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Paediatric Pulmonology and Allergology
2005 April, Vol. VIII, No. 1 (2858-2874)
PULMONARY VASCULAR DISEASE
Andrew Bush
Imperial School of Medicine at National Heart and Lung Institute and Royal Brompton Hospital, London, UK
Pulmonary hypertension has recently been the subject of reclassification by an international consensus conference. This paper reviews the new classification, and the diagnostic pathways which are followed. However, the biggest recent developments have been in therapy. Acute reversibility of pulmonary hypertension in the catheter laboratory is used to determine whether conventional treatment with high dose calcium channel blockers can safely be used in preference to novel, but expensive and potentially toxic therapies. There are a wide variety of novel treatments. Pulmonary vasoconstriction can be modulated by intravenous, inhaled, subcutaneous and oral prostanoids, phosphodiesterase V inhibitors and possibly by inhaled nitric oxide or arginine supplements. The endothelin receptor antagonists show promise not merely as vasodilators, but also in modulating pulmonary vascular remodeling. The era of combinations of powerful therapies for these patients is coming. For medical failures, lung transplantation remains an option. Also of importance are novel monitoring strategies. Inert gas methods are well established, but are becoming utilized more often. The use of exhaled breath condensate to measure mediators important in pulmonary vascular remodeling is still being explored. Care for children with pulmonary hypertension in special centres may allow a dramatically improved prognosis.