Pulmonary Hypertension in Congenital Heart Diseases

Abstract

Pulmonary hypertension (PH) is a severe disease affecting significantly the outcomes of patients with a varying incidence in different studies: from 5 to 52 cases per million population. PH affects many body organs, and is defined as the “progressive disease of the pulmonary vascular system, with a mean pulmonary artery pressure (mPAP) > 20 mmHg at rest measured by right heart catheterization.” Concurrent pulmonary arterial wedge pressure (PAWP) of ≤15 mmHg and pulmonary vascular resistance (PVR) of ≤3 Wood units/m2 are the main indicators of precapillary pulmonary hypertension; after the sixth World Symposium on Pulmonary Hypertension (WSPH) in 2018; the latter defined a new threshold for Pulmonary Hypertension with the rationale that 20 mmHg mPAP is two standard deviations above the normal mPAP (14.0 ± 3.3 mmHg). Currently, the values for mPAP ≥20 mmHg are the same in children above 3 months of age at sea level. In this chapter, the classification of PH, the clinical diagnosis and management, including the pharmacological agents used in the management of PH, alleviating and aggravating factors in PH, and the role of intravenous sedative and anesthetic agents are discussed in detail.