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The challenges in paediatric pulmonary arterial hypertension

Maurice Beghetti, Rolf M.F. Berger
European Respiratory Review 2014 23: 498-504; DOI: 10.1183/09059180.00007714
Maurice Beghetti
1Paediatric Cardiology Unit, Children’s University Hospital Geneva, Geneva, Switzerland. 2Centre for Congenital Heart Diseases, Dept of Pediatric Cardiology, Beatrix Children’s Hospital, University Medical Centre Groningen, University of Groningen, Groningen, The Netherlands
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  • For correspondence: maurice.beghetti@hcuge.ch
Rolf M.F. Berger
1Paediatric Cardiology Unit, Children’s University Hospital Geneva, Geneva, Switzerland. 2Centre for Congenital Heart Diseases, Dept of Pediatric Cardiology, Beatrix Children’s Hospital, University Medical Centre Groningen, University of Groningen, Groningen, The Netherlands
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    Figure 1.

    Multifactorial causes of paediatric pulmonary hypertensive vascular disease. Reproduced from [9] with permission from the publisher.

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    Figure 2.

    The recently proposed paediatric idiopathic/hereditable pulmonary arterial hypertension treatment algorithm. In Europe, the use of all agents is considered off-label in children, aside from sildenafil. CCB: calcium channel blocker; ERA: endothelin receptor antagonist; PDE-5i: phosphodiesterase type-5 inhibitor. Reproduced from [18] with permission from the publisher.

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  • Table 1. Determinants of risk in paediatric pulmonary arterial hypertension
    Determinants of riskLower riskHigher risk
    Clinical evidence of RV failureNoYes
    Progression of symptomsNoYes
    SyncopeNoYes
    GrowthFailure to thrive
    WHO functional classI, IIIII, IV
    BNP/NT-proBNPMinimally elevatedSignificantly elevated, increasing
    EchocardiographySevere RV enlargement/dysfunction
    Pericardial effusion
    HaemodynamicsSystemic CI >3.0 L·min−1·m−2Systemic CI <2.5 L·min−1·m−2
    mPAP/mSAP <0.75 mmHgmPAP/mSAP >0.75 mmHg
    Acute vasoreactivityRAP >10 mmHg
    PVRI >20 WU·m−2
    • RV: right ventricular; WHO: World Health Organization; BNP: B-type natriuretic peptide; NT-proBNP: N-terminal pro-BNP; CI: cardiac index; mPAP: mean pulmonary artery pressure; mSAP: mean systemic artery pressure; RAP: right atrial pressure; PVRI: pulmonary vascular resistance index; WU: Wood unit. Reproduced from [18] with permission from the publisher.

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    • M. Beghetti
    • R.M.F. Berger
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The challenges in paediatric pulmonary arterial hypertension
Maurice Beghetti, Rolf M.F. Berger
European Respiratory Review Dec 2014, 23 (134) 498-504; DOI: 10.1183/09059180.00007714

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The challenges in paediatric pulmonary arterial hypertension
Maurice Beghetti, Rolf M.F. Berger
European Respiratory Review Dec 2014, 23 (134) 498-504; DOI: 10.1183/09059180.00007714
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  • Article
    • Abstract
    • Introduction
    • Paediatric PAH
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  • Paediatric pulmonology
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