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Pulmonary veno-occlusive disease: Two children with gradual disease progression

Ronald W Day1, Parker W Clement2, Aimee O Hersh1

  • 1University of Utah Department of Pediatrics, 81 North Mario Capecchi Drive, Salt Lake City, UT 84113, USA.

Respiratory Medicine Case Reports
|January 11, 2017
PubMed

Insights

Rare pulmonary vascular diseases, pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis, may present with early pulmonary hypertension. Some children show improvement with targeted pulmonary hypertension therapy and vasodilator response may predict treatment success.

Area of Science:

  • Cardiology
  • Pulmonology
  • Pediatrics

Background:

  • Pulmonary veno-occlusive disease (PVOD) and pulmonary capillary hemangiomatosis (PCH) are rare, severe pulmonary vascular diseases.
  • These conditions often present insidiously, making early diagnosis challenging.

Observation:

  • Two pediatric cases demonstrated pulmonary hypertension for 3-5 years before radiographic signs of PVOD/PCH appeared.
  • Both patients showed a moderate decrease in pulmonary arterial pressure with acute vasodilator testing.

Findings:

  • Targeted pulmonary hypertension therapy led to improved six-minute walk performance without worsening pulmonary edema.
  • Slow progression over months to years is characteristic of PVOD and PCH in some patients.

Implications:

  • An acute vasodilator response may identify pediatric patients with PVOD/PCH who could benefit from medical therapy.
  • Early identification of pulmonary hypertension is crucial for managing these rare pediatric vascular lung diseases.

Keywords:
PCH, Pulmonary Capillary HemangiomatosisPVOD, pulmonary veno-occlusive diseasePulmonary arterial hypertensionPulmonary capillary hemangiomatosisPulmonary veno-occlusive disease

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