A growing problem: maternal death and peripartum complications are higher in women with grown-up congenital heart disease

Background: As patients with grown-up congenital heart disease (GUCH) increase, more women with GUCH will become pregnant. Heart surgeons may be involved in maternal GUCH care, yet the prevalence, characteristics, and outcomes for these women are unknown. We determined the national prevalence of GUCH parturients, their diagnostic makeup, and whether they have increased risk of peripartum complications, maternal or fetal death.

Methods: We searched the Nationwide Inpatient Sample for women undergoing delivery in the United States between 1998 and 2007, with GUCH patients indicated by a code for "congenital cardiac diagnosis complicating pregnancy," Patient and hospital characteristics were compared between women with and without GUCH. National estimates for maternal and fetal mortality, cardiac complications, induction, caesarean or surgically assisted birth, and preterm delivery were derived. Outcomes were compared between women with and without GUCH, and also within diagnostic GUCH subgroups.

Results: A total of 39.9 million births occurred, 26,973 (0.07%) of which were GUCH. Mean age was 27 years for both groups. Most common congenital diagnoses included ventricular septal defect (VSD) in 15%, aortic stenosis or insufficiency in 13%, atrial septal defect in 13%, pulmonary stenosis in 4%, and tetralogy of Fallot in 2%. Stillborn delivery was equivalent among groups. Maternal mortality was 18-fold higher in GUCH parturients (0.09%) compared with women without GUCH (0.005%; p < 0.001). Complications were higher for GUCH parturients compared with age-matched women, including cardiac complications (2.3% vs 0.2%), induction (37% vs 33%), caesarean or surgically assisted birth (45% vs 35%), and preterm delivery (10% vs 7%), p < 0.001 for all. A diagnosis of VSD was associated with the highest risk of maternal death and complications (p < 0.05 for all). More GUCH women delivered at teaching hospitals (58%) compared with women without GUCH (45%; p < 0.001).

Conclusions: The GUCH parturients, especially those with VSD, have increased risk of mortality and peripartum complications compared with other age-matched women. Despite these risks, nearly 50% of GUCH patients deliver at nonteaching hospitals. Current national practice patterns for GUCH women are inadequate, and outcomes could be improved by education and proper triage of even relatively "simple" GUCH lesions such as atrial septal defect and VSD. Further studies that investigate risk-adjusted outcomes in a variety of care settings are necessary to resolve this complex issue.