International Journal of Cardiology 177 (2014) e32–e33
Contents lists available at ScienceDirect
International Journal of Cardiology journal homepage: www.elsevier.com/locate/ijcard
Letter to the Editor
Electrocardiographic findings of secondary arrhythmogenic right ventricular cardiomyopathy in different right ventricular abnormalities Re.: Secondary arrhythmogenic right ventricular cardiomyopathy after decades of operative repair of tetralogy of Fallot Stefan Peters ⁎ St. Elisabeth Hospital Salzgitter, Germany
a r t i c l e
i n f o
Article history: Received 28 July 2014 Accepted 29 July 2014 Available online 6 August 2014 Keywords: Ebstein anomaly Uhl anomaly Tetralogy of Fallot Localised right precordial QRS prolongation
In different right ventricular abnormalities the development of secondary arrhythmogenic right ventricular cardiomyopathy is possible. This is true for Ebstein anomaly where the true right ventricle can develop aneurysms. According to old data aneurysms of the true right ventricle could be seen in nearly 2/3 of patients [1]. The same is true for operative repair of tetralogy of Fallot where the right ventricular outflow tract decades ago can develop aneurysms and outpouchings [2]. There is ongoing discussion whether Uhl anomaly and arrhythmogenic right ventricular cardiomyopathy share certain similarities. At least partial Uhl anomaly shares multiple diagnostic features of arrhythmogenic right ventricular cardiomyopathy. Uhl anomaly is often associated with atrial septum or other inborn defects. Here we describe electrocardiographic features of a total of three patients with secondary arrhythmogenic right ventricular cardiomyopathy associated with Ebstein anomaly, surgical repair of tetralogy of Fallot and atrial septum defect suggesting partial Uhl anomaly.
⁎ St. Elisabeth Hospital gGmbH Salzgitter, Liebenhaller Str. 20, 38259 Salzgitter, Germany. E-mail address: [email protected].
http://dx.doi.org/10.1016/j.ijcard.2014.07.280 0167-5273/© 2014 Elsevier Ireland Ltd. All rights reserved.
A 28-year old female patient had surgical repair of tetralogy of Fallot decades ago and developed aneurysmal sacculation of the right ventricular outflow tract. The patient suffered from palpitations. Her baseline ECG was in sinus rhythm, localised right precordial QRS prolongation, inverted T waves in right precordial leads and large Q wave, small R wave and inverted T wave in lead aVR (Fig. 1). A 33-year old female patient suffering from dyspnoea had haemodynamic significant atrial septum defect and echocardiographic signs of partial Uhl anomaly. Her ECG was in sinus rhythm, localised right precordial QRS prolongation, epsilon waves in V2, inverted T waves in V1 up to V2 and large Q wave, small R wave and inverted T wave in lead aVR were seen in V2 (Fig. 2). Sinus rhythm, localised right precordial QRS prolongation, inverted T waves in V1 and V2 and large Q wave, small R wave and inverted T waves in lead aVR were the electrocardiographic findings of the 38-year old female patient with Ebstein anomaly. Echocardiography demonstrates dilatation and aneurysm of the true right ventricle (Fig. 3). Signs of secondary arrhythmogenic right ventricular cardiomyopathy appear in different right ventricular abnormalities. These abnormalities include Ebstein anomaly, Uhl anomaly, surgical repair of tetralogy of Fallot and Brugada syndrome. In Brugada syndrome arrhythmogenic right ventricular cardiomyopathy has long been discussed. After the description of plakophilin-2 as a possible gene for Brugada syndrome [3] it is possible that patients suffer from these two diseases as described in one paper [4]. In Ebstein anomaly Anderson and Lie described long ago aneurysms of the true right ventricle resembling arrhythmogenic right ventricular cardiomyopathy [1]. Also the left ventricle can be dyskinetic [5] and left ventricular non-compaction is described in Ebstein anomaly as well [6]. In surgical repair of tetralogy of Fallot aneurysms of the right ventricular outflow tract could be revealed in several papers [2,7]. The ECG of our young patient with palpitations and typical findings in echocardiography seems to be a secondary form of arrhythmogenic right ventricular cardiomyopathy. The histologic difference in Uhl anomaly and arrhythmogenic right ventricular cardiomyopathy is trivial: in Uhl anomaly the musculature of the right ventricle is completely missing with fibrofatty
S. Peters / International Journal of Cardiology 177 (2014) e32–e33
e33
Fig. 3. Standard ECG of a female patient with Ebstein anomaly. Fig. 1. Standard ECG of a female patient after surgical repair of tetralogy of Fallot.
development of arrhythmogenic right ventricular cardiomyopathy is possible. In Uhl anomaly atrial septum defect or other inborn defects can be the first diagnosis. The ECG of all three patients confirms that secondary arrhythmogenic right ventricular cardiomyopathy is a relevant phenomenon in different right ventricular abnormalities.
Conflict of interest The authors report no relationships that could be construed as a conflict of interest. References
Fig. 2. Standard ECG of a female patient with partial Uhl anomaly and atrial septum defect.
replacement whereas in arrhythmogenic right ventricular cardiomyopathy myocardial atrophy and electroanatomic scar by fibrofatty replacement are evident. At least in partial Uhl anomaly the
[1] Anderson KR, Lie JT. Pathologic anatomy of Ebstein anomaly of the heart revisited. Am J Cardiol 1978;41:739–45. [2] Donaldson EE, Ko JM, Gonzalez-Stawinski G, Hall SA, Roberts WC. Secondary arrhythmogenic right ventricular cardiomyopathy decades after operative repair of tetralogy of Fallot. Am J Cardiol 2014;114:806–9. [3] Cerrone M, Lin X, Zhang M, et al. Missense mutations in plakophilin-2 cause sodium current deficit and associate with a Brugada syndrome phenotype. Circulation 2014;129:1092–103. [4] Peters S. Arrhythmogenic cardiomyopathy and provocable Brugada ECG in a patient caused by missense mutation in plakophilin-2. Int J Cardiol 2014;173:317–8. [5] Daliento L, Angelini A, Ho SY, et al. Angiographic and morphologic features of the left ventricle in Ebstein's anomaly. Am J Cardiol 1997;80:1051–9. [6] Fazio G, Visconti C, D'angelo L, et al. Diagnosis and definition of biventricular noncompaction associated to Ebstein's anomaly. Int J Cardiol 2011;150:e20–4. [7] George A, Ko JM, Lensing FD, Kuiper JJ, Roberts WC. “Repaired” tetralogy of Fallot mimicking arrhythmogenic right ventricular cardiomyopathy (another phenocopy). Am J Cardiol 2011;108:326–9.
Contents lists available at ScienceDirect
International Journal of Cardiology journal homepage: www.elsevier.com/locate/ijcard
Letter to the Editor
Electrocardiographic findings of secondary arrhythmogenic right ventricular cardiomyopathy in different right ventricular abnormalities Re.: Secondary arrhythmogenic right ventricular cardiomyopathy after decades of operative repair of tetralogy of Fallot Stefan Peters ⁎ St. Elisabeth Hospital Salzgitter, Germany
a r t i c l e
i n f o
Article history: Received 28 July 2014 Accepted 29 July 2014 Available online 6 August 2014 Keywords: Ebstein anomaly Uhl anomaly Tetralogy of Fallot Localised right precordial QRS prolongation
In different right ventricular abnormalities the development of secondary arrhythmogenic right ventricular cardiomyopathy is possible. This is true for Ebstein anomaly where the true right ventricle can develop aneurysms. According to old data aneurysms of the true right ventricle could be seen in nearly 2/3 of patients [1]. The same is true for operative repair of tetralogy of Fallot where the right ventricular outflow tract decades ago can develop aneurysms and outpouchings [2]. There is ongoing discussion whether Uhl anomaly and arrhythmogenic right ventricular cardiomyopathy share certain similarities. At least partial Uhl anomaly shares multiple diagnostic features of arrhythmogenic right ventricular cardiomyopathy. Uhl anomaly is often associated with atrial septum or other inborn defects. Here we describe electrocardiographic features of a total of three patients with secondary arrhythmogenic right ventricular cardiomyopathy associated with Ebstein anomaly, surgical repair of tetralogy of Fallot and atrial septum defect suggesting partial Uhl anomaly.
⁎ St. Elisabeth Hospital gGmbH Salzgitter, Liebenhaller Str. 20, 38259 Salzgitter, Germany. E-mail address: [email protected].
http://dx.doi.org/10.1016/j.ijcard.2014.07.280 0167-5273/© 2014 Elsevier Ireland Ltd. All rights reserved.
A 28-year old female patient had surgical repair of tetralogy of Fallot decades ago and developed aneurysmal sacculation of the right ventricular outflow tract. The patient suffered from palpitations. Her baseline ECG was in sinus rhythm, localised right precordial QRS prolongation, inverted T waves in right precordial leads and large Q wave, small R wave and inverted T wave in lead aVR (Fig. 1). A 33-year old female patient suffering from dyspnoea had haemodynamic significant atrial septum defect and echocardiographic signs of partial Uhl anomaly. Her ECG was in sinus rhythm, localised right precordial QRS prolongation, epsilon waves in V2, inverted T waves in V1 up to V2 and large Q wave, small R wave and inverted T wave in lead aVR were seen in V2 (Fig. 2). Sinus rhythm, localised right precordial QRS prolongation, inverted T waves in V1 and V2 and large Q wave, small R wave and inverted T waves in lead aVR were the electrocardiographic findings of the 38-year old female patient with Ebstein anomaly. Echocardiography demonstrates dilatation and aneurysm of the true right ventricle (Fig. 3). Signs of secondary arrhythmogenic right ventricular cardiomyopathy appear in different right ventricular abnormalities. These abnormalities include Ebstein anomaly, Uhl anomaly, surgical repair of tetralogy of Fallot and Brugada syndrome. In Brugada syndrome arrhythmogenic right ventricular cardiomyopathy has long been discussed. After the description of plakophilin-2 as a possible gene for Brugada syndrome [3] it is possible that patients suffer from these two diseases as described in one paper [4]. In Ebstein anomaly Anderson and Lie described long ago aneurysms of the true right ventricle resembling arrhythmogenic right ventricular cardiomyopathy [1]. Also the left ventricle can be dyskinetic [5] and left ventricular non-compaction is described in Ebstein anomaly as well [6]. In surgical repair of tetralogy of Fallot aneurysms of the right ventricular outflow tract could be revealed in several papers [2,7]. The ECG of our young patient with palpitations and typical findings in echocardiography seems to be a secondary form of arrhythmogenic right ventricular cardiomyopathy. The histologic difference in Uhl anomaly and arrhythmogenic right ventricular cardiomyopathy is trivial: in Uhl anomaly the musculature of the right ventricle is completely missing with fibrofatty
S. Peters / International Journal of Cardiology 177 (2014) e32–e33
e33
Fig. 3. Standard ECG of a female patient with Ebstein anomaly. Fig. 1. Standard ECG of a female patient after surgical repair of tetralogy of Fallot.
development of arrhythmogenic right ventricular cardiomyopathy is possible. In Uhl anomaly atrial septum defect or other inborn defects can be the first diagnosis. The ECG of all three patients confirms that secondary arrhythmogenic right ventricular cardiomyopathy is a relevant phenomenon in different right ventricular abnormalities.
Conflict of interest The authors report no relationships that could be construed as a conflict of interest. References
Fig. 2. Standard ECG of a female patient with partial Uhl anomaly and atrial septum defect.
replacement whereas in arrhythmogenic right ventricular cardiomyopathy myocardial atrophy and electroanatomic scar by fibrofatty replacement are evident. At least in partial Uhl anomaly the
[1] Anderson KR, Lie JT. Pathologic anatomy of Ebstein anomaly of the heart revisited. Am J Cardiol 1978;41:739–45. [2] Donaldson EE, Ko JM, Gonzalez-Stawinski G, Hall SA, Roberts WC. Secondary arrhythmogenic right ventricular cardiomyopathy decades after operative repair of tetralogy of Fallot. Am J Cardiol 2014;114:806–9. [3] Cerrone M, Lin X, Zhang M, et al. Missense mutations in plakophilin-2 cause sodium current deficit and associate with a Brugada syndrome phenotype. Circulation 2014;129:1092–103. [4] Peters S. Arrhythmogenic cardiomyopathy and provocable Brugada ECG in a patient caused by missense mutation in plakophilin-2. Int J Cardiol 2014;173:317–8. [5] Daliento L, Angelini A, Ho SY, et al. Angiographic and morphologic features of the left ventricle in Ebstein's anomaly. Am J Cardiol 1997;80:1051–9. [6] Fazio G, Visconti C, D'angelo L, et al. Diagnosis and definition of biventricular noncompaction associated to Ebstein's anomaly. Int J Cardiol 2011;150:e20–4. [7] George A, Ko JM, Lensing FD, Kuiper JJ, Roberts WC. “Repaired” tetralogy of Fallot mimicking arrhythmogenic right ventricular cardiomyopathy (another phenocopy). Am J Cardiol 2011;108:326–9.
