Author + information
- Kiersten Frenchu, MD∗ (, )
- Shane Flood, MD,
- Lauren Rousseau, MPAS, PA-C, CCDS,
- Athena Poppas, MD and
- Antony Chu, MD
- Department of Internal Medicine, Brown University Rhode Island Hospital, Providence, Rhode IslandDepartment of Internal Medicine, Brown University Rhode Island Hospital, Providence, Rhode Island
- ↵∗Address for correspondence:
Dr. Kiersten Frenchu, Brown Medicine, 245 Chapman Street, Providence Rhode Island 02905.
Rapid diagnosis of Brugada syndrome is critical to therapy, which is aimed at reversing provoking factors to suppress/terminate malignant arrhythmias. This case highlights the diagnosis and peri-operative management of patients with Brugada syndrome at high risk for sudden cardiac death. (Level of Difficulty: Beginner.)
Brugada syndrome is an inherited channelopathy that confers an increased risk of sudden cardiac death (SCD). Approximately 25% of individuals affected with Brugada syndrome demonstrate a mutation in the SCN5a sodium channel (1). Baseline electrocardiography (ECG) results may be electrophysiologically normal and therefore confound the diagnosis. Provoking factors may be required to unmask the Brugada ECG pattern (2). This case highlights the discovery and management of a patient with heretofore undiagnosed Brugada syndrome.
A 50-year-old female with a history of essential hypertension and hyperlipidemia presented with fever and abdominal pain. She denied any chest pain, palpitations, or syncope prior to admission, and acute appendicitis was diagnosed. Her baseline ECG was unremarkable. Pre-operatively, she developed spontaneous ventricular fibrillation (VF) and was found unresponsive and required a 200-J biphasic shock for termination. After defibrillation, her ECG revealed new ST-segment elevations, right bundle branch pattern, and coved, persistently elevated ST-segments in V1 and V2, consistent with type 1 Brugada syndrome (Figure 1).
Treatment of this patient required rapid diagnosis of Brugada syndrome by ECG findings and clinical history. This patient’s post-arrest ECG is demonstrative for type 1 Brugada, with coved ST-segment elevations of >2 mm in V1 to V2 and a negative T-wave in the setting of right bundle branch block (1,2). Importantly, the QTc complex is normal in patients with Brugada syndrome as the most common mutations involve sodium channel defects (as opposed to potassium channel defects that lead to QTc abnormalities).
Treatment of Brugada syndrome is aimed at prevention of SCD with an automatic implantable cardioverter-defibrillator (AICD); however, that was contraindicated in this patient due to her active infection (2). She was managed acutely with quinidine, the most effective general therapy, with a plan to administer isoproterenol to prevent further episodes of arrhythmia if needed for electrical storm (2). Therapy consisting of hydration, electrolyte replenishment, and antipyretic administration was optimized. In regard to management, there are currently conflicting views of the use of amiodarone and lidocaine in Brugada syndrome and evidence that procainamide should be avoided (2).
Perioperatively, the patient received an infusion of isoproterenol. She underwent an uncomplicated laparoscopic appendectomy, with slow peritoneal insufflation and deflation to avoid increased vagal tone, another trigger for ventricular arrhythmias in the setting of Brugada syndrome.
The patient had no further arrhythmia episodes. Her echocardiogram result was normal. Prior to discharge, an AICD was placed after her infection resolved. Later, she reported that her father had died suddenly at age 45 years. Genetic testing was recommended for her and her first-degree family members.
Rapid diagnosis of Brugada syndrome unmasked by triggers is critical to therapy designed to reverse provoking factors and suppress or terminate lethal episodes of ventricular arrhythmia. This case outlines perioperative management of patients with Brugada syndrome at high risk for SCD, particularly those with an index presentation secondary to acute illness. Following is an overview of Brugada management:
• Acute management of Brugada syndrome includes optimizing hydration, correcting electrolyte abnormalities, and using antipyretics and quinidine.
• A secondary preventative of SCD from type 1 Brugada syndrome following VF arrest involves placement of an AICD.
• For patients undergoing surgery, vigilant planning is required.
• Genetic testing should be recommended to the patient and the patient’s first-degree family members.
The authors have reported that they have no relationships relevant to the contents of this paper to disclose.
- Received April 1, 2019.
- Revision received April 29, 2019.
- Accepted May 2, 2019.
- 2019 The Authors
- Juang J.J.,
- Horie M.
- Antzelevitch C.,
- Fish J.M.