Clinical Applications of Inhaled Nitric Oxide in Children with Pulmonary Hypertension

Publisher Summary

Nitric oxide has recently achieved prominence as a ubiquitous endogenous substance with a myriad of biological functions. Its discovery has aided understanding of mechanisms of established therapies and suggested new treatments for a variety of diseases. Most notably as a vasodilator derived from the endothelium, this molecule has been applied to the treatment of pulmonary hypertensive disorders. This chapter describes the use of inhaled nitric oxide to selectively manipulate pulmonary vascular tone in the evaluation of the candidate for reparative surgery or transplantation and the postoperative cardiac patient and in the treatment of transient graft dysfunction after lung transplantation. In addition, the chapter includes experience with inhaled nitric oxide in the patient with congenital mitral stenosis undergoing surgery or balloon valvuloplasty and in the patient with palliated single-ventricle physiology. The use of inhaled nitric oxide in children with congenital heart disease and pulmonary hypertension indicates that nitric oxide is a selective pulmonary vasodilator that may improve patient management, particularly after surgical procedures requiring cardiopulmonary bypass. It is observed that there are several patients for whom all resuscitative maneuvers for the treatment of pulmonary hypertensive crises have been unsuccessful until inhaled nitric oxide is added to the therapeutic regimen. In addition, studies using inhaled nitric oxide as an investigational probe point toward endothelial injury as a contributor to post-cardiopulmonary bypass pulmonary vasoconstriction. Inhaled nitric oxide relieves pulmonary vasoconstriction associated both with left atrial or pulmonary venous hypertension and following the relief of mitral valve or pulmonary venous obstruction.