title: [Long-term hematological management of cyanotic congenital heart diseases].
abstract: The long-term management of cyanotic congenital heart disease requires treatment of raised haematocrit (> 65%) whilst conserving the quality of the red blood cells [mean corpuscular volume, mean corpuscular haemoglobin concentration (MCHC)]. Since 1975, the author has chosen chemotherapy (hydroxyurea, pipobroman) as first-line treatment, reserving iron supplements, phlebotomy and platelet antagonists as adjuvant therapy. Pre-treatment data of 170 patients showed: a high prevalence of hypochromic microcytosis due to iron deficiency and of thrombopaenia, in relation to the severity and duration of the polycythaemia. Hyperuricaemia was greater than 420 mumol/l in 67% of cases; effort tolerance and hyperviscosity were related to the haematocrit and iron deficiency. One hundred and forty seven patients were followed up for a total duration of 769 years of chemotherapy. Clinical tolerance was good: biological tolerance was marked by a high frequency of thrombocytopaenia requiring withdrawal (10 cases) or reduction of treatment (34 cases). The causes of the 39 deaths observed are analysed: none was related to the hydroxyurea or pipobroman. The treatment or follow-up was stopped in 29 cases: the reasons are reported. Seventy eight patients under treatment had a favourable outcome with normal social reinsertion in 74 cases. This efficiency was related to maintenance of a haematocrit < 65%, to correction of the iron deficiency and increase in the MCHC. These results were obtained with an average dosage of 19 +/- 4.5 mg/kg/day of hydroxyurea (69 patients). By slowing erythropoiesis, chemotherapy reduces the indication of phlebotomy, so reducing iron loss: it also inhibits excessive bouts of polycythaemia in cases of iron therapy.(ABSTRACT TRUNCATED AT 250 WORDS)
authors.affiliation: Clinique chirurgicale Marie-Lannelongue, Le Plessis-Robinson.
authors.forename: P
authors.initials: P
authors.lastname: Cornu
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D006330:Heart Defects, Congenital / Q000150:complications* / Q000628:therapy; D006402:Hematologic Diseases / Q000188:drug therapy / Q000209:etiology / Q000628:therapy*; D006801:Humans; D007223:Infant; D000090463:Iron Deficiencies; D008875:Middle Aged; D010975:Platelet Aggregation Inhibitors / Q000627:therapeutic use; D012189:Retrospective Studies; D013921:Thrombocytopenia / Q000209:etiology / Q000628:therapy; D013927:Thrombosis / Q000517:prevention & control; D013997:Time Factors; D014527:Uric Acid / Q000097:blood
chemical_list: D010975:Platelet Aggregation Inhibitors; D014527:Uric Acid
vernacular_title: Prise en charge hématologique au long cours des cardiopathies congénitales cyanogènes.