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- coagulation disorder
- cyanotic congenital heart disease
- magnetic resonance imaging
- neurological disorder
Haemostatic defects or thrombosis associated with secondary erythrocytosis is well known in adult patients with cyanotic congenital heart disease (CCHD).1 One of the most serious complications of this haematological derangement is a cerebrovascular event,2 although a large study of adult patients with CCHD showed no increased risk of stroke.3 Although these patients may not develop apparent stroke, they often have various neurological symptoms, such as headache, dizziness, and tinnitus, in association with blood hyperviscosity.1 To our knowledge, no systematic magnetic resonance imaging (MRI) studies of the brain in this condition have been reported. Investigating the relation between brain MRI findings and blood coagulation abnormalities may be important for adequate management of patients with CCHD.
We studied 15 consecutive patients with CCHD aged 18–45 years (median age 24) who had been examined and followed up at the Tsukuba University Hospital for more than 10 years. None of the patients had undergone a radical operation for cardiac defects because of high pulmonary vascular resistance for Fontan circulation in eight patients (three with single ventricle, two with tetralogy of Fallot, two with double outlet right ventricle, and one with corrected transposition of the great arteries and ventricular septal defect (VSD); the latter five patients also had pulmonary atresia, hypoplastic pulmonary arteries, or hypoplastic left ventricle) and because of extremely high pulmonary arterial resistance with a right to left shunt (Eisenmenger’s syndrome) in seven (two with VSD, two with VSD and ductus arteriosus, two with atrioventricular septal defect, and one with VSD and transposition of the great arteries). Two patients had undergone Blalock-Taussig anastomosis but the postoperative courses were uneventful. None of the patients had had cardiopulmonary bypass or catheter intervention. Patients with …