A 38-year-old female diagnosed with bicuspid aortic valve in infancy presents to the Philadelphia Adult Congenital Heart Center for a consultative visit regarding pregnancy. She was followed in childhood at The Children’s Hospital of Philadelphia, but was lost to care in young adulthood. She had developed progressive aortic stenosis and was last seen by Cardiology eight years ago when she was pregnant with her first child. She had moderate to severe aortic stenosis at that time with peak and mean gradients of 64 mmHg and 37 mmHg, respectively, and a calculated valve area of 0.8 cm2 by echocardiogram.
She now presents in the 22nd week of her second gestation with critical aortic stenosis. Repeat echocardiogram showed peak and mean gradients of 141 mmHg and 91 mmHg, respectively, and calculated valve area 0.5 cm2. She endorsed dyspnea on exertion and had an episode of exertional syncope walking up the stairs two weeks ago.
High-risk OB/GYN was consulted and patient’s obstetric care was moved to the Hospital of the University of Pennsylvania. Interventional cardiology was consulted for possible percutaneous balloon valvuloplasty should her symptoms progress. She was followed every two to four weeks for the remainder of her pregnancy and continued to work full-time as a nurse without worsening of symptoms. Repeat echocardiogram at 32 weeks’ gestation was unchanged.
Scheduled repeat cesarean section was planned at 37 weeks’ gestation in the main OR with a multidisciplinary team consisting of OB/GYN, OB anesthesia, cardiac anesthesia, cardiac surgery and adult congenital cardiology. A 6-pound, 13-ounce healthy baby boy was delivered without incident and the patient recovered in the cardiac SICU for 24 hours. She was discharged on postpartum day three. Aortic valve replacement is currently being planned.
Left ventricular outflow tract obstruction is a risk factor for adverse cardiac maternal events. Pregnant patients with high-risk congenital cardiac lesions such as critical aortic stenosis are best served in an adult congenital heart disease center experienced in the care of these women.