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Thoracoscopic sympathectomy for incessant polymorphic ventricular tachycardia in chronic chagasic myocarditis A case report
International Journal of Cardiology 119 (2007) 255 – 257 www.elsevier.com/locate/ijcard
Letter to the Editor
Thoracoscopic sympathectomy for incessant polymorphic ventricular tachycardia in chronic chagasic myocarditis \ A case report Oswaldo Gutiérrez a,⁎, Elliot Garita b , Carlos Salazar b a
Department of Cardiology, México Hospital, Arrhythmia Service, Universidad de Costa Rica, San José, Costa Rica b Cardiothoracic Surgery Department, Rafael Calderón Guardia Hospital, San José, Costa Rica Received 2 March 2006; received in revised form 27 July 2006; accepted 29 July 2006 Available online 31 October 2006
Abstract Management of complex ventricular arrhythmias due to chagasic myocarditis is challenging. We herein present the case of a young male who was presented with incessant well tolerated polymorphic ventricular tachycardia of unknown cause, who was unresponsive to conventional medical management and as a last resort was submitted to a thoracoscopic bilateral sympathectomy, and showed remarkable improvement in his arrhythmia control. Later on his tests returned positive for Chagas' disease and shortly thereafter he suddenly died. © 2006 Elsevier Ireland Ltd. All rights reserved. Keywords: Chagas disease; Ventricular tachycardia; Thoracoscopic sympathectomy; Chagasic myocarditis
1. Introduction
2. Case report
Chagas' disease is endemic in Central and South America. This chronic parasitic infestation produces dilated myocardiopathy, ventricular aneurysms [1] and arrhythmias. Along with sinus and atrioventricular node dysfunction and supraventricular tachyarrhythmias, chronic myocarditis due to Chagas' disease produces a wide variety of ventricular arrhythmias, ranging from isolated ventricular premature beats without any other evidence of heart disease, to sustained ventricular arrhythmias with severe contractile dysfunction and sudden death. Syncope is frequently present [1,2]. Response to antiarrhythmic drugs is usually poor and the prognosis is guarded. The case of a male with polymorphic incessant ventricular tachycardia (VT) ultimately treated with thoracoscopic sympathectomy is presented.
A 29 year-old farm worker male, had a 2 year history of intermittent paroxysmal palpitations and a syncopal episode 3 months before his hospital admission. He was empirically treated with oral mexiletine with partial symptomatic relief. His family history was positive for sudden death of a 40year-old uncle. His admission EKG is shown in Fig. 1, a polymorphic and bidirectional (V1) VT with a mean heart rate of 160 bpm is seen. This incessant ventricular rhythm showed atrioventricular dissociation and sporadic sinus beats, the QT interval was normal (0.38 s). The patient tolerated well this tachycardia, with a systolic blood pressure fluctuating between 100 and 130 mmHg, his admission serum creatinine was 1.4 mg/dl. An echocardiogram showed mild left ventricular dilatation with moderate impairment of contractile function, initially interpreted as result of this incessant ventricular rhythm. He was started on IVamiodarone and then p.o. atenolol, with no change. The latter one was then replaced by verapamil and no change was observed either. An electrophysiological study was performed, confirming the ventricular origin of his tachycardia, triggered by premature polymorphic ventricular contractions with variable coupling. Catheter ablation was attempted trying
⁎ Corresponding author. Departamento de Cardiología, sección 4-B, Hospital México, San José, Costa Rica, PO Box 471-1300, San José, Costa Rica. Tel.: +506 368 2049; fax: +506 231 3856. E-mail address: [email protected] (O. Gutiérrez). 0167-5273/$ - see front matter © 2006 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.ijcard.2006.07.138
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O. Gutiérrez et al. / International Journal of Cardiology 119 (2007) 255–257
Fig. 1. Twelve lead ECG showing polymorphic bidirectional ventricular tachycardia. This rhythm was hemodinamically well tolerated.
to suppress these triggering beats without success. Different pacing maneuvers were attempted however no therapeutic response was observed. IV adenosine, magnesium sulfate and lidocaine were also tried. As a last resort, bilateral thoracoscopic sympathectomy was undertaken, and the right and left T2, T3
and T4 ganglia were ablated. During the procedure the patient showed runs of normal sinus rhythm alternating with frequent episodes of nonsustained polymorphic VT. Apical pleural adhesions were found in the right side, a left pleural effusion and mild right atrial dilatation were also seen.
Fig. 2. The upper panel shows a 24 h heart rate tachogram, mean value above 100 bpm after admission. After sympathectomy, (lower panel) mean heart rate is 90 bpm. Both measurements were performed under antiarrhythmic drugs (amiodarone and atenolol).
O. Gutiérrez et al. / International Journal of Cardiology 119 (2007) 255–257
After the procedure, he was again started on oral amiodarone and atenolol, reestablishing a normal sinus rhythm, with normal QT interval and isolated premature ventricular beats. Before his hospital discharge, a positive serologic testing for Chagas' disease was reported. We added an ACE inhibitor to his medical therapy and the patient was discharged with only mild palpitations. A Holter study was done revealing a significant fall in sympathetic influence, determined by a decreased mean heart rate after the procedure, compared with a preoperative study (Fig. 2). Eight months after the procedure, he has had frequent unsustained, well tolerated, episodes of polymorphic VT with a mean heart rate of 90 bpm and mild palpitations. One month later, he suddenly died during working. 3. Discussion Even with moderate impairment of contractile function, initially interpreted as secondary to this incessant ventricular rhythm, this case was initially considered as one of polymorphic VT without structural heart disease because of this unusual incessant and bidirectional pattern and his young age. Since the QT interval was repeatedly normal, and there was no typical Brugada sign in the precordial leads and since there was a bidirectional pattern, it was assumed that the picture was secondary to a catecholaminergic ventricular tachycardia until serologic testing results were reported. This case with incessant form of tolerated polymorphic VT represented a therapeutic challenge, considering the high mortality rate of this condition and the relative contraindication to implant a defibrillator if persisted with that pattern. As a last resort and without knowing the definitive diagnosis, a thoracoscopic sympathectomy was undertaken, assuming that an autonomic imbalance contributed to this patient's arrhythmia. Regarding a therapeutic approach, there are only few reports in the literature of this intervention, utilized in patients with long QT syndrome [3]. However, after the procedure, there was a significant decrease in sympathetic activity and the patient showed symptomatic relief. The dysautonomic syndrome [4] in chagasic myocarditis includes postural hypotension, dizziness, fatigue, and triggering of arrhythmias, attributed to immunologic destruction of the cardiac autonomic ganglia and nerves [4,5]. Acting as “a muscarinic agonist,” chagasic IgG could induce desensitization or down-regulation of myocardial cholinergic receptors, or both, and contributes to the expression of a clinical parasympathetic denervation syndrome [6] that could appear before the clinical appearance of substantial myocardial dysfunction. There are circulating autoantibodies against β-1-adrenoreceptors (with agonist-like activity) [7] and M2 muscarinic cholinergic receptors [8], although they are also present in other chronic
257
cardiac conditions [9]. Parasympathetic dysautonomy is an early phenomenon, consequently the cardiac parasympathetic system remains unopposed resulting in cardiotoxic effects [8,9], specially in chagasic patients who, as a consequence of the acute phase, develop a ventricular aneurism of a critical size [9]. Dysfunction of the sympathetic nervous system occurs during the later stages of the disease, resulting in lower plasma norepinephrine levels in contrast to other advanced heart failure conditions [7]. There is also α-adrenoreceptors blockade that explains impairment of postural reflexes. Disautonomy can be an important determinant of triggering and maintenance of ventricular arrhythmias in chagasic patients; thoracoscopic sympathectomy might be more likely successful in this particular condition as compared to other causes of incessant VT and could give a prognosis benefit regarding the pathophysiology of heart failure and sudden cardiac death, along with other therapies, i.e. an implantable defibrillator. Other reported antiarrhythmic approaches in drug-unresponsive ventricular arrhythmias in this condition are aneurysmectomy and ethanol chemical ablation [10]. References [1] de Paola AAV, Horowitz LN, Miyamoto MH, et al. Angiographic and electrophysiologic substrates of ventricular tachycardia in chronic chagasic myocarditis. Am J Cardiol 1990;65:360–3. [2] Acquatella H, Mattia AR. A native of El Salvador with tachycardia and syncope. N Engl J Med 1993;329:488–96. [3] Turley AJ, Thambyrajah J, Harcombe AA. Bilateral thoracoscopic cervical sympathectomy for the treatment of recurrent polymorphic ventricular tachycardia. Heart 2005;91(1):15–7. [4] Iosa D, Massari DC, Dorsey FC. Chagas' cardioneuropathy: effect of ganglioside treatment in chronic dysautonomic patients-a randomized, double-blind, parallel, placebo-controlled study. Am Heart J 1991;122: 775–85. [5] Gorelik G, Genaro AM, Sterin-Borda L, Gonzalez Cappa S, Borda ES. Antibodies bind and activate beta adrenergic and cholinergic lymphocyte receptors in Chagas' disease. Clin Immunol Immunopathol 1990;55:221–36. [6] Sterin-Borda L, Gorelik G, Borda ES. Chagasic IgG binding with cardiac muscarinic cholinergic receptors modifies cholinergic-mediated cellular transmembrane signals. Clin Immunol Immunopathol 1991;61:387–97. [7] Lo Presti MS, Bustamante JM, Rivarola WH, et al. Changes in the cardiac beta-adrenergic system provoked by different T. cruzi strains. Int J Cardiol in press [Electronic publication ahead of print]. [8] Punukollu G, Gowda RM, Khan IA, Navarro VS, Vasavada BC. Clinical aspects of the Chagas' heart disease. Int J Cardiol in press [Electronic publication ahead of print]. [9] Davila DF, Donis JH, Torres A, Ferrer JA. A modified and unifying neurogenic hypothesis can explain the natural history of chronic Chagas heart disease. Int J Cardiol 2004;96:191–5. [10] de Paola AAV, Gomes JA, Miyamoto MH, Fo EEM. Transcoronary chemical ablation of ventricular tachycardia in chronic chagasic myocarditis. J Am Coll Cardiol 1992;20:480–2.
Letter to the Editor
Thoracoscopic sympathectomy for incessant polymorphic ventricular tachycardia in chronic chagasic myocarditis \ A case report Oswaldo Gutiérrez a,⁎, Elliot Garita b , Carlos Salazar b a
Department of Cardiology, México Hospital, Arrhythmia Service, Universidad de Costa Rica, San José, Costa Rica b Cardiothoracic Surgery Department, Rafael Calderón Guardia Hospital, San José, Costa Rica Received 2 March 2006; received in revised form 27 July 2006; accepted 29 July 2006 Available online 31 October 2006
Abstract Management of complex ventricular arrhythmias due to chagasic myocarditis is challenging. We herein present the case of a young male who was presented with incessant well tolerated polymorphic ventricular tachycardia of unknown cause, who was unresponsive to conventional medical management and as a last resort was submitted to a thoracoscopic bilateral sympathectomy, and showed remarkable improvement in his arrhythmia control. Later on his tests returned positive for Chagas' disease and shortly thereafter he suddenly died. © 2006 Elsevier Ireland Ltd. All rights reserved. Keywords: Chagas disease; Ventricular tachycardia; Thoracoscopic sympathectomy; Chagasic myocarditis
1. Introduction
2. Case report
Chagas' disease is endemic in Central and South America. This chronic parasitic infestation produces dilated myocardiopathy, ventricular aneurysms [1] and arrhythmias. Along with sinus and atrioventricular node dysfunction and supraventricular tachyarrhythmias, chronic myocarditis due to Chagas' disease produces a wide variety of ventricular arrhythmias, ranging from isolated ventricular premature beats without any other evidence of heart disease, to sustained ventricular arrhythmias with severe contractile dysfunction and sudden death. Syncope is frequently present [1,2]. Response to antiarrhythmic drugs is usually poor and the prognosis is guarded. The case of a male with polymorphic incessant ventricular tachycardia (VT) ultimately treated with thoracoscopic sympathectomy is presented.
A 29 year-old farm worker male, had a 2 year history of intermittent paroxysmal palpitations and a syncopal episode 3 months before his hospital admission. He was empirically treated with oral mexiletine with partial symptomatic relief. His family history was positive for sudden death of a 40year-old uncle. His admission EKG is shown in Fig. 1, a polymorphic and bidirectional (V1) VT with a mean heart rate of 160 bpm is seen. This incessant ventricular rhythm showed atrioventricular dissociation and sporadic sinus beats, the QT interval was normal (0.38 s). The patient tolerated well this tachycardia, with a systolic blood pressure fluctuating between 100 and 130 mmHg, his admission serum creatinine was 1.4 mg/dl. An echocardiogram showed mild left ventricular dilatation with moderate impairment of contractile function, initially interpreted as result of this incessant ventricular rhythm. He was started on IVamiodarone and then p.o. atenolol, with no change. The latter one was then replaced by verapamil and no change was observed either. An electrophysiological study was performed, confirming the ventricular origin of his tachycardia, triggered by premature polymorphic ventricular contractions with variable coupling. Catheter ablation was attempted trying
⁎ Corresponding author. Departamento de Cardiología, sección 4-B, Hospital México, San José, Costa Rica, PO Box 471-1300, San José, Costa Rica. Tel.: +506 368 2049; fax: +506 231 3856. E-mail address: [email protected] (O. Gutiérrez). 0167-5273/$ - see front matter © 2006 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.ijcard.2006.07.138
256
O. Gutiérrez et al. / International Journal of Cardiology 119 (2007) 255–257
Fig. 1. Twelve lead ECG showing polymorphic bidirectional ventricular tachycardia. This rhythm was hemodinamically well tolerated.
to suppress these triggering beats without success. Different pacing maneuvers were attempted however no therapeutic response was observed. IV adenosine, magnesium sulfate and lidocaine were also tried. As a last resort, bilateral thoracoscopic sympathectomy was undertaken, and the right and left T2, T3
and T4 ganglia were ablated. During the procedure the patient showed runs of normal sinus rhythm alternating with frequent episodes of nonsustained polymorphic VT. Apical pleural adhesions were found in the right side, a left pleural effusion and mild right atrial dilatation were also seen.
Fig. 2. The upper panel shows a 24 h heart rate tachogram, mean value above 100 bpm after admission. After sympathectomy, (lower panel) mean heart rate is 90 bpm. Both measurements were performed under antiarrhythmic drugs (amiodarone and atenolol).
O. Gutiérrez et al. / International Journal of Cardiology 119 (2007) 255–257
After the procedure, he was again started on oral amiodarone and atenolol, reestablishing a normal sinus rhythm, with normal QT interval and isolated premature ventricular beats. Before his hospital discharge, a positive serologic testing for Chagas' disease was reported. We added an ACE inhibitor to his medical therapy and the patient was discharged with only mild palpitations. A Holter study was done revealing a significant fall in sympathetic influence, determined by a decreased mean heart rate after the procedure, compared with a preoperative study (Fig. 2). Eight months after the procedure, he has had frequent unsustained, well tolerated, episodes of polymorphic VT with a mean heart rate of 90 bpm and mild palpitations. One month later, he suddenly died during working. 3. Discussion Even with moderate impairment of contractile function, initially interpreted as secondary to this incessant ventricular rhythm, this case was initially considered as one of polymorphic VT without structural heart disease because of this unusual incessant and bidirectional pattern and his young age. Since the QT interval was repeatedly normal, and there was no typical Brugada sign in the precordial leads and since there was a bidirectional pattern, it was assumed that the picture was secondary to a catecholaminergic ventricular tachycardia until serologic testing results were reported. This case with incessant form of tolerated polymorphic VT represented a therapeutic challenge, considering the high mortality rate of this condition and the relative contraindication to implant a defibrillator if persisted with that pattern. As a last resort and without knowing the definitive diagnosis, a thoracoscopic sympathectomy was undertaken, assuming that an autonomic imbalance contributed to this patient's arrhythmia. Regarding a therapeutic approach, there are only few reports in the literature of this intervention, utilized in patients with long QT syndrome [3]. However, after the procedure, there was a significant decrease in sympathetic activity and the patient showed symptomatic relief. The dysautonomic syndrome [4] in chagasic myocarditis includes postural hypotension, dizziness, fatigue, and triggering of arrhythmias, attributed to immunologic destruction of the cardiac autonomic ganglia and nerves [4,5]. Acting as “a muscarinic agonist,” chagasic IgG could induce desensitization or down-regulation of myocardial cholinergic receptors, or both, and contributes to the expression of a clinical parasympathetic denervation syndrome [6] that could appear before the clinical appearance of substantial myocardial dysfunction. There are circulating autoantibodies against β-1-adrenoreceptors (with agonist-like activity) [7] and M2 muscarinic cholinergic receptors [8], although they are also present in other chronic
257
cardiac conditions [9]. Parasympathetic dysautonomy is an early phenomenon, consequently the cardiac parasympathetic system remains unopposed resulting in cardiotoxic effects [8,9], specially in chagasic patients who, as a consequence of the acute phase, develop a ventricular aneurism of a critical size [9]. Dysfunction of the sympathetic nervous system occurs during the later stages of the disease, resulting in lower plasma norepinephrine levels in contrast to other advanced heart failure conditions [7]. There is also α-adrenoreceptors blockade that explains impairment of postural reflexes. Disautonomy can be an important determinant of triggering and maintenance of ventricular arrhythmias in chagasic patients; thoracoscopic sympathectomy might be more likely successful in this particular condition as compared to other causes of incessant VT and could give a prognosis benefit regarding the pathophysiology of heart failure and sudden cardiac death, along with other therapies, i.e. an implantable defibrillator. Other reported antiarrhythmic approaches in drug-unresponsive ventricular arrhythmias in this condition are aneurysmectomy and ethanol chemical ablation [10]. References [1] de Paola AAV, Horowitz LN, Miyamoto MH, et al. Angiographic and electrophysiologic substrates of ventricular tachycardia in chronic chagasic myocarditis. Am J Cardiol 1990;65:360–3. [2] Acquatella H, Mattia AR. A native of El Salvador with tachycardia and syncope. N Engl J Med 1993;329:488–96. [3] Turley AJ, Thambyrajah J, Harcombe AA. Bilateral thoracoscopic cervical sympathectomy for the treatment of recurrent polymorphic ventricular tachycardia. Heart 2005;91(1):15–7. [4] Iosa D, Massari DC, Dorsey FC. Chagas' cardioneuropathy: effect of ganglioside treatment in chronic dysautonomic patients-a randomized, double-blind, parallel, placebo-controlled study. Am Heart J 1991;122: 775–85. [5] Gorelik G, Genaro AM, Sterin-Borda L, Gonzalez Cappa S, Borda ES. Antibodies bind and activate beta adrenergic and cholinergic lymphocyte receptors in Chagas' disease. Clin Immunol Immunopathol 1990;55:221–36. [6] Sterin-Borda L, Gorelik G, Borda ES. Chagasic IgG binding with cardiac muscarinic cholinergic receptors modifies cholinergic-mediated cellular transmembrane signals. Clin Immunol Immunopathol 1991;61:387–97. [7] Lo Presti MS, Bustamante JM, Rivarola WH, et al. Changes in the cardiac beta-adrenergic system provoked by different T. cruzi strains. Int J Cardiol in press [Electronic publication ahead of print]. [8] Punukollu G, Gowda RM, Khan IA, Navarro VS, Vasavada BC. Clinical aspects of the Chagas' heart disease. Int J Cardiol in press [Electronic publication ahead of print]. [9] Davila DF, Donis JH, Torres A, Ferrer JA. A modified and unifying neurogenic hypothesis can explain the natural history of chronic Chagas heart disease. Int J Cardiol 2004;96:191–5. [10] de Paola AAV, Gomes JA, Miyamoto MH, Fo EEM. Transcoronary chemical ablation of ventricular tachycardia in chronic chagasic myocarditis. J Am Coll Cardiol 1992;20:480–2.