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 Table of Contents  
CASE REPORT
Year : 2016  |  Volume : 13  |  Issue : 2  |  Page : 148-151

Isolated left ventricular noncompaction: Report of a case from Ibadan, Nigeria


1 Department of Medicine, Division of Cardiology, University College Hospital, College of Medicine, University of Ibadan, Ibadan, Oyo State, Nigeria
2 Department of Medicine, University College Hospital, College of Medicine, University of Ibadan, Ibadan, Oyo State, Nigeria
3 Department of Family Medicine, University College Hospital, College of Medicine, University of Ibadan, Ibadan, Oyo State, Nigeria
4 Department of Anatomy; Department of Medicine, College of Medicine, University of Ibadan, Ibadan, Oyo State, Nigeria

Date of Web Publication4-Aug-2016

Correspondence Address:
Okechukwu S Ogah
Department of Medicine, Division of Cardiology, University College Hospital Ibadan, PMB 5116, Ibadan, Oyo State
Nigeria
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0189-7969.187741

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  Abstract 

Isolated left ventricular noncompaction (ILVNC) is a form of cardiomyopathy caused by the intrauterine failure of the myocardium to compact (endomyocardial morphogenesis) resulting in multiple trabeculations of the left ventricular myocardium and deep intertrabecular recesses and thickened myocardium. The condition is relatively uncommon and is often complicated by heart failure, malignant arrhythmias, and thromboembolism. There have been a few reports of this condition from some parts of Africa (Djibouti, Sudan, and South Africa). To the best of our knowledge, there has not been previous report from Nigeria. The purpose of this paper is to report a case of ILVNC from Ibadan, Nigeria.

Keywords: Cardiac failure, cardiomyopathy, heart disease, heart failure, isolated left ventricular noncompaction, left ventricular noncompaction


How to cite this article:
Ogah OS, Adebayo O, Aje A, Koya FK, Towoju O, Adesina JO, Adeoye AM, Adebiyi AA, Oladapo OO, Falase AO. Isolated left ventricular noncompaction: Report of a case from Ibadan, Nigeria. Nig J Cardiol 2016;13:148-51

How to cite this URL:
Ogah OS, Adebayo O, Aje A, Koya FK, Towoju O, Adesina JO, Adeoye AM, Adebiyi AA, Oladapo OO, Falase AO. Isolated left ventricular noncompaction: Report of a case from Ibadan, Nigeria. Nig J Cardiol [serial online] 2016 [cited 2022 Sep 26];13:148-51. Available from: https://www.nigjcardiol.org/text.asp?2016/13/2/148/187741


  Introduction Top


Isolated left ventricular noncompaction (ILVNC) is a form of cardiomyopathy characterized by prominent ventricular trabeculations and deep intertrabecular recesses or sinusoids in communication with the left ventricular (LV) cavity. The disorder is often complicated by heart failure, arrhythmias, and thromboembolic events. [1]

The first description of this condition was in 1975 by Dusek et al. [2] when he reported the persistence of spongy myocardium in a pathological specimen. Engberding and Bender [3] were the first to report this condition with the use of transthoracic echocardiography (TTE). The pathological basis of this disease is the failure of compaction during cardiac embryogenesis. This results in myocardial dysfunction due to "failure of the efficient rotational ventricular system for contractile performance." [1]


  Case report Top


A 19-year-old male undergraduate Nigerian (of Yoruba descent) presented at the Emergency Department of University College Hospital on the April 7, 2016, with 3 months history of easy fatigability, 2 months history of dyspnea, 2 weeks history of cough, and 5 days history of leg swelling.

He was apparently well until 3 months before presentation when he became easily fatigued on moderate exertion. About a month later, he developed dyspnea on mild exertion which progressed to dyspnea at rest. This was associated with leg edema, orthopnea, paroxysmal nocturnal dyspnea, and nocturnal cough which was productive of frothy sputum.

There was associated palpitation and easy satiety, but there was no chest pain, syncope, or dizziness. No history of reduction in his urine output and no hematuria or yellowness of the eyes.

He is not a known hypertensive, diabetic, or asthmatic patient. He has not had any surgery or blood transfusion in the past and has no known drug allergy.

He is the first of parent's five children in a monogamous family setting. There is no history of similar illness in the family. There is a positive history of hypertension in his maternal grandmother. He has never smoked cigarette or drank alcohol in the past.

Physical examination revealed a young man, dyspneic, not pale, anicteric, afebrile with bilateral pitting pedal edema up to middle-third of the leg. He was conscious and alert, fully oriented with no focal neurological deficits.

His respiratory rate was 36 cycles/min; trachea was central; percussion note was resonant, and he had bibasal crepitations.

His pulse rate was 80 beats/min irregularly irregular and small volume. Heart rate was 128 beats/min with a pulse deficit of 48. Blood pressure measured was 100/70 mmHg. His jugular venous pressure was raised. The first and second heart sounds were heard. There were mitral and tricuspid regurgitant murmurs.

His abdomen was full, soft, and moved with respiration, with mild epigastric and right hypochondrial tenderness. There was hepatomegaly (liver span was 18 cm). The spleen and kidneys were not enlarged.

His 12-lead electrocardiogram showed coarse atrial fibrillation with Ashman's phenomenon [Figure 1].
Figure 1: Twelve-lead electrocardiogram of the patient

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Prothrombin time and international normalized ratio were normal. HIV screening was nonreactive. Hepatitis B surface antigen and anti-hepatitis C were negative. His electrolytes were within normal limits.

Echocardiography performed on him showed dilated left atrium, increased LV wall thickness, and prominent trabeculations in the left ventricle with deep intertrabecular recesses. The LV wall was thickened [Figure 2] and [Figure 3]. There was impaired LV systolic function (LV ejection fraction of 30%). There was moderate mitral regurgitation and mild to moderate tricuspid regurgitation.
Figure 2: (a and b) Two-dimensional echocardiogram showing excessive trabeculation in the left ventricular

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Figure 3: (a and b) Doppler echocardiography showing direct blood flow from ventricular cavity into deep intratrabecular recess

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He was placed on the standard heart failure treatment which included diuretics, beta-blocker, angiotensin converting enzyme inhibitor (ACEI), and aldosterone antagonist to which he responded very well and was discharged after 5-day of hospital admission. He is now being followed up at our outpatient cardiac clinic.


  Discussion Top


ILVNC can be defined as a rare genetic, congenital cardiomyopathy that is characterized by "a pattern of excessively prominent and multiple trabecular meshwork and deep intertrabecular recesses within the LV myocardium in the absence of other structural heart diseases." [1],[4] It was first described just about two decades ago. [2],[3]

In general, ILVNC is a subtype of LV noncompaction (LVNC). The other type is nonisolated type. [4] Nonisolated LVNC is associated with anomalous origin of the left coronary artery and pulmonary atresia among others. [4] Cases of ILVNC can be x-linked or autosomal in inheritance. Genetic mutations associated with the disorder include mutations in ACTC E101K mutation, G4.5 (x-linked), alpha-dystrobrevin, ZASP, actin, lamin A/C, and the locus is on chromosome 11 p 15 [5] ACTC E101K mutations are not specific for the disease. This has been identified in apical hypertrophic cardiomyopathy and septal defects. [6] There are suggestions that the disorder may be acquired. [7]

In the development of the fetus, cardiac trabeculation starts at about the 4 th week when the length of the embryo is about 4 mm. By the 8 th week of embryogenesis, trabecular remodeling occurs. During normal development, the intratrabecular recess condenses into capillaries and by birth, the trabeculation in left ventricle should remain as capillaries (although some trabeculation is expected in the right ventricles after birth). [8] Trabeculation in the left ventricle during extra-uterine life is due to arrested normal compaction process.

Pathologically, there is LV hypertrophy (LVH), LV trabeculation, two-layer structure in the left myocardium, and the latter feature is usually in the apical and midventricular segment of the left ventricle. [1]

Without a high sense of suspicion, the condition can be mis-or under-diagnosed.

Isolated ventricular noncompaction (IVNC) was initially grouped as one of the "unclassified" cardiomyopathies by World Health Organization (WHO). WHO diagnostic criteria for IVNC include the following: Absence of coexisting cardiac abnormalities, a two layer structure with a compacted thin epicardial band and a much thicker noncompacted endocardial layer of trabecular meshwork with deep endomyocardial spaces (A maximal end systolic ratio of noncompacted to compacted layers of >2 is diagnostic), the predominant localization of the pathology was to mid-lateral (seven of seven patients), apical (six), and mid-inferior (seven) areas confirmed at echocardiographically and color Doppler evidence of deep perfused intertrabecular recesses [Figure 3]a and b].

More recently, Jenni et al., Chin et al. and Stöllberger et al. have proposed newer diagnostic criteria [7],[8] [Table 1].
Table 1: Echocardiographic diagnostic criteria for isolated left ventricular noncompaction


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Cases of IVNC may present with systolic and/or diastolic dysfunction. It may be asymptomatic initially, and some patients may even be diagnosed during routine echocardiography. The complications include malignant arrhythmias, LV failure, and cardioembolic events which "constitute the classical triad of complications." [5],[7] Cause of death is usually progressive ventricular dysfunction, arrhythmias and systemic, and pulmonary embolism. [1]

In the year 2006, Ker and his colleagues reported the first case of ILVNC from sub-Saharan Africa. [11] Since then several cases have been reported from the continent, (Sudan and South Africa). [12],[13],[14]

In a prospective registry of cases in Johannesburg, Peters et al. [15] showed that the mean age of presentation was 45.4 ± 13.1 years. About 44.4% were women, and 63% were in New York Heart Association Class II. In addition, they also reported a prevalence of 6.9% in their dedicated cardiomyopathy clinic. The site of noncompaction in the order of decreasing frequency was apical (100%), mid-inferior (74.1%), and mid lateral wall (64.8%). Furthermore, they observed the right ventricular noncompaction in 22.2%. Some of the complications noted by the workers include pulmonary hypertension (88.3%), right ventricular dilatation (74.1%), and depressed right ventricular function.

Several diagnostic modalities can be used in diagnosing LVNC, but the TTE remains the imaging modality of choice. [5],[8] Essentially diagnosis is based on the identification of multiple prominent ventricular trabeculations with intertrabecular spaces communicating with the ventricular cavity. Cardiac magnetic resonance imaging is also relevant and shows increased LV mass >20%, with trabeculation and compact LV mass as defined at end diastole and noncompact myocardium/compact myocardium ratio also in diastole. [5],[11] Doppler echocardiography will show direct blood flow from ventricular cavity into deep intratrabecular recess; this will not occur in LVH. [1] Common electrocardiographic finding includes biventricular hypertrophy with extreme QRS voltages, isolated or diffuse T-wave changes; Wolf-Parkinson-White with premature atrial and ventricular contractions. [12] Cardiac catheterization may be used to exclude other cardiac or coronary abnormalities.

Early diagnosis is necessary for prompt treatment without such there is increase in morbidity and mortality due early heart failure, life-threatening ventricular arrhythmias, and systemic embolic events. [1] Beta-blocker therapy (e.g., carvedilol is relevant in management in addition to diuretics, digitalis, and ACEI. [13]


  Conclusion Top


LVNC may not be that rare in Nigeria. A high sense of suspicion is needed, especially in many cases of dilated cardiomyopathy with malignant arrhythmias and/or thromboembolic complications.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
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. 1
    
2.
Dusek J, Ostádal B, Duskova M. Postnatal persistence of spongy myocardium with embryonic blood supply. Arch Pathol 1975;99:312-7.  Back to cited text no. 2
    
3.
Engberding R, Bender F. Identification of a rare congenital anomaly of the myocardium by two-dimensional echocardiography: Persistence of isolated myocardial sinusoids. Am J Cardiol 1984;53:1733-4.  Back to cited text no. 3
[PUBMED]    
4.
Varnava AM. Isolated left ventricular non-compaction: A distinct cardiomyopathy? Heart 2001;86:599-600.  Back to cited text no. 4
[PUBMED]    
5.
Elliott P, Andersson B, Arbustini E, Bilinska Z, Cecchi F, Charron P, et al. Classification of the cardiomyopathies: A position statement from the European society of cardiology working group on myocardial and pericardial diseases. Eur Heart J 2008;29:270-6.  Back to cited text no. 5
    
6.
Monserrat L, Hermida-Prieto M, Fernandez X, Rodríguez I, Dumont C, Cazón L, et al. Mutation in the alpha-cardiac actin gene associated with apical hypertrophic cardiomyopathy, left ventricular non-compaction, and septal defects. Eur Heart J 2007;28:1953-61.  Back to cited text no. 6
    
7.
Captur G, Nihoyannopoulos P. Left ventricular non-compaction: Genetic heterogeneity, diagnosis and clinical course. Int J Cardiol 2010;140:145-53.  Back to cited text no. 7
    
8.
Oechslin E, Jenni R. Left ventricular non-compaction revisited: A distinct phenotype with genetic heterogeneity? Eur Heart J 2011;32:1446-56.  Back to cited text no. 8
    
9.
Chin TK, Perloff JK, Williams RG, Jue K, Mohrmann R. Isolated noncompaction of left ventricular myocardium. A study of eight cases. Circulation 1990;82:507-13.  Back to cited text no. 9
    
10.
Stöllberger C, Gerecke B, Finsterer J, Engberding R. Refinement of echocardiographic criteria for left ventricular noncompaction. Int J Cardiol 2013;165:463-7.  Back to cited text no. 10
    
11.
Ker J, Van Der Merwe C. Isolated left ventricular non-compaction as a cause of thrombo-embolic stroke: A case report and review. Cardiovasc J S Afr 2006;17:146-7.  Back to cited text no. 11
    
12.
Ali SK. Unique features of non-compaction of the ventricular myocardium in Arab and African patients. Cardiovasc J Afr 2008;19:241-5.  Back to cited text no. 12
    
13.
Peters F, Dos Santos C, Essop R. Isolated left ventricular non-compaction with normal ejection fraction. Cardiovasc J Afr 2011;22:90-3.  Back to cited text no. 13
    
14.
Peters F, Essop M. Isolated left ventricular non-compaction in Africa: Elucidating myths. Cardiovasc J Afr 2013;24:188.  Back to cited text no. 14
[PUBMED]    
15.
Peters F, Khandheria BK, dos Santos C, Matioda H, Maharaj N, Libhaber E, et al. Isolated left ventricular noncompaction in Sub-Saharan Africa: A clinical and echocardiographic perspective. Circ Cardiovasc Imaging 2012;5:187-93.  Back to cited text no. 15
    


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