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

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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“…Pulmonary hypertension (PHTN) diagnosis was made according to the current literature in pediatric population …”

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“…Pulmonary hypertension (PH) is a rare condition in infants and children that results in substantial morbidity and mortality . According to international guidelines, the definition of PH in children (aged ≥ 3 months), as in adults, is a mean pulmonary artery pressure (mPAP) ≥ 25 mm Hg.…”

“…In the current international classification, five broad subgroups of PH are defined: pulmonary arterial hypertension (PAH) (Group 1), PH due to left heart disease (Group 2), PH due to lung diseases and/or hypoxia (Group 3), chronic thromboembolic pulmonary hypertension (Group 4), and PH with unclear and/or multifactorial mechanisms (Group 5) . PAH is specifically defined as PH with pulmonary arterial wedge pressure (PAWP) ≤15 mm Hg and pulmonary vascular resistance (PVR) ≥3 Wood units × m 2 (≈240 dyn s cm –5 ), in the absence of lung disease and Group 4 or 5 PH . The 6th World Symposium on Pulmonary Hypertension (WSPH) (2018) proposed an mPAP cutoff of >20 mmHg and suggested a new definition for Group 4 to include chronic thromboembolic pulmonary hypertension (CTEPH) and other pulmonary artery obstructions …”

“…PAH is specifically defined as PH with pulmonary arterial wedge pressure (PAWP) ≤15 mm Hg and pulmonary vascular resistance (PVR) ≥3 Wood units × m 2 (≈240 dyn s cm –5 ), in the absence of lung disease and Group 4 or 5 PH . The 6th World Symposium on Pulmonary Hypertension (WSPH) (2018) proposed an mPAP cutoff of >20 mmHg and suggested a new definition for Group 4 to include chronic thromboembolic pulmonary hypertension (CTEPH) and other pulmonary artery obstructions …”

“…The two most common forms of PH in children are PAH (Group 1), which accounts for the majority of cases, and PH associated with lung disease (Group 3), of which the most common form is bronchopulmonary dysplasia …”