Long-term installation of a central venous catheter for dialysis in a patient with heterotaxia syndrome : A case with a persistent left superior vena cava and absent right superior and inferior vena cava

Abstract

Hemodialysis was introduced in a 72-year-old female patient with diabetic end-stage kidney disease. Due to internal shunt occlusion, synthetic grafts were implanted in the bilateral brachia; however, she showed repeated steal phenomenon, so insertion of the indwelling central venous catheter for dialysis was attempted via the right internal jugular vein. At this time, a persistent left superior vena cava (PLSV) was suspected because of the unusual passage of the catheter through the blood vessels. The end of the inserted angiographic catheter was found to reach the right atrium via the PLSV. Polysplenia in the category of visceral heterotaxia syndrome was diagnosed with a CT scan showing an absent right superior and inferior vena cava with hemiazygos continuation and the existence of 3 spleens. Subsequently, hemodialysis has been successfully conducted on an outpatient basis for 3 years with a one-time exchange of the catheter due to a catheter embolus. Visceral heterotaxia syndrome should be considered when the unusual passage of a guidewire is observed. A finding of multiple spleens or spleen absence is helpful for this diagnosis.