Europace Advance Access originally published online on November 24, 2007
Europace 2008 10(1):86-90; doi:10.1093/europace/eum244
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BRUGADA SYNDROME
Pilsicainide-induced Brugada-type ECG and ventricular arrhythmias originating from the left posterior fascicle in a case with Brugada syndrome associated with idiopathic left ventricular tachycardia
1 Division of Cardiology, Yamaguchi University Graduate School of Medicine, 1-1-1 Minami-Kogushi, Ube 755-8505, Japan; 2 Division of Cardiology and Faculty of Health Sciences, Yamaguchi University Graduate School of Medicine, Ube, Japan
Manuscript submitted 11 September 2007. Accepted after revision 8 October 2007.
* Corresponding author. Tel: +81-836-22-2248; fax: +81-836-22-2246. E-mail address: ueyama23{at}yahoo.co.jp
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Abstract |
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The patient was a 50-year-old male in 2002, who was first suspected of having a Brugada-type electrocardiogram (ECG). A drug challenge test using pilsicainide was performed and unmasked a typical coved type ST elevation followed by ventricular arrhythmias (VAs) manifesting a QRS pattern with a right bundle branch block and left axis deviation. Three years later, he was transferred to the emergency room due to a wide QRS tachycardia with the same QRS morphology as the VA that previously occurred in the drug challenge test. An ECG just after the recorded termination of the tachycardia exhibited a typical Brugada-type ECG. In an electrophysiological study, ventricular fibrillation could be easily induced with reproducibility. Since the clinical tachycardia could not be sustained by an isoproterenol infusion, mapping and catheter ablation targeting the pilsicainide-induced VAs was performed. The successful ablation site was the left mid-lower septal wall where a Purkinje potential was recorded and a false tendon was attached just to it.
Key Words: Idiopathic ventricular tachycardia, Brugada syndrome, Pilsicainide
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Introduction |
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Brugada syndrome is a clinical entity characterized by syncope or sudden cardiac death due to polymorphic ventricular tachycardia or ventricular fibrillation (VF) and specific electrocardiographic (ECG) features consisting of a right bundle branch block (RBBB) and ST-segment elevation in the right precordial leads in patients with structurally normal hearts.1
Idiopathic left ventricular tachycardia (ILVT) manifesting a QRS pattern with an RBBB and left axis deviation (LAD), so called left posterior fascicular tachycardia, is also another clinical entity usually occurring in young adults without any structural heart disease.2,3 We report a case of a patient with both types of arrhythmic disorders. |
Case report |
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The patient was a 50-year-old male in which a Brugada-type ECG (saddle-back type) was recorded when he was admitted to our hospital for atopic dermatitis in June of 2002. Since a drug challenge test with pilsicainide at a dose of 25 mg (0.42 mg/kg intravenously) revealed a typical coved type ST elevation followed by ventricular arrhythmias (VAs) exhibiting a QRS pattern with an RBBB and LAD, we recommended a further examination for ECG abnormalities but he refused it at that time.
Three years later, for the first time, he felt palpitations that lasted during the all night and he pointed out that he had had a tachycardia episode at a prior hospital. The ECG revealed a wide QRS tachycardia, with a QRS pattern exhibiting an RBBB and LAD, and a rate of 185 bpm (Figure 1A). After his arrival to our emergency room, the tachycardia spontaneously terminated and was followed by sinus rhythm. The ECG during sinus rhythm recorded just after the tachycardia termination exhibited a typical Brugada-type ECG (Figure 1B). The patient had no abnormal physical findings except for his atopic skin condition. He was in an afebrile state and his blood tests including the cardiac enzymes and serum electrolytes were also within normal limits. Late potentials were considered positive on the signal-averaged ECG (Fukuda Denshi Co. Ltd. FDX-6531, Tokyo, Japan); and the filtered QRS duration was 150 ms, duration of low amplitude signals of <40 µV, 53 ms, and root mean square voltage of the signals in the last 40 ms, 8 µV. There were no obvious structural abnormalities as assessed by echocardiography, cardiac enhanced computed tomography and magnetic resonance imaging. Only a false tendon across the left ventricular (LV) mid-lower septum to the infero-lateral wall was observed on the echocardiogram.
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As the typical Brugada sign disappeared from his ECG recorded a few days later, a pilsicainide challenge test was performed once again (Figure 1C and D) as described previously.4
The pilsicainide unmasked a typical Brugada-type ECG once again and the infusion at a dose of 0.45 mg/kg (27 mg) was immediately stopped because of the occurrence of a VA with a QRS pattern similar to that of the clinical wide QRS tachycardia that lasted for 
12 min. Coronary angiography and right ventriculography revealed no abnormal findings. An electrophysiological study (EPS) was performed under sedation using propofol. The EPS revealed normal values for the sinus and atrioventricular nodal function and a normal HV interval of 50 ms. No retrograde conduction was observed and no supraventricular tachyarrhythmias other than atrial fibrillation could be induced by rapid atrial pacing or programmed pacing from the high right atrium. Ventricular fibrillation was induced twice by programmed double extra stimuli (basic cycle length, 600 ms; S1S2, 250 ms; S2S3, 190 ms) applied from the right ventricular outflow tract (RVOT) under controlled conditions (Figure 2A). During an isoproterenol infusion, the VF could not be re-induced, but a non-sustained ventricular tachycardia (VT) with the same QRS morphology as the clinical tachycardia was induced reproducibly with programmed stimulation (Figure 2B). However, the induced VT could not be sustained, and we administered a low-dose of pilsicainide intravenously. After the administration of 20 mg (0.33 mg/kg) of pilsicainide, a VA occurred that had an RBBB morphology with LAD. The intracardiac electrograms recorded in the LV during the VA exhibited Purkinje potentials (PPs) preceding the ventricular activation (Figure 3). Although the PPs could be recorded during the VA, no diastolic potentials could be found. We performed endocardial activation mapping of the LV using an electroanatomic mapping system (CARTOTM, Biosense-Webster, Johnson & Johnson, USA) and radiofrequency (RF) catheter ablation, applied to the earliest sites of the PPs was performed. Finally, no more pilsicainide-induced VAs could be induced after several RF applications.
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One week after the catheter ablation, we once again performed a pilsicainide challenge test. The administration of 50 mg (0.83 mg/kg) of pilsicainide could reproducibly reveal a typical Brugada-type ECG but never induced any VAs. Although he may have been cured of the ILVT by eliminating the pilsicainide-induced ILVT-like arrhythmia, it was unlikely to have reduced the potential risk for a more ominous and life-threatening polymorphic ventricular tachycardia (PVT) or VF associated with Brugada syndrome, and therefore, we recommended that the patient receive an implantable cardioverter defibrillator but he refused. Although he has remained free of any symptoms or arrhythmia recurrence without any antiarrhythmic therapy for over 24 months, a long-term follow-up will still be required. During the observed period, there were no ECG findings such as PR prolongation suggesting an SCN5A mutation and none of the mutation identified in the SCN5A gene were found in the present case.
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Discussion |
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We reported the present case with two rare associated conditions: (i) Brugada syndrome and (ii) ILVT displaying a QRS pattern with RBBB and LAD. In addition, the intravenous administration of pilsicainide, a class 1C agent, reproducibly revealed a typical coved type ST elevation followed by VAs typical of ILVT. Finally, the use of that medication for provocation testing to induce the VAs in the present case with no inducible sustained ILVT during the EPS, enabled us to cure the VAs with RF applications.
It is well recognized that either ILVT or Brugada syndrome is two distinct clinical and ECG entities; the former was first reported by Zipes2 and Belhassen3 and the latter by the Brugada brothers in 1992.1 Brugada syndrome is usually associated with PVT or VF as a result of sudden cardiac death or syncope. Although sustained monomorphic VT (SMVT) is infrequent in Brugada syndrome, there have been several reports describing Brugada syndrome associated with SMVT.5–14 Among the eight available patients in those reports, the VT morphology exhibited an RBBB pattern in four patients and an LBBB pattern in another four. The SMVT in those reports was more prevalent in children and infants and was more affected by certain specific conditions, febrile states, sodium channel blockers and cathecolaminergic conditions such as during exercise, alcohol consumption or an isoproterenol infusion. In our case, the clinical SMVT was documented without any febrile state or the use of any drugs or alcohol. To the best of our knowledge, to date there have been no reports about Brugada syndrome coexisting with SMVT typical of ILVT exhibiting an RBBB and LAD pattern.
The documentation of the spontaneous coved type ST elevation after the termination of the VT and reproducible induction of VF in the VT provocation test under the control state suggested that the present case's electrophysiological characteristics were compatible with Brugada syndrome. Pilsicainide-induced VAs are often observed in patients with Brugada syndrome, but most of them originate from the RV, especially from the outflow tract.15 In contrast, pilisicainide-induced VAs have very rarely been associated with idiopathic VT or VF without Brugada syndrome. Nagai et al.16 were first to report the facilitation of the induction of a sustained ILVT following the administration of pilsicainide in patients with spontaneous VT who had no inducible arrhythmias during the EPS. Non-sustained polymorphic VT following the administration of pilsicainide was also reported in a patient with an apparent idiopathic VF without Brugada syndrome.17 Nagai et al.16 succeeded to induce a sustained ILVT after the intravenous administration of pilsicainide, at a much lower dose (0.14 mg/kg) compared to that used in our case (0.33 mg/kg). We continued to observe for spontaneous non-sustained ILVT-like VAs and did not perform a VT provocation test during the EPS after the administration of the pilsicainide in order to avoid any further proarrhythmic risks of Brugada syndrome.
In regard to the therapeutic technique to treat ILVTs, the sustained tachycardias in most of the cases with ILVT can be induced by an adequate stimulus protocol with or without an ISP infusion, and favorable results can be expected from an RF ablation targeting the earliest PPs18 or diastolic potentials during the VT.19 However, during the EPS in the present case no sustained clinical VT could be induced, but VF could be induced due to the coexistence of an arrhythmogenic substrate of Brugada syndrome. In such cases with ILVT who have no inducible or only non-sustained VAs, the classic endocardial pace mapping method, linear ablation of the left septal wall guided by the PPs during sinus rhythm20,21 or RF ablation of the VAs22 using a three-dimensional mapping system would be attempted. Our successful strategy of the RF ablation, i.e. the observation that potential proarrhythmic effects of pilsicainide could be used to facilitate the induction and cure of the arrhythmia, may be a novel clinical approach for the treatment of patients with no inducible sustained ILVT as Nagai et al.16 reported. Pro-arrhythmias due to antiarrhythmic drugs are a well-known phenomenon in patients with VT related to organic heart disease but are very unusual in patients with idiopathic VT. However, since a Brugada-type ECG can occur with either repolarization or depolarization abnormalities,23,24 even though no genetic mutations of the SCN5A gene were found in this case, it would not be surprising if such an arrhythmogenic substrate produced by the pilsicainide may have existed around the Purkinje network and may have lead to the VA. Presumably, an arrhythmogenic substrate may not only exist in the RVOT, but also somewhere else in some cases with Brugada syndrome. Thus, that might be one of the possible reasons that various arrhythmias,25,26 as well as VF and PVT, coexist in patients with Brugada syndrome.
Conflict of interest: none declared.
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