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Pulmonary hypertensive diseases.

A Bush1

  • 1Department of Paediatric Respirology Medicine, Imperial School of Medicine at National Heart and Lung Institute, Royal Brompton Hospital, London, UK.

Paediatric Respiratory Reviews
|November 3, 2005
PubMed
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Pulmonary hypertension (PH) in children often stems from lung or heart conditions. Identifying less common causes and primary pulmonary vascular diseases is crucial for diagnosis and treatment, though prognosis remains poor without lung transplantation.

Area of Science:

  • Pediatric Cardiology
  • Pulmonology
  • Medical Diagnostics

Background:

  • Pulmonary hypertension (PH) is a serious condition with diverse etiologies in children.
  • Common causes include end-stage pulmonary disease and congenital heart disease.
  • Less frequent causes involve sleep-disordered breathing, neuromuscular disease, and occult interstitial lung disease.

Purpose of the Study:

  • To outline the differential diagnosis of pediatric pulmonary hypertension.
  • To emphasize the importance of considering primary pulmonary vascular diseases when common causes are excluded.
  • To discuss diagnostic modalities and treatment challenges for pediatric PH.

Main Methods:

  • Review of common and less obvious causes of pulmonary hypertension.

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  • Description of primary pulmonary vascular diseases: primary pulmonary hypertension, pulmonary veno-occlusive disease, pulmonary embolic disease, and invasive pulmonary capillary haemangiomatosis.
  • Discussion of diagnostic tools including clinical signs, chest X-ray, echocardiography, right heart catheterization, and open lung biopsy.

    • Main Results:

    • Clinical signs and chest X-ray findings for PH are often non-specific.
    • Echocardiography aids in estimating pulmonary artery pressure and excluding congenital heart disease.
    • Right heart catheterization is essential for diagnosis confirmation, assessing reversibility, and excluding other causes.
    • Open lung biopsy may be required for definitive diagnosis.

    Conclusions:

    • Accurate diagnosis of pediatric pulmonary hypertension requires a systematic approach, excluding common causes before considering primary pulmonary vascular diseases.
    • Diagnostic workup involves a combination of imaging, hemodynamic assessment, and potentially biopsy.
    • Treatment remains challenging, with lung transplantation often being the only viable option for improving prognosis.