Molecular Mechanism of Congenital Heart Disease and Pulmonary Hypertension

Department of Pediatrics and Cell and Developmental Biology,Vanderbilt University Medical Center,Nashville,USA Dr. Baldwin’s research efforts have concentrated on delineating the molecular basis of vascular development in the mammalian embryo as an approach to understanding the etiology of congenital heart diseases. His laboratory efforts are based on the hypothesis that the developing vasculature provides important patterning information that directs subsequent cardiac and pulmonary morphogenetic events. Investigations have focused on two areas: 1) the role of endothelial cell adhesion molecules, particularly PECAM-1, in regulating vascular ontogeny and 2) the role of NFATc-1 in specification of endocardial development during early organogenesis. 

اظهر أقل

UCSF Benioff Children’s Hospital,University of California,San Francisco,USA   Research Interests: Endothelial regulation of the pulmonary circulation during normal development and during the development of pediatric pulmonary hypertension disorders. Endothelial dysfunction in pediatric pulmonary hypertension Summary: Pulmonary hypertension, high blood pressure in the lungs, is a serious disorder in subsets of neonates, infants, and children. These include newborns with persistent pulmonary hypertension of the newborn (PPHN), children with congenital heart defects, and teenagers and young adults with primary pulmonary hypertension. The vascular endothelium (the cells that line the blood vessels in the lungs), via the production of vasoactive factors such as nitric oxide and endothelin-1, are important regulators of the tone and growth of pulmonary blood vessels. We utilize an integrated physiologic, biochemical, molecular, and anatomic approach, to study the potential role of aberrant endothelial function in the pathophysiology of pulmonary hypertensive disorders. To this end, we utilize fetal surgical techniques to create animal models of congenital heart disease, and investigate the early role of endothelial alterations in the pathophysiology of pulmonary hypertension secondary to congenital heart disease with increased pulmonary blood flow. Our clinical research interests include the use of pulmonary vasodilator therapy for pediatric pulmonary hypertension, and the use of peri-operative BNP levels as marker of outcome following repair of congenital heart disease.

اظهر أقل

Division of Pediatric Cardiology, Department of Pediatrics,Keio University ,School of Medicine,Tokyo,Japan