transforming-growth-factor-beta and Eisenmenger-Complex

There have been remarkable advances in our understanding of the pathobiology of pulmonary hypertension. A region on chromosome 2 encoding bone morphogenetic receptor type 2 has been identified to underlie familial and many cases of sporadic primary pulmonary arterial hypertension. The vasoactive mediators, discovered and defined by vascular biologists, have been translated into promising treatments of human disease. Prostacyclin, endothelin receptor blockers, sildenafil, and nitric oxide have been applied therapeutically to limit, and occasionally reverse, the inexorable damage to the pulmonary circulation initiated by recently identified genetic and environmental triggers of pulmonary arterial hypertension.

Topics: Antihypertensive Agents; Bone Morphogenetic Protein Receptors, Type II; Bronchodilator Agents; Child; Chromosomes, Human, Pair 2; Eisenmenger Complex; Epoprostenol; Humans; Hypertension, Pulmonary; Mutation; Nitric Oxide; Piperazines; Protein Serine-Threonine Kinases; Pulmonary Artery; Purines; Receptors, Endothelin; Sildenafil Citrate; Sulfones; Transforming Growth Factor beta; Vasodilator Agents