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Summer / Fall 2025

Dr. Vinicio de Jesus Perez discusses The Hidden Epidemic: Meth and PH and the need for compassionate, stigma-free screening on episode 539: I'm Aware That I'm Rare: the phaware® podcast.


Congratulations Drs. Zamanian and Rabinovitch!

The NIH/NHLBI has awarded Roham Zamanian, MD, Professor, Pulmonary, Allergy and Critical Care Medicine, Director, Adult PH Program and Associate Director of the Vera Moulton Wall Center and Marlene Rabinovitch, MD, Dwight and Vera Dunlevie Professorship in Pediatric Cardiology, and Director, Basic Science and Engineering (BASE) Initiative, Betty Irene Moore Children's Heart Center, a 5-year, US$ 12.5 million grant for their proposal for “A Phase 2 Study of Elafin (Tiprelestat) for the Treatment of Pulmonary Arterial Hypertension (PAH)”. The Data Coordinating Center for the trial, led by Dr. Cathie Spino at the SABER unit at the University of Michigan, was funded by a companion application.

PAH is a disease of progressive obliteration of the lung vasculature that results from elastase mediated degradation of elastin, endothelial dysfunction, smooth muscle cell proliferation and chronic peri- and intravascular inflammation. There is an unmet need to find a therapy that is disease modifying in that it addresses these underlying cellular and molecular features of PAH. This proposal tests the safety and efficacy of human recombinant Elafin (tiprelestat) as a treatment for PAH in a randomized placebo-controlled clinical trial across 10 centers. Elafin is ideally suited for this role as it inhibits neutrophil elastase, suppresses cytokine mediated inflammation, and activates BMPR2 receptor signaling resulting in favorable profile of gene expression in hereditary and other forms of PAH. Beyond the primary endpoint of pulmonary vascular resistance (PVR) and numerous secondary endpoints, the study will evaluate the potential for disease modification – a “first-ever” in the field in PAH Clinical Trials.  Beyond the clinical study enrolling centers, the study involves collaborations with three major cores: Harvard University for non-invasive pulmonary vascular imaging and blood volume quantification, UCSF for PK/PD modeling & assessments, and Temple University as the investigational pharmacy.  

What makes this proposal even more special is that it is based on the life-long work of Dr. Marlene Rabinovitch and her leadership in investigating the role of elastase inhibitors in vascular diseases and their pleotropic impact on beneficial BMPR2 mediated gene regulation, combined with theleadership of Dr. Roham Zamanian in clinical investigation and clinical trials for new and promising PAH therapies.

Original post by Mark Nicolls, MD 
Chief, Division of Pulmonary, Allergy & Critical Care Medicine
Associate Dean for ResearchEndowed Chair, The Stanford Professor of Pulmonary & Critical Care Medicine

Research reported in this publication was supported by the National Heart, Lung, And Blood Institute of the National Institutes of Health under Award Number UG3HL180990. The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Institutes of Health.


Maya Kumar, PhD named The Lucile Packard Foundation for Children’s Health Faculty Scholar

Maya Kumar, PhD, Assistant Professor, Pediatrics (Pulmonary Medicine) was named The Lucile Packard Foundation for Children’s Health Faculty Scholar by the Stanford Maternal & Child Health Research Institute (MCHRI) for 2025–2028 for the study: Uncovering the Cellular and Molecular Basis of Pathologic Vascular Changes in Pulmonary Arterial Hypertension and Cystic Fibrosis.

She was also awarded the CF Foundation Research Grant for the study: Uncovering the cellular and molecular basis of bronchial artery dysfunction in cystic fibrosis


Krasnow Lab Publishes 2 Journal Articles in Nature


Dr. Roham Zamanian, discusses the concept of "disease modification" in the treatment of pulmonary hypertension in episode 527: I'm Aware That I'm Rare: the phaware® podcast.


Rachel K. Hopper, MD appointed Director of the Pediatric PH Program, Vera Moulton Wall Center, July 2025

Dr. Hopper is a Clinical Professor in the Division of Pediatric Cardiology in the Department of Pediatrics and Director of the Pediatric Pulmonary Vascular Disease Program in the Children’s Heart Center and Vera Moulton Wall Center for Pulmonary Vascular Disease at Stanford.

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