Brugada syndrome treatment
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Implantation of a cardiac defibrillator is the only proven method of treatment in Brugada syndrome.Patients with aborted sudden cardiac death are at high risk for recurrence and should undergo AICD implantation, and do not require an electrophysiologic study to assess inducibility. Patients with symptoms (either syncope, seizures or nocturnal agonal respirations) should undergo implantation of a defibrillator if no other cause of their symptoms can be identified. Asymptomatic patients should undergo electrophysiologic testing, and if VT / VF can be induced, they should undergo implantation of an ICD. Asymptomatic patients who cannot be induced should followed-up closely. Patients who are asymptomatic with no family history of Brugada syndrome can be followed-up closely.
The Two Patient Groups
The 2005 consensus statement divides patients into two groups:
- Higher risk patients with spontaneous Type I Brugada pattern
- A less high risk cohort of patients who require infusion of a sodium channel blocker to induce a Type I Brugada pattern.
The management of these two groups of patients will be discussed separately.
Management of Patients with a Spontaneous Type I Brugada Pattern
Implantation of a cardiac defibrillator should be considered in the following patients:
Symptomatic Patients
- Patients with aborted sudden cardiac death
- Patients with syncope, seizures or nocturnal agonal respirations who have no other identifiable cause for their symptoms
The flowchart below summarizes the recommendations of the 2005 consensus panel:
Asymptomatic Patients
- Patients with a family history of sudden cardiac death that is suspected to be due to Brugada syndrome in whom VT VF can be induced on electrophysiologic testing.
- Patients with no family history of sudden cardiac death in whom VT VF can be induced on electrophysiologic testing.
In essence, if VT VF can be induced on electrophysiologic testing in these patients, a cardiac defibrillator should be implanted. It is unclear if the same recommendations apply to those patients who require that the electrodes be placed one to two intercostal spaces higher to demonstrate a Brugada type I electrocardiographic pattern.
The flowchart below summarizes the recommendations of the 2005 consensus panel:
Management of Patients with a Sodium Channel Induced Type I Brugada Pattern
Implantation of a cardiac defibrillator should be considered in the following patients:
Symptomatic Patients
- Patients with aborted sudden cardiac death
- Patients with syncope, seizures or nocturnal agonal respirations who have no other identifiable cause for their symptoms
The flowchart below summarizes the recommendations of the 2005 consensus panel:
Asymptomatic Patients
- Patients with a family history of sudden cardiac death that is suspected to be due to Brugada syndrome in whom VT VF can be induced on electrophysiologic testing.
The flowchart below summarizes the recommendations of the 2005 consensus panel.
Pharmacotherapy
- Pharmacotherapy alone may not be sufficient to treat Brugada syndrome, but it may be required in regions of the world where ICD implantation is cost prohibitive or in infants.[1]
- Quinidine reduces the number of VF episodes and corrects spontaneous ECG changes, possibly via inhibiting I(to) channels. No drug has demonstrated long term efficacy in the prevention of sudden cardiac death.[2][3][4][5][6][7]
- Pharmacological tests using a sodium-channel blocker drug should be used to evaluate suspected Brugada syndrome. Intravenous ajmaline and flecainide are the most widely used agents. The test is considered positive if a type 1 ECG pattern is identified during drug infusion.
Drugs with Potential Antiarrhythmic Effect
(Alphabetical order generic name)
| Generic name | Brand name® | Class / Clinical use | References | Recommendation |
| Cilostazol | e.g. Pletal® |
Phosphodiesterase inhibitor | Tsuchiya 2002 Abud 2006 Matsui 1999 |
Class IIb |
| Isoproterenol Isoprenaline |
e.g. Isuprel® |
Beta-adrenergic receptor stimulation | Miyazaki 1996 Suzuki 2000 Watanabe 2006 Ohgo 2007 Ganesan 2006 |
Class I |
| Orciprenaline | e.g. Alotec® Metaprel® Novasmasol® |
Beta-adrenergic receptor stimulation | Kyriazis 2009 | Class IIa |
| Quinidine | e.g. Quinalan® Chinidin® |
Antiarrhythmic Agent | Suzuki 2000 Alings 2001 Belhassen 2004 Mizusawa 2006 Probst 2007 Ohgo 2007 Yan 1999 |
Class I |
- Recommendation: Class I: convincing evidence/opinion; Class IIa: evidence/opinion less clear; Class IIb: conflicting evidence/opinion; Class III: very little evidence.
Treatment of VT Storm
- VT storm has been successfully treated with isoproterenol. The mechanism is thought to be augmenting the cardiac L type channel.
Treatment of Coronary Ischemia
- Patients with risk factors for coronary artery disease may require an angiogram before ICD implantation.
Treatment of Factors that may Precipitate Brugada Type EKG Changes and Clinical Symptoms
- Fever in a Brugada syndrome patient should be treated with an antipyretic.
- Brugada syndrome patients should avoid hot tubs, very hot baths or extremely hot climates.
- Hypokalemia, hyperkalemia, and hypercalcemia should be treated aggressively.
- Carbohydrate loading should be avoided.
Contraindicated medications
Brugada syndrome is considered an absolute contraindication to the use of the following medications:
ACC/AHA/ESC 2006 Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death (DO NOT EDIT) [8]
Recommendations for Brugada Syndrome
| Class I |
| Suggested phrases for writing recommendations:
-1. In asymptomatic patients with only inducible type 1 Brugada electrocardiographic pattern, observation without therapy is recommended 2. In patients with Brugada syndrome with spontaneous type 1 Brugada electrocardiographic pattern and cardiac arrest, sustained VA or a recent history of syncope presumed due to VA, an ICD is recommended if meaningful survival of greater than 1 year is expected. 3. In patients with Brugada syndrome experiencing recurrent ICD shocks for polymorphic VT, intensification of therapy with quinidine or catheter ablation is recommended 4. In patients with spontaneous type 1 Brugada electrocardiographic pattern and symptomatic VA who either are not candidates for or decline an ICD, quinidine or catheter ablation is recommended |
| Class IIa |
| In patients with suspected long QT syndrome, ambulatory electrocardiographic monitoring, recording the ECG lying and immediately on standing, and/or exercise treadmill testing can be useful for establishing a diagnosis and monitoring the response to therapy. |
| "1. An ICD is reasonable for Brugada syndrome patients with spontaneous ST-segment elevation in V1, V2, or V3 who have had syncope with or without mutations demonstrated in the SCN5A gene and who have reasonable expectation of survival with a good functional status for more than 1 y. (Level of Evidence: C)" |
| "2. Clinical monitoring for the development of a spontaneous ST-segment elevation pattern is reasonable for the management of patients with ST-segment elevation induced only with provocative pharmacological challenge with or without symptoms. (Level of Evidence: C)" |
| "3. An ICD is reasonable for Brugada syndrome patients with documented VT that has not resulted in cardiac arrest and who have reasonable expectation of survival with a good functional status for more than 1 y. (Level of Evidence: C)" |
| "4. Isoproterenol can be useful to treat an electrical storm in the Brugada syndrome. (Level of Evidence: C)" |
| Class IIb |
| In patients with asymptomatic Brugada syndrome and a spontaneous type 1 Brugada electrocardiographic pattern, an electrophysiological study with programmed ventricular stimulation using single and double extrastimuli may be considered for further risk stratification. |
| In patients with suspected or established Brugada syndrome, genetic counseling and genetic testing may be useful to facilitate cascade screening of relatives |
| "1. EP testing may be considered for risk stratification in asymptomatic Brugada syndrome patients with spontaneous ST elevation with or without a mutation in the SCN5A gene. (Level of Evidence: C)" |
| "2. Quinidine might be reasonable for the treatment of electrical storm in patients with Brugada syndrome.(Level of Evidence: C)" |
|issue=41 |pages=2873–926 |date=November 2014 |pmid=25173340 |doi=10.1093/eurheartj/ehu281 |url=}}</ref>===
| Recomendacations | Class | Level | References |
|---|---|---|---|
| History and clinical assessment | |||
| In all patients with suspected AAS, pre-test probability assessment is recommended, according to the patient’s condition, symptoms, and clinical features. | I | B | [9] |
| Laboratory testing | |||
| In case of suspicion of AAS, the interpretation of biomarkers should always be considered along with the pretest clinical probability. | IIa | B | |
| In case of low clinical probability of AAS, negative D-dimer levels should be considered as ruling out the diagnosis. | IIa | C | [10][11][12][13] |
| In case of intermediate clinical probability of AAS with a positive (point-of-care) D-dimer test, further imaging tests should be considered. | IIa | B | [10][11] |
| In patients with high probability (risk score 2 or 3) of AD, testing of D-dimers is not recommended. | III | C | |
| Imaging | |||
| TTE is recommended as an initial imaging investigation. | I | C | |
| In unstabled patients with a suspicion of AAS, the following imaging modalities are recommended according to local availability and expertise: | |||
| TOE | I | C | |
| CT | I | C | |
| In stable patients with a suspicion of AAS, the following imaging modalities are recommended (or should be considered) according to local availability and expertise: | |||
| CT | I | C | |
| MRI | I | C | |
| TOE | IIa | C | |
| In case of initially negative imaging with the persistence of suspicion of AAS, repetitive imaging (CT or MRI) is recommended. | I | C | |
| Chest X-ray maybe considered in cases of low clinical probability of AAS. | IIb | C | |
| In case of uncomplicated Type B AD treated medically, repeated imaging (CT or MRI)e during the first days is recommended. | I | C | |
References
- ↑ Al-Khatib, Sana M.; Stevenson, William G.; Ackerman, Michael J.; Bryant, William J.; Callans, David J.; Curtis, Anne B.; Deal, Barbara J.; Dickfeld, Timm; Field, Michael E.; Fonarow, Gregg C.; Gillis, Anne M.; Granger, Christopher B.; Hammill, Stephen C.; Hlatky, Mark A.; Joglar, José A.; Kay, G. Neal; Matlock, Daniel D.; Myerburg, Robert J.; Page, Richard L. (2018). "2017 AHA/ACC/HRS Guideline for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death". Circulation. 138 (13). doi:10.1161/CIR.0000000000000549. ISSN 0009-7322.
- ↑ Belhassen B, Glick A, Viskin S (2004). "Efficacy of quinidine in high-risk patients with Brugada syndrome". Circulation. 110 (13): 1731–7. doi:10.1161/01.CIR.0000143159.30585.90. PMID 15381640.
- ↑ Tsuchiya, Takeshi; Ashikaga, Keiichi; Honda, Toshihiro; Arita, Makoto (2002). "Prevention of Ventricular Fibrillation by Cilostazol, an Oral Phosphodiesterase Inhibitor, in a Patient with Brugada Syndrome". Journal of Cardiovascular Electrophysiology. 13 (7): 698–701. doi:10.1046/j.1540-8167.2002.00698.x. ISSN 1045-3873.
- ↑ Abud, Atilio; Bagattin, Daniel; Goyeneche, Raul; Becker, Carlos (2006). "Failure of Cilostazol in the Prevention of Ventricular Fibrillation in a Patient with Brugada Syndrome". Journal of Cardiovascular Electrophysiology. 17 (2): 210–212. doi:10.1111/j.1540-8167.2005.00290.x. ISSN 1045-3873.
- ↑ Matsui, Kazunori; Kiyosue, Tatsuto; Wang, Jin-Cheng; Dohi, Kazuhiro; Arita, Makoto (1999). Cardiovascular Drugs and Therapy. 13 (2): 105–113. doi:10.1023/A:1007779908346. ISSN 0920-3206. Missing or empty
|title=(help) - ↑ Miyazaki, Toshihisa; Mitamura, Hideo; Miyoshi, Shunichiro; Soejima, Kyoko; Aizawa, Yoshifusa; Ogawa, Satoshi (1996). "Autonomic and antiarrhythmic drug modulation of ST segment elevation in patients with Brugada syndrome". Journal of the American College of Cardiology. 27 (5): 1061–1070. doi:10.1016/0735-1097(95)00613-3. ISSN 0735-1097.
- ↑ Suzuki, Hiroshi; Torigoe, Katsumi; Numata, Osamu; Yazaki, Satoshi (2000). "Infant Case with a Malignant Form of Brugada Syndrome". Journal of Cardiovascular Electrophysiology. 11 (11): 1277–1280. doi:10.1046/j.1540-8167.2000.01277.x. ISSN 1045-3873.
- ↑ Zipes DP, Camm AJ, Borggrefe M, Buxton AE, Chaitman B, Fromer M; et al. (2006). "ACC/AHA/ESC 2006 Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death: a report of the American College of Cardiology/American Heart Association Task Force and the European Society of Cardiology Committee for Practice Guidelines (writing committee to develop Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death): developed in collaboration with the European Heart Rhythm Association and the Heart Rhythm Society". Circulation. 114 (10): e385–484. doi:10.1161/CIRCULATIONAHA.106.178233. PMID 16935995.
- ↑ Evangelista, Arturo; Isselbacher, Eric M.; Bossone, Eduardo; Gleason, Thomas G.; Eusanio, Marco Di; Sechtem, Udo; Ehrlich, Marek P.; Trimarchi, Santi; Braverman, Alan C.; Myrmel, Truls; Harris, Kevin M.; Hutchinson, Stuart; O’Gara, Patrick; Suzuki, Toru; Nienaber, Christoph A.; Eagle, Kim A. (2018). "Insights From the International Registry of Acute Aortic Dissection". Circulation. 137 (17): 1846–1860. doi:10.1161/CIRCULATIONAHA.117.031264. ISSN 0009-7322.
- ↑ 10.0 10.1 Eggebrecht, Holger; Mehta, Rajendra H.; Metozounve, Huguette; Huptas, Sebastian; Herold, Ulf; Jakob, Heinz G.; Erbel, Raimund (2008). "Clinical Implications of Systemic Inflammatory Response Syndrome Following Thoracic Aortic Stent-Graft Placement". Journal of Endovascular Therapy. 15 (2): 135–143. doi:10.1583/07-2284.1. ISSN 1526-6028.
- ↑ 11.0 11.1 Sutherland, Alexander; Escano, Jude; Coon, Troy P. (2008). "D-dimer as the Sole Screening Test for Acute Aortic Dissection: A Review of the Literature". Annals of Emergency Medicine. 52 (4): 339–343. doi:10.1016/j.annemergmed.2007.12.026. ISSN 0196-0644.
- ↑ Suzuki, Toru; Bossone, Eduardo; Sawaki, Daigo; Jánosi, Rolf Alexander; Erbel, Raimund; Eagle, Kim; Nagai, Ryozo (2013). "Biomarkers of aortic diseases". American Heart Journal. 165 (1): 15–25. doi:10.1016/j.ahj.2012.10.006. ISSN 0002-8703.
- ↑ Taylor, R. Andrew; Iyer, Neel S. (2013). "A decision analysis to determine a testing threshold for computed tomographic angiography and d-dimer in the evaluation of aortic dissection". The American Journal of Emergency Medicine. 31 (7): 1047–1055. doi:10.1016/j.ajem.2013.03.039. ISSN 0735-6757.