Epidemiology of pediatric pulmonary hypertension

Abstract

Pulmonary hypertension (PH) is a rare condition. Data on adult PH patients is abundant, but data on pediatric PH are scarce. Nevertheless, in the past decade, increasing data concerning pediatric PH are becoming available, enabling this review of the epidemiology, characterization, and outcome of pediatric pulmonary hypertension.PH is a condition that is associated with a variety of diseases. It is a rare condition in children with a complex and age-specific presentation. Transient forms of pulmonary arterial hypertension (PAH), i.e., potentially reversible PAH, specifically occur in children and form the largest group of pediatric PH. Advanced PAH in children is predominantly idiopathic or associated with congenital heart disease. Other forms of PAH seldom occur in children. WHO PH groups 2-5 are rare in childhood; however, they may be underreported.The clinical presentation of pediatric PH is relatively nonspecific as it is in adults, with dyspnea on exertion as the most common presenting symptom. However, the clinical presentation of pediatric PH includes symptoms specific for children such as syncope. Pediatric PH frequently co-occurs with genetic and syndromal abnormalities, including Down syndrome. Right heart failure is less common in children despite a severe hemodynamic profile.PAH is a detrimental disease with a poor prognosis. Outcome of pediatric PAH differs between patients with different types of congenital heart disease. In the last decades, new PAH-targeted therapies have been developed, which have improved outcome in pediatric pulmonary arterial hypertension. However, currently reported survival rates vary and critical appraisal of patient populations, survival data, and proposed treatment approaches is necessary.