State-of-the-Art Paper
Pediatric Pulmonary Hypertension

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Pulmonary hypertension (PH) is a rare disease in newborns, infants, and children that is associated with significant morbidity and mortality. In the majority of pediatric patients, PH is idiopathic or associated with congenital heart disease and rarely is associated with other conditions such as connective tissue or thromboembolic disease. Incidence data from the Netherlands has revealed an annual incidence and point prevalence of 0.7 and 4.4 for idiopathic pulmonary arterial hypertension and 2.2 and 15.6 for pulmonary arterial hypertension, respectively, associated with congenital heart disease (CHD) cases per million children. The updated Nice classification for PH has been enhanced to include a greater depth of CHD and emphasizes persistent PH of the newborn and developmental lung diseases, such as bronchopulmonary dysplasia and congenital diaphragmatic hernia. The management of pediatric PH remains challenging because treatment decisions continue to depend largely on results from evidence-based adult studies and the clinical experience of pediatric experts.

Key Words

congenital heart disease
pediatrics
pulmonary hypertension

Abbreviations and Acronyms

APAH-CHD
pulmonary arterial hypertension associated with congenital heart disease
AVT
acute vasodilator testing
CHD
congenital heart disease
HPAH
hereditary pulmonary arterial hypertension
IPAH
idiopathic pulmonary arterial hypertension
PAPm
mean pulmonary artery pressure
PH
pulmonary hypertension
PHVD
pulmonary hypertensive vascular disease
PPHN
persistent pulmonary hypertension of the newborn
PVR
pulmonary vascular resistance
SVR
systemic vascular resistance

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The University of Colorado School of Medicine has received consulting fees for Dr. Ivy from Actelion, Bayer, Gilead, Eli Lilly, Pfizer, and United Therapeutics. The University Medical Center Groningen has received consulting fees for Dr. Berger from Actelion, Bayer, GlaxoSmithKline, Lilly, Novartis, and Pfizer. Dr. Berger has performed consultancies for Actelion, Bayer, GlaxoSmithKline, Lilly, Novartis, Pfizer, and United Therapeutics. Dr. Bonnet has received lecture and consulting honoraria from Actelion, Eli Lilly, Pfizer, and Bayer. Dr. Fleming has served as a consultant to Actelion and Pfizer. Dr. Haworth has received consulting fees from GlaxoSmithKline. Dr. Rosenzweig has received research grant support from Actelion, Gilead, GlaxoSmithKline, Eli Lilly, Bayer, and United Therapeutics; and consulting honoraria from United Therapeutics and Actelion. The University of California has received consulting fees for Dr. Steinhorn from Ikaria and United Therapeutics, and she has served as an unpaid consultant to Actelion. Dr. Beghetti has served as an advisory board member for Actelion, Bayer, Eli Lilly, GlaxoSmithKline, Novartis, and Pfizer; has received grants from Actelion and Bayer; has receiving lecture fees from Actelion, Bayer, and Pfizer; has developed educational materials for Actelion and Pfizer; and has receiving consulting fees from Actelion, Bayer, GlaxoSmithKline, Pfizer, and Novartis. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.