Case reports of pregnancies in women with Shone syndrome: role for multidisciplinary planning

Shone syndrome is a complex congenital cardiac condition describing multiple obstructive left-sided heart lesions. We present four cases of pregnancies in three women with Shone syndrome and the role for preconception counseling and multidisciplinary delivery planning.

Single-centre chart review of pregnant women with Shone syndrome over 5 years, including maternal and foetal outcomes and delivery plans. There were four deliveries amongst three nulliparous women ages 25-31 years with prior surgical repairs for Shone syndrome. Multidisciplinary delivery planning occurred in the third trimester of each pregnancy. There were four term deliveries (vaginal deliveries for three, caesarean for one) with cardiac monitoring and adjustments to typical peripartum protocols. One patient required acute intensive care unit care for postpartum tachycardia and pulmonary oedema, and another required medical management of gestational hypertension. One infant required neonatal intensive care for respiratory distress. All patients and infants were discharged by postpartum Day 3 without early readmission. One patient developed acute decompensated heart failure at 7 months postpartum and died following cardiac arrest.

Women with Shone syndrome possess multiple obstructive cardiac lesions often considered in isolation as high risk for cardiac events in pregnancy, though not completely captured in risk prediction algorithms and with heterogeneity requiring case-by-case management. Multidisciplinary delivery planning including cardiologists, obstetricians, and obstetric anaesthesiologists can safely navigate immediate maternal and neonatal care needs. Pre-conception counselling and close follow-up are imperative.