European Society of Cardiology
Skip navigation links
Home
About the ESC
Membership
Communities
Congresses
Education
Guidelines & Surveys
Journals
Initiatives
Welcome to the European Society of Cardiology. Our mission: to reduce the burden of cardiovascular disease in Europe
 
01 Mar 2011

Non-dilated left ventricle with moderate systolic dysfunction 

Topics: Myocardial Disease
Authors: Petr Kuchynka, Tomas Palecek, Ales Linhart
A 37- year old woman with a 5-year history of treated hypothyreosis was admitted to the local hospital for shortness of breath.



Echocardiographic examination revealed non-dilated left ventricle (LV) with moderate systolic dysfunction (ejection fraction 35%). Coronary angiography excluded any coronary artery pathology and the patient was treated as a new-onset heart failure due to non-ischemic dilated cardiomyopathy, possibly acute myocarditis.  Within 3 weeks of therapy including ACE-inhibitor, diuretics and betablocker she felt much better. Echocardiography showed recovery of the LV systolic function with calculated ejection fraction 56%. However, after 5 weeks she again started to suffer from progressive dyspnea with worsening of the LV systolic function as documented by echocardiography. Based on these findings she has been referred to our department for further thorough evaluation of the etiology of the  LV dysfunction.
Upon admission the patient was haemodynamically stable, with blood pressure of 120/70 mmHg and heart rate of 60 beats/min. Apart from obesity no other abnormality was revealed by  physical examination. Her family history was not suggestive of familiar cardiomyopathy.

ECG

Fig. 1. ECG showed sinus rhythm with normal PQ and QT intervals, low voltage in limb leads and negative T  waves in leads I, II, aVL ,V5, V6.

Echocardiographic examination

Fig. 2. Echocardiographic examination demonstrated diffuse LV hypokinesis with moderate systolic dysfunction (ejection fraction 40%).

The LV was mildly dilated with end-diastolic diameter of 61mm. The left atrium was also mildly dilated (33ml/m2), while the right chambers were of normal size. There were no moderate or severe valvular abnormalities. Pulmonary artery systolic pressure was not elevated. Pericardial effusion was not detected. Chest X-ray revealed no abnormality. Laboratory examinations showed elevated  erythrocyte sedimentation rate (FW 64 mm/h) and mild increase in CRP ( 24mg/l). Troponin I was mildly elevated (0,18ug/l) and CK level was in a normal range. Minerals, glucose, urea, creatinine, liver enzymes and thyroid hormones were normal. A mild elevation of B-type natriuretic peptide was observed (414 ng/l). Blood count was normal. Serological tests for Borrelia burgdorferi, HIV and for hepatitis A, B and C were negative.

 Cardiac MRI of the patientCardiac MRI  examination confirmed the presence of diffuse hypokinesis of the mildly dilated LV with ejection fraction 42%. T2- weighted imaging did not reveal signs of myocardial oedema. Late gadolinium enhacement (LGE) was present in midwall of the middle part of the interventricular septum and anterolateral wall of the LV, some  LGE could be found also in the basal part of the anterolateral papillary muscle (Fig. 3).

Fig. 3. Cardiac MRI of the patient.

 

 



QUESTIONS
1) Can we make the final diagnosis based on the above cited results?
2) If not, which examination would you recommend to perform further?       

Conclusion

1) Can we make the final diagnosis based on the above cited results?

Based on the patient’s history and results of echocardiographic and MRI exams, our patient suffers from new-onset DCM of so far unknown etiology.
The clinical course has shown deterioration of the LV systolic function accompanied by signs of heart failure despite conventional heart failure therapy.  In addition, the pattern of late gadolinium enhancement observed on cardiac MRI exam does not completely exclude the presence of myocardial inflammation, although T2-weighted images do not reveal clear signs of myocardial edema. Therefore we believe endomyocardial biopsy is clearly indicated in this patient in order to elucidate the nature of underlying pathological myocardial process and consequently to make the final diagnosis. 

2) If not, which examination would you recommend to perform further?

Endomyocardial biopsy (EMB) was performed and eight samples from the right ventricle were taken and examined by histology, immunohistochemistry and by PCR focused on HSV 1, CMV, EBV, HHV6, parvovirus B19, adenovirus, enteroviruses and Borrelia burgdorferi (Bb). PCR was positive only for Bb genome. Immunohistochemistry revealed noncaseating granulomas consisting of aggregates of epiteloid histiocytes with mild infiltration of lymphocytes and presence of large multinuclear giant cells (Figure 1).



Fig. 1.
Noncaseating granulomas with  large multinuclear giant cells in the  EMB (hematoxylin and eosin  staining, x 400)

According to the EMB results we established the diagnosis of cardiac sarcoidosis with the concomitant presence of Bb genome in EMB.
To determine the involvement of extracardiac organs by sarcoidosis, CT scan of the thorax and abdomen was performed and the presence of mild mediastinal lymphadenopathy corresponding most probably to initial form of pulmonary sarcoidosis was found.
Based on the EMB results, combination therapy consisting of antibiotics and corticosteroids was added to the conventional heart failure therapy. The patient was put on ceftriaxone given intravenously 2g daily for 3 weeks and prednisone administered orally 40mg daily for 6 months with subsequent dose of 10 mg daily for the next half of the year. Even after 2 months of  above mentioned treatment the patient was asymptomatic and echocardiographic examination revealed normalization of the LV diameters and complete recovery of the LV  systolic function (Figure 2).



Fig. 2.
LV  M-mode from the parasternal long axis-view showing LVEDD and LVESD

Discussion

Cardiac sarcoidosis is reported to be relatively rare being present in only about 2% of patients with this multisystem disease (1). However, among patients dying with sarcoidosis, in whom an autopsy was performed, the prevalence has been estimated to be between 20% and 25% (2).
PET and MRI have been considered to be the most sensitive noninvasive tools for the diagnosis of cardiac sarcoidosis. Cardiac MRI can demonstrate both scar and myocardial edema. Acute myocardial inflammation presents as focal areas of thickening and increased signal intensity on T2-weighted images and/or early gadolinium-enhanced images. On the other hand,  delayed gadolinium enhancement representing scaring of the myocardium  is found predominantly in the midmyocardium and epicardial areas  and the  predilection for abnormalities is in basal and lateral segments of the LV or within the papillary muscles (3,4).
As shown in our case, it would be almost impossible to make the correct diagnosis of cardiac sarcoidosis if EMB was not being performed. EMB showing noncaseating granulomas is referred to be patognomonic. However, EMB may be negative due to patchy distribution of granulomas and therefore negative biopsy should not be considered a clear exclusion of myocardial involvement if the results of other exams strongly support the presence of cardiac sarcoidosis.
 Lyme myocarditis is a rare manifestation of the infection caused by Borrelia burgdorferi which occurs in 0.5- 4% of the cases in Europe (5). Among conduction disturbances and other cardiac complications of the disease pericarditis and inflammatory dilated cardiomopathy can be also present. Although Bb was isolated from the myocardium of a patient with unexplained DCM as early as in 1990 (6) , the exact prevalence of Bb genome in EMB of patients with DCM is still  unknown and differs substantially among published studies (7,8).
Association between cardiac sarcoidosis and the presence of Bb genome in EMB seems to be unclear. In our series of patients, who underwent EMB because of new-onset DCM, Bb genome was present in 21% of subjects and in none of them granulomatous type of myocardial inflammation was  detected (8).


Notes to editor
Presented by: Petr Kuchynka, Tomas Palecek and Ales Linhart
2nd Department of Internal Medicine –
Clinical Department of Cardiology and Angiology,
First Faculty of Medicine and General University Hospital,
Charles University, Prague, Czech Republic
References

1.Baughman RP, Teirstein AS, Judson MA, et al: Clinical characteristics of patients in a case control study of sarcoidosis. Am J Respir Crit Care Med 2001;15:1885-1889.

2.Roberts WC, McAllister Jr HA, Ferrans VJ: Sarcoidosis of the heart. A clinicopathologic study of 35 necropsy patients (group 1) and review of 78 previously described necropsy patients (group 2). Am J Med 1977;63:86-108.

3.Smedema JP, Snoep G, van Kroonenburgh MP, et al: Evaluation of the accuracy of gadolinium-enhanced cardiovascular magnetic resonance in the diagnosis of cardiac sarcoidosis. J Am Coll Cardiol  2005;45:1683-1690

4.Doughan AR, Williams BR: Cardiac sarcoidosis. Heart 2006;92:282-288.

5.Wang G, van Dam AP, Schwartz I, et al. Molecular typing of Borrelia burgdorferi
sensulato: taxonomic, epidemiological, and clinical implications. Clin Microbiol Rev 1999;12:633–53.

6.Stanek G, Klein J, Bittner R, et al. Isolation of Borrelia burgdorferi from the myocardium of a patient with longstanding cardiomyopathy. N Engl J Med 1990;322:249–52.

7.Suedkamp M, Lissel C, Eiffert H, et al. Cardiac myocytes of heart from patients with end-stage dilated cardiomyopathy do not contain Borrelia burgdorferi DNA. Am Heart J 1999;138:269–72.

8. Palecek T, Kuchynka P, Hulinska D, et al. Presence of Borrelia burgdorferi in endomyocardial biopsies in patients with new-onset unexplained dilated cardiomyopathy.Med Microbiol Immunol 2010 ;199:139-43.

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.