The importance of evaluating anatomy below the diaphragm in staged single-ventricle patients presenting with cyanosis.

OBJECTIVE

To highlight veno-venous collaterals and portosystemic shunts as possible causes of cyanosis requiring evaluation or treatment below the diaphragm in patients with single-ventricle physiology during staged palliation.

BACKGROUND

Cyanosis can complicate staged single-ventricle palliation and is often evaluated with imaging isolated to the thorax. As a result, lesions below the diaphragm, such as veno-venous collaterals and portosystemic shunts, may be missed. Identification is important since these lesions can be treated via transcatheter techniques.

METHODS

This study is a retrospective, single-centre case series of six patients.

RESULTS AND DISCUSSION: Two patients following stage 2 palliation had multiple small veno-venous collaterals connecting to a reconstituted azygos with retrograde flow to below the diaphragm. Accessing veno-venous collaterals closer to their entry points below the diaphragm was more optimal for occlusion. Two patients following stage 3 palliation had veno-venous collaterals originating below the diaphragm and connecting to the pulmonary veins. Both patients required infrarenal inferior vena cava contrast injection to identify the veno-venous collateral. The final two patients had heterotaxy with either stage 2 or 3 palliation and were found to have bilateral pulmonary arteriovenous malformations secondary to a portosystemic shunt diverting hepatic factor away from the lungs. All patients had successful device closure of their veno-venous collaterals or portosystemic shunts.

CONCLUSION

The six patients discussed demonstrate the importance of evaluating below the diaphragm in single-ventricle physiology patients presenting with cyanosis.