Penn Cardiovascular Institute

Pulmonary Vascular Disease Research Program at Penn

Director: Steven Kawut, MD, MS

The Pulmonary Vascular Disease Program at the Penn Cardiovascular Institute (CVI) is focused on understanding the mechanisms of a variety of diseases characterized by pulmonary vascular dysfunction and pulmonary hypertension and the subsequent effects on the right side of the heart.

Image of small, muscular pulmonary artery

Small Muscular Pulmonary Artery

Showing endothelial hypertrophy, medial thickening, and increased adventitia.

Comprehensive Pulmonary Vascular Disease Research at Penn

This Pulmonary Vascular Disease Program includes researchers and clinicians from various disciplines, including:

  • Pulmonology
  • Cardiology
  • Pathology
  • Developmental and cell biology
  • Radiology
  • Surgery
  • Biostatistics
  • Genetics

Extensive experience in cell biology, cardiovascular function, developmental biology, electrophysiology, high-throughput genetics, physiology, epidemiology, biostatistics, clinical trials, and clinical care of patients with a variety of pulmonary vascular diseases and/or right heart failure enables the translation of research questions into feasible studies and new approaches to the treatment of lung disease.

Basic and Translational Pulmonary Research Themes at Penn

The basic and translational laboratories within this program unit provide an established infrastructure for studies of cellular function, myocardial physiology, animal models of right ventricular failure, transcriptional profiling, and molecular genetics. Several research themes form the backbone of the basic science program. These themes include studies of molecular determinants of pulmonary vascular development.

Other areas of interest include studies of cell-matrix interactions, endothelial cell-cell adhesion and the regulation of the perivascular environment by vascular endothelium. Investigators are performing translational research studies of the mTOR signaling pathway in pulmonary artery smooth muscle from both animal models and patients with pulmonary arterial hypertension.

The Penn Center for Cell and Tissue Studies in Pulmonary Vascular Disease

The Cardiovascular Medical Research and Education Fund supports a multicenter study of pulmonary arterial hypertension (Pulmonary Hypertension Breakthrough Initiative) and helped establish the Penn Center for Cell and Tissue Studies in Pulmonary Vascular Disease, directed by Horace Delisser, MD, Steven Kawut, MD, MS, and Kaori Ihida-Stansbury, PhD.

The center acquires, isolates, and phenotypes lung vascular cells from normal individuals and those with significant pulmonary vascular disease. The center then distributes these biologic materials to the U.S. multicenter network and collaborates with a variety of investigators at Penn.

Clinical Pulmonary Vascular Disease Research at Penn

Investigators in the Pulmonary Vascular Disease Program have led National Institutes of Health (NIH)-sponsored, multicenter randomized clinical trials as well as industry trials in pulmonary arterial hypertension. The established infrastructure facilitates initiation of clinical trials with precise data collection on well-phenotyped patients with pulmonary vascular disease. MESA-RV is a NIH-sponsored study of right ventricular function in a cohort of several thousand individuals without clinical cardiovascular disease.

Other Clinical Research Areas

  • A large, NIH-funded multicenter epidemiologic study of the role of sex hormones in portopulmonary hypertension.
  • Studies of quality-of-life and symptom complexes in patients with pulmonary arterial hypertension.
  • Novel approaches to cardiac imaging. Penn is a member of the ongoing REVEAL Registry, designed to study the current practices in diagnosis and treatment of pulmonary arterial hypertension in the U.S.
  • Program members also study the contribution of the pulmonary vasculature to acute lung injury after lung transplantation (Lung Transplant Outcomes Group).

Integrated with the Established Penn Pulmonary Clinical Program

Many of the research projects depend on the seamless integration of investigators with the pulmonary vascular disease clinical program at the University of Pennsylvania. The Penn Pulmonary Vascular Disease Clinical Program was started more than 25 years ago and is one of the nation's largest pulmonary vascular disease programs. The program has multiple locations:

The clinical program evaluates patients with all types of pulmonary vascular disorders, including pulmonary arterial hypertension, chronic thromboembolic disease, hepatopulmonary syndrome, and pulmonary hypertension due to left-sided heart disease.