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  Table of Contents    
CASE REPORT  
Year : 2011  |  Volume : 54  |  Issue : 1  |  Page : 164-166
Isolated idiopathic right ventricular dilated cardiomyopathy


1 Department of Histopathology, Post Graduate Institute of Medical Education and Research, Chandigarh - 160 012, India
2 Department of Cardiology, Post Graduate Institute of Medical Education and Research, Chandigarh - 160 012, India

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Date of Web Publication7-Mar-2011
 

   Abstract 

Isolated idiopathic right ventricular dilated cardiomyopathy is rare and is a diagnosis by exclusion. There is a distinct male predominance. The usual clinical presentations are syncope, ventricular tachycardia, left bundle branch block on ECG and right heart failure. Diagnosis is usually established based on the clinical and laboratory parameters. Confirmation of the same is done by studying the pathological features of the heart in an endomyocardial biopsy or at post-mortem. Herein we report a case of this rare entity which was diagnosed clinically and was further confirmed at autopsy.

Keywords: Cardiomyopathy, dilated, right ventricle

How to cite this article:
Samanta S, Vijayverghia R, Vaiphei K. Isolated idiopathic right ventricular dilated cardiomyopathy. Indian J Pathol Microbiol 2011;54:164-6

How to cite this URL:
Samanta S, Vijayverghia R, Vaiphei K. Isolated idiopathic right ventricular dilated cardiomyopathy. Indian J Pathol Microbiol [serial online] 2011 [cited 2019 Nov 18];54:164-6. Available from: http://www.ijpmonline.org/text.asp?2011/54/1/164/77392



   Introduction Top


Dilated cardiomyopathy (DCM) is the most common form of cardiomyopathy and is characterized by increased myocardial mass and volume. Ventricular wall becomes thin and stretched out, thus compromising the cardiac contractility and resulting in poor ventricular function. [1] DCM is recognized clinically by ventricular dilatation associated with low ejection fraction on echocardiography. Left ventricle (LV) is the chamber which gets frequently involved by this clinical condition. [2] However; there are few reports of familial DCM affecting the right ventricle as well. [3] Isolated idiopathic right ventricular DCM is an equally rare condition like the familial right ventricular DCM. Besides the few reports which had been mentioned casually, there are only a few documented cases in the English literature. [4],[5],[6],[7] Here, we report a rare case of isolated dilated right ventricular cardiomyopathy with well-preserved left ventricular function.


   Case Report Top


A 37-year-old male patient presented with history of palpitation of 15 days duration followed by oliguria. There was no history of associated chest pain, breathlessness, or syncope at the time of presentation. He gave history of palpitation off and on at rest, associated with chest pain and breathlessness in the last 2 years. There was no definite aggravating or relieving factor. There was no history of diabetes mellitus, hypertension, coronary artery disease, pulmonary tuberculosis, or family history of heart disease. The patient was a known alcoholic, chronic smoker, and an opioid addict. At admission, he had a blood pressure of 78/60 mmHg, pulse rate of 140/min, raised jugular venous pressure, and pitting bilateral pedal edema. There was no evidence of anemia, jaundice, cyanosis, or clubbing. The respiratory system was within normal limits. Cardio-vascular system examination showed normal S1 and S2 with no added sounds. Liver was palpable 3 cm below the right costal margin. There was no evidence of ascites or any bruit. Routine hemogram revealed a hemoglobin value of 142 g/l, total leukocyte count of 8×10 9 /l with normal differential count. Coagulogram was deranged with prothrombin time of 36 s, activated partial thromboplastin time of 47 s, prothrombin time index of 28% and an international normalized ratio was 3.5. Biochemical parameters showed low sodium (125 mEq/l) and normal potassium (4.8 mEq/l). Blood urea (143 mg/dl) and creatinine (3.1 mg/dl) were raised. ABG showed blood pH of 7.18 and SaO 2 - 96.9%. Chest X-ray showed a grossly enlarged heart with marked dilatation of the right ventricle [Figure 1]. Electrocardiography (ECG) showed right axis deviation with a wide complex tachycardia (140/min), inferior axis deviation and right bundle branch block. Echocardiography examination showed a markedly dilated right ventricle (RV) and right atrium (RA) along with severely hypokinetic RV and grade III tricuspid regurgitation. Interventricular and interatrial septa were pushed toward the left side with cavity compression. No pericardial effusion or mural thrombus was observed in any of the vessels or the heart chambers. With the clinical possibility of ventricular tachycardia, cardioversion by direct current of 200 J and 300 J was done and he was reverted to normal sinus rhythm. He continued to be hypotensive in spite of being on inotropes and died after 17 h of hospital admission. Following demise of the patient subjected to a partial autopsy.
Figure 1: Chest X-ray PA view: hugely enlarged cardiac silhouette with rounding of the left and right borders

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At autopsy, the body cavities did not show any excess of serous fluid. As expected, the heart showed significant finding. It was grossly enlarged measuring 16×12×10 cm and weighing 532 g. (RA) and right ventricle (RV) were largely dilated and contained multiple mural thrombi. The RA thombus was seen extending to the enlarged and discolored right auricle [Figure 2]. RV wall thickness was 4 mm. Pulmonary artery was normal. The LV wall thickness was 11 mm with no significant cavity dilatation or mural thrombus. Left atrium was slightly dilated. Aorta showed grade II atherosclerosis. Microscopy of the RV revealed thin out wall with individual myocardial fiber size variation with marked anisonucleosis, interstitial edema, patchy interstitial and perivascular fibrosis, and endocardial sclerosis. Section from the LV was of normal histology except for mild myocardial anisonucleosis. There was no feature to suggest chronic or acute ischemia. Coronary arteries were essentially within normal limits except the left anterior descending with 50% narrowing by atheromatous plaque. Liver, spleen, and lungs showed features of chronic passive venous congestion. The histology of liver did not show any feature to suggest a possibility of alcohol-related liver injury.
Figure 2: Apical slice of the heart: showing markedly dilated RV with mural thrombi and relatively normal sized LV with few mural thrombi

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   Discussion Top


In view of a well-maintained wall thickness and cavity size of the LV with normal histology, the present case has all the classic features for the diagnosis of isolated right ventricular DCM, similar to the criteria for the diagnosis of RVDCM that had been reported in the literature. [4],[5],[6] The cardiac pathology in the present case was restricted to the right heart. Though there was thinning of the RV wall, the histology of the RV wall showed the presence of myocardial fibers and no replacement by adipocyte thereby excluding a diagnosis of arrythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C), which is a condition that would be considered in the differential diagnosis in such a patient. [8] The pathological changes observed in RV involved the whole RV rather than a specific area called "the dysplasia triangle." There was no family history of a similar illness in the siblings. Isolated RV cardiomyopathy may incite the possibility of Uhl's anomaly. Uhl's anomaly is characterized by virtually complete absence of the myocardium of the parietal layer of RV which is composed of apposing endocardial and epicardial layers with no interposed adipose tissue with no evidence of inflammation or necrosis. [9] Isolated right ventricular noncompaction that would be in the differential diagnosis is an extremely rare congenital cardiomyopathy resulting from an arrest in normal endomyocardial embryogenesis. The clinical syndrome includes systolic and diastolic dysfunctions with ventricular arrhythmias in some of the patients. Echocardiography or pathology examination of the heart at autopsy did not reveal RV sinuses. [10] The patient gave history of chronic alcohol and opioid abuse, cardiomyopathy resulting from long standing utilization of such substances would involve either LV or biventricles, and sometimes could also be associated with myocarditis. The absence of histological features that could be suggestive of alcohol-related liver disease and a normal LV, a diagnosis of an isolated idiopathic right ventricular DCM would be the most appropriate diagnosis in this case.

In conclusion, we report a rare case of isolated idiopathic right ventricular DCM in a young male patient who presented with long standing history of palpitation off and on at rest associated with chest pain and breathlessness. A detail genetic and proteomic analysis is a requirement to elucidate the underlying mechanism in such type of a chronic debilitating and potentially fatal disease.

 
   References Top

1.Luk A, Ahn E, Soor GS, Butany J. Dilated cardiomyopathy: A review. J Clin Pathol 2009;62:219-25.  Back to cited text no. 1
[PUBMED]  [FULLTEXT]  
2.Olsen EG. Pathology of primary cardiomyopathies. Postgrad Med J 1972;48:732-7.  Back to cited text no. 2
[PUBMED]  [FULLTEXT]  
3.Ibsen HH, Baandrup U, Simonsen EE. Familial right ventricular dilated cardiomyopathy. Br Heart J 1985;54:156-9.  Back to cited text no. 3
[PUBMED]  [FULLTEXT]  
4.Mokhobo KP, Mntla PS. Lone ventricular cardiomyopathy, 1993-1996. S Afr Med J 1997;87:892-6.  Back to cited text no. 4
[PUBMED]    
5.Hatle L, Stake G, Storstein O. Chronic myocardial disease. 2. Haemodynamic findings related to long-term prognosis. Acta Med Scand 1976;199:407-11.   Back to cited text no. 5
    
6.Fitchett DH, Sugrue DD, Mac Arthur CG, Oakley Celia M. Right ventricular dilated cardiomyopathy. Br Heart J 1984;51:25-9.  Back to cited text no. 6
    
7.Rowland E, McKenna WJ, Sugrue D, Barclay R, Foale RA, Krikler DM. Ventricular tachycardia of left bundle branch block configuration in patients with isolated right ventricular dilatation. Clinical and electrophysiological features. Br Heart J 1984;51:15-24.   Back to cited text no. 7
[PUBMED]  [FULLTEXT]  
8.Marcus FI, Fontaine GH, Guiraudon G, Frank R, Laurenceau JL, Malergue C, et al. Right ventricular dysplasia: A report of 24 adult cases. Circulation 1982;65:384-98.  Back to cited text no. 8
[PUBMED]  [FULLTEXT]  
9.Uhl HS. Uhl's anomaly revisited. Circulation 1996;93:1483-4.   Back to cited text no. 9
[PUBMED]  [FULLTEXT]  
10.Zhang XJ, Zhi G, Hou HJ, Zhou X. A rare case of isolated non-compaction right ventricular myocardium. Chin Med J 2009;122:1718-20.  Back to cited text no. 10
[PUBMED]  [FULLTEXT]  

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Correspondence Address:
Kim Vaiphei
No 127/C Sector 24/A, Chandigarh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.77392

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