HRCR Rare Diseases Article Collection
- 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.
- 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.5,6
- 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.1
- 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.1,2
- 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.