Objective: To investigate the regional flow distribution in patients with Fontan circulation by using magnetic resonance imaging (MRI). Materials and Methods: We identified 39 children (18 females and 21 males; mean age, 9.3 years; age range, 3.3–17.0 years) with Fontan circulation in whom flow volumes across the thoracic and abdominal arteries and veins were measured by using MRI. The patients were divided into three groups: fenestrated Fontan circulation group with MRI performed under general anesthesia (GA) (Group 1, 15 patients; average age, 5.9 years), completed Fontan circulation group with MRI performed under GA (Group 2, 6 patients; average age, 8.7 years), and completed Fontan circulation group with MRI performed without GA (Group 3, 18 patients; average age, 12.5 years). The patient data were compared with the reference ranges in healthy controls. Results: In comparison with the controls, Group 1 showed normal cardiac output (3.92 ± 0.40 vs. 3.72 ± 0.69 L/min/m2, p = 0.30), while Group 3 showed decreased cardiac output (3.24 ± 0.71 vs. 3.96 ± 0.64 L/min/m2, p = 0.003). Groups 1 and 3 showed reduced abdominal flow (1.21 ± 0.28 vs. 2.37 ± 0.45 L/min/m2, p < 0.001 and 1.89 ± 0.39 vs. 2.64 ± 0.38 L/min/m2, p < 0.001, respectively), which was mainly due to the diversion of the cardiac output to the aortopulmonary collaterals in Group 1 and the reduced cardiac output in Group 3. Superior mesenteric and portal venous flows were more severely reduced in Group 3 than in Group 1 (ratios between the flow volumes of the patients and healthy controls was 0.26 and 0.37 in Group 3 and 0.63 and 0.53 in Group 1, respectively). Hepatic arterial flow was decreased in Group 1 (0.11 ± 0.22 vs. 0.34 ± 0.38 L/min/m2, p = 0.04) and markedly increased in Group 3 (0.38 ± 0.22 vs.-0.08 ± 0.29 L/min/m2, p < 0.0001). Group 2 showed a mixture of the patterns seen in Groups 1 and 3. Conclusion: Fontan circulation is associated with reduced abdominal flow, which can be attributed to reduced cardiac output and portal venous return in completed Fontan circulation, and diversion of the cardiac output to the aortopulmonary collaterals in fenestrated Fontan circulation.
- Abdominal flow
- Fenestrated Fontan
- Fontan associated liver disease
- Fontan operation
- Protein-losing enteropathy
ASJC Scopus subject areas
- Radiology Nuclear Medicine and imaging