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Inappropriate management of polycythaemia in adults with cyanotic congenital heart disease
  1. Adult Congenital Heart Disease Clinic,
  2. Guy’s Hospital, London SE1 9RT, UK
  3. email: e.rosenthal{at}

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Sir,—The editorial by Thorne1highlights misconceptions in the management of polycythaemia in adults with congenital heart disease. Inappropriate venesection is not only encountered in patients with inoperable cyanotic heart disease. Venesection and bone marrow suppression with radioactive phosphorus are also used in patients with operable congenital heart disease. Two recent cases are illustrative.

Case 1 is a 38 year old man with double outlet right ventricle, a straddling tricuspid valve, and pulmonary atresia who had a classic Blalock-Taussig shunt at age 11 years. After many years of venesection for his “inoperable congenital heart disease” he suffered a cerebrovascular accident from which he made a good recovery. Following this he received two courses of radioactive phosphorus in an attempt to suppress red cell production. After being referred to our unit he had a fenestrated Fontan operation with an increase in his exercise tolerance. He is currently awaiting transcatheter occlusion of the atrial fenestration.

Case 2 is a patient with classic tricuspid atresia, a restrictive ventricular septal defect, and pulmonary stenosis. After repeated venesections, he was referred to our unit at the age of 45 years, after he had become unwell during a venesection. He had a fenestrated Fontan operation, which was followed by transcatheter occlusion of his fenestration. Four years later he had oxygen saturations of 95% and a greatly improved exercise tolerance. (On a lighter note, his aged but sprightly mother “complains” that she is no longer able to keep up with him when walking—a reversal of the situation before surgery.)

These cases are typical of patients who have been “lost to follow up” from congenital heart disease centres and are seen only in general medical, adult cardiology, or haematology departments. Adults with congenital heart disease should be managed in conjunction with congenital heart disease units. In particular, patients labelled as “inoperable” or with “complex” congenital heart disease need to be reviewed in light of modern approaches to treatment.