Ann Thorac Surg 2009;88:982-983. doi:10.1016/j.athoracsur.2009.01.066
© 2009 The Society of Thoracic Surgeons
Case Reports
Use of ECMO to Temporize Circulatory Instability During Severe Brugada Electrical Storm
Paul S. Pagel, MD, PhDa,*,
R. Eric Lilly, MDb,
Alfred C. Nicolosi, MDb
a Department of Anesthesiology, Medical College of Wisconsin, Milwaukee, Wisconsin
b Department of Cardiothoracic Surgery, Medical College of Wisconsin, Milwaukee, Wisconsin
Accepted for publication January 27, 2009.
* Address correspondence to Dr Pagel, Clement J. Zablocki Veterans Affairs Medical Center, Anesthesia Service, 5000 W National Ave, Milwaukee, WI 53295 (Email: pspagel{at}mcw.edu).
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Abstract
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We describe the use of extracorporeal membrane oxygenation to temporize circulatory instability during almost incessant ventricular fibrillation in a patient with Brugada syndrome who had electively discontinued chronic amiodarone therapy. The extracorporeal membrane oxygenation was continued for 3 days after emergent delivery of the neonate, during which time the number of ventricular fibrillation episodes and internal defibrillations markedly decreased concomitant with administration of intravenous amiodarone and verapamil. Oral anti-arrhythmic therapy was subsequently reinstituted, and the remainder of the patient's hospital course was uneventful.
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Introduction
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Brugada syndrome (BrS) is an inherited disease with low penetrance autosomal dominant transmission characterized by right bundle-branch block, ST-segment elevation in leads V1 through V3, and increased risk of sudden death without demonstrable structural heart abnormalities [1]. Brugada syndrome results from mutations in the SCN5A gene located on chromosome 3p21 that encodes the alpha subunit of voltage-gated cardiac Na+ channel (Nav1.5) or the glycerol-3-phosphate dehydrogenase 1-like gene that affects trafficking of the channel [2]. The defect produces dysfunctional Nav1.5 current by impairing channel expression and kinetics, and affects the cardiac action potential by causing more pronounced myocyte repolarization at the end of phase 1, thereby contributing to ventricular arrhythmogenesis. An implantable cardioverter-defibrillator (ICD) is the recommended treatment for patients with BrS based on a history of resuscitated sudden cardiac death, syncope, a positive electrophysiology study, or a strong family history of the disease [3]. Many factors may precipitate repetitive ventricular arrhythmias (termed "electrical storm") in patients with BrS, including fever, bradycardia, anti-arrhythmic medications (eg, Na+ channel blockers, flecainide), beta-adrenoceptor antagonists, psychotropic drugs, antihistamines, and electrolyte abnormalities (eg, hypokalemia, hypercalcemia) [1]. Quinidine and isoproterenol are the two major drugs used for pharmacological suppression of BrS electrical storm by restoring action potential morphology and normalizing ST-segment elevation [1]. In this report, we describe the use of extracorporeal membrane oxygenation (ECMO) to temporize circulatory instability during almost incessant ventricular fibrillation (VF) that was refractory to antiarrhythmic therapy in a parturient with BrS.
A 24-year-old gravida 2, para 0, woman with a history of type 1 BrS was admitted to our institution for treatment of accelerated VF concomitant with appropriate ICD firing. The diagnosis of type 1 BrS was established when the patient was 12 years old, after she was resuscitated following a sudden cardiac death episode. An ICD was placed, and the patient was treated with oral amiodarone and verapamil, which successfully suppressed further episodes of VF. The patient suffered a miscarriage 3 years before the current admission that she attributed to chronic amiodarone therapy, and as a result, she decided to electively discontinue this anti-arrhythmic when she became pregnant again. During week 20 of gestation, the patient experienced two episodes of VF associated with ICD discharge that were treated with oral quinidine. An elective VF induction confirmed adequate arrhythmia suppression with quinidine therapy. The patient had no further episodes of VF during the following 4 weeks, but then she received seven ICD shocks for VF on the morning of admission. She was treated with intravenous amiodarone and isoproterenol, but continued to experience progressively more frequent episodes of VF that were often initially refractory to internal defibrillation. She was taken to the electrophysiology laboratory, where ablation of a suspected arrhythmogenic focus within the right ventricular outflow track was unsuccessful. An atrial pacemaker was inserted because atrial pacing seemed to at least temporarily control the ventricular arrhythmias. The ICD generator was also replaced because the battery was nearly depleted. Despite these interventions, the patient experienced accelerating VF and appropriate ICD discharge. She became progressively hypoxemic, requiring endotracheal intubation and mechanical ventilation. Chest compressions were also required on several occasions to maintain systemic and placental perfusion, and because of this remarkable hemodynamic instability, an emergent Cesarean section was performed. The male infant survived and was transported in critical condition to the neonatal intensive care unit. The infant was premature, but subsequently he grew and thrived without apparent adverse sequelae.
The patient continued to experience nearly incessant VF and ICD firing after delivery, requiring intermittent chest compressions because of the ongoing hemodynamic instability. She was brought to the operating room, where transesophageal echocardiography demonstrated normal cardiac structure and function. Intravenous heparin was administered and 17-French and 19-French Bio-Medicus cannulae (Medtronic Inc, Minneapolis, MN) were inserted into the left femoral vein and right femoral artery, respectively, tunneled subcutaneously, and exteriorized on the anterior aspect of each thigh. The ECMO was then instituted at a flow rate of 3.2 L/min with satisfactory flow characteristics. The patient had four episodes of VF during surgery that responded to internal defibrillation. Hemostasis was obtained, the incisions were closed, and the patient was taken to the intensive care unit. The ECMO was continued for 3 days, during which the number of VF episodes and ICD discharges markedly decreased, concomitant with administration of intravenous amiodarone and verapamil. Postpartum hemorrhage was minimal despite anticoagulation, but the patient did require re-exploration of the right groin for evacuation of a large hematoma and repair of a femoral pseudoaneurysm. Oral anti-arrhythmic therapy was eventually reinstituted, the ECMO cannulae were removed, and the remainder of the patient's course was uneventful. She was released from the hospital 1 week after she experienced her final episode of VF and internal defibrillation.
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Comment
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We believe that the current case is the first to report the use of ECMO to provide cardiovascular and respiratory support during electrical storm of VF in a patient with type 1 BrS. The ECMO therapy has been successfully used for circulatory support in patients with refractory malignant ventricular arrhythmias resulting from intrinsic cardiac disease or drug overdose [4]. Emergency ECMO-supported percutaneous coronary interventions were reported in 2 patients with ventricular fibrillation and facilitated successful restoration of normal sinus rhythm and stable hemodynamics [5]. Similarly, ECMO was used to terminate refractory ventricular tachycardia in 11 patients with acute myocarditis, coronary artery spasm, or hypoxemia [6]. Unlike the current case in which hemodynamics and arterial oxygenation were sustained with ECMO for 3 days, ECMO was used for relatively brief periods of time (< 2 hours) in these previous cases [5, 6].
Brugada syndrome is most frequently described in middle-aged men, and this male predominance may be attributed, in part, to the electrophysiological effects of testosterone. Men with BrS experienced more frequent syncopal episodes, had a higher incidence of aborted sudden cardiac death, demonstrated greater ST-segment elevation, and were more likely to sustain inducible VF compared with women [7]. The mechanisms responsible for these gender-related differences remain unclear despite extensive study. Calcium-independent transient outward K+ current (Ito) density was shown to be greater in male canine right ventricular myocardium compared with the female counterpart [8]. This finding may exacerbate abnormal Nav1.5-mediated loss of the epicardial action potential dome, produce greater ST-segment elevation, and favor VF in men with BrS [7]. Notably, estrogen inhibits, whereas testosterone enhances, rapidly inactivating potassium channel (Kv4.3) expression (the major determinant of Ito current), thereby providing a relative protective effect in women. These data suggest that the balance of sex hormones contribute to the male predominance in BrS. Plasma testosterone concentrations increase throughout pregnancy as a result of fetal-placental production and reduced metabolic clearance rate. In the current case, ECMO was chosen instead of a ventricular assist device, because the electrophysiologist opined that that patient's recurrent VF would resolve relatively rapidly after delivery of the infant and placenta because of a reduction in plasma testosterone concentration. Thus, the development of repetitive VF in the current patient with type 1 BrS may have been related not only to her elective discontinuation of amiodarone therapy but also to the adverse electrophysiological effects of increases in plasma testosterone concentration.
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References
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- Antzelevitch C. Brugada syndrome Pacing Clin Electrophysiol 2006;29:1130-1159.[Medline]
- London B, Michalec M, Mehdi H, et al. Mutation in glycerol-3-phosphate dehydrogenase 1 like gene (GPD1-L) decreases cardiac Na+ current and causes inherited arrhythmias Circulation 2007;116:2260-2268.[Abstract/Free Full Text]
- Sarkozy A, Boussy T, Kourgiannides G, et al. Long-term follow-up of primary prophylactic implantable cardioverter-defibrillator therapy in Brugada syndrome Eur Heart J 2007;28:334-344.[Abstract/Free Full Text]
- Cohen MI, Gaynor JW, Ramesh V, et al. Extracorporeal membrane oxygenation for patients with refractory ventricular arrhythmias J Thorac Cardiovasc Surg 1999;118:961-963.[Free Full Text]
- Ricciardi MJ, Moscucci M, Knight BP, Zivin A, Bartlett RH, Bates ER. Emergency extracorporeal membrane oxygenation (ECMO)-supported percutaneous coronary interventions in the fibrillating heart Catheter Cardiovasc Interv 1999;48:402-405.[Medline]
- Tsai FC, Wang YC, Huang YK, et al. Extracorporeal life support to terminate refractory ventricular tachycardia Crit Care Med 2007;35:1673-1676.[Medline]
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- Di Diego JM, Cordeiro JM, Goodrow RJ, et al. Ionic and cellular basis for the predominance of the Brugada syndrome phenotype in males Circulation 2002;106:2004-2011.[Abstract/Free Full Text]
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Abstract
Introduction
