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Intrahepatic right-to-left shunting after the Fontan operation
Alessandro Giamberti1, Robert H Anderson, Marc R de Leval
1Cardiothoracic Unit, Great Ormond Street Hospital for Children NHS Trust, Great Ormond Street, London WC1N 3JH, UK. DelevM@gosh.nhs.uk
Insights
Two patients with right isomerism experienced severe cyanosis after a fenestrated Fontan operation. A massive shunt from the inferior vena cava through the liver to the intra-atrial baffle was identified and successfully treated by ligating the anomalous hepatic vein.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Surgical Innovation
Background:
- Right isomerism presents complex congenital heart defects.
- The Fontan operation is a palliative procedure for single-ventricle physiology.
- Fenestrated Fontan procedures aim to improve outcomes but can have complications.
Observation:
- Two patients with corrected right isomerism developed severe progressive cyanosis post-fenestrated Fontan operation.
- Cardiac catheterization revealed a significant right-to-left shunt.
- The shunt originated from the inferior vena cava, traversed the liver, and connected to the left side of the intra-atrial baffle via an anomalous hepatic vein.
Findings:
- A massive intrapulmonary shunt was identified as the cause of cyanosis.
- The shunt pathway involved an anomalous hepatic venous connection to the systemic venous pathway.
- Successful ligation of the anomalous hepatic vein resolved the cyanosis in both patients.
Implications:
- Highlights a rare but serious complication of fenestrated Fontan operations.
- Underscores the importance of detailed anatomical and hemodynamic assessment in complex congenital heart disease.
- Demonstrates the efficacy of targeted surgical intervention for specific shunt anomalies in Fontan patients.
Abstract:
We describe two patients with right isomerism, corrected with a fenestrated Fontan operation, who suffered severe progressive cyanosis. Cardiac catheterisation in both revealed a massive right-to-left shunt from the inferior caval vein, through the liver, to a hepatic vein draining directly to the left side of the intra-atrial baffle. The anomalous vein was successfully ligated in both patients.