Aims: To describe smoking habits in adults with congenital heart disease (ACHD) and to assess the relationship between smoking exposure and cardiovascular mortality. Methods: Data on smoking history and cardiovascular mortality were extracted from the Euro Heart Survey on adult congenital heart disease - a retrospective cohort study, that included patients diagnosed with 1 of 8 subgroups of ACHD (Atrial Septal Defects, Ventricular Septal Defects, Marfan Syndrome, Aortic Coarctation, Tetralogy of Fallot (ToF), Transposition of the Great Arteries (TGA), Fontan circulation, and Cyanotic disease). Results: Complete data of 3375 ACHD patients (median age 28 years) were available for analysis. At inclusion, 9.3% (n = 314) were current smokers and 4.2% (n = 142) of the patients had smoked in the past. During a median follow-up of 5.1 years, 101 patients (3%) died. In the majority of cases the cause of death was cardiovascular (n = 81; 80%). Kaplan-Meier and Cox survival analysis for each of the defects separately showed a significantly increased age and sex-adjusted cardiovascular mortality associated with smoking exposure in TGA patients (Hazard ratio 4.2 (95% CI 1.0-16.8); P = 0.044). Also in ToF mortality was higher amongst smokers, though not significantly (HR 3.4 (95% CI 0.6-18.5); P = 0.15). In the remaining defects no relationship between smoking and cardiovascular mortality was observed. Conclusion: The prevalence of smoking amongst ACHD patients is relatively low. Smoking exposure is associated with increased cardiovascular mortality in patients with TGA. Prospective long-term follow-up studies are necessary.

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Keywords Adults, Congenital heart disease, Euro Heart Survey, Mortality, Smoking
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Journal International Journal of Cardiology
Engelfriet, P.M, Drenthen, W, Pieper, P.G, Tijssen, J.G.P, Yap, S-C, Boersma, H, & Mulder, B.J.M. (2008). Smoking and its effects on mortality in adults with congenital heart disease. International Journal of Cardiology, 127(1), 93–97. doi:10.1016/j.ijcard.2007.05.008