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

Abstract

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.

Hematologic Derangements among Children with Unoperated Cyanotic Congenital Heart Disease in Ethiopia

“…In cyanotic CHDs, uninhibited erythropoietin secretion triggered by chronic hypoxemia, leads to uninhibited erythrocyte production irrespective of depletion of iron stores in a bid to increase oxygen-carrying capacity ( 22 , 23 ). The erythrocytes produced under such conditions are usually iron-deficient, leading to “anemia in the face of polycythemia”( 3 , 11 ). But this iron deficiency is often overlooked by practitioners ( 23 ).…”

“…But this iron deficiency is often overlooked by practitioners ( 23 ). Such erythrocytes increase the risk of CVAs and chronic end-organ damage due to intravascular sludging ( 11 ).…”

“…Among the many kinds of possible CHD complications, hematologic derangements and coagulopathies are the most important and can be life-threatening. Secondary polycythemia and increased blood viscosity, impaired blood flow dynamics and red cell deformation, iron deficiency anemia precipitated by polycythemia, thrombocytopenia, and coagulopathies are all well-described in patients with cyanotic CHD (( 3 – 11 )). Endothelial dysfunction, vascular dysfunction, and altered structure may also play a role in increasing the risk of cardiopulmonary insufficiency as well as thromboembolic events in children with CHD ( 12 , 13 ).…”

Isolated cerebellar infarction in a case of JAK 2 mutation-negative polycythemia vera: A case report

“…are less frequently associated with ischemic stroke[ 1 ] and there the treatment consists in addressing the cause causing elevated hematocrit. [ 6 7 ]…”

Concomitant Cerebral Venous Thrombosis and Intracranial Hemorrhages in Presentation of a Patient with Secondary Polycythemia: A Case Report

“…A low-risk PV patient is defined as age <60 years old without a previous thrombosis event [ 14 ]. Although uncommon, secondary polycythemia can lead to cerebrovascular events, such as ischemic stroke and cerebral venous thrombosis (CVT) [ 15 – 18 ]. Here, we present a rare case of a 35-year-old man with secondary polycythemia who developed concomitant CVT, which led to hemorrhagic transformation of acute cerebral infarction, and subarachnoid hemorrhage.…”