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 ::  Abstract
 ::  Introduction
 ::  Case report
 ::  Discussion
 ::  Acknowledgment
 ::  References
 ::  Article Figures

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CASE REPORT
Year : 1996  |  Volume : 42  |  Issue : 3  |  Page : 84-6

Embolic stroke in a child with idiopathic dilated cardiomyopathy.


Department of Pediatrics, Seth GS Medical College, Parel, Mumbai.

Correspondence Address:
S C Karande
Department of Pediatrics, Seth GS Medical College, Parel, Mumbai.

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Source of Support: None, Conflict of Interest: None


PMID: 0009715325

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 :: Abstract 

An eleven year old boy presented with sudden onset right-sided hemiplegia and ipsilateral lower facial weakness. Two-dimensional echo revealed the diagnosis of idiopathic dilated cardiomyopathy with multiple intracardiac clots. MRI scan of head showed infarctions in the area of caudate nuclei, putamen, brain stem and cerebellum. On anticoagulation therapy, all thrombi, except one, disappeared. The child died 2.5 months later due to resistant cardiac failure.


Keywords: Anticoagulants, therapeutic use,Cardiomyopathy, Congestive, complications,ultrasonography,Case Report, Cerebral Infarction, diagnosis,drug therapy,etiology,Child, Fatal Outcome, Human, Intracranial Embolism and Thrombosis, diagnosis,drug therapy,etiology,Magnetic Resonance Imaging, Male,


How to cite this article:
Karande S C, Kulthe S G, Lahiri K R, Jain M K. Embolic stroke in a child with idiopathic dilated cardiomyopathy. J Postgrad Med 1996;42:84

How to cite this URL:
Karande S C, Kulthe S G, Lahiri K R, Jain M K. Embolic stroke in a child with idiopathic dilated cardiomyopathy. J Postgrad Med [serial online] 1996 [cited 2019 Nov 15];42:84. Available from: http://www.jpgmonline.com/text.asp?1996/42/3/84/430





  ::   Introduction Top


Idiopathic dilated cardiomyopathy (IDC) is a chronic heart muscle disease that predominantly affects young men and causes dilatation and contractile dysfunction of the left or the right ventricle, or both[1]. At present there are no reliable data on the prevalence of IDC in children[2]. To our knowledge there is only one earlier report of a child with IDC in Indian literature[3]. We report a child with IDC whose presenting manifestation was an embolic stroke.


  ::   Case report Top


An 11-year-old boy presented with sudden right-sided weakness and ipsilateral lower facial weakness. He had intermittent cough, breathlessness and palpitations on exertion since 7 months. He was on treatment with digoxin, furosemide and captopril. There was no history of fever, altered sensorium, convulsions, head injury, hematuria, pain in abdomen or contact with tuberculosis.

At admission, physical examination revealed sinus tachycardia normal peripheral pulses blood pressure 100/170 mm/Hg, elevated jugular venous pressure, bilateral basal crepitations, tender hepatomegaly and oedema feet. Cardiovascular examination revealed marked cardiomegaly and S3 gallop. Neurological examination revealed right hypotonic hemiplegia with ipsilateral upper motor neuron facial palsy.

Relevant laboratory investigations were normal. Chest X-ray showed cardiomegaly (cardiothoracic ratio 0.75) with pulmonary congestion. ECG showed sinus tachycardia, biventricular hypertrophy and S-T segment depression pattern. 2-1) ECHO revealed dilatation of all 4 chambers with global left ventricular dysfunction, a poor ejection fraction of 15-20% (normal 55-65%) and multiple clots on the lateral and posterior walls of the left ventricle.

The child was administered intravenous heparin bolus therapy, along with other medications. After 7 days heparin was omitted and oral warfarin substituted. The child's general condition stabilised with these measures. MRI scan of head done 1 month after admission showed old infarcts in the area of the caudate nuclei, putamen [Figure - 1]& [Figure - 2], brain stem and cerebellum [Figure - 2] On anticoagulation therapy, serial 2-D Echo showed disappearance of the clots and at discharge 1.5 months after admission, only a single clot (2.1 cm) remained. But the left ventricular ejection fraction had further deteriorated to 15%. Cardiac catheterisation and endomyocardial biopsy were not considered safe procedures at this point of time. Mild residual right spastic hemiparesis with ipsilateral upper motor neurone facial paresis remained as sequelae of the embolic phenomena. Digoxin, furosemide, captopril and warfarin were continued

One month later the child was readmitted with worsening of the cardiac failure (left ventricular ejection fraction 10%) without any new neurological findings or Weeding from any site. On 2D-Echo, a suggestion of a clot 1 em in size was seen in the left ventricle. A dopamine drip was started but within 20 hours the child died. Post-mortem examination was refused by the parents.


  ::   Discussion Top


Identifying the aetiology, duration of pre-symptomatic phase, and managing patients with IDC is a difficult clinical problem[4] Conventional treatment is restricted to the relief of symptoms of cardiac failure with diuretics, captopril, digoxin and the prevention of thromboembolic complications with warfarin[1].

Lewis and Chabot[5] reviewed 81 children with IDC (1975-90) and found that mortality was highest (19%) during the first 6 months after presentation. Survival was 70% at 2 yrs, 64% at 5 yrs. 52% after 11.5 yrs, with age at initial presentation not having any impact on survival. Wiles et al[6] reviewed 39 children with IDC (1966-90) and found that age at presentation, sex, cardiomegaly, cardiac failure and degree of systolic ventricular dysfunction did not consistently predict the final outcome. Risk factors reported for a poor prognosis are presence of arrhythmias[5],[6],[7], and marked elevation in the left ventricular end-diastolic pressure (> 25 torr)[5].

Systemic embolism is a life threatening complication. Patients often have evidence of ventricular thrombi at autopsy[8]. Till recently, there was a controversy regarding the necessity of routine anticoagulation because of lack of prospective studies defining the prevalence of embolization and the tear of an increased risk of anticoagulant-associated bleeding in patients with a congested liver[9]. Ciaccheri et al[10] have now recommended anticoagulant treatment in patients having severe cardiac failure, intra-cardiac thrombosis and previous systemic embolism. Falk et al[11] have recommended long-term anticoagulation in patient with severely impaired left ventricular 5, function corresponding to fractional shortening of the left ventricle of < 10% as determined from the M-mode echocardiogram. In our case, embolization had already occurred at time of presentation, and both initiation and continuation of anticoagulation treatment were indicated. Recently, cardiac transplantation has been utilised successfully in 2 out of 3 (66.7%) and in all 4 (100%)[5] children with IDC.


  ::   Acknowledgment Top


We thank our Dean, Dr (Mrs) P M Pai for granting us permission to publish this case report

 
 :: References Top

1. Abelmann WH, Loreil BH. The challenge of cardiomyopathy. J Am Coll Cardiol 1989; 13:1219-1239.  Back to cited text no. 1    
2.Colan SD, Spevak PJ, Parness IA, Nadas AS. Cardiomyopathies. In: Fyier DC, editor. Nadas’ Paediatric Cardiology, 1st edition. New Delhi: Jaypee Brothers; 1992, pp 329-361.  Back to cited text no. 2    
3.Moghe KV Ahuja SP. Idiopathic cardiomegaly with pulmonary tuberculosis and severe anaemia in a female child of 12 years - a case report. Indian Pediatr 1980; 17:97-100.  Back to cited text no. 3    
4.Caforio ALP, Stewart JT, McKenna WJ. Idiopathic dilated cardiomyopathy rational treatment awaits better understanding of pathogenesis Br Med J 1990; 300:890-891  Back to cited text no. 4    
5.Lewis AB, Chabot M. Outcome of infants and children with dilated cardiomyopathy. Am J Cardiol 1991; 68:365-369.  Back to cited text no. 5    
6.Wiles HB, McArthur PD, Taylor AB. Prognostic features of children with idiopathic, dilated cardiomyopathy. Am J Cardiol 1991; 68:1372-1376.  Back to cited text no. 6    
7.Griffin ML, Hernandez A, Martin TC. Dilated cardiomyopathy in infants and children. J Am Coll Cardiol 1988; 11:139-144.  Back to cited text no. 7    
8.Roberts WC, Siegel RJ, McManus BM. Idiopathic dilated cardiomyopathy, analysis of 152 necropsy patients. Am J Cardiol 1987; 60:1340-1355.  Back to cited text no. 8    
9.Stevenson LW, Perloff JK. The dilated cardiomyopathies: clinical aspects Cardiol Clin 1988; 6:197-218.  Back to cited text no. 9    
10.Ciaccheri M, Castelli G, Ceechi F. Lack of correlation between intracavitary thrombosis detected by cross sectional echocardiography and systemic emboli in patients with dilated cardio- myopathy. Br Heart J 1989; 62:26-29.  Back to cited text no. 10    
11.Falk RH, Foster E, Coats MH. Ventricular thrombi and thromboembolism in dilated cardio-myopathy: a prospective follow-up study. Am Heart J 1992; 123:136-142.   Back to cited text no. 11    


    Figures

[Figure - 1], [Figure - 2]

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