Marfan Syndrome Research Today is a free monthly online journal that collates and summarizes the latest research about Marfan Syndrome, including details on genetics, causes, symptoms, treatment.

Smoking and its effects on mortality in adults with congenital heart disease.

Engelfriet PM, Drenthen W, Pieper PG, Tijssen JG, Yap SC, Boersma E, Mulder BJ

Department of Cardiology, Academic Medical Centre, Amsterdam, The Netherlands.

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.

Published 27 May 2008 in Int J Cardiol, 127(1): 93-7.
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