State-of-the-Art Lecture Addresses Vascular Disorders

Harry (Hal) Dietz


A state-of-the-art lecture will provide insights into vascular disorders on Nov. 8. Harry (Hal) Dietz, MD, will speak on “Found in Translation: New Insights into the Pathogenesis and Treatment of Marfan Syndrome and Related Disorders.”

Dr. Dietz is Victor A. McKusick Professor of Pediatrics, Medicine, and Molecular Biology & Genetics in the Institute of Genetic Medicine at the Johns Hopkins University School of Medicine. He is also an investigator in the Howard Hughes Medical Institute.

Dr. Dietz heads a multidisciplinary clinic for the diagnosis and management of individuals with heritable forms of cardiovascular disease, with a special emphasis on Marfan syndrome and related connective tissue disorders. Dr. Dietz’s research team has investigated the development and homeostasis of the arterial wall, with an emphasis on understanding the genetic factors that predispose patients to aortic aneurysm, a condition that accounts for up to 2 percent of deaths in industrialized countries. An understanding of Marfan syndrome could provide insight into vascular wall biology because it is caused by mutations in a single gene and its etiology includes aortic aneurysms.

Dr. Dietz’s group linked an error in the gene that encodes fibrillin-1, a connective tissue protein, to Marfan syndrome, and has helped uncover genes underlying four other conditions that cause aortic aneurysms. Dr. Dietz is currently conducting a clinical trial of a drug approved for hypertension treatment to test its effectiveness in treating Marfan syndrome.

In addition to Marfan syndrome, his team is studying the vascular disorders familial tetralogy of Fallot, cerebral cavernous malformation, a premature aging syndrome, and Loeys-Dietz syndrome. The latter syndrome is named for Dr. Dietz and a Johns Hopkins colleague.

Dr. Dietz received his undergraduate training in biomedical engineering at Duke University and his medical degree from the Health Sciences University of Syracuse. He obtained clinical and research training in pediatrics, pediatric cardiology, and genetics at the Johns Hopkins University School of Medicine.

Dr. Dietz has received several prestigious awards, including the Curt Stern Award from the American Society of Human Genetics and the Taubman Prize for excellence in translational medical science. He is an inductee of the American Society for Clinical Investigation, American Association for the Advancement of Science, Institute of Medicine, Association of American Physicians, and the National Academy of Sciences.

October-November 2013 (Vol. 5, Number 10 & 11)