Persistent Left Superior Vena Cava with Absent Right Superior Vena Cava and Discrete Subaortic Stenosis Diagnosed in a Patient with Sick Sinus Syndrome: A Case Report and Brief Review of the Literature

Abstract

A persistent left superior vena cava (PLSVC) is the most frequent anomaly of the venous drainage system. While both a right and left superior vena cava (SVC) are usually present, a unique, left-sided SVC, also known as an isolated PLSVC, accounts for only 10–20% of cases. It is frequently associated with arrhythmias and other congenital cardiac anomalies. Though it is usually an asymptomatic condition, it may pose significant problems whenever central venous access is needed. We report a case of an isolated PLSVC that was diagnosed incidentally during pacemaker implantation for sinus node dysfunction. The venous anomaly was associated with subvalvular aortic stenosis determined by a subaortic membrane; this particular association of congenital cardiovascular anomalies is a rare finding, with only a few cases reported in the literature. We aim to highlight the clinical and practical implications of this condition, as well as to discuss the embryonic development and diagnostic methods of this congenital defect.

Cardiac Implantable Electronic Devices in Different Anatomical Types of Persistent Left Superior Vena Cava: Case Series and Brief Review of the Literature

“…Another associated congenital anomaly, with stronger clinical implications, was described in a previously published case. In this case, the patient was also diagnosed with subvalvular aortic stenosis, determined by a subaortic membrane, which is a rare finding, with only few similar cases being reported in the literature [ 5 ]. It has been observed that patients with PLSVC can also present conduction disorders, which arise as a result of the histological abnormalities caused by the dilated coronary sinus [ 23 ].…”

“…Among these cases, we identified six patients who had PLSVC, which leads to an incidence of at least 0.15% of this anomaly in the group of patients who required implantable devices in our clinic. It should be mentioned that, in these cases, we met several types of this congenital anomaly (two cases of type II, two cases of type III A, and two cases of type III B according to Schummer’s classification); however, in this article, we will present only four cases, since two of them have already been published [ 5 , 6 ]. None of these patients met the criteria for cardiac resynchronization therapy.…”

Implantation of a Dual-Chamber Automatic Cardioverter Defibrillator in a Patient with Persistent Left Superior Vena Cava: Case Report and Brief Literature Review

“…However, as PLSVC is mostly asymptomatic, the actual prevalence of this anomaly might be underestimated. In association with congenital heart disease (CHD), the incidence is even higher, reaching 9 to 10% [ 3 , 9 ]. Reports regarding CHD and PLSVC vary, due to demographics and the clinical characteristics of the studied population.…”

Pacemaker Implantation in a Patient with Isolated Persistent Left Superior Vena Cava Draining into the Left Atrium: A Case Report and Brief Literature Review

“…Persistent left superior vena cava (PLSVC) is the most common venous drainage anomaly of the systemic thoracic veins, with its prevalence among the general population ranging from 0.5 to 2%. In 80–90% of the cases, the PLSVC drains into the right atrium via a dilated coronary sinus [ 1 , 2 , 3 , 4 ].…”

“…PLSVC is commonly associated with a normal right-sided superior vena cava. However, in 10–20% of cases, an isolated PLSVC may be present, frequently coexisting with other cardiac congenital disorders or arrhythmias [ 4 , 5 , 6 , 7 ].…”