Pulmonary circulation
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Pulmonary circulation · Apr 2012
Clinical perspectives with long-term pulsed inhaled nitric oxide for the treatment of pulmonary arterial hypertension.
Pulmonary arterial hypertension (PAH) is a chronic, progressive disease of the pulmonary vasculature with a high morbidity and mortality. Its pathobiology involves at least three interacting pathways - prostacyclin (PGI(2)), endothelin, and nitric oxide (NO). Current treatments target these three pathways utilizing PGI(2) and its analogs, endothelin receptor antagonists, and phosphodiesterase type-5 (PDE-5) inhibitors. ⋯ Consistent with a deficiency in endogenously produced NO, long-term pulsed iNO dosing appears to favorably affect hemodynamics in PAH patients, observations that appear to correlate with benefit in uncontrolled settings. Clinical studies and case reports involving patients receiving long-term continuous pulsed iNO have shown minimal risk in terms of adverse events, changes in methemoglobin levels, and detectable exhaled or ambient NO or NO(2). Advances in gas delivery technology and strategies to optimize iNO dosing may enable broad-scale application to long-term treatment of chronic diseases such as PAH.