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Catastrophic abrupt deterioration of left ventricular function

in a patient with typical arrhythmogenic right ventricular cardiomyopathy

Case Presentation:

Abrupt catastrophic decrease of cardiac function can be observed in some patients with ascertained well tolerated arrhythmogenic right ventricular cardiomyopathy (ARVC). The following case is a typical well documented illustration of this situation.

Myocardial Disease


A 28 years old male patient had no personal history until he experienced palpitations while playing soccer. Some months later a near syncopal episode of ventricular tachycardia (VT) showing a left bundle branch block (LBBB) pattern was documented (see Figure 1). The physical examination was normal.



Figure 1. ECG during ventricular tachycardia.





QUESTIONS

 

What is your diagnosis? What is the diagnostic work-up you propose?

Diagnosis of ARVC was performed. Left ventricular (LV) systolic function was normal on echocardiography. Short term evolution was good with sotalol therapy. One month later the patient experienced chest pain. He had ST segment elevation in DII, DIII and AVF on ECG. Troponin 1C level was strongly elevated at 120 ng/ml. Anti-virus serology (IgM) was positive for Para Influenza III.

How to explain chest pain and signs of inflammation?

Three months later LVEF was 57% during radionuclide evaluation, with increase of right ventricle size. Four months later he had severe episode of VT with syncope despite sotalol therapy.

How to treat these resistant VT episodes?

Three months later a new radionuclide evaluation showed a drop of LVEF to 45%. Troponin level was 5 ng/ml. After three additional months LVEF decreased to 25%. Echocardiography exhibited a thrombus inside LV and the patient developed major clinical signs of liver dysfunction.

How to explain this continuous and major decrease of LVEF in 27 months? What to do?



 

What is your diagnosis?   What are the next diagnostic tools?
The diagnosis of ARVC was suspected after recording an exercise-induced ventricular tachycardia with LBBB pattern (figure 1, case presentation).
Ventricular tachycardia is generally related to ischemic heart disease. In a young patient with normal physical examination other diagnoses have to be discussed. In particular a pseudo VT pattern in WPW syndrome which is in fact atrial fibrillation transmitted to the accessory pathway. However, in this situation the heart rate which can be as high as 300/min is slightly irregular with slight changes in morphology. Here we have another marker which is the LBBB pattern suggesting an origin in the right ventricle in a possible case of ARVC.
Echocardiography, as the first line examination, showed dilated right atrium (RA) and moderate RV dilatation. Late potentials were positive for three criteria. MRI exhibited dilated RV. Radionuclide: LVEF 65%. Contrast angiogram showed RV infundibular aneurysm and increased thickness of trabeculations.

How to explain chest pain and signs of inflammation?
The diagnosis of pericardo-myocarditis could explain chest pain and was also suggested by anti-virus serology. It could be that the inflammatory process not studied after the first episode of VT was already the trigger in the first episode of arrhythmia in addition to the autonomous nervous system involved in effort and stress during soccer play.

How to treat these resistant VT episodes?
ICD was implanted.

How to explain this continuous drop of LVEF? What to do?
This rapid deterioration of left ventricular function suggests a major destruction of LV myocardium by an autoimmune process triggered by acute myocarditis. Heart transplantation was the only available solution.

The proof of this diagnosis was made by histologic examination of the specimen after heart tx.

1. Typical ARVC structure was observed on the right ventricle only (see Figure 2)


Figure 2. Typical pattern of almost transmural replacement of myocardium by fat and fibrosis with surviving cardiomyocytes within fat. Thin rims of myocardium are only visible along the endocardium (on the left).






2. Lymphocytes were present in both left and right ventricles with major destruction of cardiomyocytes and interstitial as well as replacement fibrosis in other areas (Figure 3).





Figure 3. Zone of chronic-active myocarditis in the LV in the same patient as above. Major loss of cardiac function led to progressive deterioration of heart function and transplantation.







Conclusion:


We describe a typical clinical case of ARVC with severe ventricular arrhythmias but unusual acute signs of clinical inflammation suggesting pericardo-myocarditis with LVEF drop from 57 to 25% in 27 months. Biventricular invasion by lymphocytes suggests a superimposed chronic-active myocarditis involving both ventricles and explaining the abrupt catastrophic drop of cardiac function (Figure 4).
The authors think that the heart of patients with all forms of cardiomyopathies including ARVC are more susceptible than normal to superimposed infection, especially related to Coxsackie B viruses. This is an illustration of interactions between environment and a genetic disease, and this can explain the rapid evolution of the disease.



Figure 4. Role of superimposed myocarditis on the LVEF in patients with ARVC and DCM. (modified from reference 2)

References


  1. Bowles NE, Ni J, Marcus F, Towbin JA. The detection of cardiotropic viruses in the myocardium of patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy. J Am Coll Cardiol. 2002 Mar 6;39(5):892-5.
  2. Fontaine G, Fontaliran F, Frank R. Arrhythmogenic right ventricular cardiomyopathies: clinical forms and main differential diagnoses. Circulation. 1998 Apr 28;97(16):1532-5.
  3. Basso C, Thiene G, Corrado D, Angelini A, Nava A, Valente M. Arrhythmogenic right ventricular cardiomyopathy. Dysplasia, dystrophy, or myocarditis? Circulation. 1996 Sep 1;94(5):983-91.
  4. Bonny A, Lellouche N, Ditah I, Hidden-Lucet F, Yitemben MT, Granger B, Larrazet F, Frank R, Fontaine G. C-reactive protein in arrhythmogenic right ventricular dysplasia/cardiomyopathy and relationship with ventricular tachycardia. Cardiol Res Pract. 2010 Aug 24;2010.
  5. Campian ME, Verberne HJ, Hardziyenka M, de Groot EA, van Moerkerken AF, van Eck-Smit BL, Tan HL. Assessment of inflammation in patients with arrhythmogenic right ventricular cardiomyopathy/dysplasia. Eur J Nucl Med Mol Imaging. 2010 Nov;37(11):2079-85.

Notes to editor


Presented by Dr. Guy Fontaine & Dr. Philippe Charron
Centre de référence Maladies cardiaques héréditaires,
Hôpital Pitié-Salpêtrière & Université Paris 6, France
The content of this article reflects the personal opinion of the author/s and is not necessarily the official position of the European Society of Cardiology.

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