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 Table of Contents  
Year : 2021  |  Volume : 7  |  Issue : 1  |  Page : 83-84

Double whammy: Rheumatic heart disease associated with left ventricular noncompaction

1 Department of Anesthesia, P D Hinduja Hospital, Mumbai, Maharashtra, India
2 Department of Cardiology, Apex Heart Institute, Ahmedabad, Gujarat, India

Date of Submission19-Aug-2020
Date of Decision01-Feb-2020
Date of Acceptance11-Feb-2021
Date of Web Publication24-Apr-2021

Correspondence Address:
Pradyot Tiwari
Apex Heart Institute, Ahmedabad, Gujarat
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jpcs.jpcs_82_20

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We present a rare case of a 48 year old man, coming with Rheumatic heart disease, mitral regurgitation, aortic regurgitation and LV non compaction. This is a very rare combination of diseases.

Keywords: LV Non-compaction, MR. AR, rheumatic heart disease

How to cite this article:
Banerji A, Tiwari P. Double whammy: Rheumatic heart disease associated with left ventricular noncompaction. J Pract Cardiovasc Sci 2021;7:83-4

How to cite this URL:
Banerji A, Tiwari P. Double whammy: Rheumatic heart disease associated with left ventricular noncompaction. J Pract Cardiovasc Sci [serial online] 2021 [cited 2021 Sep 25];7:83-4. Available from: https://www.j-pcs.org/text.asp?2021/7/1/83/314478

A 48-year-old male presented to us with dyspnea on exertion and palpitations for the past 8 months. On cardiovascular examination, a loud pansystolic murmur of mitral regurgitation was heard at apex and a long blowing diastolic murmur suggestive of aortic regurgitation was heard in the neo-aortic area. EKG was suggestive left ventricular volume overload and chest X-ray demonstrated cardiomegaly with a cardiothoracic ratio 0.65.

Transthoracic echocardiography revealed valvular changes suggestive of rheumatic heart disease. Anterior mitral leaflet was thickened with diastolic doming while posterior mitral leaflet had restricted mobility with mal-coaptation giving rise to moderate mitral regurgitation [Figure 1]a and Video 1]. Aortic valve was tricuspid with thickened and retracted leaflet tips giving rise to severe aortic regurgitation [Figure 1]b. Left ventricle (LV) was globally hypokinetic (ejection fraction = 25%–30%) with prominent and broad trabeculae with deep intertrabecular recess in the apical and inferolateral areas of LV [[Figure 1]c and [Figure 1]d and Videos 2 and 3]. Color Doppler evaluation revealed perfusion of intertrabecular recess [Figure 1]e and Video 4]. The final diagnosis of rheumatic severe aortic regurgitation and moderate mitral regurgitation with LV noncompaction was made. The patient was advised aortic valve replacement along with mitral valve repair but refused by the patient and is currently stabilized on torsemide, spironolactone, carvedilol, and ramipril.
Figure 1: (a) Apical 4 chambered view with colour Doppler demonstrating moderate mitral regurgitation. (b) Parasternal long axis view revealing severe aortic regurgitation. (c) Modified apical 4 chambered view clearly depicting the prominent noncompact layer with the ratio of noncompact to compact layer >2 and more prominent in apical and lateral areas of left ventricle. (d) Parasternal short axis view depicting the same observation and with prominent changes in inferior and lateral walls. (e) Apical 4 chambered view with colour Doppler demonstrating perfusion of the intertrabecular recess.

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Left ventricular noncompaction cardiomyopathy (LVNC) is a rare myocardial disorder characterized by prominent left ventricular trabeculae, deep intertrabecular recesses, and the thin compacted layer.[1] It results from the arrest of the normal compaction process of the developing myocardium, resulting in deep intertrabecular recesses which communicate with the ventricular cavity unlike sinusoids.[2]

LVNC may be isolated or associated with a number of congenital and valvular heart diseases. Heart failure, cardioembolic events, and ventricular arrhythmias are the common modes of presentation.

Echocardiographic diagnostic criteria have been laid for LVNC and include noncompacted endocardial layer with trabeculations and deep recesses, noncompaction to compaction ratio of ≥2:1 at end systole, color Doppler evidence of flow in intertrabecular recesses, and predominant localization in the apical, mid-lateral, and mid-inferior regions.[3] However, the diagnosis is increasingly verified or made by the use of cardiac magnetic resonance imaging. Available data indicate that LVNC generally has a poor prognosis.[4]

Our case is a very rare case depicting the association of rheumatic aortic and mitral regurgitation with left ventricular noncompaction. This association is of clinical significance because this might exacerbate the left ventricular dysfunction caused by chronic aortic and mitral regurgitation. Postoperative outcomes might also be worse as recovery of cardiac mechanical function is less likely in the presence of a preexisting myocardial disease (LVNC).

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed

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Conflicts of interest

There are no conflicts of interest.

  References Top

Jenni R, Oechslin EN, Van Der Loo B. Isolated ventricular non-compaction of the myocardium in adults. Heart 2007;93:11-5.  Back to cited text no. 1
Weiford BC, Subbarao VD, Mulhern KM. Noncompaction of the ventricular myocardium. Circulation 2004;109:2965-71.  Back to cited text no. 2
Jenni R, Oechslin E, Schneider J, Attenhofer Jost C, Kaufmann PA. Echocardiographic and pathoanatomical characteristics of isolated left ventricular non-compaction: A step towards classification as a distinct cardiomyopathy. Heart 2001;86:666-71.  Back to cited text no. 3
Aras D, Tufekcioglu O, Ergun K, Ozeke O, Yildiz A, Topaloglu S, et al. Clinical features of isolated ventricular noncompaction in adults long-term clinical course, echocardiographic properties, and predictors of left ventricular failure. J Card Fail 2006;12:726-33.  Back to cited text no. 4


  [Figure 1]

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