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Inhaled nitric oxide in adults with pulmonary hypertension

Darren B Taichman, MD, PhD
Section Editor
Jess Mandel, MD
Deputy Editor
Geraldine Finlay, MD


The inhalation of nitric oxide (NO) has been proposed as long-term therapy for pulmonary arterial hypertension (PAH), and possibly other types of pulmonary hypertension (PH), because NO-mediated signaling is decreased in patients with PAH. Such a role, however, has not been established due in part to practical limitations associated with its administration, an area of continued study [1]. Currently, the only widely accepted role for inhaled NO is as a vasodilator during a vasoreactivity trial. Its use has also been reported in acutely ill patients with severe PH.

In this topic review, the role of NO in the pathogenesis of PAH is described. Diagnostic and therapeutic uses of inhaled NO in adults with PH are then discussed.

The definition, classification, epidemiology, etiology, pathogenesis, clinical manifestations, diagnosis, treatment, and prognosis of PH are discussed elsewhere. The use of inhaled NO in the management of patients with acute respiratory distress syndrome (ARDS) and in the management of infants with persistent PH are also discussed separately. (See "Overview of pulmonary hypertension in adults" and "Acute respiratory distress syndrome: Investigational or ineffective pharmacotherapy in adults" and "Persistent pulmonary hypertension of the newborn".)


The World Health Organization (WHO) classifies patients with pulmonary hypertension (PH) into five groups based upon etiology. Patients in the first group are considered to have pulmonary arterial hypertension (PAH), whereas patients in the remaining four groups are considered to have PH (table 1). When all five groups are discussed collectively, PH is generally used. We adhere to this nomenclature in this topic review. The WHO classification system is described in detail elsewhere. (See "Overview of pulmonary hypertension in adults", section on 'Classification'.)


Endogenous nitric oxide (NO) has a normal role in healthy patients, which is interrupted in patients with PAH.


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