HRCR Rare Diseases Article Collection
Localized intramural reentry confined within the ventricular septum in lamin cardiomyopathy
High-resolution electroanatomic mapping systems have greatly improved the ability to characterize the size and dimensions of the reentrant circuit responsible for human ventricular tachycardia (VT). The minimal dimension of critical isthmus regions may be less than 1 cm in more than 25% of circuits mapped.1 Despite advanced, detailed simultaneous epicardial and endocardial mapping, detection of intramural circuit components remains challenging. Epicardial mapping through coronary venous branches has gained popularity owing to refinement of mapping catheters and novel use of transcoronary venous ethanol.Masquerade: An unusual accessory pathway with ventricular insertion at the right–left sinus of Valsalva mimicking outflow tract ventricular tachycardia
Although rare, an accessory pathway (AP) can have a ventricular insertion in the region of the aortic sinuses of Valsalva.1–4 This can be difficult to diagnose and may resemble outflow tract ventricular tachycardia (VT).4 Despite the existence of various algorithms that can differentiate wide-complex supraventricular tachycardia (SVT) from VT, these conditions may be impossible to differentiate based on surface QRS morphology alone.5,6Ventricular tachycardia ablation in a patient with Ehlers-Danlos syndrome
Ventricular tachycardia (VT) is a well-recognized complication of postinfarct-related scar. Catheter ablation is a guideline-recommended therapy in patients with frequent VT episodes. Ablation aims to target regions of slow conduction within ventricular scar that support reentry.1Late-onset Fabry disease revealed by ventricular tachycardia: A case report
Fabry disease (FD) is an X-linked lysosomal storage disorder due to mutations in the GLA gene leading to deficiency of lysosomal α-galactosidase A (α-Gal A). Classic FD causes multiorgan failure, whereas the later-onset phenotype is characterized by predominantly cardiac manifestations. Ventricular arrhythmias are among the complications.1,2Ventricular tachycardia as the initial presentation of missed Kawasaki disease in a teenager
Ventricular tachycardia (VT) has been reported in acute cases of Kawasaki disease. VT secondary to ischemic sequelae is also a known long-term complication of Kawasaki disease, typically seen 2 decades after initial disease onset.1,2 We report the first case, to our knowledge, of an otherwise healthy teenager with sustained VT as the presenting symptom for missed Kawasaki disease and describe the management approach by our team that had not considered Kawasaki disease in the differential. This case broadens our understanding of Kawasaki disease presentations in the young and timing of long-term sequelae.
