Persistent Left Superior Vena Cava: A Rare Case with Clinical Significance

Abstract

A 55-year-old Indian lady presented to the emergency department with a history of fever and breathing difficulty for the last four days. She had a recent history of admission (four days prior) in a local hospital for recurrent syncope attacks, breathing difficulty, chest heaviness and giddiness and on evaluation was found to have complete heart block on electrocardiogram (ECG) for which permanent dual chamber cardiac pacemaker was implanted through the right subclavian vein. Her chest radiograph (A-P view) done at the local hospital show bilateral chest infiltrates and also the pacemaker leads were seen on the left side of the chest [ Fig-2b]. Her medical history included type 2 diabetes mellitus and hypertension for which she was on regular medicines for past 4 years. She was admitted to the Intensive Care Unit (ICU) for the management of the sepsis due to clinical evidence of pneumonia. On examination, she was drowsy but arousable, moving all limbs, following simple commands with no nuchal rigidity and both pupils equal in size and reacting to light. Her blood pressure was150/80mmHg, pulse 120/minute, random blood sugar 300mg%, no pedal oedema, normal jugular venous pulse, chest-bilaterally clear, heart sounds normal. Her liver function tests, renal function test was normal with leukocytosis. Her echocardiogram showed pacing leads in the right atrium and ventricle with a dilated coronary sinus and left ventricle ejection aBstRaCt Persistent Left Superior Vena Cava (PLSVC) is a rare congenital vascular anomaly (incidence of 0.3-0.5% of the general population) which being mostly asymptomatic in its presentation, is usually detected incidentally. There are many practical clinical implications associated with it including arrhythmias. We report a rare case of PLSVC with absent Right Superior Vena Cava (RSVC) (isolated PLSVC), in a 55-year-old lady who had complete heart block followed by sepsis and was diagnosed to have this condition during the permanent cardiac pacemaker implantation and central venous catheter insertion showing an abnormal path of the catheter/pacing leads. The authors also give an insight into its clinical relevance.

Permanent pacemaker implantation in a patient with persistent left superior vena cava with an absent right superior vena cava: A case report

“…This case report described the successful implantation of a permanent pacemaker in a patient with isolated PLSVC. PLSVC is a persistent left-sided venous vasculature that drains into the right atrium through the CS due to failure of the left superior cardinal vein to form the "ligament of Marshall" during the early weeks of embryologic development [6] . Venous return through the CS increases, which causes dilatation of the CS.…”

A Case of Sudden Death Due to Persistent Severe Pulmonary Arterial Hypertension After Late Atrial Septal Defect Closure

“…It is the most common congenital thoracic venous anomaly with a prevalence of 0.3–0.5% in the general population . Although it is usually asymptomatic, it has been associated with structural abnormalities of the sinus node and conduction tissues due to dilation of the coronary sinus, which can result in cardiac rhythm disorders . Failure in identifying this anomaly at the autopsy could have been due to several reasons: since the left superior vena cava dimensions have an inverse relationship with those of the right one, the regular size of the right superior vena cava observed during the autopsy could explain a small caliber of the left one.…”

Acute pontine infarction in a patient with persistent left superior vena cava

“…It is a rare disease with a prevalence of 0.3-0.5% in the general population. 4 In over 90% of PLSVC cases, it drains into the right atrium through the coronary sinus and does not form a RLS or lead to hemodynamic problems. Affected patients are at higher risk of air embolism associated with intravenous injection as well as stroke or intracranial abscess associated with septic embolism or thromboembolism.…”