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Epidemiology of Pediatric Pulmonary Hypertension

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Pediatric and Congenital Cardiology, Cardiac Surgery and Intensive Care

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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 decades, increasing data concerning pediatric PH have become 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.

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Authors and Affiliations

  1. Center for Congenital Heart Diseases, Department of Pediatric Cardiology, Beatrix Children’s Hospital, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands

    Johannes M. Douwes & Rolf M. F. Berger

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  1. Johannes M. Douwes
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  2. Rolf M. F. Berger
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Corresponding author

Correspondence to Johannes M. Douwes .

Editor information

Editors and Affiliations

  1. Children’s Hospital Colorado University of Colorado Denver, School of, Aurora, CO, USA

    Eduardo M. da Cruz

  2. Children's Hospital Colorado University of Colorado School of Medicin, Aurora, CO, USA

    Dunbar Ivy

  3. Department of Cardiac Surgery, German Pediatric Heart Center, Sankt Augustin, Germany

    Viktor Hraska

  4. Pediatric Cardiac Surgery, Children's Hospital Colorado University of Colorado, Aurora, CO, USA

    James Jaggers

Section Editor information

  1. Center for Congenital Heart Diseases, Department of Pediatric Cardiology, Beatrix Children’s Hospital, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands

    Rolf M. F. Berger

  2. Dept. Pediatric Cardiology, Children's Hospital Colorado, Univ. of Colorado, Denver, Aurora, Colorado, USA

    Dunbar Ivy

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Douwes, J.M., Berger, R.M.F. (2020). Epidemiology of Pediatric Pulmonary Hypertension. In: da Cruz, E., Ivy, D., Hraska, V., Jaggers, J. (eds) Pediatric and Congenital Cardiology, Cardiac Surgery and Intensive Care. Springer, London. https://doi.org/10.1007/978-1-4471-4999-6_204-2

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  • DOI: https://doi.org/10.1007/978-1-4471-4999-6_204-2

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