence variations
23	Confirm BMPR2 sequence variations