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In this chapter, we describe the development of inhaled NO as a therapeutic strategy to increase pulmonary vascular NO and cGMP concentrations. Inhaled NO induces pulmonary vasodilation without the side effects of systemic hypotension or hypoxemia. An overview of how inhaled NO elicits its effects is provided, and alternate approaches to increasing pulmonary NO and cGMP concentrations are considered. Several of the clinical applications of inhaled NO are presented, as is a discussion of the methods required for the safe administration of NO gas. Finally, we present experimental and clinical observations highlighting the potential applications of inhaled NO to the treatment of systemic vascular diseases.

Heart transplantation carries significant risks for the recipient in the peri- and postoperative period. These include primary graft dysfunction (PGD), right ventricular (RV) failure, rejection, and infectious complications. Allograft ischemia-reperfusion and recipient pulmonary hypertension are common causes of PGD and RV failure. The use of inotropes, pulmonary vasodilators, and mechanical circulatory support (MCS) for the management of these issues is common. Early acute rejection is typically characterized by allograft dysfunction and is most commonly diagnosed by endomyocardial biopsy. Depending on the severity of presentation, management of acute rejection ranges from short-term corticosteroid therapy to MCS. Infectious complications are also a significant source of early morbidity and mortality in heart transplant recipients.

The presence of pulmonary arterial hypertension (PAH) increases morbidity and mortality in patients with congenital heart disease (CHD). PAH associated with CHD is a heterogeneous condition, depending on the type of the underlying defect and previous repair strategies. There is growing evidence of the benefits of pulmonary vasodilators in this population, but, despite recent advances, mortality remains relatively high. Currently, there are limited data to guide the use of pulmonary vasodilators in this population. Furthermore, particular subgroups such as patients with Fontan circulation or “segmental” pulmonary hypertension are challenging to manage and even more challenging to treat. The current chapter reviews the different therapies for PAH in this growing and challenging population.