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CONGENITAL HEART DISEASE

Anemia in Adults With Congenital Heart Disease Relates to Adverse Outcome

Konstantinos Dimopoulos, MD, MSc, PhD^_*,,_*, Gerhard-Paul Diller, MD, PhD^_*,, Georgios Giannakoulas, MD, PhD^_*, Ricardo Petraco, MD^_*, Aikaterini Chamaidi, MD^_*, Evaggelia Karaoli, MD^_*, Michael Mullen, MD^_*, Lorna Swan, MD, PhD^_*, Massimo F. Piepoli, MD, PhD, Philip A. Poole-Wilson, MD, Darrel P. Francis, MA and Michael A. Gatzoulis, MD, PhD^_*

^_* Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, United Kingdom
Department of Clinical Cardiology, National Heart and Lung Institute, Imperial College School of Medicine, London, United Kingdom
International Centre for Cardiocirculatory Health, St. Mary's Hospital, London, United Kingdom

Manuscript received February 20, 2009; revised manuscript received May 28, 2009, accepted June 28, 2009.

^_* Reprint requests and correspondence: Dr. Konstantinos Dimopoulos, Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital, Sydney Street, London SW3 6NP, United Kingdom (Email: k.dimopoulos02{at}ic.ac.uk).

Objectives: To assess the relation of anemia in noncyanotic adults with congenital heart disease (ACHD) to functional capacity and mortality.

Background: Anemia is common in acquired heart failure and affects prognosis. The presence of anemia and its relation to outcome in ACHD remain unknown.

Methods: Data were collected on consecutive noncyanotic ACHD patients attending our tertiary center between 2001 and 2006 in whom hemoglobin concentration was measured. Anemia was defined as hemoglobin concentration <13 g/dl in males and <12 g/dl in females. Cyanotic patients were excluded to avoid confounding from secondary erythrocytosis.

Results: Overall, 830 noncyanotic ACHD patients (age 36.5 ± 15.0 years, 49.6% male) fulfilled the inclusion criteria. The prevalence of anemia was 13.1% and was highest in patients with congenitally corrected transposition of great arteries and Ebstein anomaly of the tricuspid valve. Anemic patients were more likely to be receiving diuretics (p < 0.0001) and have a lower mean corpuscular volume (p = 0.0001), with a trend toward a higher New York Heart Association functional class (p = 0.06). During a median follow-up of 47 months, 55 patients died. Anemic patients had a 3-fold higher mortality risk compared with nonanemic patients, even after propensity score adjustment for clinical variables such as systemic ventricular function, renal impairment, and diuretic therapy (adjusted hazard ratio: 3.00; 95% confidence interval: 1.46 to 6.13).

Conclusions: Anemia is not uncommon in ACHD patients attending tertiary services and is associated with a 3-fold increased risk of death. Screening for anemia should be part of the routine assessment of ACHD patients for risk stratification and treatment when correctable causes are identified.

Key Words: congenital heart defects • anemia • prognosis

Abbreviations and Acronyms   ACHD = adults with congenital heart disease   ccTGA = congenitally corrected transposition of great arteries   MCV = mean corpuscular volume   NYHA = New York Heart Association

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