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European Journal of Echocardiography 2005 6(6):470-472; doi:10.1016/j.euje.2005.02.005
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Copyright © 2005, The European Society of Cardiology

Contrast echocardiography for perfusion in right ventricular cardiomyopathy

Attila Nemesa, Willem B. Vletterb, Marcoen F. Scholtenb and Folkert J. ten Cateb,*

a2nd Department of Medicine and Cardiology Centre, University of Szeged, Szeged, Hungary
bDepartment of Cardiology, Thoraxcentre, Erasmus MC, Rotterdam, The Netherlands

Received 24 September 2004; accepted after revision 12 February 2005.

f.j.tencate{at}erasmusmc.nl

* Corresponding author. Department of Cardiology, Thoraxcenter, Erasmus Medical Center Rotterdam, Dr Molewaterplein 40, 3015 GD, Room Ba304, Rotterdam, The Netherlands. Tel.: +31 10 4635669; fax: +31 10 4635498.


   Abstract
Top
 Abstract
Introduction
 Case study
 Discussion
 Conclusion
 References
 
Right ventricular (RV) cardiomyopathy is a familiar myocardial disease of RV characterized by extensive fatty replacement of the myocardium. Conventional echocardiography is able to show abnormalities in myocardial contractility, but fat on the images appears to be similar to the surrounding tissue or fluid. The present case suggests the clinical role of contrast echocardiography showing perfusion abnormalities in patients with RV cardiomyopathy in the region of the fat depositions.

Keywords: 2D, two-dimensional; ECG, electrocardiogram; ICD, implantable cardioverter defibrillator; IVS, interventricular septum; LBBB, left bundle branch block; LV, left ventricle; LVFW, left ventricular lateral free wall; MR, magnetic resonance; MV, mitral valve; RBBB, right bundle branch block; RV, right ventricle; RVW, right ventricular wall; TV, tricuspid valve; VT, ventricular tachycardia


   Introduction
Top
 Abstract
 Introduction
Case study
 Discussion
 Conclusion
 References
 
Right ventricular (RV) cardiomyopathy is a familiar myocardial disease of RV characterized by extensive fatty replacement of the myocardium.1,2 Previously mainly MR and CT studies verified their diagnostic value in the evaluation of these characteristics in RV cardiomyopathy.3–6 The aim of this report is to demonstrate the clinical usefulness of contrast echocardiography in the evaluation of perfusion in right ventricular cardiomyopathy.


   Case study
Top
 Abstract
 Introduction
 Case study
Discussion
 Conclusion
 References
 
A 40-year-old man was referred to the Thoraxcenter, Erasmus MC, Rotterdam, with suspected right ventricular cardiomyopathy. His medical history started in September 2001 with left and right sided cardiac decompensation on the basis of hypertensive cardiomyopathy. Secondary hypertension was excluded, the severity was moderate. The coronary angiography showed normal epicardial coronary arteries, the left and right heart hemodynamic results are not available. The left ventricle (LV) ejection fraction was 30% during heart failure which improved to more than 40% after medical treatment of high blood pressure. During this admission, frequent nonsustained ventricular tachycardias (VT) with 160/min heart rate up to 5 times a day during 5 or 6 min with left bundle branch block (LBBB) pattern developed with symptoms of palpitations, while the resting ECG showed right bundle branch block (RBBB) (Fig. 1a,b). Ventricular tachycardia episodes continued, despite medical treatment. He was admitted for ICD implantation. His mother died suddenly of unknown cause at age 51. Transthoracic two-dimensional echocardiography showed a severe dilation and extensive dyskinesia of the anterior right ventricle wall, the RV ejection fraction was reduced. The basal segments of RV were dyskinetic with diastolic bulging (Fig. 2). Contrast echocardiographic examination showed normally perfused interventricular septum, while the anterior wall of the RV was not perfused (Fig. 3). The contrast examination was carried out using Sonovue (Bracco, Milan, Italy) and Philips Sonos 5500 (Philips Medical Systems, Best, Netherlands) using power modulation imaging. The contrast agent was given as slow infusion with a speed of 0.5 ml per second. A total dose of 2 ml Sonovue was given.


Figure 1
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  		Figure 1 A 12-lead ECG showing RBBB at resting condition (a), during the palpitation episode, high frequency VT with LBBB pattern was detected (b).

 


Figure 2
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  		Figure 2 Conventional 2D echo showing severe dilation of the right ventricle and bulging above TV (see arrow).

 


Figure 3
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  		Figure 3 Contrast echocardiography demonstrating no perfusion in RV free wall (see arrows), while the interventricular septum is normally perfused.

 

   Discussion
Top
 Abstract
 Introduction
 Case study
 Discussion
Conclusion
 References
 
According to the nature of the disease, RV cardiomyopathy is characterized by partial or total replacement of the myocardium by adipose or fibro-adipose tissue. Several diagnostic methods were used to evaluate patients with suspected RV cardiomyopathy. Conventional echocardiography is able to show irregular dilation of the outflow tract with an increased right ventricle/left ventricle ratio, abnormalities in myocardial contractility, but fat on the images appears to be similar to the surrounding tissue or fluid.7,8 MR imaging3–5 and electron-beam computed tomography6 have demonstrated their capability to detect these characteristics. The result of the present study is in accordance with Lopez-Fernandez et al. who concluded that color Doppler contrast enhancement of the RV improves the ability to assess RV cardiomyopathy.9


   Conclusion
Top
 Abstract
 Introduction
 Case study
 Discussion
 Conclusion
References
 
The present case suggests the clinical role of contrast echocardiography showing perfusion abnormalities in patients with RV cardiomyopathy in the region of the fat depositions.


   References
Top
 Abstract
 Introduction
 Case study
 Discussion
 Conclusion
 References
 

  1. Gemayel C., Pelliccia A., Thompson P.D. Arrhythmogenic right ventricular cardiomyopathy. J Am Coll Cardiol (2001) 38:1773–1781.[Abstract/Free Full Text]
  2. Fontaine G., Fontaliran F., Hébert J.L., Chemla D., Zenati O., Lecarpentier Y., et al. Arrhythmogenic right ventricular dysplasia. Annu Rev Med (1999) 50:17–35.[CrossRef][Web of Science][Medline]
  3. Midiri M., Finazzo M. MR imaging of arrhythmogenic right ventricular dysplasia. Int J Cardiovasc Imaging (2001) 17:297–304.[CrossRef][Web of Science][Medline]
  4. van der Wall E.E., Kayser H.W., Bootsma M.M., de Roos A., Schaly M. Arrhythmogenic right ventricular dysplasia: MRI findings. Herz (2000) 25:356–364.[CrossRef][Web of Science][Medline]
  5. Molinari G., Sardanelli F., Gaita F., Ottonello C., Richiardi E., Parodi R.C., et al. Right ventricular dysplasia as a generalized cardiomyopathy? Findings on magnetic resonance imaging. Eur Heart J (1995) 16:1619–1624.
  6. Tada H., Shimizu W., Ohe T., Hamada S., Kurita T., Aihara N., et al. Usefulness of electron-beam computed tomography in arrhythmogenic right ventricular dysplasia: relationship to electrophysiological abnormalities and LV involvement. Circulation (1996) 94:437–444.[Abstract/Free Full Text]
  7. Kisslo J. Two-dimensional echocardiography in arrhythmogenic right ventricular dysplasia. Eur Heart J (1989) 10:22–26.[Abstract/Free Full Text]
  8. Robertson J.H., Bardy G.H., German L.D., Gallagher J.J., Kisslo J. Comparison of two-dimensional echocardiographic and angiographic findings in arrhythmogenic right ventricular dysplasia. Am J Cardiol (1985) 55(13 Pt 1):1506–1508.[CrossRef][Web of Science][Medline]
  9. Lopez-Fernandez T., Garcia-Fernandez M.A., Perez D., Moreno Y. Usefulness of contrast echocardiography in arrhythmogenic right ventricular dysplasia. J Am Soc Echocardiogr (2004) 17:391–393.[CrossRef][Web of Science][Medline]

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