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Cesarean Delivery in a Parturient with Severe Pulmonary HTN secondary to congenital PAPVR
Abstract Number: SU-85
Abstract Type: Case Report/Case Series
Intro: Pregnancy in patients with adult congenital heart disease is increasingly common and outcomes are typically favorable.(1) When a congenital lesion remains uncorrected, pregnancy can be especially risky for the mother.(2) Our case of unrepaired partial anomalous pulmonary venous return (PAPVR) highlights the importance of patient counseling, a multidisciplinary approach, and continuous delivery planning.
Case: A 36 yo G6P4 with severe PH and RV dysfunction secondary to PAPVR presented at 11 wks gestation. Her OB history included 4 prior cesarean deliveries (CD) with her last CD in 2006. In 2012, she presented with SOB and was diagnosed with severe PH and RV failure. Prior to her current pregnancy, she was managed with Tadalafil, Mecitentan, Treprostinil, Lasix, Digoxin, and O2 therapy. At that time she was being evaluated for a transplant, however she became pregnant. A 1st trimester ECHO showed normal LV size and function and severe RVE with severely reduced function RVFAC 9%, RVSP 94mmHg (RAP=15). She tolerated early pregnancy well and remained NYHA Class II-III. Delivery was tentatively planned for 32 wks, however she required admission at 31 wks for worsening SOB. Over the course of her pregnancy, she was continually discussed at meetings including MFM, cardiology, CT surgery and anesthesia. Given her worsening clinical status the above teams urgently met to prepare a more immediate delivery plan. Her symptoms improved with diuresis and we proceeded with a CD under regional anesthesia. On arrival to the OR, an arterial line and pulmonary artery catheter (PAC) were placed. She was also started on high flow iNO. After the PAC was placed, she began having short runs of VT and amiodarone was administered. Given the concern for decompensation during her anesthetic induction and CD and the inability to medically resuscitate her if decompensation were to occur, the decision was made to place femoral ECMO cannulas prior to surgical incision. Due to the need for heparin anticoagulation for cannulation, her epidural had been placed 2 hrs prior to presenting to the OR. Femoral ECMO cannulas were placed while the epidural was slowly dosed with lidocaine 2%/bicarbonate/epinephrine. Low-dose epinephrine, vasopressin, and phenylephrine were used to balance the decreased SVR. Incision was made after achieving a T5 level. Delivery was uneventful. Estimated blood loss was 1L and she received 500mL of crystalloid post-delivery. She was stable throughout the procedure and transferred to ICU postoperatively following ECMO decannulation. Her postoperative course was uncomplicated.
Discussion: Our case highlights the importance of a multidisciplinary approach and careful peripartum planning including a plan if decompensation occurs. We believe the constant communication among team members throughout pregnancy played a critical role in the positive outcome.
1. J Am Coll Cardiol. 2007;49(24):2303–2315
2. J Cardiothorac Vasc Anesth. 2013 Oct;27(5):1041-7