Author + information
- Received November 2, 2016
- Revision received December 8, 2016
- Accepted December 15, 2016
- Published online August 21, 2017.
- Ashish N. Doshi, MD, PhD∗ (, )
- Bradley C. Clark, MD and
- Charles I. Berul, MD
- ↵∗Address for correspondence:
Dr. Ashish N. Doshi, Department of Cardiology, Children's National Medical Center, 111 Michigan Avenue Northwest, Washington, DC 20010.
Ventricular tachycardia (VT) is a known sequela in patients with repaired congenital heart disease (CHD) (1). The mechanism frequently involves anatomic isthmuses created during surgical repair (2,3). Disruption of normal tissue architecture from artificial patches and baffles as well as fibrosis from prior surgical incisions provide this substrate for re-entry (4). We report a unique focal VT in an adult CHD patient that originated from an unusual congenital anatomic substrate rather than secondary to the surgical repair.
The patient was a 24-year-old woman born with truncus arteriosus type I and quadricuspid truncal valve with additional rudimentary noncoronary cusp (Figure 1). She underwent initial repair in infancy, involving pericardial patch closure of a large ventricular septal defect, excision of the main pulmonary artery from the truncus, and placement of a right ventricular to pulmonary artery conduit, which had been last revised 16 years prior. She presented with palpitations, and a Holter monitor demonstrated 7% isolated monomorphic premature ventricular contractions (PVCs) and short runs of nonsustained VT. She was referred for electrophysiology study and VT ablation. The electrophysiology study was performed nonfluoroscopically using 3-dimensional electroanatomic guidance and intracardiac echocardiography. Activation mapping of spontaneous PVCs and pace mapping using the PaSo system (Biosense Webster, Diamond Bar, California), demonstrated a discrete focus in the rudimentary noncoronary cusp of the quadricuspid truncal valve. There was no early site within the heart. Radiofrequency ablation was performed, with acceleration of ventricular ectopy during initial radiofrequency application and no further ectopy during the 2 subsequent lesions. There were no spontaneous or inducible PVCs with programmed stimulation. She had no further ventricular ectopy in the recovery room or on follow-up Holter monitoring.
This case highlights the importance of considering both the original anatomic substrate as well as post-surgical changes when evaluating arrhythmias in patients with repaired CHD.
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 2, 2016.
- Revision received December 8, 2016.
- Accepted December 15, 2016.
- 2017 American College of Cardiology Foundation
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