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A cure for pulmonary arteriovenous fistulas?
1Royal Children's Hospital, Melbourne, Australia.
Insights
Pulmonary arteriovenous fistulas developed in a child with left atrial isomerism after surgery. These fistulas regressed following a surgical revision connecting hepatic veins to pulmonary arteries.
Area of Science:
- Cardiology
- Pediatric Surgery
- Medical Imaging
Background:
- Left atrial isomerism is a complex congenital heart defect.
- The Kawashima operation is a surgical procedure for specific congenital heart conditions.
- Pulmonary arteriovenous fistulas (PAVs) are abnormal connections between pulmonary arteries and veins.
Observation:
- A cyanotic child with left atrial isomerism developed diffuse bilateral pulmonary arteriovenous fistulas post-Kawashima operation.
- The hepatic veins were initially draining into the common atrium.
- The patient presented with cyanosis and respiratory distress.
Findings:
- Diffuse bilateral pulmonary arteriovenous fistulas were diagnosed.
- Surgical intervention rerouted hepatic venous drainage.
- The fistulas showed significant regression after the hepatic veins were connected to the confluent pulmonary arteries via a left lateral atrial tunnel.
Implications:
- This case highlights a potential complication of the Kawashima procedure.
- Successful management involved a tailored surgical approach to reroute venous drainage.
- Understanding venous anatomy and surgical modifications is crucial for managing complex congenital heart disease.
Abstract:
Diffuse bilateral pulmonary arteriovenous fistulas developed in a cyanotic child with left atrial isomerism, consisting of an interrupted inferior vena cava with continuation of the hemiazygos vein to the isolated left superior vena cava, soon after she underwent a Kawashima operation that left the hepatic veins draining into the common atrium. The fistulas regressed after the hepatic veins were connected to the confluent pulmonary arteries with a left lateral atrial tunnel.