Pulmonary hypertension: advances in pathogenesis and treatment

• Published in: British medical bulletin Vol. 94; no. 1; pp. 21 - 32
• Main Authors: Toshner, M., Tajsic, T., Morrell, N. W.
• Format: Journal Article
• Language: English
• Published: Oxford Oxford University Press 01.06.2010
• Subjects: Autoantibodies - metabolism; Biological and medical sciences; Bone Morphogenetic Protein Receptors, Type II - genetics; Endothelial Growth Factors - antagonists & inhibitors; General aspects; Humans; Hypertension, Pulmonary - etiology; Hypertension, Pulmonary - therapy; Inflammation - immunology; Medical sciences; Mutation; Pneumology; pulmonary hypertension; Pulmonary hypertension. Acute cor pulmonale. Pulmonary embolism. Pulmonary vascular diseases; Stem Cell Transplantation; vascular remodelling; Vasodilator Agents - therapeutic use; Medicine; Cardiovascular disease; Treatment; Respiratory disease; Pathogenesis; Pulmonary hypertension
• ISSN: 0007-1420; 1471-8391
• DOI: 10.1093/bmb/ldq012

Pulmonary hypertension is an orphan disease that until recently has received limited attention within the wider medical community. This has changed distinctly in the last 10 years with the advent of new classes of therapy and a renewed interest in mechanisms of pathogenesis. This review utilized information gathered from recent conferences, and a review of the literature was conducted using MedLine and Pubmed. Accepted mechanisms of pathogenesis and currently available treatments are presented. We will discuss interesting new concepts in pathogenesis, including the importance of genetic forms of the disease and in particular the transforming growth factor receptor superfamily and the evolving evidence of the contribution of dysregulated immunity. Areas of research may yield therapeutic benefits in the not-too-distant future, including anti-proliferative therapies and stem cell therapy.