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Table of Contents
CASE REPORT
Year : 2022  |  Volume : 12  |  Issue : 1  |  Page : 40-42

Takayasu aortoarteritis masquerading left ventricular noncompaction syndrome


1 Department of Medicine, Lokmanya Tilak Municipal Medical College and General Hospital, Mumbai, Maharashtra, India
2 Department of Cardiology, Lokmanya Tilak Municipal Medical College and General Hospital, Mumbai, Maharashtra, India
3 Department of Radiodiagnosis, Lokmanya Tilak Municipal Medical College and General Hospital, Mumbai, Maharashtra, India

Date of Submission25-Jan-2021
Date of Decision15-Feb-2021
Date of Acceptance16-Feb-2021
Date of Web Publication08-Feb-2022

Correspondence Address:
Dr. Aditi Sanjiv Patankar
1st Floor, Department of Medicine, Lokmanya Tilak Municipal Medical College and General Hospital, Sion, Mumbai - 400 022, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jicc.jicc_7_21

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  Abstract 


We report an interesting case of a 20-year-old man who was admitted in intensive cardiac care unit with congestive cardiac failure along with thromboembolic manifestations and a raised erythrocyte sedimentation rate. On the basis of two-dimensional echocardiography, he was diagnosed as left ventricular noncompaction syndrome. However, cardiac magnetic resonance imaging (MRI) ruled out noncompaction. After extensive workup, the patient was diagnosed with Takayasu aortoarteritis. Post therapy, the patient has normal cardiac function and cardiac MRI without thromboembolic event in 8 years' follow-up.

Keywords: Cardiac magnetic resonance imaging, cardiac transplant, left ventricular noncompaction syndrome


How to cite this article:
Gokhale Y, Patankar AS, Mahajan AU, Firke V. Takayasu aortoarteritis masquerading left ventricular noncompaction syndrome. J Indian coll cardiol 2022;12:40-2

How to cite this URL:
Gokhale Y, Patankar AS, Mahajan AU, Firke V. Takayasu aortoarteritis masquerading left ventricular noncompaction syndrome. J Indian coll cardiol [serial online] 2022 [cited 2022 May 27];12:40-2. Available from: https://www.joicc.org/text.asp?2022/12/1/40/337354




  Introduction Top


Left ventricular noncompaction syndrome (LVNC) is a rare congenital cardiomyopathy. Specific criteria on two-dimensional echocardiography (2DECHO) for diagnosis of LVNC are lacking leading to overdiagnosis. We report one such case mistaken as LVNC on 2DECHO initially but later found to have Takayasu arteritis.


  Case Report Top


A 20-year-old male presented to emergency department with sudden onset of breathlessness. On examination, he had tachycardia with absent left lower limb pulsations. Blood pressure was 190/110 mmHg in the right upper limb. Respiratory rate was 44/min with saturation of 90% on room air. Jugular venous pressure was raised. On auscultation, gallop rhythm and bilaterally rales were heard. Electrocardiogram revealed left axis deviation with T wave inversions in leads V1-V6. Chest roentgenogram showed cardiomegaly with bilateral reticulonodular infiltrates. Complete hemogram, liver function tests, and renal function tests were normal. Erythrocyte sedimentation rate (ESR) was 122 mm/h (normal range 0–12). Ultrasonography of abdomen showed unequal kidney size with right kidney measuring 11.3 cm × 4.5 cm and left kidney measuring 8.5 cm × 3.6 cm with maintained corticomedullary differentiation. In view of ultrasonography findings, renal artery Doppler was performed which confirmed left renal artery stenosis. Absent left lower limb pulsations prompted us to do a lower limb Doppler which showed an echogenic thrombus with 80% luminal compromise of left common iliac artery, left external iliac artery and proximal left internal iliac artery which needed an urgent corrective thrombectomy. Differential diagnosis of hypertensive failure secondary to renal artery stenosis or systemic embolization from an underlying cardiac pathology or thrombophilia state was considered. The patient was shifted to intensive cardiac care unit for the management of hypertensive cardiac failure and was treated with injectable nitroglycerin, diuretics, and noninvasive ventilation. Thrombophilia and connective tissue diseases were ruled out by a negative ANA, anti β2 glycoprotein, ACLA, ANCA, Sr homocysteine and Factor V mutation. 2DECHO ruled out thrombus or vegetation and revealed global left ventricular hypokinesia with severe tricuspid regurgitation and pulmonary hypertension with ejection fraction of 10%–15% and evidence of LVNC. Computed tomography (CT) chest showed thrombosis of distal segmental pulmonary artery branches with ground glass opacities in peri-bronchial distribution seen in right middle, right upper and superior segment of the left lower lobe. The same day patient developed focal convulsion in left upper limb along with weakness and seventh cranial nerve palsy. CT of the brain confirmed multiple infarcts in right parietal, frontal, temporal lobe either secondary to embolus or due to vasculitis. In view of multiple thrombotic events, provisional diagnosis of systemic embolization due to noncompaction of left ventricle was made and the patient was started on unfractionated heparin. We faced a dilemma regarding continuation of life long anticoagulation. However, young age, unexplained raised ESR in a patient of congestive cardiac failure (CCF), presence of renal artery stenosis with hypertension and pulmonary vasculature thrombosis were more in favor of the diagnosis of Takayasu aortoarteritis (TA). Hence, a cardiac magnetic resonance imaging (MRI) was performed to reconfirm the diagnostic findings of 2DECHO. Cardiac MRI showed ratio of noncompacted to compacted myocardium (N/C) to be <2 (N/C of >2.3 is needed for diagnosis) [Figure 1]a and [Figure 1]b and LVNC was ruled out. As TA was a diagnostic consideration, we proceeded with CT aortography. It showed eccentric wall thickening with aneurismal dilation of supraceliac aorta with mild stenosis of celiac artery along with luminal narrowing of left renal artery [Figure 2]a and [Figure 2]b. Takayasu arteritis was confirmed and patient was started on corticosteroids and injection methotrexate. At 1 monthly follow-up, the patient was asymptomatic with ESR of 6. Follow-up at 3 months showed an improved ejection fraction of 60%. At 8 years' follow-up, cardiac MRI revealed a well compacted heart with normal ejection fraction [Figure 3]a and [Figure 3]b.
Figure 1: Bright blood BFFE sequence images in 4ch (a) and short axis (b) views showing moderate dilatation of the left ventricle with prominence of inter-trabecular spaces. Ratio of thickness of noncompacted myocardium to compacted myocardium is <2.3

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Figure 2: (a and b) Axial MIP image of computed tomography aortogram showing severe diffuse narrowing of left renal artery with small-sized scarred left kidney; Coronal MIP image of computed tomography aortogram showing diffuse nonenhancing wall thickening in left common iliac artery causing mild luminal narrowing in distal aspect. No acute thrombus is seen

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Figure 3: Bright blood BFFE sequence images in 4ch (a) and short axis (b) views showing normal sized left ventricle with normal trabeculations. Anterior and posterior papillary muscles are seen distinctly in short axis (b) view

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  Discussion Top


LVNC is a rare congenital cardiomyopathy characterized by increased number of trabeculations in the left ventricle.[1] When an embryonic heart develops, the muscle fibers of myocardium start by being “non-compacted” before compressing to form the thick muscular wall. LVNC happens when this part of the normal development of the heart (called the “remodeling phase”) is interrupted. Even though, its congenital, patients with LVNC are not diagnosed until later in life, when they start to develop symptoms. Symptoms usually occur because of pump failure, arrhythmias due to abnormal electrical signaling or thromboembolic phenomena Chin et al.[2] evaluated the size of trabeculations relative to the thickness of the compacted wall of the LV in end diastole. Jenni[3] found a ratio of noncompacted to compacted myocardium of >2 on short axis view as significant. Stöllberger and Finsterer[4] criteria were based on the number of prominent trabeculations visible in the apical views of the LV in diastole. LVNC in our case was diagnosed using Jenni's criteria. Uniform criteria for diagnosis of LVNC by 2DECHO are lacking. This might lead to overdiagnosis of this untreatable condition.

Conditions mimicking noncompaction on 2DECHO are prominent normal myocardial trabeculations, false tendons, aberrant bands, cardiac tumors, and hypertrophic cardiomyopathy.[5] Cardiac MRI is frequently used now a days as it provides a better ratio of noncompacted to compacted myocardium. In a study done by Peterson et al.[6] he found that the degree of noncompaction of the LV was far more frequent in healthy, dilated, and hypertrophied hearts than previously reported; presumably due to increased sensitivity of CMR. The diastolic ratio of 2.3 had sensitivity of 86% and specificity of 99%. Jacquie et al.[7] described another method to diagnose this entity: A trabeculated left ventricular mass above 20% of total mass with a sensitivity of 91.6% and a specificity of 86.5% is predictive of LVNC. A study done by Thuny et al.[8] compared two modalities of diagnosis and found that cardiac MRI was superior to standard echocardiography in assessing extent of noncompaction and providing morphological information beyond that obtained with conventional echocardiography. LVNC is managed symptomatically by anti-failure drugs; arrhythmias with pacemaker or implantable cardiac defibrillator and thromboembolism with anticoagulation, definitive management being cardiac transplant. In CCF, ESR is low.[9],[10] In this case, ESR of 122 mm/h in the presence of CCF made us consider and investigate for an underlying systemic disease.

Declaration of patient consent

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

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Rooms I, Dujardin K, De Sutter J. Non-compaction cardiomyopathy: A genetically and clinically heterogeneous disorder. Acta Cardiol 2015;70:625-31.  Back to cited text no. 1
    
2.
Chin TK, Perloff JK, Williams RG, Jue K, Mohrmann R. Isolated noncompaction of left ventricular myocardium. A study of eight cases. Circulation [Internet]. 1990 ;82:507–13. Available from: https://pubmed.ncbi.nlm.nih.gov/2372897/.  Back to cited text no. 2
    
3.
Jenni R, Oechslin EN, Van Der Loo B. Isolated ventricular non-compaction of the myocardium in adults [Internet]. Heart. BMJ Publishing Group; 2007;93:11–5. Available from: /pmc/articles/PMC1861330/.  Back to cited text no. 3
    
4.
Stöllberger C, Finsterer J. Diagnostic problem of lv hypertrabeculation/noncompaction? JACC Cardiovasc Imaging 2014;7:532-3.  Back to cited text no. 4
    
5.
Song ZZ. Echocardiography in the diagnosis left ventricular noncompaction. Cardiovasc Ultrasound 2008;6:64.  Back to cited text no. 5
    
6.
Petersen SE, Selvanayagam JB, Wiesmann F, Robson MD, Francis JM, Anderson RH, et al. Left ventricular non-compaction: Insights from cardiovascular magnetic resonance imaging. J Am Coll Cardiol 2005;46:101-5.  Back to cited text no. 6
    
7.
Amzulescu MS, Rousseau MF, Ahn SA, Boileau L, de Meester de Ravenstein C, Vancraeynest D, et al. Prognostic impact of hypertrabeculation and noncompaction phenotype in dilated cardiomyopathy: A CMR study. JACC Cardiovasc Imaging 2015;8:934-46.  Back to cited text no. 7
    
8.
Thuny F, Jacquier A, Jop B, Giorgi R, Gaubert JY, Bartoli JM, et al. Assessment of left ventricular non-compaction in adults: Side-by-side comparison of cardiac magnetic resonance imaging with echocardiography. Arch Cardiovasc Dis 2010;103:150-9.  Back to cited text no. 8
    
9.
Malgutte D, Waghmare I, Bhute V, Joshi A, Gokhale Y. Non-ischemic cardiomyopathy: Role of immunologic work-up and cardiac MRI in etiologic diagnosis and outcomes. J Assoc Physicians India 2019;67:30-4.  Back to cited text no. 9
    
10.
Haber HL, Leavy JA, Kessler PD, Kukin ML, Gottlieb SS, Packer M. The erythrocyte sedimentation rate in congestive heart failure. Engl J Med 1991;324:353-8.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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