Author + information
- Received February 8, 2018
- Revision received May 17, 2018
- Accepted May 24, 2018
- Published online September 17, 2018.
- Wim T.J. Aanhaanen, MD, PhD,
- Jaap Jan J. Smit, MD, PhD,
- Arif Elvan, MD, PhD and
- Ahmet Adiyaman, MD, PhD∗ ()
- ↵∗Address for correspondence:
Dr. Ahmet Adiyaman, Isala Heart Center, Dokter van Heesweg 2, 8025 AB, Zwolle, the Netherlands.
A 52-year-old woman was diagnosed with Brugada syndrome in 2014, confirmed by an ajmaline challenge and the presence of a SCN5A mutation. Ajmaline induces coved-type ST-segment elevation with beat-to-beat variation, as a sign of imminent ventricular instability (1), and nonsustained ventricular tachycardias (VTs) (Online Figures 1 and 2). In 2017, we scheduled an epicardial ablation by Nademanee’s approach (2) after recurrent episodes of sustained VT.
Fractionated potentials were found in the epicardial right ventricular outflow tract (RVOT) with expansion of the area after ajmaline 55 mg intravenous (Figure 1A) (2). Moreover, coved-type ST-segment elevation with beat-to-beat variation was found (Figure 1C, Online Figure 3). After ablation of the epicardial substrate, we challenged the patient with 45 mg of ajmaline, and marked QRS lengthening and instability of the QRS complex was apparent with subsequent premature ventricular contractions and sustained VT (Online Figures 4 and 5). After hemodynamic stabilization and normalization of the electrocardiogram, frequent premature ventricular contractions and nonsustained VT remained. We hypothesized that endocardial ablation was required for transmural lesions to reach noninducibility (3).
The next day, we performed a procedure with an epi-endocardial approach (2,3). There were no residual fractionated potentials at the epicardium; however, fractionated and late potentials were found on the endocardial side throughout the endocardial RVOT, including the posteroseptal region, which cannot be the result of the epicardial ablation. A pace map similar to the clinical VT (99% match) was found in the posteroseptal RVOT at the location where the fractionated and late potentials were found (Figure 1B, Online Video 1). Noninducibility was reached only after ablation in this area and in the remaining endocardial substrate. Ajmaline 55 mg intravenous no longer produced a Brugada electrocardiogram (Online Figure 6). The patient remained free of VT during follow-up (Online Figure 7).
This case illustrates the occurrence and impact of structural abnormalities, besides a pathological SCN5A mutation, in patients with Brugada syndrome (4). Moreover, these abnormalities are not exclusive to the epicardium and the endocardium adjacent to epicardium, but can also include the myocardium of the posteroseptal RVOT.
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 February 8, 2018.
- Revision received May 17, 2018.
- Accepted May 24, 2018.
- 2018 American College of Cardiology Foundation
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