International Journal of Cardiology 176 (2014) 1072–1073
Contents lists available at ScienceDirect
International Journal of Cardiology journal homepage: www.elsevier.com/locate/ijcard
Letter to the Editor
Variability of right ventricular angiography in arrhythmogenic right ventricular cardiomyopathy S. Peters St. Elisabeth Hospital gGmbH Salzgitter, Liebenhaller Str. 20, 38259 Salzgitter, Germany
a r t i c l e
i n f o
Article history: Received 26 May 2014 Accepted 26 July 2014 Available online 2 August 2014 Keywords: Arrhythmogenic right ventricular cardiomyopathy Imaging technique Right ventricular angiography MRI scan
The optimal imaging technique to diagnose arrhythmogenic right ventricular cardiomyopathy is under discussion. Cardiac MRI has advanced to be the dominant imaging technique as morphology and function can be assessed. Fatty infiltrates can de detected although it is not easy to differentiate whether fat infiltration is within the ventricle or surrounding the ventricle. Fibrose infiltration can be best seen with gadolinium scan. Segmental contraction impairment is difficult to analyse. There are a few centres in the world where cardiac MRI can definitely diagnose arrhythmogenic right ventricular cardiomyopathy. Up to now right ventricular angiography was the method of choice in order to diagnose ARVC. Morphology of the right ventricle is difficult to assess. Segmental contraction impairment can be best seen by right ventricular angiography. Tissue imaging is not possible. Right ventricular angiography is the method of choice to analyse trabecular structure. In the centre of Ivry-sur-Seine Paris right ventricular angiography remains the gold standard to diagnose arrhythmogenic right ventricular cardiomyopathy [1]. With the help of two case reports the variability of right ventricular angiography should be demonstrated. Case no. 1 was a 30 year-old male patient who was admitted to hospital with chest pain, palpitations and dizziness. Syncope did not appear. The right ventricle was dilated and deep horizontal fissures were seen in the apex and in the right ventricular outflow tract. In other areas fissural structure was normal (Fig. 1).
E-mail address: [email protected].
http://dx.doi.org/10.1016/j.ijcard.2014.07.135 0167-5273/© 2014 Elsevier Ireland Ltd. All rights reserved.
Standard ECG demonstrates localised right precordial QRS prolongation and progressive R wave reduction in right precordial leads. Holter monitoring was uneventful. Case no. 2 was a 24 year-old male patient who suffered syncope. Paroxysmal atrial fibrillation was evident several years ago. The right ventricle was dilated and the inferior and apical region presented with bulges. The right ventricular outflow tract revealed deep horizontal fissures. Trabecular structure was reduced at inferior and apical region (Fig. 2). The apex of the left ventricle was akinetic although the overall function of the left ventricle was within normal limits. Standard ECG revealed localized right precordial QRS prolongation, epsilon waves in V1 and V2 and QRS fragmentation. Holter monitoring revealed an AV block of 1° and an episode of complete AV block with a cardiac arrest of more than 1 min. Ventricular tachycardia was not documented. Arrhythmogenic right ventricular cardiomyopathy with left ventricular involvement was the diagnosis in case no. 2. The variability of right ventricular angiography can be best presented by these two cases. Sacculation and dilatation (bulging) of the so-called “triangle of dysplasia” in arrhythmogenic right ventricular cardiomyopathy can be seen in most cases [2]. Completely missing or reduced structure of trabeculae was published several years ago [3]. In 1990 Daliento published a paper where deep horizontal fissures (hypertrophied fissures) were the best parameter in arrhythmogenic right ventricular cardiomyopathy with high sensitivity and specificity [4]. In case no. 1 bulging of inferior, apical and right ventricular outflow tract cannot be seen, but deep horizontal fissures of the apical and right ventricular outflow tract region. Echocardiography demonstrates within the right ventricular outflow tract two different bulgings with a diameter of upper limits (Fig. 3). In this case echocardiography is a very good marker of arrhythmogenic right ventricular cardiomyopathy. In case no. 2 a biventricular involvement is presented. This patient suffered from syncope of unknown reason and the therapy of choice is ICD implantation [5]. Recurrent ventricular tachycardia, ventricular fibrillation and syncope mostly occur in cases with arrhythmogenic right ventricular cardioymyopathy with significant left ventricular involvement [6]. Sudden cardiac death occurs predominantly in cases with biventricular arrhythmogenic disease [7]. In our patient an event recorder was implanted for diagnostic reasons. In conclusion, there is a certain variability of angiographic findings in arrhythmogenic right ventricular cardiomyopathy making a better analysis of segmental contraction impairment and trabecular structure available.
S. Peters / International Journal of Cardiology 176 (2014) 1072–1073
1073
Fig. 3. Two bulges of the right ventricular outflow tract demonstrated by echocardiography.
References Fig. 1. Deep horizontal fissures at the apex and at the right ventricular outflow tract.
In the hand of experienced physicians cardiac MRI is a very good option in the analysis of arrhythmogenic right ventricular cardiomyopathy with regard to morphology and tissue presentation.
Conflict of interest The authors report no relationships that could be construed as a conflict of interest.
Fig. 2. Bulges of the inferior, apical and right ventricular outflow tract region with loss of trabecular structure.
[1] Duthoit G, Fressart V, Hidden-Lucet F, et al. Brugada ECG pattern: a physiopathological prospective study based on clinical, electrophysiological, angiographic and genetic findings. Front Physiol 2012;3:47. [2] Marcus FI, Fontaine GH, Guiraudon G, et al. Right ventricular dysplasia: a report of 24 adult cases. Circulation 1982;65:384–98. [3] Peters S, Weber B, Hartwig CA, Reil GH. Incidence and significance of morphologic and morphometric variants in selective right ventricular angiography in diagnosis of arrhythmogenic right ventricular disease. Med Klinik 1994;89:175–83. [4] Daliento L, Rizzoli G, Thiene F, et al. Diagnostic accuracy of right ventriculography in arrhythmogenic right ventricular cardiomyopathy. Am J Cardiol 1990;66:741–5. [5] Corrado D, Calkins H, Link MS, et al. Prophylactic implantable defribrillator in patients with arrhythmogenic right ventricular cardiomyopathy/dysplasia and no prior ventricular fibrillation or sustained ventricular tachycardia. Circulation 2010;122: 1144–52. [6] Peters S. Left ventricular impairment in arrhythmogenic right ventricular dysplasia: what we can learn from angiography. Cardiology 1995;86:473–6. [7] Tavora F, Zhang M, Franco M, et al. Distribution of biventricular disease in arrhythmogenic cardiomyopathy: an autopsy study. Hum Pathol 2012;43:592–5.
Contents lists available at ScienceDirect
International Journal of Cardiology journal homepage: www.elsevier.com/locate/ijcard
Letter to the Editor
Variability of right ventricular angiography in arrhythmogenic right ventricular cardiomyopathy S. Peters St. Elisabeth Hospital gGmbH Salzgitter, Liebenhaller Str. 20, 38259 Salzgitter, Germany
a r t i c l e
i n f o
Article history: Received 26 May 2014 Accepted 26 July 2014 Available online 2 August 2014 Keywords: Arrhythmogenic right ventricular cardiomyopathy Imaging technique Right ventricular angiography MRI scan
The optimal imaging technique to diagnose arrhythmogenic right ventricular cardiomyopathy is under discussion. Cardiac MRI has advanced to be the dominant imaging technique as morphology and function can be assessed. Fatty infiltrates can de detected although it is not easy to differentiate whether fat infiltration is within the ventricle or surrounding the ventricle. Fibrose infiltration can be best seen with gadolinium scan. Segmental contraction impairment is difficult to analyse. There are a few centres in the world where cardiac MRI can definitely diagnose arrhythmogenic right ventricular cardiomyopathy. Up to now right ventricular angiography was the method of choice in order to diagnose ARVC. Morphology of the right ventricle is difficult to assess. Segmental contraction impairment can be best seen by right ventricular angiography. Tissue imaging is not possible. Right ventricular angiography is the method of choice to analyse trabecular structure. In the centre of Ivry-sur-Seine Paris right ventricular angiography remains the gold standard to diagnose arrhythmogenic right ventricular cardiomyopathy [1]. With the help of two case reports the variability of right ventricular angiography should be demonstrated. Case no. 1 was a 30 year-old male patient who was admitted to hospital with chest pain, palpitations and dizziness. Syncope did not appear. The right ventricle was dilated and deep horizontal fissures were seen in the apex and in the right ventricular outflow tract. In other areas fissural structure was normal (Fig. 1).
E-mail address: [email protected].
http://dx.doi.org/10.1016/j.ijcard.2014.07.135 0167-5273/© 2014 Elsevier Ireland Ltd. All rights reserved.
Standard ECG demonstrates localised right precordial QRS prolongation and progressive R wave reduction in right precordial leads. Holter monitoring was uneventful. Case no. 2 was a 24 year-old male patient who suffered syncope. Paroxysmal atrial fibrillation was evident several years ago. The right ventricle was dilated and the inferior and apical region presented with bulges. The right ventricular outflow tract revealed deep horizontal fissures. Trabecular structure was reduced at inferior and apical region (Fig. 2). The apex of the left ventricle was akinetic although the overall function of the left ventricle was within normal limits. Standard ECG revealed localized right precordial QRS prolongation, epsilon waves in V1 and V2 and QRS fragmentation. Holter monitoring revealed an AV block of 1° and an episode of complete AV block with a cardiac arrest of more than 1 min. Ventricular tachycardia was not documented. Arrhythmogenic right ventricular cardiomyopathy with left ventricular involvement was the diagnosis in case no. 2. The variability of right ventricular angiography can be best presented by these two cases. Sacculation and dilatation (bulging) of the so-called “triangle of dysplasia” in arrhythmogenic right ventricular cardiomyopathy can be seen in most cases [2]. Completely missing or reduced structure of trabeculae was published several years ago [3]. In 1990 Daliento published a paper where deep horizontal fissures (hypertrophied fissures) were the best parameter in arrhythmogenic right ventricular cardiomyopathy with high sensitivity and specificity [4]. In case no. 1 bulging of inferior, apical and right ventricular outflow tract cannot be seen, but deep horizontal fissures of the apical and right ventricular outflow tract region. Echocardiography demonstrates within the right ventricular outflow tract two different bulgings with a diameter of upper limits (Fig. 3). In this case echocardiography is a very good marker of arrhythmogenic right ventricular cardiomyopathy. In case no. 2 a biventricular involvement is presented. This patient suffered from syncope of unknown reason and the therapy of choice is ICD implantation [5]. Recurrent ventricular tachycardia, ventricular fibrillation and syncope mostly occur in cases with arrhythmogenic right ventricular cardioymyopathy with significant left ventricular involvement [6]. Sudden cardiac death occurs predominantly in cases with biventricular arrhythmogenic disease [7]. In our patient an event recorder was implanted for diagnostic reasons. In conclusion, there is a certain variability of angiographic findings in arrhythmogenic right ventricular cardiomyopathy making a better analysis of segmental contraction impairment and trabecular structure available.
S. Peters / International Journal of Cardiology 176 (2014) 1072–1073
1073
Fig. 3. Two bulges of the right ventricular outflow tract demonstrated by echocardiography.
References Fig. 1. Deep horizontal fissures at the apex and at the right ventricular outflow tract.
In the hand of experienced physicians cardiac MRI is a very good option in the analysis of arrhythmogenic right ventricular cardiomyopathy with regard to morphology and tissue presentation.
Conflict of interest The authors report no relationships that could be construed as a conflict of interest.
Fig. 2. Bulges of the inferior, apical and right ventricular outflow tract region with loss of trabecular structure.
[1] Duthoit G, Fressart V, Hidden-Lucet F, et al. Brugada ECG pattern: a physiopathological prospective study based on clinical, electrophysiological, angiographic and genetic findings. Front Physiol 2012;3:47. [2] Marcus FI, Fontaine GH, Guiraudon G, et al. Right ventricular dysplasia: a report of 24 adult cases. Circulation 1982;65:384–98. [3] Peters S, Weber B, Hartwig CA, Reil GH. Incidence and significance of morphologic and morphometric variants in selective right ventricular angiography in diagnosis of arrhythmogenic right ventricular disease. Med Klinik 1994;89:175–83. [4] Daliento L, Rizzoli G, Thiene F, et al. Diagnostic accuracy of right ventriculography in arrhythmogenic right ventricular cardiomyopathy. Am J Cardiol 1990;66:741–5. [5] Corrado D, Calkins H, Link MS, et al. Prophylactic implantable defribrillator in patients with arrhythmogenic right ventricular cardiomyopathy/dysplasia and no prior ventricular fibrillation or sustained ventricular tachycardia. Circulation 2010;122: 1144–52. [6] Peters S. Left ventricular impairment in arrhythmogenic right ventricular dysplasia: what we can learn from angiography. Cardiology 1995;86:473–6. [7] Tavora F, Zhang M, Franco M, et al. Distribution of biventricular disease in arrhythmogenic cardiomyopathy: an autopsy study. Hum Pathol 2012;43:592–5.
