Author + information
- Received November 8, 2017
- Revision received December 4, 2017
- Accepted December 7, 2017
- Published online April 16, 2018.
- Christopher M. Janson, MDa,∗ (, )
- Veneel Bhupathiraju, MDb,
- Saurabh Talathi, MDc and
- Kristi Glotzbach, MDa
- aDivision of Cardiology, Children’s Hospital at Montefiore/Albert Einstein College of Medicine, Bronx, New York
- bDepartment of Pediatrics, Albert Einstein College of Medicine, Bronx, New York
- cDepartment of Pediatrics, Lincoln Medical Center, Bronx, New York
- ↵∗Address for correspondence:
Dr. Christopher M. Janson, Children’s Hospital of Philadelphia, 8 NW, 3401 Civic Center Boulevard, Philadelphia, Pennsylvania 19104.
A 4-week-old infant with a history of tuberous sclerosis, cardiac rhabdomyomas, and Wolff-Parkinson-White syndrome (Figures 1A and 1B) presented with wide QRS tachycardia (Figure 1C). Vagal maneuvers, adenosine, and esmolol had no effect on the tachycardia, suggesting that the atrioventricular (AV) node was not a critical component. Multiple accessory pathways (APs) were suspected, with a pathway-to-pathway AV re-entry tachycardia. A pre-excited atrial tachycardia was less likely. With procainamide, tachycardia gradually slowed, followed by a sudden change from wide to narrow QRS morphology, without a pause (Figure 1D). The narrow QRS complex suggested transition to orthodromic AV re-entry tachycardia, with antegrade AV nodal conduction. Adenosine was repeated, and tachycardia terminated transiently, with recurrence of wide complex tachycardia after 3 sinus beats (Figure 1E). The infant’s tachycardia was ultimately controlled on amiodarone and propranolol.
Cardiac rhabdomyomas, seen in up to 50% of patients with tuberous sclerosis, can create substrate for arrhythmia, depending on their location, with supraventricular tachycardia, ventricular tachycardia, sinus node dysfunction, and complete heart block as described (1,2). Ventricular pre-excitation is seen in up to 10% of children with cardiac rhabdomyomas (2). The tumors are hypothesized to function as accessory electrical AV connections, analogous to the traditional AP. In this patient, a mass at the septal aspect of the tricuspid valve correlated with an electrocardiogram pre-excitation pattern of a right posteroseptal or mid-septal AP (Figures 1A and 1B). Because multiple masses often coexist in the same patient, there is a theoretical substrate for multiple APs. In general, the presence of multiple APs is discovered at an electrophysiology study; multiple APs have been observed at an electrophysiology study in up to 10% to 13% of children with AV re-entry tachycardia, more often in association with structural heart disease (3), and in 13% of pediatric patients presenting with antidromic tachycardia (4). In this case, there was clear electrocardiographic evidence for at least 2 distinct accessory AV connections.
The authors have reported that they have no relationships relevant to the contents of this paper to disclose.
All authors attest they are in compliance with human studies committees and animal welfare regulations of the authors’ institutions and Food and Drug Administration guidelines, including patient consent where appropriate. For more information, visit the JACC: Clinical Electrophysiology author instructions page.
- Received November 8, 2017.
- Revision received December 4, 2017.
- Accepted December 7, 2017.
- 2018 American College of Cardiology Foundation
- Hinton R.B.,
- Prakash A.,
- Romp R.,
- et al.,
- for the International Tuberous Sclerosis Consensus Group
- Miyake C.Y.,
- Del Nido P.J.,
- Alexander M.E.,
- et al.