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 Table of Contents  
CONTEMPORARY TOPIC
Year : 2020  |  Volume : 4  |  Issue : 1  |  Page : 33-35

Hypereosinophilic Syndrome: What to Expect in Echo


1 Department of Preventive and Non Invasive Cardiology, Jaipur Heart Institute, Jaipur, Rajasthan, India
2 Department of Cardiology, Jaipur Heart Institute, Jaipur, Rajasthan, India

Date of Submission18-Apr-2019
Date of Decision26-May-2019
Date of Acceptance16-Jun-2019
Date of Web Publication11-Apr-2020

Correspondence Address:
Dr. Deepak Agrawal
301, Sangam Residency, Opposite Kartarpura Phatak, Vijay Nagar, Jaipur - 302 006, Rajasthan
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jiae.jiae_18_19

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  Abstract 

Hypereosinophilic syndrome is characterized by eosinophilic tissue damage. The clinical presentation is variable. When there is cardiac involvement, which occurs in 40%–60% of cases, it is referred commonly as Loeffler's endocarditis. Recently, a unique case report was published by Dr. Garg et al., in which they described pulmonary valve involvement with thickening and stenosis detected by two-dimensional transthoracic echocardiography in an adult presenting with Loeffler's endocarditis. Based on this case report, we review this topic to elucidate various manifestations of hypereosinophilic syndrome, which we should expect during echocardiography.

Keywords: Hypereosinophilic syndrome, Loeffler's endocarditis, restrictive cardiomyopathy


How to cite this article:
Garg A, Agrawal D, Sharma GL. Hypereosinophilic Syndrome: What to Expect in Echo. J Indian Acad Echocardiogr Cardiovasc Imaging 2020;4:33-5

How to cite this URL:
Garg A, Agrawal D, Sharma GL. Hypereosinophilic Syndrome: What to Expect in Echo. J Indian Acad Echocardiogr Cardiovasc Imaging [serial online] 2020 [cited 2020 Jun 7];4:33-5. Available from: http://www.jiaecho.org/text.asp?2020/4/1/33/282196




  Introduction Top


Hypereosinophilic syndrome is characterized by eosinophilic tissue damage. The clinical presentation is variable. When there is cardiac involvement, which occurs in 40%–60% of cases, it is referred commonly as Loeffler's endocarditis. We review this topic to elucidate various manifestations of hypereosinophilic syndrome, which should be expected during echocardiography.


  Review Top


Hypereosinophilic syndrome is characterized by eosinophilic tissue damage. The clinical presentation is variable. The patient may be completely asymptomatic or present with a multisystem disorder.[1] When there is cardiac involvement, which occurs in 40%–60% of cases, it is referred commonly as Loeffler's endocarditis.[1],[2],[3],[4],[5] Early diagnosis is crucial to avoid serious complications and irreversible damage to the heart.

  • The hallmark of echocardiography finding is the obliteration of the apex of the left or right ventricle, or both, by laminar thrombus [Figure 1][6]
  • The early stage of cardiac involvement begins with eosinophilic infiltration, followed by an intermediate thrombotic stage, and finally, a late fibrotic stage, resulting in endomyocardial fibrosis. Endomyocardial fibrosis leads to a restrictive cardiomyopathy


  • Doppler indices of left ventricular compliance should be evaluated and can be used to follow the severity of the restrictive process [Figure 2] and [Figure 3].
    Figure 1: Two-dimensional transthoracic echocardiography (modified apical 4-chamber view) shows the hyperechogenic thrombus within the left ventricular apex (arrow). The right side of the heart is not affected. LA: Left atrium, LV: Left ventricle, RA: Right atrium, RV: Right ventricle

    Click here to view
    Figure 2: Transmitral flow pulsed-wave Doppler echocardiography shows high filling pressures consistent with a restrictive filling pattern. E =1.1 m/s; A = 0.5 m/s; E/A ratio > 2

    Click here to view
    Figure 3: Pulsed-wave tissue Doppler echocardiography at the septal mitral annulus shows that the E' velocity is <7 cm/s. The E/E' ratio is higher than 15, indicating that the pulmonary capillary wedge pressure exceeds 20 mmHg. This finding is consistent with a restrictive filling pattern

    Click here to view


  • Left atrium and/or right atrium dilatation results from restrictive cardiomyopathy and Mitral valve/Tricuspid valve regurgitation. It produces a typical combination of small ventricles and large atria[6]
  • In addition, hypereosinophilic syndrome tends for thickening of valves, commonly MV, next TV, less commonly AV, and very rarely PV.


  • There occurs involvement of the left ventricular posterior wall, the papillary muscles, and the posterior mitral valve leaflet, leading to mitral regurgitation[7] [Figure 4]. Subannular thrombus causing PML fixity leading to MR is described in HES.
Figure 4: Two-dimensional transthoracic echocardiography. Parasternal long- axis view shows severe mitral regurgitation. The aortic valve is mildly thickened. RV: Right ventricle, LV: Left ventricle, AO: Aorta, PE: Pericardium, LA: Left atrium, MR: Mitral regurgitation

Click here to view


Rupture chordae and flail MV have been reported.[8]

  • In addition, HES tends for thickening of valves, commonly MV, next TV, less commonly AV, very rarely PV.[9]
  • Recently, a unique case report was published by Dr. Garg et al., in which they described pulmonary valve involvement with thickening and stenosis detected by two-dimensional transthoracic echocardiography in an adult presenting with Loeffler's endocarditis[10] [Figure 5]
  • In an NIH study of 22 HES patients, 68% showed echocardiographic features of left ventricular free wall thickening, 37% showed an increase in left atrial transverse dimension, and 27% showed an increase in right ventricular transverse dimension[11]
  • Unsuspected pericardial effusions (from acute eosinophilic pericarditis, heart failure) were found by echocardiography in 32% of these patients.[11] Cardiac tamponade is rare.
Figure 5: Aortic short-axis view shows marked thickening of the pulmonary valve. Color Doppler-guided continuous wave Doppler interrogation shows a peak gradient of 48.50 mmHg consistent with moderate PV stenosis (arrow). LA: Left atrium, LV: Left ventricle, PA: Pulmonary artery, RA: Right atrium

Click here to view


Acknowledgment

I, Dr. Ashok Garg, am thankful to Dr. G. L. Sharma for collecting all relevant and useful data from various resources for writing this review article.

I am also thankful to Dr. Deepak Agrawal for his valuable support in preparing this review article as per criteria of journal and also for proofreading and submission of the article.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Kleinfeldt T, Nienaber CA, Kische S, Akin I, Turan RG, Körber T, et al. Cardiac manifestation of the hypereosinophilic syndrome: New insights. Clin Res Cardiol 2010;99:419-27.  Back to cited text no. 1
    
2.
Loeffler W. Mural fibroplastic endocarditis with eosinophilia: A peculiar disease. Schweiz Med Wochenschr 1936;66:817-20.  Back to cited text no. 2
    
3.
Karnak D, Kayacan O, Beder S, Delibalta M. Hypereosinophilic syndrome with pulmonary and cardiac involvement in a patient with asthma. CMAJ 2003;168:172-5.  Back to cited text no. 3
    
4.
Ommen SR, Seward JB, Tajik AJ. Clinical and echocardiographic features of hypereosinophilic syndromes. Am J Cardiol 2000;86:110-3.  Back to cited text no. 4
    
5.
Shah R, Ananthasubramaniam K. Evaluation of cardiac involvement in hypereosinophilic syndrome: Complementary roles of transthoracic, transesophageal, and contrast echocardiography. Echocardiography 2006;23:689-91.  Back to cited text no. 5
    
6.
Acquatella H, Schiller NB, Puigbó JJ, Gómez-Mancebo JR, Suarez C, Acquatella G, et al. Value of two-dimensional echocardiography in endomyocardial disease with and without eosinophilia. A clinical and pathologic study. Circulation 1983;67:1219-26.  Back to cited text no. 6
    
7.
Feigenbaum H, Armstrong WF, Ryan T. Feigenbaum's Echocardiography. 6th ed. Philadelphia: Lippincott Williams and Wilkins; 2005. p. 752.  Back to cited text no. 7
    
8.
Madhwal S, Goldberg J, Barcena J, Guha A, Gogate P, Cmolik B, et al. Unusual cause of acute mitral regurgitation: Idiopathic hypereosinophilic syndrome. Ann Thorac Surg 2012;93:974-7.  Back to cited text no. 8
    
9.
Hernandez CM, Arisha MJ, Ahmad A, Oates E, Nanda NC, Nanda A, et al. Usefulness of three-dimensional echocardiography in the assessment of valvular involvement in Loeffler endocarditis. Echocardiography 2017;34:1050-6.  Back to cited text no. 9
    
10.
Garg A, Nanda NC, Sungur A, Sharma GL, Mehta KJ, Öz TK, et al. Transthoracic echocardiographic detection of pulmonary valve involvement in Löeffler's endocarditis. Echocardiography 2014;31:83-6.  Back to cited text no. 10
    
11.
Parrillo JE, Borer JS, Henry WL, Wolff SM, Fauci AS. The cardiovascular manifestations of the hypereosinophilic syndrome. Prospective study of 26 patients, with review of the literature. Am J Med 1979;67:572-82.  Back to cited text no. 11
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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