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Management of Severe Pulmonary Stenosis in Tetrology of Fallot Parturient
Abstract Number: RF4AD-467
Abstract Type: Case Report Case Series
Tetralogy of Fallot (TOF) is the most common congenital heart disease (CHD) with an incidence of 4 in 10,000 live births (1). From a reported 30-yr survival of 77% in a cohort of 106 pts in 1954 (2), the surgical technique has substantially improved 25-yr survival rate to 94.5% (3). The number of adults with CHD continues to grow as therapies and surgical interventions become more effective. The majority of women with CHD reach reproductive years, and despite the moderate to high risks associated with pregnancy, recommendations are available to navigate these parturients through pregnancy (4). In this case report, we describe the management of a TOF pt with severe pulmonary stenosis.
22 yo G4P0030 with history of TOF with pulmonary atresia s/p Ross procedure with prosthetic valve replacement in 2007 presents to the hospital at 34w3d for her 4th cordocentesis and PUBS procedure in the setting of persistently elevated MCA dopplers and IUGR with anti-Kell and anti-S antibodies. Complicating her medical history are late care at 21w GA, autism with learning disabilities, chromosomal 19p13.11 2.2m deletion (present in three generations, including fetus), severe anxiety, and PSA.
Her pre-pregnancy TTE, approximately 6 months prior, revealed peak gradient across her homograft of 65mmHg and a RVSP of 42mmHg. Her first pregnancy TTE occurred at 26w1d with peak gradient of 71mmHg and RVSP of 100mmHg. With each subsequent ECHO, she continued to have further accentuation of her pulmonary stenosis: at 34w2d a peak gradient of 116mmHg and RVSP 135mmHg and at 37w0d a peak gradient unobtainable due to positioning and RVSP 149mmHg.
She was admitted to the L&D unit at 37w0d for early neuraxial placement via DPE technique and arterial line placement after midazolam 4mg and fentanyl 100mcg. Her labor course was unremarkable and she was transferred to the OR for FAVD after 36 hours. She received a bolus of 2% lidocaine with epinephrine 10mL and required support with phenylephrine gtt and vasopressin boluses. However, immediately after delivery with 1.2L EBL, the pt became tachycardic to the 130s. A central line was placed, 1u PRBC was administered, a Nexplanon was implanted and pt was transferred to the CICU. 6 weeks postpartum, the pt had a repeat TTE showing a peak gradient of 89mmHg with RVSP 100mmHg. She received a replacement 23mm SAPIEN transcatheter valve with a subsequent gradient of 20mmHg across the new valve and RVSP 28mmHg.
While the medical management of this pt is remarkable, her psychosocial situation offered additional challenges questioning her ability to offer informed consent for her labor and postpartum contraception. She required hours of coaching before each neuraxial placement and developed PTSD related to the multiple procedure required in the setting of fetal anemia. This poses an interesting discussion of pre-conception counseling in high risk patients associated with significant mental health and cognitive impairment.