Isolated Persistent Left Superior Vena Cava Associated With Autosomal Dominant Polycystic Kidney Disease (ADPKD): Challenges and Clinical Significance

Abstract

Persistent left superior vena cava (PLSVC) is the most common venous anomaly of the thorax that usually coexists with the right superior vena cava. However, in a minority of cases, there is only a PLSVC without a right superior vena cava which is called an isolated PLSVC. There are some reported anomalies that can cooccur with PLSVC; yet, none have reported an association with autosomal dominant polycystic kidney disease (ADPKD). In this case report, we describe a 41-year-old man with end-stage renal disease from ADPKD who underwent central venous catheterization (permacath) to initiate hemodialysis. Upon catheterization, a complete right internal jugular vein septum (bicuspid valve) was found, along with an isolated PLSVC that drained directly in the coronary sinus. We demonstrate the multiple challenges encountered during the catheterization procedure and we illustrate the anomaly with detailed images and supplementary videos. Furthermore, we discuss the coexistence of PLSVC with renal anomalies in the context of previous literature. To conclude, interventional radiologists should keep the possibility of PLSVC in mind, especially when difficulties are encountered during catheterization where proper characterization of the PLSVC type and associated anomalies is crucial for tailoring the best management approach. Moreover, an association between venous anomalies including left superior vena cava and renal anomalies may co-exist, and further studies are needed to explore this possible association.

Case report: A novel occurrence of persistent left cranial vena cava coexisting with polycystic kidney disease in a cat

“…Because PKD in cats is known to be rooted in genetic causes ( 14 ), it is likely that these diseases occur together because of genetic defects. According to reports in humans, PLCVC and renal anomalies including PKD, horseshoe kidney, and unilateral absent kidney occur simultaneously ( 5 , 33 , 34 ). In addition, among humans, autosomal-dominant PKD (ADPKD) patients have a higher frequency of congenital heart disease than the general population ( 35 ).…”

“…In humans, persistent left superior vena cava (PLSVC) is used as an alternative term for PLCVC. PLSVC is the most frequently identified congenital thoracic vascular anomaly in humans, with a prevalence ranging from 0.2 to 3% in the general population, increasing to 1.3 to 11% in individuals with congenital heart disease (3,5). It has also been reported in several animal species, including dogs, with relatively few occurrences in cats (1,(6)(7)(8).…”