title: Replacement therapy for iron deficiency improves exercise capacity and quality of life in patients with cyanotic congenital heart disease and/or the Eisenmenger syndrome.
abstract: BACKGROUND
  Iron deficiency is common in cyanotic congenital heart disease (CHD) and results in reduced exercise tolerance. Currently, iron replacement is advocated with limited evidence in cyanotic CHD. We investigated the safety and efficacy of iron replacement therapy in this population.
  
  
  METHODS
  Twenty-five iron-deficient cyanotic CHD patients were prospectively studied between August 2008 and January 2009. Oral ferrous fumarate was titrated to a maximum dose of 200mg thrice-daily. The CAMPHOR QoL questionnaire, 6 minute walk test (6MWT) and cardiopulmonary exercise testing were conducted at baseline and after 3 months of treatment.
  
  
  RESULTS
  Mean age was 39.9 ± 10.9 years, 80% females. Fourteen had Eisenmenger syndrome, 6 complex cyanotic disease and 5 Fontan circulation. There were no adverse effects necessitating termination of treatment. After 3 months of treatment, hemoglobin (19.0 ± 2.9 g/dL to 20.4 ± 2.7 g/dL, p<0.001), ferritin (13.3 ± 4.7 μg/L to 54.1 ± 24.2 μg/L, p<0.001) and transferrin saturation (17.8 ± 9.6% to 34.8 ± 23.4%, p<0.001) significantly increased. Significant improvements were also detected in the total CAMPHOR score (20.7 ± 10.9 to 16.2 ± 10.4, p=0.001) and 6MWT distance (371.7 ± 84.7 m to 402.8.0±74.9m, p=0.001). Peak VO(2) remained unchanged (40.7 ± 9.2% to 43.8 ± 12.4% of predicted, p=0.15).
  
  
  CONCLUSIONS
  Three months of iron replacement therapy in iron-deficient cyanotic CHD patients was safe and resulted in significant improvement in exercise tolerance and quality of life. Identification of iron deficiency and appropriate replacement should be advocated in these patients.
authors.affiliation: Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital, Sydney Street, London SW3 6NP, UK. edgartay1@gmail.com
authors.forename: Edgar L W
authors.initials: EL
authors.lastname: Tay
authors.forename: Ana
authors.initials: A
authors.lastname: Peset
authors.forename: Maria
authors.initials: M
authors.lastname: Papaphylactou
authors.forename: Ryo
authors.initials: R
authors.lastname: Inuzuka
authors.forename: Rafael
authors.initials: R
authors.lastname: Alonso-Gonzalez
authors.forename: Georgios
authors.initials: G
authors.lastname: Giannakoulas
authors.forename: Aphrodite
authors.initials: A
authors.lastname: Tzifa
authors.forename: Sara
authors.initials: S
authors.lastname: Goletto
authors.forename: Craig
authors.initials: C
authors.lastname: Broberg
authors.forename: Konstantinos
authors.initials: K
authors.lastname: Dimopoulos
authors.forename: Michael A
authors.initials: MA
authors.lastname: Gatzoulis
mesh_terms: D000328:Adult; D018798:Anemia, Iron-Deficiency / Q000188:drug therapy* / Q000503:physiopathology / Q000523:psychology; D004541:Eisenmenger Complex / Q000188:drug therapy* / Q000503:physiopathology / Q000523:psychology; D005080:Exercise Test / Q000187:drug effects / Q000379:methods; D017079:Exercise Tolerance / Q000187:drug effects* / Q000502:physiology; D005260:Female; D005296:Ferrous Compounds / Q000008:administration & dosage*; D005500:Follow-Up Studies; D006330:Heart Defects, Congenital / Q000188:drug therapy / Q000503:physiopathology / Q000523:psychology; D006801:Humans; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies; D011788:Quality of Life / Q000523:psychology; D016896:Treatment Outcome
chemical_list: D005296:Ferrous Compounds; C031621:ferrous fumarate