Anesthetic management in pregnant women with Fontan circulation: a single-center retrospective study (2011-2026)

BACKGROUND Pregnancy in women with Fontan circulation presents substantial anesthetic and hemodynamic challanges with limited evidence to guide peripartum management. METHODS This retrospective single-center cohort study (2011-2026) included all pregnancies in women with Fontan circulation managed at a tertiary referral center. The primary outcome was anesthetic technique and the incidence of anesthesia-related hemodynamic instability during delivery. Maternal characteristics, anesthetic management, and maternal and neonatal outcomes were reviewed. RESULTS Twelve women experienced 32 pregnancies, including 17 deliveries. Cesarean delivery accounted for 76% (13/17). Among 12 cesarean with full anesthetic details, neuraxial anesthesia was used in 83% (10/12), predominantly low-dose combined spinal-epidural anesthesia with prophylactic phenylephrine infusion. Invasive arterial pressure monitoring was used in 75% (9/12). Anesthesia-related complications were limited to one transient hypotensive episode and two transient desaturation events. Two patients experiances thromboembolic events post-cesarean delivery; one patient developed postpartum pulmonary embolism, and another patient experianced transient ischemic attack. No maternal deaths occurred. Prematurity occurred in 65% (11/17) of deliveries, and 56% (10/18) of neonates required neonatal intensive care admission. CONCLUSIONS In women with Fontan circulation managed within a structured multidisciplinary program, a standardized anesthetic approach incorporating low-dose combined spinal epidural anesthesia and prophylactic phenylephrine infusion was associated with favorable maternal outcomes. These findings should be interpreted in the context of comprehensive multidisciplinary care, careful patient selection, and institutional expertise rather than attributed to the anesthetic technique alone.