Epicardially placed implantable cardioverter‐defibrillator for a child with congenital long QT syndrome

Abstract

A 7-year-old boy presented at our hospital with syncope. At birth, electrocardiography had shown a long QT interval with torsade de pointes (TdP). Congenital long QT syndrome (LQTS) had been diagnosed by genetic testing, and was successfully controlled with oral propranolol. At age 7, TdP had recurred with syncope. Electrocardiography revealed a prominent long QT interval with T-wave alternans. The propranolol dose was increased, but TdP remained uncontrolled. A cardioverter-defibrillator (ICD) was implanted epicardially, and TdP completely resolved with atrial pacing. We report this rare case of ICD implantation in a child with LQTS.

A rare case of tetralogy of Fallot with catecholaminergic polymorphic ventricular tachycardia

“…This procedure was previously performed in a 7-year-old patient with long QT syndrome type 2 in our hospital. 8 …”

Minimally invasive, pericardial implantable cardioverter‐defibrillator placement in a young child

“…As previously described, pericardial placement of a defibrillation coil provides a safe and efficacious approach to ICD therapy in young children when a transvenous system is not feasible [3] , [4] . The configuration of a posteriorly and superiorly placed coil in the oblique sinus and abdominal generator demonstrates low and stable defibrillator energy and stable impedance.…”

Effect of beta‐blockade on quantitative microvolt T‐wave alternans in 24‐hour continuous 12‐lead ECG recordings in patients with long QT syndrome

“…The effect of beta-blockade on quantitative microvolt TWA has not previously been investigated in LQTS patients, although a few cases of reduction or elimination of macroscopic TWA by betablockade and improvement in symptoms in pediatric LQTS patients have been reported (Bosi et al, 2002;Mache et al, 1996;Sugiyama et al, 2017). Japanese Circulation Society guidelines recommend microvolt TWA for prediction of sudden cardiac death in patients with ischemic (JCS Joint Working Group, 2012; Klingenheben, Grönefeld, Li, & Hohnloser, 2001;Rashba et al, 2002) or non-ischemic cardiomyopathy (JCS Joint Working Group, 2012; Klingenheben et al, 2001); in these populations, metoprolol (Klingenheben et al, 2001), d,l-sotalol (Klingenheben et al, 2001, and esmolol (Rashba et al, 2002) have been reported to suppress microvolt TWA along with arrhythmia (Verrier & Ikeda, 2013).…”

“…Although a few case reports demonstrated that beta-blockade reduces "macroscopic" TWA in association with arrhythmias in LQTS (Bosi, Cappato, Priori, & Stramba-Badiale, 2002;Mache et al, 1996;Sugiyama et al, 2017), the effect of beta-blockade on microvolt levels of TWA in LQTS patients has not been systemically investigated.…”