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Review
. 2019 Jan 24;53(1):1801916.
doi: 10.1183/13993003.01916-2018. Print 2019 Jan.

Paediatric pulmonary arterial hypertension: updates on definition, classification, diagnostics and management

Affiliations
Review

Paediatric pulmonary arterial hypertension: updates on definition, classification, diagnostics and management

Erika B Rosenzweig et al. Eur Respir J. .

Abstract

Paediatric pulmonary arterial hypertension (PAH) shares common features of adult disease, but is associated with several additional disorders and challenges that require unique approaches. This article discusses recent advances, ongoing challenges and distinct approaches for the care of children with PAH, as presented by the Paediatric Task Force of the 6th World Symposium on Pulmonary Hypertension. We provide updates of the current definition, epidemiology, classification, diagnostics and treatment of paediatric PAH, and identify critical knowledge gaps. Several features of paediatric PAH including the prominence of neonatal PAH, especially in pre-term infants with developmental lung diseases, and novel genetic causes of paediatric PAH are highlighted. The use of cardiac catheterisation as a diagnostic modality and haemodynamic definitions of PAH, including acute vasoreactivity, are addressed. Updates are provided on issues related to utility of the previous classification system to reflect paediatric-specific aetiologies and approaches to medical and interventional management of PAH, including the Potts shunt. Although a lack of clinical trial data for the use of PAH-targeted therapy persists, emerging data are improving the identification of appropriate targets for goal-oriented therapy in children. Such data will likely improve future clinical trial design to enhance outcomes in paediatric PAH.

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Conflict of interest statement

Conflict of interest: E.B. Rosenzweig reports institutional grants from Actelion, GSK, Gilead, Bayer and United Therapeutics/Lung Biotech, outside the submitted work. Conflict of interest: S.H. Abman reports grants from Shire and United Therapeutics, outside the submitted work. Conflict of interest: I. Adatia reports personal fees (for data and safety monitoring board work for selexipag trial in children, and for clinical oversight committee work for macitentan trial in children) from Actelion, personal fees (for data and safety monitoring board work for citruline trial in children) from Asklepion, and personal fees (for clinical oversight committee work for tadalafil trial in children) from Eli Lilly, outside the submitted work. Conflict of interest: M. Beghetti reports grants and personal fees from Actelion, grants, personal fees and non-financial support from Bayer Healthcare, and personal fees from GSK, Eli Lilly, MSD and Pfizer, during the conduct of the study. Conflict of interest: D. Bonnet reports personal fees from Actelion Pharmaceuticals Novartis, Bayer Healthcare and Eli Lilly, outside the submitted work. Conflict of interest: S. Haworth has nothing to disclose. Conflict of interest: D.D. Ivy: the University of Colorado School of medicine contracts with Actelion, Bayer, Lilly and United Therapeutics for D.D. Ivy to be a consultant and perform research studies. Conflict of interest: R.M.F. Berger: University Medical Center Groningen contracts with Actelion and Lilly for consultancy and advisory board activities of R.M.F. Berger.

Figures

FIGURE 1
FIGURE 1
Diagnostic algorithm for pulmonary hypertension (PH) in children. CHD: congenital heart disease; PFT: pulmonary function test; DLCO: diffusing capacity of the lung for carbon monoxide; CT: computed tomography; RV: right ventricular; V/Q: ventilation/perfusion; CTEPH: chronic thromboembolic PH; CTA: CT angiography; PA: pulmonary artery; PEA: pulmonary endarterectomy; AVT: acute vasodilator testing; mPAP: mean pulmonary arterial pressure; PAWP: pulmonary arterial wedge pressure; PVRI: pulmonary vascular resistance index; WU: Wood Units; 6MWT: 6-min walk test; CPET: cardiopulmonary exercise test; MRI: magnetic resonance imaging; CTD: connective tissue disease; PVOD: pulmonary veno-occlusive disease; PCH: pulmonary capillary haemangiomatosis; IPAH/FPAH: idiopathic/familial pulmonary arterial hypertension.
FIGURE 2
FIGURE 2
Paediatric idiopathic/familial pulmonary arterial hypertension treatment algorithm. CCB: calcium channel blocker; ERA: endothelin receptor agonist; PDE5i: phosphodiesterase type 5 inhibitor. #: deterioration or not meeting treatment goals.

Comment in

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