Successful implantation of a resynchronization implantable cardioverter defibrillator in a patient with persistent left superior vena cava with absent right SVC

摘要

Introduction: Persistent left superior vena cava (PLSVC) withabsent right SVC is a rare congenital anomaly of the vena cava,which poses challenges during performing transvenous pacing.We report a case of a successful biventricular pacing and defibrillatordevice implantation (CRT-D) via a persistent left superiorvena in a patient with dilated cardiomyopathy and severe LVsystolic dysfunction with absent right SVC.Case report: A 53-yr-old male patient presented to us with suddenonset of breathlessness with orthopnea. On evaluation ECG doneshowed LBBB with QRS duration of 120 ms with secondary ST-Tchanges. 2D Echo done showed dilated LV with global hypokinesia,ventricular dyssynchrony and severe LV systolic dysfunction with2D LVEF 15–20%. Patient was treated conservatively and lateradvised coronary angiography (CAG). CAG done showed nonobstructivecoronary artery disease. Patient was later dischargedon diuretics, ACE inhibitors, spiranolactone and beta blockers.Despite being on optimal medical therapy for 3 months patientremained in NYHA class II, hence CRT-D implantation wasplanned.Venography done revealed a PLSVC with absent right SVC withPLSVC draining into an extremely dilated coronary sinus. A 5 FJudkins-type angiographic catheter (JR 4.0) was used to performselective coronary sinus tributaries venography via the left subclavianvein. Then with the help of guide wire lateral vein wascannulated. Retaining the wire, the angiographic catheter wasreplaced with a guiding sheath, and then the left ventricular leadsuccessfully positioned in a lateral vein. A dual-coil active-fixationdefibrillator lead was positioned in the right ventricular apex,followed by a SonR active fixation lead in the right atrial free wall.All three leads were implanted through the PLSVC. Acute thresholdswere checked and all leads were eventually connected to aCRT -D device. Later ECG done showed biventricular pacing andchest X-ray done confirmed the lead positions. The patient wasdischarged from the hospital without complications.Discussion: Persistent left SVC (PLSVC) is a rare anomaly of thevenous circulation, and it occurs in 0.5% of the general population,0.3% of healthy persons, and 4.3% of those with congenital heartdisease. In most patients with left SVC, a right SVC is present. PLSVC with absent right SVC occurs in only 0.09–0.13% of patientswho have congenital heart defects. Although this rare condition isfrequently associated with situs inversus, our patient was situssolitus and lacked a right SVC and had a persistent left SVC thatdrained into the right atrium via an extremely dilated coronarysinus. This condition is typically asymptomatic, usually incidentallydiscovered during pacemaker implantation and it can complicatelead placement through the subclavian approach. Although there are many case reports about successful defibrillator or pacemakerlead implantations through PLSVC, there are only fewcase reports of CRT-D implantation in patients with PLSVC withabsent SVC.