Cardiac Transplantation in a Patient With Situs Inversus Totalis and Extracardiac Fontan.

OBJECTIVE

This case report describes a successful dextrocardia-preserving orthotopic heart transplant in a patient with situs inversus totalis and an extracardiac Fontan, utilizing a levocardia donor heart.

KEY STEPS

The donor heart was rotated 90° counterclockwise along its longitudinal axis to optimize inferior venous connection and incorporate existing extracardiac Fontan. Superior venous connections were secured using a harvesting technique preserving the innominate vein. A GORE-TEX graft was wrapped around the innominate vein to prevent its compression as it passed through the aortic arch.

POTENTIAL PITFALLS

Counterclockwise rotation requires adequate pulmonary artery length. In the absence of extra aortic tissue to allow mild aortic bowing and prevent superior venous obstruction, a GORE-TEX graft offers an effective alternative to maintain unobstructed superior venous drainage.

TAKE-HOME MESSAGES: Dextrocardia-preserving transplantation is feasible with a 90° counterclockwise rotation. Reusing the extracardiac Fontan reduces ischemic time; reinforcing the innominate vein with GORE-TEX prevents compression, securing venous integrity.