Multiple intrapulmonary arteriovenous fistulas in childhood
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Published on: April 1, 2022
1Department of Paediatric Cardiology, Royal Belfast Hospital for Sick Children, United Kingdom.
Insights
Pulmonary arteriovenous fistulas (AVFs) can cause childhood cyanosis. Nifedipine effectively treated two young boys with AVFs, demonstrating its therapeutic potential for this rare condition.
Area of Science:
- Cardiology
- Pediatrics
- Pulmonology
Background:
- Pulmonary arteriovenous fistulas (AVFs) are uncommon vascular malformations.
- They can be congenital or acquired, often presenting with cyanosis in children.
- AVFs are associated with multisystem disorders and conditions like hepatopulmonary syndrome.
Observation:
- Two pediatric cases of boys in their first decade of life presenting with cyanosis due to AVFs.
- Bubble echocardiography strongly indicated the presence of AVFs in both patients.
- Cardiac catheterization revealed normal pulmonary artery pressure and diffuse pulmonary telangiectasis.
Findings:
- Both patients diagnosed with pulmonary arteriovenous fistulas.
- Nifedipine therapy proved effective in managing the condition for both individuals.
- The study highlights nifedipine as a viable treatment option for pediatric AVFs.
Implications:
- Early diagnosis of AVFs using echocardiography is crucial.
- Nifedipine offers an effective treatment strategy for pediatric pulmonary arteriovenous fistulas.
- Understanding AVF pathophysiology aids in managing cyanotic heart conditions in children.
Abstract:
Pulmonary arteriovenous fistulas (AVFs) are a rare but recognized cause of cyanosis in childhood. Lesions may be acquired as in hepatopulmonary syndrome or they may be congenital, particularly in association with certain multisystem disorders. Large fistulas are more common than multiple small connections. Two cases. both boys, presenting in the first decade of life are described. "Bubble" echocardiography was the most telling investigation and strongly suggested the presence of AVFs in both cases. Each patient then underwent cardiac catheterization, which demonstrated normal pulmonary artery pressure and diffuse pulmonary telangiectasis. Both patients were treated effectively with nifedipine and continue with this mode of therapy.
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