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Stroke due to arterial disease in congenital heart disease
  1. Neurosciences Unit
  2. Institute of Child Health
  3. The Wolfson Centre
  4. Mecklenburgh Square
  5. London WC1N 2AP

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    Editor,—A 1 year old girl with pulmonary atresia developed an acute right hemiparesis. She had had an intact ventricular septum at birth. Ventricular septostomy and insertion of a Blalock-Taussig shunt were carried out at 1 week. She had otherwise been well and was developmentally age appropriate. At the time of the hemiparesis, she was only mildly polycythaemic (packed cell volume = 0.426). There were no new findings on praecordial echocardiography; no intracardiac thrombus was seen. Thirty six hours later, magnetic resonance imaging showed an acute infarct in the territory of the left middle cerebral artery (MCA). A small, mature lesion was present in the right corona radiata. Magnetic resonance angiography revealed reduced flow in the petrous and cavernous left internal carotid artery, with a high density rim, suggestive of dissection, with reduced flow in the left MCA (see fig 1). She was treated with warfarin; at six weeks she had a significant residual hemiparesis.

    Figure 1

    T2 weighted magnetic resonance image (axial section) showing a high intensity rim around the left internal carotid artery which has a smaller lumen than the contralateral vessel. The rim represents the intramural haematoma which has resulted from dissection of the vessel.

    Cervicocephalic arterial dissection is a significant cause of stroke in young patients1 but the diagnosis is considered often only when there is a history of antecedent trauma, although this is not invariable, and dissection may occur spontaneously or after relatively innocuous injuries. Diagnosis is important as anticoagulation is of proved benefit if given before infarction has occurred2and the risk of recurrent dissection over 10 years is 12%.3

    Although an embolic origin for stroke is frequent assumed in children with known cardiac disease, after excision of endocarditis, polycythaemia and right to left shunts, of 25 such patients we have seen, intracardiac thrombus was demonstrated in only five cases (20%). Fifteen of these children (60%) were found to have structural cerebrovascular abnormalities.

    Children with acute stroke should be thoroughly investigated on each occasion in order to detect all potential risk factors, some of which may contribute to the already significant risk of recurrent cerebral ischaemic events.


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