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Severe Portopulmonary Hypertension Complicated by Congenitally Interrupted Inferior Vena Cava
Abstract Number: SU-22
Abstract Type: Case Report/Case Series
Introduction: Pulmonary hypertension (PH) has been associated with maternal mortality rates of 30-50%.(1) Early targeted therapy and multidisciplinary management are essential to optimize patient outcomes. We present the management of a patient with severe PH secondary to liver disease complicated by congenitally interrupted inferior vena cava (IVC).
Case: A 24 year old G1 female presented with a history of biliary atresia status post segmental liver transplant as an infant, portal vein stenosis resulting in liver failure, and portopulmonary hypertension. She also had a history of polysubstance abuse and noncompliance with her immunosuppressants and sildenafil. She initially presented to pulmonology at 20 weeks gestational age (GA) for right heart catheterization, which revealed PAP 90/44 and PCWP 14 mmHg. Transthoracic echocardiogram (TTE) showed a severely dilated RV with moderate systolic dysfunction. She was admitted for initiation of IV epoprostenol, which was titrated based on serial TTEs. Cardiac surgery was consulted for delivery planning in case ECMO was required. Cannulation strategy was complicated by interrupted IVC, which would likely result in inadequate venous drainage. Delivery was planned at 38 weeks GA. Left internal jugular (IJ) central line was placed preoperatively. Left radial arterial line was placed prior to induction. Cesarean delivery and tubal ligation were performed under general anesthesia. She was not a candidate for neuraxial anesthesia due to platelet dysfunction associated with IV epoprostenol as well as coagulopathy due to concomitant liver failure. Intraoperatively, she was maintained on IV and inhaled epoprostenol, as well as milrinone and epinephrine infusions. These were titrated based on intraoperative TEE and maternal hemodynamics. A right femoral arterial line was placed for arterial access, with plans for femoral venous and left IJ venous cannulation if ECMO was required for refractory RV failure. She remained hemodynamically stable, was extubated in the OR and taken to the ICU postoperatively. She was discharged on postoperative day 7. She was weaned off of her IV epoprostenol over 7 months and transitioned to oral PH medications.
Discussion: Pulmonary hypertension can occur in liver transplant recipients with or without portal hypertension, possibly from microscopic portosystemic shunts or even primary PH.(2) Severe portopulmonary hypertension treatment (anticoagulation, IV epoprostenol) requires strict patient adherence.(3) The optimum timing of delivery is uncertain and involves assessment of both maternal and neonatal risk. Cesarean delivery avoids the pushing during the second stage of labor which can cause adverse hemodynamic effects, although both general and neuraxial anesthesia also require vigilance to minimize peripheral vasodilatation.(4)
1. BJOG 2010; 227:565-74.
2. Liver Transpl 2006; 12:870-5
3. Lancet 2004; 363: 1461–1468
4. Int J Obstet Anesth 2009; 18:156-64