Heterotaxy syndrome with azygous continuation-causing pseudo Budd-Chiari syndrome after cardiopulmonary bypass

Area of Science:

• Cardiology
• Vascular Surgery
• Pediatric Surgery

Background:

• Heterotaxy syndrome is a congenital disorder affecting organ placement.
• Interruption of the inferior vena cava (IVC) is a known anomaly in some heterotaxy cases.
• Direct hepatic vein (HV) drainage into the right atrium (RA) bypasses the typical IVC pathway.

Observation:

• A patient with heterotaxy syndrome and IVC interruption presented with atypical venous drainage.
• Hepatic veins were observed to drain directly into the right atrium.
• The patient underwent cardiopulmonary bypass for cardiac surgery.

Findings:

• Post-cardiopulmonary bypass, the patient developed symptoms consistent with Budd-Chiari syndrome.
• This condition, termed pseudo Budd-Chiari syndrome, arose due to altered venous hemodynamics.
• The direct HV-to-RA shunt complicated the management of post-operative venous congestion.

Implications:

• This case highlights the critical implications of IVC interruption and direct HV-to-RA inflow in cardiac surgical planning.
• Understanding these anatomical variations is crucial for preventing post-operative complications like pseudo Budd-Chiari syndrome.
• Surgical strategies may need adaptation to manage altered venous return in affected patients.