Dr. Robyn J. Barst Pediatric PH Research and Mentoring Fund Grant Winner 2014

Rebecca Johnson Kameny, MDRebecca Johnson Kameny, MD

Clinical Instructor in the Division of Pediatrics
University of California, San Francisco
Title: “Right Ventricular Performance in Pediatric Pulmonary Hypertension and Congenital Heart Disease”
Term: December 1, 2014 – November 30, 2015

This Barst Fund Award was made possible through a generous, anonymous donation in honor of pediatric PH patient Cordelia.

Summary of Research Project:

Right ventricle function is the primary determinant of both functional capacity and survival in pulmonary hypertension. Patients with pulmonary hypertension secondary to congenital heart disease have improved survival (for a given degree of pulmonary vascular disease) compared with patients with primary pulmonary hypertension. The mechanisms of this survival benefit are unknown, but might be related to a differential right ventricular response to increased afterload. Similarly, certain subpopulations of patients with pulmonary hypertension have an “adaptive” right ventricular response – with concentric hypertrophy and preserved systolic function – while other patients with pulmonary hypertension progress more rapidly to right ventricular failure. Dr. Kameny’s project seeks an improved understanding of the mechanisms underlying adaptive right ventricular responses to afterload challenge.

Curriculum Vitae

Education/Training

1999-2003: B.A. in Art and Archaeology, Princeton University
2003-2008: Doctorate in Medicine, Boston University
2008-2011: Resident, Boston Combined Residency Program, Harvard University/Boston Univesrity
2011-2014: Fellow, CVRI, University of California, San Francisco

Honors/Awards

2007: Alpha Omega Alpha, American Pathology Honors Society
2008: New England Pediatric Society Award
2011: Benjamin Seigel Teaching Award
2014: Society for Pediatric Research Fellow Basic Research Award
2014: American Heart Association Fellow to Faculty Transition Award
2014: National Institutes of Health Loan Repayment Program Award

Professional Memberships

2008: American Academy of Pediatrics
2011: Pediatric Society of Critical Care Medicine
2011: Society of Critical Care Medicine
2013: American Heart Association
2014: Society for Pediatric Research

Selected Publications

  1. Johnson RC, Leopold JA, Loscalzo J. Vascular calcification: pathobiological mechanisms and clinical implications. Circulation Research, 99(10): 1044-1059, 2006. PMID: 17095733
  2. Liberman M, Johnson RC, Handy DE, Loscalzo J, Leopold JA. Bone morphogenetic protein-2 activates NADPH oxidase to increase endoplasmic reticulum stress and human coronary artery smooth muscle cell calcification. Biochemical and Biophysical Research Communications, 413(3): 436-441, 2011. PMID: 21907184
  3. Johnson RC, Datar SA, Oishi PE, Bennett S, Maki J, Sun C, Johengen M, He Y, Raff GW, Redington AN, Fineman JR. Adaptive right ventricular performance in response to acutely increased afterload in a lamb model of congenital heart disease: evidence for enhanced Anrep effect. American Journal of Physiology: Heart and Circulatory Physiology, 306(8):H1222-30. 2014. PMID: 24561861
  4. Datar SA, Oishi PE, Gong W, Bennett SH, Sun CE, Johengen M, Maki J, Johnson RC, Raff GW, Fineman JR. Altered reactivity and nitric oxide signaling in the isolated thoracic duct from an ovine model of congenital heart disease with increased pulmonary blood flow. American Journal of Physiology: Heart and Circulatory Physiology, 306(7):H954-62. 2014. PMID: 24531811