Pregnancy in Women with Congenital Heart Disease: The Impact of a Systemic Right Ventricle

Objective. Individuals with a systemic right ventricle develop cardiac complications earlier in life. Limited data exists regarding the effect of a maternal systemic right ventricle on cardiac events during pregnancy. We sought to assess the effect of a systemic right ventricle on cardiac events and pregnancy outcomes. Design. The study was designed as a retrospective cohort study of pregnant women with maternal congenital heart disease. Setting. The study was set in a university, academic tertiary care referral center. Patients. Study subjects were identified by International Statistical Classification of Diseases and Related Health Problems-9 codes. Women with mitral valve prolapse only or noncongenital cardiac disease were excluded. The exposure was defined by systemic ventricle. Outcome Measures. The primary outcome was a composite of congestive heart failure, arrhythmia, stroke, cardiac arrest/death during pregnancy or postpartum (CARDCOMP). The secondary outcome (PREGCOMP) was a composite of preterm delivery, preeclampsia, growth restriction, and stillbirth/pregnancy loss (PREGCOMP). Student's t-test or chi-square/Fisher's exact tests were used for comparison of continuous/categorical variables. Multivariable logistic regression was performed to control for possible confounders. Results. One hundred forty-six pregnancies in 114 women were included; 15 (10.3%) pregnancies involved a systemic right ventricle. CARDCOMP complicated 12.3% of these pregnancies. Women with a systemic right ventricle were more likely to develop CARDCOMP even after adjustment for confounders (odds ratio [OR] 6.32 [1.7-23.5], P=.006). PREGCOMP complicated 40.4% of all pregnancies. Women with a systemic right ventricle were also more likely to develop PREGCOMP (OR 5.37 [1.4-20.7], P=.015) compared with women with a systemic left ventricle after controlling for confounders. Conclusion. In women with congenital heart disease, a systemic right ventricle is associated with adverse cardiac and pregnancy outcomes. This information is critical for counseling and caring for these women. Further investigation is warranted regarding the effect of pregnancy on long-term health for this unique cohort of women.