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Brugada Phenocopy Is an Important Differential Diagnosis in Patients with Type 1 Brugada ECG Patterns
LETTER
Brugada Phenocopy Is an Important Differential Diagnosis in Patients with Type 1 Brugada ECG Patterns To the Editor: We read the case presentation by Yamagami et al1 and correspondence by Manohar and Gitler2 with great interest and noted that both groups did not mention Brugada phenocopy as an important differential diagnosis in patients presenting with type 1 Brugada electrocardiogram (ECG) patterns. Brugada syndrome is an inherited syndrome characterized by type 1 or type 2 Brugada ECG patterns in precordial leads V1-V3 that predispose individuals to ventricular arrhythmias and sudden cardiac death.3 Brugada phenocopies are clinical entities that have ECG patterns identical to true congenital Brugada syndrome, but are elicited by various clinical conditions such as metabolic derangements, mechanical mediastinal compression, myocardial ischemia, and pulmonary embolism.4 We agree with Manohar et al2 in that attributing the ECG findings to right ventricular infarction secondary to pulmonary embolism seems plausible but premature. Febrile states are known to unmask true congenital Brugada syndrome3 and further investigation is warranted, as this patient may be at risk of sudden cardiac death and may require an implantable cardioverter-defibrillator. Interestingly, this patient presented with concurrent pulmonary embolism and evidence suggestive of right ventricular infarction. Type 1 Brugada ECG patterns have been previously reported in the context of pulmonary embolism5 and right ventricular infarction.6 Differentiating between Brugada syndrome and Brugada phenocopy is important and requires a systematic diagnostic approach, as failure to identify patients with Brugada syndrome may have negative implications on morbidity and mortality. We have established a systematic diagnostic Funding: None. Conflict of Interest: None. Authorship: All authors had access to the data and a role in writing the manuscript.
0002-9343/$ -see front matter Ó 2015 Elsevier Inc. All rights reserved.
approach to Brugada phenocopy5 and suggest that Yamagami et al1 apply our approach to this patient. Most importantly, this patient should undergo provocative challenge testing with a sodium channel blocker such as flecainide, ajmaline, or procainamide to rule out myocardial sodium channel dysfunction.3 A positive challenge would suggest Brugada syndrome and may be an indication for an implantable cardioverter-defibrillator. A negative personal and family history, along with a negative provocative challenge, would suggest Brugada phenocopy; the etiology of which could be either the pulmonary embolism or right ventricular infarction. We invite these authors, and future authors, to use the term “Brugada phenocopy” to facilitate literature searches and future research in this area. In addition, should this patient meet Brugada phenocopy diagnostic criteria, we invite Yamagami et al1 to submit this and future cases to our online international registry at www.brugadaphenocopy.com. Daniel D. Anselm, MD Byron H. Gottschalk, BMSc Adrian Baranchuk, MD Department of Cardiology Kingston General Hospital Queen’s University Kingston, Ont Canada
http://dx.doi.org/10.1016/j.amjmed.2015.04.045
References 1. Yamagami F, Mizuno A, Shirai T, Niwa K. A savage sequence: ST-segment elevations with pulmonary embolism. Am J Med. 2014;127:820-822. 2. Manohar S, Gitler B. Is this right ventricular infarction or Brugada syndrome? Am J Med. 2015;128(4):e25. 3. Bayes de Luna A, Brugada J, Baranchuk A, et al. Current electrocardiographic criteria for diagnosis of Brugada pattern: a consensus report. J Electrocardiol. 2012;45:433-442. 4. Anselm DD, Gottschalk BH, Baranchuk A. Brugada phenocopies: consideration of morphologic criteria and early findings from an international registry. Can J Cardiol. 2014;30:1511-1515. 5. Wynne J, Littmann L. Brugada electrocardiogram associated with pulmonary embolism. Int J Cardiol. 2013;162:e32-e33. 6. Anselm DD, Barbosa-Barros R, de Sousa Belém L, Nogueira de Macedo R, Pérez-Riera AR, Baranchuk A. Brugada phenocopy induced by acute inferior ST-segment elevation myocardial infarction with right ventricular involvement. Inn Card Rhythm Manag. 2013;4:1092-1094.
Brugada Phenocopy Is an Important Differential Diagnosis in Patients with Type 1 Brugada ECG Patterns To the Editor: We read the case presentation by Yamagami et al1 and correspondence by Manohar and Gitler2 with great interest and noted that both groups did not mention Brugada phenocopy as an important differential diagnosis in patients presenting with type 1 Brugada electrocardiogram (ECG) patterns. Brugada syndrome is an inherited syndrome characterized by type 1 or type 2 Brugada ECG patterns in precordial leads V1-V3 that predispose individuals to ventricular arrhythmias and sudden cardiac death.3 Brugada phenocopies are clinical entities that have ECG patterns identical to true congenital Brugada syndrome, but are elicited by various clinical conditions such as metabolic derangements, mechanical mediastinal compression, myocardial ischemia, and pulmonary embolism.4 We agree with Manohar et al2 in that attributing the ECG findings to right ventricular infarction secondary to pulmonary embolism seems plausible but premature. Febrile states are known to unmask true congenital Brugada syndrome3 and further investigation is warranted, as this patient may be at risk of sudden cardiac death and may require an implantable cardioverter-defibrillator. Interestingly, this patient presented with concurrent pulmonary embolism and evidence suggestive of right ventricular infarction. Type 1 Brugada ECG patterns have been previously reported in the context of pulmonary embolism5 and right ventricular infarction.6 Differentiating between Brugada syndrome and Brugada phenocopy is important and requires a systematic diagnostic approach, as failure to identify patients with Brugada syndrome may have negative implications on morbidity and mortality. We have established a systematic diagnostic Funding: None. Conflict of Interest: None. Authorship: All authors had access to the data and a role in writing the manuscript.
0002-9343/$ -see front matter Ó 2015 Elsevier Inc. All rights reserved.
approach to Brugada phenocopy5 and suggest that Yamagami et al1 apply our approach to this patient. Most importantly, this patient should undergo provocative challenge testing with a sodium channel blocker such as flecainide, ajmaline, or procainamide to rule out myocardial sodium channel dysfunction.3 A positive challenge would suggest Brugada syndrome and may be an indication for an implantable cardioverter-defibrillator. A negative personal and family history, along with a negative provocative challenge, would suggest Brugada phenocopy; the etiology of which could be either the pulmonary embolism or right ventricular infarction. We invite these authors, and future authors, to use the term “Brugada phenocopy” to facilitate literature searches and future research in this area. In addition, should this patient meet Brugada phenocopy diagnostic criteria, we invite Yamagami et al1 to submit this and future cases to our online international registry at www.brugadaphenocopy.com. Daniel D. Anselm, MD Byron H. Gottschalk, BMSc Adrian Baranchuk, MD Department of Cardiology Kingston General Hospital Queen’s University Kingston, Ont Canada
http://dx.doi.org/10.1016/j.amjmed.2015.04.045
References 1. Yamagami F, Mizuno A, Shirai T, Niwa K. A savage sequence: ST-segment elevations with pulmonary embolism. Am J Med. 2014;127:820-822. 2. Manohar S, Gitler B. Is this right ventricular infarction or Brugada syndrome? Am J Med. 2015;128(4):e25. 3. Bayes de Luna A, Brugada J, Baranchuk A, et al. Current electrocardiographic criteria for diagnosis of Brugada pattern: a consensus report. J Electrocardiol. 2012;45:433-442. 4. Anselm DD, Gottschalk BH, Baranchuk A. Brugada phenocopies: consideration of morphologic criteria and early findings from an international registry. Can J Cardiol. 2014;30:1511-1515. 5. Wynne J, Littmann L. Brugada electrocardiogram associated with pulmonary embolism. Int J Cardiol. 2013;162:e32-e33. 6. Anselm DD, Barbosa-Barros R, de Sousa Belém L, Nogueira de Macedo R, Pérez-Riera AR, Baranchuk A. Brugada phenocopy induced by acute inferior ST-segment elevation myocardial infarction with right ventricular involvement. Inn Card Rhythm Manag. 2013;4:1092-1094.