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European Journal of Heart Failure 2005 7(6):1023-1026; doi:10.1016/j.ejheart.2005.06.004
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© 2005 European Society of Cardiology

Ventricular remodeling in Loeffler endocarditis: Implications for therapeutic decision making

Carla Lofiego, Marinella Ferlito, Guido Rocchi, Elena Biagini, Enrica Perugini, Angelo Branzi and Claudio Rapezzi*

Institute of Cardiology, University of Bologna Policlinico S. Orsola-Malpighi, Via Massarenti 9, 40138 Bologna, Italy

* Corresponding author. Tel.: +39 051 349858; fax: +39 051 344859. E-mail address: crapezzi{at}aosp.bo.it


   Abstract
Top
 Abstract
1. Background
 2. Methods and results
 3. Discussion
 Acknowledgment
 References
 
Background: Little is known about the morphological and functional evolution of ventricular abnormalities in Loeffler endocarditis.

Methods and Results: We describe 5 patients, including 3 with long-term echocardiographic follow-up, in whom apical obliteration due to fibro-thrombotic thickening of the endocardium showed favorable patterns of evolution. In one patient there was almost complete resolution of the obliterative process with consequently increased effective ventricular volume. In two patients formation of a flow-passage in the fibrocalcific apical ‘floor’ between the main medioventricular cavity and the apical chamber, leading to a ‘double-chambered’ left ventricle was observed.

Conclusions: Medical therapy and appropriate anticoagulation, can induce favorable long-term ventricular remodeling in Loeffler endocarditis.

Key Words: Loeffler endocarditis • Doppler echocardiography • Thrombosis • Hypereosinophilia • Endomyocardial fibrosis

Received December 25, 2004; Revised May 18, 2005; Accepted June 15, 2005


   1. Background
Top
 Abstract
 1. Background
2. Methods and results
 3. Discussion
 Acknowledgment
 References
 
Loeffler endocarditis is an uncommon myocardial disease, thought to be secondary to eosinophil damage, often due to eosinophilic leukemia or idiopathic hypereosinophilic syndrome. Loeffler endocarditis is characterized by fibrous thickening of the endocardium of one or both ventricles, leading to apical obliteration and a restrictive pathophysiology leading to heart failure, thromboembolic events and/or atrial fibrillation. Prognosis is poor due to high mortality from heart failure, sudden death or thromboembolism [1]. Little is known about the evolution of the morphological ventricular abnormalities and their possible clinical implications. Recent improvements in the medical treatment of hypereosinophilic diseases [2] highlight the need for detailed knowledge of their various sequelae.

The aim of this study was to report on the evolution of the echocardiographic pattern in five patients diagnosed with Loeffler endocarditis at our center.


   2. Methods and results
Top
 Abstract
 1. Background
 2. Methods and results
3. Discussion
 Acknowledgment
 References
 
Two-dimensional and Doppler echocardiograms were performed according to the recommendations of the American Society of Echocardiography. Volumes of the left and right atria were calculated from the apical 4-chamber view by the area-length method (with values indexed to body surface area being expressed as ml/m2). We evaluated the morphology of the fibrotic zone. Based on planimetric measurements (from the apical 4-chamber view) of the areas of the total and effective cavities, we calculated the percentage of obliteration. Contrast echocardiography was performed whenever possible for visualization of the ventricular cavity.

Since 1989, five patients referred to our institute fitted the following diagnostic criteria for Loeffler endocarditis: apical obliteration of one or both ventricles by echogenic material suggestive of fibrosis or thrombosis; bright echoes at the cavity surface of the apical obliteration suggesting calcification; preserved left apical systolic inward motion; preserved ventricular contractile function and a combination of normal-to-small ventricles with large atria [3–5]. Table 1 reports the patients' main characteristics at diagnosis. Echocardiographically, two patients exhibited homogeneous and hypoechogenic material suggestive of thrombosis (Fig. 1 A), while in the remaining three patients the apex of one or both ventricles was obliterated by fibrocalcific thickening of the endocardium. No patient presented signs of ischemic heart disease. All 5 patients had symptoms/signs of heart failure at diagnosis.


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  		Table 1 Patients' characteristics at diagnosis of Loeffler endocarditis [and anamnesis]

 


Figure 1
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  		Fig. 1 Patient 3. A. Apical 4-chamber view at diagnosis shows apical obliteration of the left ventricle by echogenic material suggestive of thrombosis extending to the mid-lateral wall (arrow). B. The same view at 7 years from diagnosis reveals almost complete reabsorption of the apical mass: the fibrocalcific lesions (arrow) are limited to the mid-apical lateral wall with consequent enlargement of the effective ventricular cavity. LA: left atrium; LV: left ventricle; RA: right atrium; RV: right ventricle.

 
Two patients had a previous diagnosis of eosinophilic leukemia, which was treated by standard chemotherapy protocols (anthracycline plus cytosine arabinoside, 2–3 cycles). Patient 1, who was cured before the cardiac diagnosis, underwent (at 2 years from diagnosis) surgical resection of endocardial fibrous tissue for congestive heart failure refractory to medical therapy, and subsequently underwent heart transplantation (at 12 years from diagnosis). Patient 2 died from leukemia-related complications 12 months after diagnosis. The remaining three patients all had an anamnesis of unexplained hypereosinophilia (already successfully treated with 1–2 months of prednisone). These patients had a long-term echocardiographic follow up (7, 3 and 4 years, respectively). Chronic heart failure either remained stable or improved (to NYHA Class II), and no relapsing hypereosinophilia was detected. All 3 patients have been on permanent treatment with warfarin (INR range 2–3) since diagnosis. Echocardiographically, patient 3 (Fig. 1) initially exhibited apical thrombotic obliteration of the left ventricle (LV), which was largely reabsorbed at 7 years after diagnosis, the fibrocalcific mass was limited to the apical-lateral zone with consequent enlargement of the effective LV cavity. The remaining two patients showed a different echocardiographic evolution. Patient 4 (Fig. 2) initially showed apical obliteration of both ventricles due to fibrocalcific thickening of the endocardium. At 3 years from diagnosis, both color-Doppler and contrast echocardiography documented flow communication in the apical fibrocalcific ‘floor— of the LV between the main ventricular cavity and the apical chamber, leading to a ‘double-chambered— appearance (the right ventricle remained unchanged). A similar evolution occurred in patient 5 (Fig. 3), who initially showed a fibrocalcific obliteration confined to the LV. At 4 years from diagnosis (when the patient was reevaluated by us following transitory cerebral events), contrast echocardiography revealed opacification of the apical chamber (accompanied by small thrombotic masses within the apical chamber).


Figure 2
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  		Fig. 2 Patient no. 4. The apical 4-chamber view at diagnosis (A) shows apical obliteration (asterisk) of both ventricles by fibrocalcific thickening of the endocardium. At 3 years from diagnosis (B, C), the color-Doppler reveals a flow communication (arrow) in the apical fibrocalcific ‘floor— between main ventricular cavity and apical chamber, producing a sort of double chambered left ventricle; contrast echocardiography (D) confirms the echo-Doppler diagnosis showing full opacification of the apical chamber. Abbreviations as in Fig. 1.

 


Figure 3
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  		Fig. 3 Patient no. 5. The apical 4-chamber view at diagnosis (A) shows apical fibrocalcific obliteration (asterisk) confined to the left ventricle. At 4 years from diagnosis (B, C), the color-Doppler shows a flow communication between the main cavity of the left ventricle and the apical chamber (arrow); contrast echocardiography (D) confirms the echo-Doppler diagnosis, showing full opacification of the apical chamber. Abbreviations as in Fig. 1.

 

   3. Discussion
Top
 Abstract
 1. Background
 2. Methods and results
 3. Discussion
Acknowledgment
 References
 
Very little is known about the morphological evolution of Loeffler endocarditis and published data are limited to angiographic follow-up and to the tropical variant of the disease, endomyocardial fibrosis [1,3–5]. To our knowledge, this is the first report on the morphological evolution of Loeffler endocarditis. It provides new information on the natural history of this rare but clinically relevant disease.

Gupta et al. previously reported the angiographic follow-up of a small subgroup of endomyocardial fibrosis patients, describing a pattern of predominantly progressive worsening, due to either increasing obliteration, involvement of the contralateral ventricle, or progression of valvular regurgitation following impairment of the subvalvular apparatus of atrio-ventricular valves [1]. By comparison, our echocardiographic follow-up of three Loeffler endocarditis patients demonstrated the existence of favorable evolutive patterns; in one patient there was considerable resolution of the apical obliteration, with enlargement of the effective ventricular cavity and in two patients development of a flow-communication in the apical fibrocalcific ‘floor— between the main ventricular cavity and the apical chamber of the LV occurred. Both these evolutions were associated with enlargement of the effective LV cavity and consequent reduction of the ventricular filling pressure and symptoms of heart failure. On technical grounds, it should be noted that contrast echocardiography played a very important role in two of our patients, enabling accurate definition of the effective volume of the cavity, revealing the passage of flow between the main cavity and the apical chamber and enhancing the visualization of thrombi. Clinically, it is likely that anticoagulant therapy played a fundamental role in our patients by limiting progressive thrombotic apposition, and consequently allowing favorable remodeling of the apical fibro-thrombotic mass.

In conclusion, our findings suggest that medical therapy and appropriate anticoagulation can induce favorable long-term LV remodeling associated with a very stable clinical outcome. We believe that therapeutic decision making has to weigh a surgical approach (which is still burdened by high morbidity and mortality and disease recurrence [6] against the possibility of a favorable clinical outcome on medical therapy alone.


   Acknowledgment
Top
 Abstract
 1. Background
 2. Methods and results
 3. Discussion
 Acknowledgment
References
 
We thank Robin MT Cooke for writing assistance.


   References
Top
 Abstract
 1. Background
 2. Methods and results
 3. Discussion
 Acknowledgment
 References
 

  1. Gupta P.N., Valiathan M.S., Balakrishnan K.G., Kartha C.C., Ghosh M.K. Clinical course of endomyocardial fibrosis. Br Heart J (1989) 62:450–454.[Abstract/Free Full Text]
  2. Cools J., DeAngelo D.J., Gotlib J., Stover E.H., Legare R.D., Cortes J., et al. A tyrosine kinase created by fusion of the PDGFRA and FIP1L1 genes as a therapeutic target of imatinib in idiopathic hypereosinophilic syndrome. N Engl J Med (2003) 348:1201–1214.[Abstract/Free Full Text]
  3. Acquatella H., Schiller N.B., Puigbo J.J., Gomez-Mancebo J.R., Suarez C., Acquatella G. Value of two-dimensional echocardiography in endomyocardial disease with and without eosinophilia. A clinical and pathologic study. Circulation (1983) 67:1219–1226.[Abstract/Free Full Text]
  4. Davies J., Gibson D.G., Foale R., Heer K., Spry C.J., Oakley C.M., et al. Echocardiographic features of eosinophilic endomyocardial disease. Br Heart J (1982) 48:434–440.[Abstract/Free Full Text]
  5. Berensztein C.S., Pineiro D., Marcotegui M., Brunoldi R., Blanco M.V., Lerman J. Usefulness of echocardiography and Doppler echocardiography in endomyocardial fibrosis. J Am Soc Echocardiogr (2000) 13:385–392.[Web of Science][Medline]
  6. Schneider U., Jenni R., Turina J., Turina M., Hess O.M. Long-term follow up of patients with endomyocardial fibrosis: effects of surgery. Heart (1998) 79:362–367.[Abstract/Free Full Text]

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