Brugada phenocopies are the leading differential diagnosis of Brugada syndrome
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Brugada phenocopies are the leading differential diagnosis of Brugada syndrome
Editor – We read the literature review on Brugada syndrome (BrS) by Sheikh and Ranjan with great interest (Clin Med 2014;5:482–9). Their manuscript provides a concise review of BrS and identifies associated differential diagnoses. We would like to direct the authors to our work on Brugada phenocopies (BrP). 1 BrP are the leading differential diagnosis of true congenital BrS. Many of the underlying BrS differential diagnoses that the authors mention are actually various underlying etiologies of BrP. We would like to provide the authors with a brief explanation of BrP and its clinical distinction from BrS.
BrP are clinical entities that present with identical ECG patterns to those of true congenital BrS but are elicited by various other clinical circumstances. 1 Conditions that induce BrP are characterised into six etiological categories: metabolic conditions, mechanical compression, myocardial ischemia and pulmonary embolism, myocardial and pericardial disease, ECG modulations and miscellaneous. 1 Our international registry and online educational portal provides an updated registry of BrP cases along with the diagnostic criteria. 2
Many of the conditions that the authors mention as part of their differential diagnosis for a Brugada ECG pattern are confirmed causes of BrP, including acute pericarditis, myocardial ischemia, pulmonary embolism, hyperkalemia, hypercalcemia, hypothermia and pectus excavatum. BrP form a group of heterogeneous conditions that are perhaps the most difficult to differentiate from true congenital BrS due to identical ECG patterns. Distinguishing between BrP and BrS relies on a series of clinical and ECG characteristics (Table 1). A systematic diagnostic approach to differentiate between BrP and BrS is imperative because the same clinical condition can elicit BrP or unmask true congenital BrS. For example, hyperkalemia has been associated with BrS 3 and BrP. 2 Similarly, myocardial ischemia has been found to unmask true BrS patients 4 and also present as BrP. 2 Important criteria that differentiate BrP and BrS are that patients with BrP have a negative provocative challenge with a sodium channel blocker, along with an absence of clinical history suggestive of a sudden cardiac death syndrome.
We encourage authors to use the term Brugada phenocopy for consistency in the literature and to facilitate future research.
- © Royal College of Physicians 2015. All rights reserved.
References
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Brugada phenocopy international registry and online educational portal, 2014. Available online at www.brugadaphenocopy.com [Accessed 27 February 2015].
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- Postema PG
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