Surgical intervention for congenital heart disease (CHD) can be complicated by pulmonary hypertension (PH), which increases morbidity, mortality, and medical burden. Consequently, postoperative management of PH is an important clinical consideration to improve outcomes. Inhaled nitric oxide (iNO) is a widely accepted standard of care for PH and has been studied in the context of cardiac surgery for CHD. However, large randomized, double-blind, placebo-controlled, multicenter clinical trials in pediatric patients are limited. This review will provide an overview of the clinical studies in this setting and will discuss general treatment considerations to facilitate a better understanding of the clinical use of iNO for PH after pediatric cardiac surgery.

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 (PGI2), endothelin, and nitric oxide (NO). Current treatments target these three pathways utilizing PGI2 and its analogs, endothelin receptor antagonists, and phosphodiesterase type-5 (PDE-5) inhibitors. Inhaled nitric oxide (iNO) is approved for the treatment of hypoxic respiratory failure associated with pulmonary hypertension in term/near-term neonates. As a selective pulmonary vasodilator, iNO can acutely decrease pulmonary artery pressure and pulmonary vascular resistance without affecting cardiac index or systemic vascular resistance. In addition to delivery via the endotracheal tube, iNO can also be administered as continuous inhalation via a facemask or a pulsed nasal delivery. 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 NO2. 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.

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