Related Experiment Videos
Multiple pulmonary arteriovenous fistulas in childhood
W B Knight1, A Bush, C M Busst
1Department of Paediatric Cardiology, Brompton Hospital, London, U.K.
Insights
Pediatric pulmonary arteriovenous fistulas can be challenging to treat. While some interventions show promise, extensive disease may lead to poor outcomes in children.
Area of Science:
- Cardiology
- Pulmonology
- Pediatric Surgery
Background:
- Pulmonary arteriovenous fistulas (PAVs) are abnormal connections between pulmonary arteries and veins.
- Multiple PAVs in children are rare and can lead to significant intrapulmonary shunting and hypoxemia.
Observation:
- Three pediatric cases of multiple PAVs are presented, with ages at presentation ranging from five months to nine years.
- Diagnostic methods included mass spectrometry for pulmonary blood flow and shunt fraction measurement, and cardiac catheterization.
- Clinical presentations varied, with some cases exhibiting significant intrapulmonary shunting.
Findings:
- Treatment modalities included surgical ligation, balloon embolization, and steel coil embolization.
- The oldest child treated with embolization showed positive outcomes, with resolution of cyanosis.
- The two younger children with more extensive disease experienced disease progression and mortality despite interventions.
Implications:
- Early diagnosis and intervention are crucial for managing pediatric PAVs.
- Treatment strategies should be tailored to the extent and type of PAVs.
- Extensive PAV disease in children carries a significant risk of mortality, highlighting the need for further research into effective treatments.
Abstract:
Three cases of multiple pulmonary arteriovenous fistulas are described in children who presented at five months, two and nine years of age. Mass spectrometry was used to measure pulmonary blood flow and, in two cases, the intrapulmonary right-to-left shunt. The shunt fractions were 51% and 35%, with no significant change on breathing 100% oxygen. In one case, effective pulmonary blood flow was measured during cardiac catheterisation by the argon-freon rebreathing method and agreed closely with that found from the Fick, principle with measured oxygen consumption. Treatment consisted of surgical ligation of a lower lobe pulmonary artery in the youngest child, balloon embolisation in the second, and initial surgical oversewing of a single large fistula followed twenty months later by steel coil embolisation in the third. The last and oldest child is well and no longer cyanosed. The first two children died seven months after treatment with evidence of progression of their pulmonary arteriovenous fistulas. The first of these, who also had an atrial septal defect and discordant thoraco-abdominal arrangement, died of heart failure. Autopsies on both children confirmed extensive involvement of both lungs by arteriovenous fistulas. In one case who had a diffuse, telangiectatic form of pulmonary arteriovenous fistulas, microscopic serial reconstructions of lung tissue revealed that anastomoses occurred between arteries accompanying terminal bronchioles and intra-acinar arteries and adjacent veins. Occlusion of the pulmonary arteries supplying the fistulas led to extensive fibrosis within them, and was associated with enlargement of the corresponding bronchial arterial circulation.