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Updated: Aug 16, 2026

Increasing Pulmonary Artery Pulsatile Flow Improves Hypoxic Pulmonary Hypertension in Piglets
Published on: May 11, 2015
[A child with pulmonary hypertension: from etiological study to therapeutic decisions]
Micaela Guardiano1, Ana Maria Maia, Cíntia Correia
1Interna Complementar de Pediatria - Serviço de Pediatria, Hospital de S. João. mguardiano@hotmail.com
Insights
This case study highlights a rare pediatric pulmonary hypertension diagnosis in a 4-year-old. Obliterans bronchiolitis was identified as the underlying cause, leading to specific treatment interventions.
Area of Science:
- Pediatric Cardiology
- Pulmonology
- Rare Diseases
Background:
- Pulmonary hypertension (PH) is a severe condition with high mortality, often linked to other diseases.
- Pediatric PH is particularly rare, presenting diagnostic challenges.
- This report details a unique case of PH in a young child.
Observation:
- A 4-year-old child presented with syncope, leading to a PH diagnosis via echocardiogram.
- Further investigations revealed obliterans bronchiolitis as the underlying pulmonary condition.
- The precipitating event for the pulmonary disease remains undetermined.
Findings:
- The study documents the first instance of obliterans bronchiolitis diagnosed during the etiological investigation of pediatric pulmonary hypertension in their department.
- Successful management involved a combination of diltiazem, bosentan, and oxygen therapy.
- This case underscores the importance of comprehensive etiological workup for pediatric PH.
Implications:
- This case expands the understanding of rare causes of pediatric pulmonary hypertension.
- It emphasizes the need for early diagnosis and tailored treatment strategies for children with PH.
- Further research into the link between obliterans bronchiolitis and PH is warranted.
Abstract:
Pulmonary hypertension is a rare but devastating disease, with poor prognosis and high mortality. The disease may present as Primary Pulmonary Hypertension where no clear aetiology can be identified, or be related to a variety of underlying conditions. This is a rare disease in paediatric age. The authors present the clinical case of a 4 years old child, admitted in Paediatric Department of S. João Hospital for syncope episode. The diagnosis of pulmonary hypertension was made by echocardiogram. Ulterior studies revealed pulmonary disease (obliterans bronchiolitis). The treatment included a calcium channel blocker (diltiazem), an endothelin antagonist (bosentan) and supplemental oxygen. This is the first case of obliterans bronchiolitis diagnosed during etiological study for pulmonary hypertension, in our Pediatrics Department. In this case we could not determine the pulmonary disease precipitating event.
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