Replacement therapy for iron deficiency improves exercise capacity and quality of life in patients with cyanotic congenital heart disease and/or the Eisenmenger syndrome.
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.
Tay
Edgar L W
EL
Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital, Sydney Street, London SW3 6NP, UK. edgartay1@gmail.com
Peset
Ana
A
Papaphylactou
Maria
M
Inuzuka
Ryo
R
Alonso-Gonzalez
Rafael
R
Giannakoulas
Georgios
G
Tzifa
Aphrodite
A
Goletto
Sara
S
Broberg
Craig
C
Dimopoulos
Konstantinos
K
Gatzoulis
Michael A
MA
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
D005296:Ferrous Compounds; C031621:ferrous fumarate