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 Table of Contents  
CASE REPORT
Year : 2022  |  Volume : 8  |  Issue : 1  |  Page : 55-58

Silent rheumatic severe mitral stenosis with left ventricular noncompaction cardiomyopathy and ramifying normal coronaries: A double whammy in an octogenarian


Department of Cardiology, AIIMS, Bhubaneswar, Odisha, India

Date of Submission04-Mar-2022
Date of Decision03-Apr-2022
Date of Acceptance06-Apr-2022
Date of Web Publication26-Apr-2022

Correspondence Address:
Debasish Das
Department of Cardiology, AIIMS, Bhubaneswar - 751 019, Odisha
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jpcs.jpcs_15_22

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  Abstract 


We report a unique association of rheumatic heart disease with left ventricular (LV) noncompaction cardiomyopathy in an octogenarian. He had severe mitral stenosis with severe LV systolic dysfunction secondary to noncompaction. Although the association of rheumatic heart disease with LV, noncompaction cardiomyopathy has been described sparsely in the literature, our case is a unique illustration of the presence of LV noncompaction cardiomyopathy contributing toward severe LV systolic dysfunction in rheumatic mitral stenosis in an octogenarian with no coronary artery disease.

Keywords: Cardiomyopathy, left ventricular noncompaction, rheumatic mitral stenosis


How to cite this article:
Das D, Banerjee A, Kumar A, Singh S, Dixit M. Silent rheumatic severe mitral stenosis with left ventricular noncompaction cardiomyopathy and ramifying normal coronaries: A double whammy in an octogenarian. J Pract Cardiovasc Sci 2022;8:55-8

How to cite this URL:
Das D, Banerjee A, Kumar A, Singh S, Dixit M. Silent rheumatic severe mitral stenosis with left ventricular noncompaction cardiomyopathy and ramifying normal coronaries: A double whammy in an octogenarian. J Pract Cardiovasc Sci [serial online] 2022 [cited 2022 Aug 16];8:55-8. Available from: https://www.j-pcs.org/text.asp?2022/8/1/55/344126




  Introduction Top


Left ventricular (LV) noncompaction cardiomyopathy is a rare disorder characterized by loss of compaction of myofiber meshwork during intrauterine life.[1],[2] Because of varied clinical presentation and nonavailability of widespread echocardiography, this entity is underdiagnosed in clinical practice. We report an extremely rare case of the unique presence of LV noncompaction cardiomyopathy in an octogenarian presenting with severe rheumatic mitral stenosis and severe LV systolic dysfunction with acute pulmonary edema. Our case is the first illustration of the association of LV noncompaction cardiomyopathy with rheumatic mitral stenosis in such an extreme age.


  Case Report Top


An 82-year-old male presented to the cardiology emergency with acute-onset shortness of breath for the past 4 h without any history of angina, palpitation, presyncope, or syncope. During the presentation, he had a pulse rate of 110 beats/min and regular low volume in nature with a blood pressure of 110/70 mmHg in the right arm supine position. Cardiovascular system examination revealed the presence of huge cardiomegaly with LV type of apex in left sixth intercostal space 1 inch lateral to the midclavicular line, forceful and well sustained in nature with loud first heart sound and long rough rumbling middiastolic murmur at the apex. Chest X-ray revealed cardiomegaly with cardiothoracic computed tomography ratio of 0.7 with features of acute pulmonary edema. Electrocardiography (ECG) revealed the presence of left atrial enlargement with left bundle branch block with LV hypertrophy [Figure 1]. The presence of huge cardiomegaly and LV hypertrophy in ECG could not be explained with the presence of mitral stenosis. Echocardiography revealed the presence of severe rheumatic mitral stenosis with valve area of 0.4 cm2 [Figure 2] and [Figure 3]. Spongiform myocardium was well evident also in the parasternal short-axis view of the left ventricle with prominent trabeculae and recesses [Figure 3]. Apical four-chamber view revealed the presence of spongiform myocardium with prominent trabeculae and recesses [Figure 4] with blood flow into the recesses [Figure 5] with Jenni ratio (ratio of the thickness of the noncompacted layer of the left ventricle/thickness of the compacted layer of the left ventricle >2) suggestive of LV noncompaction cardiomyopathy. In view of the acute deterioration of LV systolic function in mitral stenosis, coronary angiogram was done to rule out the presence of severe form of coronary artery disease. Coronary angiogram was apparently peculiar with normal coronaries and ramifying branches like the bars of a parrot cage surrounding the whole ventricle [Figure 6] and [Figure 7] suggestive of adequate perfusion.[3] LV angiogram delineated the characteristic spongiform appearance of the left ventricle [Figure 8]. Right heart catheterization revealed that he had mild pulmonary hypertension with mean pulmonary artery pressure of 35 mmHg. He was managed with a diuretic, beta-blocker, angiotensin-converting enzyme inhibitor, and oxygen therapy. He improved over 3 days and was discharged home with a diuretic, beta-blocker, and vasodilator therapy. In view of elderly age with severe LV systolic dysfunction, he was not considered for high-risk mitral valve replacement and was decided to put on medical management.
Figure 1: ECG showing LAE, LBBB, and LVH. ECG: Electrocardiography, LAE: Left atrial enlargement, LBBB: Left bundle branch block, LVH: Left ventricular hypertrophy

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Figure 2: Hockey stick rheumatic AML. AML: Acute myeloid leukemia

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Figure 3: Fish mouth severe mitral stenosis with trabeculae and deep recesses of LVNC in parasternal short axis (PSAX) view

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Figure 4: Spongiform myocardium

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Figure 5: Blood flow into the recesses

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Figure 6: Arborizing RCA. RCA: right coronary artery

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Figure 7: Ramifying left circulation

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Figure 8: LV angiogram showing the spongiform appearance of the LV, LV: Left ventricular

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  Discussion Top


Our case is the first literature description of the presence of LV noncompaction causing severe LV systolic dysfunction in a case of severe rheumatic mitral stenosis in an octogenarian. Mitral stenosis per se does not lead to LV systolic dysfunction. The presence of LV systolic dysfunction in rheumatic mitral stenosis mandates excluding the presence of significant coronary artery disease, associated severe valvular regurgitation in the form of severe mitral regurgitation or aortic regurgitation, and associated nutritional anemia causing dilatation of heart with LV systolic dysfunction. Our case is a teaching to the young generation that isolated mitral stenosis if presents with severe LV systolic dysfunction, LV noncompaction cardiomyopathy can also be a possibility. The World Health Organization has labeled LV noncompaction as unclassified type of cardiomyopathy[4] characterized by deep intertrabecular recesses in hypertrophied and hyperkinetic segments of the ventricle.[5] The clinical presentation of LV noncompaction cardiomyopathy includes progressive LV systolic dysfunction, thromboembolism, and life-threatening ventricular arrhythmias.[6] The most common clinical presentation of LV noncompaction cardiomyopathy is LV systolic dysfunction with pulmonary edema as noted in our case. One peculiar finding in our case was that coronary angiogram revealed ramifying coronaries surrounding the heart-like bars of a parrot cage in left anterior oblique (LAO) cranial view. In the event of associated coronary artery disease in the elderly, it would have presented much earlier rather than presenting in the 80s. Noncompaction may involve both ventricles[7] or may involve the apex only;[8] but our case was the isolated involvement of the left ventricle without involving the right one. These subsets of patients disgustingly present with apical septal ventricular tachycardia with narrow QRS resembling paroxysmal supraventricular tachycardia, and inadvertent addition of verapamil which is a myocardial depressant further depresses the LV systolic function.[9] Jenni proposed a simpler and popular criterion of the ratio of thickness of noncompacted myocardium to compacted myocardium more than two to be diagnostic of noncompaction cardiomyopathy. It is hypothetical that as the left ventricle had robust blood supply with ramifying coronaries, the noncompaction cardiomyopathy with robust perfusion presented with severe LV systolic dysfunction in such an extremely elderly age. LV cineangiogram reveals a characteristic honeycomb appearance of the left ventricle in LV noncompaction.[10] One unusual aspect about the clinical presentation in our case was although severe mitral stenosis presents in the second or third decade of life, the index patient was quite asymptomatic up to this age; he had no effort dyspnea before this episode behaving as silent mitral stenosis. LV noncompaction cardiomyopathy usually presents in the fourth or fifth decade of life but the index case presented much later, i.e., in 80s like a smoldering form of cardiomyopathy; the octogenarian has been hit with a double whammy, i.e., the additive insult of severe rheumatic mitral stenosis and severe LV systolic dysfunction secondary to non-compaction. Our case is the first literature description of rheumatic mitral stenosis with LV noncompaction at such an extreme age.


  Conclusion Top


Our case is the first literature description of the unique association of rheumatic heart disease with LV noncompaction at an extreme age. Isolated mitral stenosis per se does not cause LV systolic dysfunction; in the presence of normal coronaries, associated LV noncompaction can also be a possibility.

Ethics clearance

Institutional Ethical Committee (IEC) clearance has been obtained.

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.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Vijayvergiya R, Jha A, Pandian RP, Sharma R, Grover A. Isolated left ventricular noncompaction in association with rheumatic mitral stenosis. Int J Cardiol 2008;123:e54-6.  Back to cited text no. 1
    
2.
Freedom RM, Yoo SJ, Perrin D, Taylor G, Petersen S, Anderson RH. The morphological spectrum of ventricular noncompaction. Cardiol Young 2005;15:345-64.  Back to cited text no. 2
    
3.
Das D, Acharya D, Mahanta DS, Singh S, Das T, Pramanik S. Anomalous origin of the right coronary artery from the left coronary sinus with medusa head left coronaries: Plethora of left coronary circulation with paucity of right one. Res Cardiovasc Med 2021;10:91-5.  Back to cited text no. 3
  [Full text]  
4.
Richardson P, McKenna W, Bristow M, Maisch B, Mautner B, O'Connell J, et al. Report of the 1995 World Health Organization/International Society and Federation of Cardiology Task Force on the Definition and Classification of cardiomyopathies. Circulation 1996;93:841-2.  Back to cited text no. 4
    
5.
Jenni R, Oechslin E, Schneider J, Attenhofer Jost C, Kaufmann PA. Echocardiographic and pathoanatomical characteristics of isolated left ventricular noncompaction: A step towards classification as a distinct cardiomyopathy. Heart 2001;86:666-71.  Back to cited text no. 5
    
6.
Lofiego C, Biagini E, Pasquale F, Ferlito M, Rocchi G, Perugini E, et al. Wide spectrum of presentation and variable outcomes of isolated left ventricular non-compaction. Heart 2007;93:65-71.  Back to cited text no. 6
    
7.
Sinkovec M, Kozelj M, Podnar T. Familial biventricular myocardial noncompaction associated with Ebstein's malformation. Int J Cardiol 2005;102:297-302.  Back to cited text no. 7
    
8.
Alehan D. Clinical features of isolated left ventricular noncompaction in children. Int J Cardiol 2004;97:233-7.  Back to cited text no. 8
    
9.
Bosnjak ZJ, Marijic J, Roerig DL, Stowe DF, Murthy VS, Kampine JP. Chronic verapamil treatment depresses automaticity and contractility in isolated cardiac tissues. Anesth Analg 1991;72:462-8.  Back to cited text no. 9
    
10.
Jenni R, Rojas J, Oechslin E. Isolated noncompaction of the myocardium. N Engl J Med 1999;340:966-7.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8]



 

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