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///2018 Abstract Details
2018 Abstract Details2019-08-02T15:57:01-05:00

Intrapartum management of a patient with pulmonary atresia with associated severe pulmonary hypertension

Abstract Number: S4D-10
Abstract Type: Case Report/Case Series

Amanda K Knutson MD, PhD1 ; Luke Liang MD2; Samir K Patel MD3; Nicole Higgins MD4

Introduction: Patients with pulmonary hypertension (PH) during pregnancy pose a particular challenge for anesthesia providers. Pregnancy may be contraindicated, given a maternal mortality rate as high as 36% across all WHO groups (1-3). The fetus is also at a higher risk of morbidity and mortality, including death, IUGR and preterm delivery (4).

Case: 34 year old G3P2 at 34.4 weeks with history of congenital pulmonary atresia (severe PH, most likely WHO 2), left lung hypoplasia, asthma, and aberrant left subclavian artery causing tracheal compression presented to L&D for elevated blood pressures, worsening DOE, orthopnea requiring supplemental oxygen, and lower extremity edema.

A CXR demonstrated pulmonary edema, and TTE showed RVP of 65/15mmHg, and mildly decreased RV systolic function. She was treated with labetalol for HTN and steroids in anticipation of preterm delivery.

At 35 weeks, her oxygenation status worsened and the decision was made to proceed with induction of labor; however, on exam, fetus was breech. External cephalic version (ECV) was planned in the operating room with preprocedure arterial line and a single dose of sildenafil. Neuraxial analgesia with epidural catheter was performed and subsequently dosed incrementally to obtain an appropriate level. After successful ECV, the patient was transferred to L&D for induction of labor with continuous pulse oximetry, invasive blood pressure monitoring and EKG. Emergency vasoactive medications were kept at bedside. The patient delivered uneventfully and, at the time of this report, is without complication.

Discussion: Patients with PH are at increased risk of peripartum morbidity and mortality. Special precautions must be taken in patients who desire to continue the pregnancy to delivery, including arterial catheterization, continuous EKG and pulse oximetry and emergency medications in case of cardiovascular collapse at bedside. Additionally, initiation of nitric oxide should be considered. Caring for these complex patients necessitates a multidisciplinary team familiar with the care of high risk patients.


1. Pieper PG, Hoedermis ES. Neth Heart J 2011: 19(12):504-8.

2. Bonnin M,et al. Anesthesiology 2005: 102:1133-7.

3. Simonneau Get al. J Am Coll Cardiol 2013; 62:S34.

4. Weiss BM, et al. J Am Coll Cardiol 1998; 31:1650-7.3.

SOAP 2018