Acute ischaemic stroke in secondary polycythaemia due to complex congenital cyanotic heart disease

A 65-year-old woman presented to the emergency department with an acute onset of left-sided hemiparesis and slurred speech for 6 hours. Physical examination was notable for clubbing and peripheral cyanosis. Brain MRI showed an acute infarct involving the right gangliocapsular region. Investigations showed an elevated haemoglobin level, a haematocrit level of >65% and thrombocytopaenia. Transthoracic echocardiography revealed tricuspid atresia, ventricular septal defect and atrial septal defect. A diagnosis of cyanotic congenital heart disease was made. Studies have indicated an association between raised haematocrit and vascular occlusive episodes in patients with polycythaemia vera. However, a relationship between raised haematocrit due to polycythaemia secondary to complex congenital heart disease and vascular thrombosis has not been reported. The improvement of neurological deficits in our patient coincided with a decline in haematocrit. The objective of this report was to highlight an association between elevated haematocrit and ischaemic stroke in patients with secondary polycythaemia due to complex congenital heart disease.