Homozygous frameshift variant in desmoglein 2 gene causes biventricular arrhythmogenic right ventricular cardiomyopathy.

Arrhythmogenic right ventricular cardiomyopathy caused by a homozygous frameshift variant (c.2358delA) in DSG2.

Positional syncope occurring just at the right time in a patient with a permanent pacemaker: A case report.

We present an unusual case of positional syncope occurring years after pacemaker implantation. Syncope was caused by a pacemaker lead fracture, which resulted from subclavian crush syndrome. The syncope occurred incidentally during hospital admission and was timely diagnosed using an integrated approach of history taking, examination findings, device interrogation and radiographic parameters. The patient subsequently underwent lead and device alteration which led to resolution of her symptoms.

Two Cases of Cardiac Implantable Electronic Device Placement via Persistent Left Superior Vena Cava.

Persistent left superior vena cava (PLSVC) is the most common variation of anomalous venous return to the heart and present in 0.1-0.5% of the general population. The left anterior cardinal veins typically obliterate during early cardiac development but failure of involution results in PLSVC. It is an asymptomatic congenital anomaly, usually discovered while performing interventions through the left subclavian vein or during cardiovascular imaging. PLSVC can be associated with cardiac arrhythmias and congenital heart disease. We present two cases of PLSVC: first, a 68-year-old male who presented with complete heart block, for which a temporary pacemaker was initially inserted followed by a permanent pacemaker; second, a 53-year-old female with a history of hypertension and ischemic cardiomyopathy with a left ventricular ejection fraction of 25%, and a survivor of sudden cardiac death, who underwent an implantable cardioverter-defibrillator (ICD) for secondary prevention. Both cases of PLSVC were detected incidentally during the transvenous approach to the heart. PLSVC was suspected by the unusually left medial position of the lead, while cineflouroscopy showed the venous trajectory toward the coronary sinus and drainage into the right atrium. It is technically difficult to cross the wire through the tricuspid valve when coming from the PLSVC and coronary sinus without making a loop in the right atrium, which is known as a wide loop technique. PLSVC is an uncommon anomalous anatomical variant and should be recognized appropriately by specialists who frequently carry out procedures through the left subclavian vein, such as implantation of permanent pacemaker, ICD and cardiac resynchronization therapy. It should also be recognized that wide loop formation of the right ventricular lead in the right atrium is helpful to cross the tricuspid valve and to affix the lead in the right ventricle. LEARNING POINTS: Persistent left superior vena cava is an anatomical variant that should be recognized by specialists who frequently carry procedures through the left subclavian vein (e.g. implantation of a permanent pacemaker, implantable cardioverter-defibrillator and cardiac resynchronization therapy).Maneuvers like wide loop formation of the right ventricular lead in the right atrium is helpful to cross the tricuspid valve and to affix the lead in the right ventricle.The cardiac imaging specialist should also suspect and rule out PLSVC on encountering a dilated coronary sinus on any imaging modality.