Pregnancy Outcomes in Marfan's Syndrome

Abstract

INTRODUCTION: Marfan's syndrome is a rare connective tissue disease with significant risk for adverse cardiovascular outcomes. Our objective was to evaluate pregnancy and cardiovascular outcomes in pregnant women with Marfan's syndrome. METHODS: We conducted a population-based retrospective cohort study using the Healthcare Cost and Utilization Project Nationwide Inpatient Sample database from 2003 to 2010. We used unconditional regression analyses to compare maternal and fetal outcomes among pregnancies in women with Marfan's syndrome with pregnancies without to estimate the adjusted relative risk. RESULTS: Among 7,094,400 births in our cohort, 339 deliveries were to women with Marfan's syndrome. There was one maternal death and six aortic dissections or ruptures among births to women with Marfan's syndrome. Births to women with Marfan's syndrome were more likely to be premature (odds ratio [OR] 2.15, 95% confidence interval [CI] 1.60–2.89) and have intrauterine growth-restricted and small-for-gestational-age neonates (OR 2.06, 95% CI 1.24–3.43). Births among women with Marfan's syndrome were more commonly by cesarean delivery (OR 1.91, 95% CI 1.53–2.38) with an increased risk of forceps deliveries (OR 6.35, 95% CI 4.10–9.83) and vacuum deliveries (OR 2.01, 95% CI 1.36–2.96). Women with Marfan's syndrome were at higher risk of major morbidities including cardiac arrhythmias (OR 10.64, 95% CI 5.49–20.61), venous thromboembolisms (OR 5.24, 95% CI 1.96–14.04), disseminated intravascular coagulation (OR 7.33, 95% CI 1.83–29.38), and pneumothorax (OR 51.95, 95% CI 6.18–437.10). CONCLUSION: Women with Marfan's syndrome are at particularly high risk of adverse pregnancy and cardiovascular events during pregnancy. Preconceptional counseling should take into consideration these risks and appropriate pregnancy care in tertiary centers should be considered.