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Segmental dyskinesia in Wolff–Parkinson–White syndrome: A possible cause of dilatative cardiomyopathy
International Journal of Cardiology 123 (2008) e31 – e34 www.elsevier.com/locate/ijcard
Letter to the Editor
Segmental dyskinesia in Wolff–Parkinson–White syndrome: A possible cause of dilatative cardiomyopathy Giovanni Fazio ⁎, Maurizio Mongiovi′, Loredana Sutera, Giuseppina Novo, Salvatore Novo, Salvatore Pipitone Department of Cardiology, Casa del Sole Hospital, Palermo, Italy Department of Cardiology, University of Palermo, Italy Received 29 August 2006; accepted 11 November 2006 Available online 12 February 2007
Abstract Wolff–Parkinson–White (WPW) is a syndrome characterized by the presence of an accessory pathway that skipping A–V node may lead the electrical stimulus from the atrium directly to the ventricle. Some studies reported the finding of myocardial dyskinesia in the segments precociously activated by the accessory pathway, at echocardiogram and at nuclear cardiac study. Soria et al. reported, in 1985, an increased incidence of dilative cardiomyopathy in patients with WPW. The pathophysiological pathway that leads to ventricular dilation may be due to the increase of end-diastolic pressure secondary to a tachycardia-induced cardiomyopathy. Tachycardia-induced cardiomyopathy is usually secondary to frequent and prolonged tachycardia episodes. In this paper we report the cases of three patients affected by WPW who developed dilative cardiomyopathy during the follow-up. Particularly dyskinetic segments, working such as a functional aneurysm, could induce deep modifications of intraventricular haemodynamics, leading to remodelling and progressive ventricular dilation. This hypothesis could have important empirical consequences because it could imply the necessity of a precocious ablative therapy in this kind of patients. © 2007 Elsevier Ireland Ltd. All rights reserved. Keywords: WPW; Dyskinetic segments; Echocardiogram findings; Remodelling; Dilatative cardiomyopathy
Wolff–Parkinson–White (WPW) is a syndrome characterized by the presence of an accessory pathway that skipping A–V node may lead the electrical stimulus from the atrium directly to the ventricle [1,2]. Some studies reported the finding of myocardial dyskinesia in the segments precociously activated by the accessory pathway, at echocardiogram [3] and at nuclear cardiac study [4]. It is not well known which are long time effects of this kinesis impairment. Soria et al. reported, in 1985, an increased incidence of dilative cardiomyopathy in patients with WPW [5]. The pathophysiological pathway that leads to ventricular dilation may be due to the increase of end-
⁎ Corresponding author. Via santa Maria di Gesu' 25, 90124, Italy. E-mail address: [email protected] (G. Fazio). 0167-5273/$ - see front matter © 2007 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.ijcard.2006.11.109
diastolic pressure secondary to a tachycardia-induced cardiomyopathy [6]. Tachycardia-induced cardiomyopathy is usually secondary to frequent and prolonged tachycardia episodes [7]. In this paper we report the cases of the three patients affected by WPW who developed dilative cardiomyopathy during the follow-up [8]. On admission patients' mean age was 5.8 years old (respectively 1, 6 and 10 years old). ECG of every patient showed the presence of an accessory pathway (antero-septal in two cases and anterior in the last). Echocardiogram documented a structurally normal heart but with an evident dyskinesia of either anterior or posterior interventricular basal septum. Patients were followed-up for a mean time of 4.3 years (respectively 2, 5 and 6 years). Two of them never complained palpitations, and periodical ECG Holter control never showed tachycardia episodes. The third
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Fig. 1. An echocardiogram of patient 1 at the first control is shown in panel A. This frame shows a 4-chamber view, where several left ventricular aneurysms are present. The ECG shows an anterior accessory pathway (B panel).
patient, at the age of 8, complained some palpitations, and at ECG Holter monitoring, some episodes of paroxysmal supraventricular tachycardia (PSVT), unresponsive to medical treatment, were documented. PSVT had a maximum duration of 2 min and caused the child's weakness and haemodynamic instability. During this period, in the absence of the arrhythmia, an echocardiogram was performed that showed a mildly dilated left ventricle with a mildly reduced systolic function. After ineffective therapy with propafenone first and amiodarone later, in a six months time, catheter ablation was performed. After the procedure, the patient was completely asymptomatic. During the follow-up all the patients developed a dilative cardiomyopathy. Because the tachycardia-induced mechanism could not explain this relief we hypothesised that dyskinesia could be involved in pathophysiology of this form of cardiomyopathy. Particularly dyskinetic segments, working such as a functional
aneurysm, could induce deep modifications of intraventricular haemodynamics, leading to remodelling and progressive ventricular dilation. This hypothesis could have important empirical consequences because it could imply the necessity of a precocious ablative therapy in this kind of patients. In fact, the ablation of the accessory pathway performed in our patients after showing initial signs of left ventricular dysfunction did not elude the restoration of a normal LV function nor prevented the development of dilative cardiomyopathy. However this suggestive hypothesis needs to be confirmed by further studies (Figs. 1, 2, and 3). References [1] Lebovitz JA, Mandel WJ, Laks MM, Kraus R, Weinstein S. Relationship between the electrical (electrocardiographic) and mechanical (echocardiographic) events in Wolff–Parkinson–White syndrome. Chest 1977;7:463–9.
G. Fazio et al. / International Journal of Cardiology 123 (2008) e31–e34 [2] Ticzon AR, Damato AN, Caracta AR, Russo G, Foster JR, Lau SH. Interventricular septal motion during preexcitation and normal conduction in Wolff–Parkinson–White syndrome: echocardiographic and electrophysiologic correlation. Am J Cardiol 1976;37:840–7. [3] DeMaria AN, Vera Z, Neumann A, Mason DT. Alterations in ventricular contraction pattern in the Wolff–Parkinson–White syndrome. Detection by echocardiography. Circulation 1976;53:249–57. [4] Botvinick EH, Frais MA, Shosa DW, et al. An accurate means of detecting and characterizing abnormal patterns of ventricular activation by phase image analysis. Am J Cardiol 1982;50:289–98.
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[5] Soria R, Fernandez F, Heller J, et al. Wolff–Parkinson–White syndrome and cardiopathies. Arch Mal Coeur Vaiss 1984;77:1468–80. [6] Otsuji Y, Osame M, Tei C, et al. Cardiac involvement in congenital myopathy. Int J Cardiol 1985;9:311–22. [7] Markhasin VS, Glasman AA, Chestukhin VV, Gol'dberg SI, Katsnel'son LB, Makhanek AO. The contribution of the myocardial segmental nonhomogeneity of the left ventricular walls to its contractile and pumping functions. Fiziol Z Im I M Sechenova 1994;80(4):72–80. [8] Emmel Mathis, Balaji Seshadri, Sreeram Narayanswami. Ventricular preexcitation associated with dilated cardiomyopathy: a causal relationship? Cardiol Young 2004;14(6):594–9.
Fig. 2. Echocardiogram of patient 2. A and B panels show a telediastolic frames captured at the first observation and after 5 years of follow-up. C and D panel show a telesystolic frames captured at the same time. All panels show an aneurysm of basal segments of interventricular septum. A left ventricular dilatation was present only in B and D panels, captured after 6 years of follow-up. In the E panel we observe the ECG that shows an antero-septal pathway.
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Fig. 3. Echocardiogram of patient 3 at the beginning of the follow-up and after 4 years. The frames A and B show a 4-chamber view of the left ventricle, with an aneurysm of the basal segments and without a dilatation of the chambers. The same aneurysm is represented in a 4-chamber view and in M-Mode analysis in the C and D frames: here we can observe a dilatation of the left ventricle. All reported frames are telesystolic frames.
Letter to the Editor
Segmental dyskinesia in Wolff–Parkinson–White syndrome: A possible cause of dilatative cardiomyopathy Giovanni Fazio ⁎, Maurizio Mongiovi′, Loredana Sutera, Giuseppina Novo, Salvatore Novo, Salvatore Pipitone Department of Cardiology, Casa del Sole Hospital, Palermo, Italy Department of Cardiology, University of Palermo, Italy Received 29 August 2006; accepted 11 November 2006 Available online 12 February 2007
Abstract Wolff–Parkinson–White (WPW) is a syndrome characterized by the presence of an accessory pathway that skipping A–V node may lead the electrical stimulus from the atrium directly to the ventricle. Some studies reported the finding of myocardial dyskinesia in the segments precociously activated by the accessory pathway, at echocardiogram and at nuclear cardiac study. Soria et al. reported, in 1985, an increased incidence of dilative cardiomyopathy in patients with WPW. The pathophysiological pathway that leads to ventricular dilation may be due to the increase of end-diastolic pressure secondary to a tachycardia-induced cardiomyopathy. Tachycardia-induced cardiomyopathy is usually secondary to frequent and prolonged tachycardia episodes. In this paper we report the cases of three patients affected by WPW who developed dilative cardiomyopathy during the follow-up. Particularly dyskinetic segments, working such as a functional aneurysm, could induce deep modifications of intraventricular haemodynamics, leading to remodelling and progressive ventricular dilation. This hypothesis could have important empirical consequences because it could imply the necessity of a precocious ablative therapy in this kind of patients. © 2007 Elsevier Ireland Ltd. All rights reserved. Keywords: WPW; Dyskinetic segments; Echocardiogram findings; Remodelling; Dilatative cardiomyopathy
Wolff–Parkinson–White (WPW) is a syndrome characterized by the presence of an accessory pathway that skipping A–V node may lead the electrical stimulus from the atrium directly to the ventricle [1,2]. Some studies reported the finding of myocardial dyskinesia in the segments precociously activated by the accessory pathway, at echocardiogram [3] and at nuclear cardiac study [4]. It is not well known which are long time effects of this kinesis impairment. Soria et al. reported, in 1985, an increased incidence of dilative cardiomyopathy in patients with WPW [5]. The pathophysiological pathway that leads to ventricular dilation may be due to the increase of end-
⁎ Corresponding author. Via santa Maria di Gesu' 25, 90124, Italy. E-mail address: [email protected] (G. Fazio). 0167-5273/$ - see front matter © 2007 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.ijcard.2006.11.109
diastolic pressure secondary to a tachycardia-induced cardiomyopathy [6]. Tachycardia-induced cardiomyopathy is usually secondary to frequent and prolonged tachycardia episodes [7]. In this paper we report the cases of the three patients affected by WPW who developed dilative cardiomyopathy during the follow-up [8]. On admission patients' mean age was 5.8 years old (respectively 1, 6 and 10 years old). ECG of every patient showed the presence of an accessory pathway (antero-septal in two cases and anterior in the last). Echocardiogram documented a structurally normal heart but with an evident dyskinesia of either anterior or posterior interventricular basal septum. Patients were followed-up for a mean time of 4.3 years (respectively 2, 5 and 6 years). Two of them never complained palpitations, and periodical ECG Holter control never showed tachycardia episodes. The third
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G. Fazio et al. / International Journal of Cardiology 123 (2008) e31–e34
Fig. 1. An echocardiogram of patient 1 at the first control is shown in panel A. This frame shows a 4-chamber view, where several left ventricular aneurysms are present. The ECG shows an anterior accessory pathway (B panel).
patient, at the age of 8, complained some palpitations, and at ECG Holter monitoring, some episodes of paroxysmal supraventricular tachycardia (PSVT), unresponsive to medical treatment, were documented. PSVT had a maximum duration of 2 min and caused the child's weakness and haemodynamic instability. During this period, in the absence of the arrhythmia, an echocardiogram was performed that showed a mildly dilated left ventricle with a mildly reduced systolic function. After ineffective therapy with propafenone first and amiodarone later, in a six months time, catheter ablation was performed. After the procedure, the patient was completely asymptomatic. During the follow-up all the patients developed a dilative cardiomyopathy. Because the tachycardia-induced mechanism could not explain this relief we hypothesised that dyskinesia could be involved in pathophysiology of this form of cardiomyopathy. Particularly dyskinetic segments, working such as a functional
aneurysm, could induce deep modifications of intraventricular haemodynamics, leading to remodelling and progressive ventricular dilation. This hypothesis could have important empirical consequences because it could imply the necessity of a precocious ablative therapy in this kind of patients. In fact, the ablation of the accessory pathway performed in our patients after showing initial signs of left ventricular dysfunction did not elude the restoration of a normal LV function nor prevented the development of dilative cardiomyopathy. However this suggestive hypothesis needs to be confirmed by further studies (Figs. 1, 2, and 3). References [1] Lebovitz JA, Mandel WJ, Laks MM, Kraus R, Weinstein S. Relationship between the electrical (electrocardiographic) and mechanical (echocardiographic) events in Wolff–Parkinson–White syndrome. Chest 1977;7:463–9.
G. Fazio et al. / International Journal of Cardiology 123 (2008) e31–e34 [2] Ticzon AR, Damato AN, Caracta AR, Russo G, Foster JR, Lau SH. Interventricular septal motion during preexcitation and normal conduction in Wolff–Parkinson–White syndrome: echocardiographic and electrophysiologic correlation. Am J Cardiol 1976;37:840–7. [3] DeMaria AN, Vera Z, Neumann A, Mason DT. Alterations in ventricular contraction pattern in the Wolff–Parkinson–White syndrome. Detection by echocardiography. Circulation 1976;53:249–57. [4] Botvinick EH, Frais MA, Shosa DW, et al. An accurate means of detecting and characterizing abnormal patterns of ventricular activation by phase image analysis. Am J Cardiol 1982;50:289–98.
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[5] Soria R, Fernandez F, Heller J, et al. Wolff–Parkinson–White syndrome and cardiopathies. Arch Mal Coeur Vaiss 1984;77:1468–80. [6] Otsuji Y, Osame M, Tei C, et al. Cardiac involvement in congenital myopathy. Int J Cardiol 1985;9:311–22. [7] Markhasin VS, Glasman AA, Chestukhin VV, Gol'dberg SI, Katsnel'son LB, Makhanek AO. The contribution of the myocardial segmental nonhomogeneity of the left ventricular walls to its contractile and pumping functions. Fiziol Z Im I M Sechenova 1994;80(4):72–80. [8] Emmel Mathis, Balaji Seshadri, Sreeram Narayanswami. Ventricular preexcitation associated with dilated cardiomyopathy: a causal relationship? Cardiol Young 2004;14(6):594–9.
Fig. 2. Echocardiogram of patient 2. A and B panels show a telediastolic frames captured at the first observation and after 5 years of follow-up. C and D panel show a telesystolic frames captured at the same time. All panels show an aneurysm of basal segments of interventricular septum. A left ventricular dilatation was present only in B and D panels, captured after 6 years of follow-up. In the E panel we observe the ECG that shows an antero-septal pathway.
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Fig. 3. Echocardiogram of patient 3 at the beginning of the follow-up and after 4 years. The frames A and B show a 4-chamber view of the left ventricle, with an aneurysm of the basal segments and without a dilatation of the chambers. The same aneurysm is represented in a 4-chamber view and in M-Mode analysis in the C and D frames: here we can observe a dilatation of the left ventricle. All reported frames are telesystolic frames.