Recent advances in pulmonary vascular disease.
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.
Adatia
Ian
I
Department of Critical Care Medicine, The Hospital for Sick Children, Toronto, Ontario, Canada. ian.adatia@sickkids.ca
D000959:Antihypertensive Agents / Q000627:therapeutic use*; D052006:Bone Morphogenetic Protein Receptors, Type II; D001993:Bronchodilator Agents / Q000627:therapeutic use*; D002648:Child; D002889:Chromosomes, Human, Pair 2; D004541:Eisenmenger Complex / Q000235:genetics; D011464:Epoprostenol / Q000627:therapeutic use; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000235:genetics; D009154:Mutation; D009569:Nitric Oxide / Q000627:therapeutic use; D010879:Piperazines / Q000627:therapeutic use; D017346:Protein Serine-Threonine Kinases / Q000235:genetics; D011651:Pulmonary Artery / Q000503:physiopathology; D011687:Purines; D017466:Receptors, Endothelin / Q000627:therapeutic use; D000068677:Sildenafil Citrate; D013450:Sulfones; D016212:Transforming Growth Factor beta / Q000378:metabolism; D014665:Vasodilator Agents / Q000627:therapeutic use*
D000959:Antihypertensive Agents; D001993:Bronchodilator Agents; D010879:Piperazines; D011687:Purines; D017466:Receptors, Endothelin; D013450:Sulfones; D016212:Transforming Growth Factor beta; D014665:Vasodilator Agents; D009569:Nitric Oxide; D000068677:Sildenafil Citrate; D011464:Epoprostenol; D017346:Protein Serine-Threonine Kinases; C498218:BMPR2 protein, human; D052006:Bone Morphogenetic Protein Receptors, Type II