NM-309
Liver Transplantation in an Infant with a Large Unrepaired Secundum ASD
Salamon Y, Prifti D, Seif J
Cleveland Clinic Foundation, Cleveland, OH, USA
Introduction
Our patient is an 8 month old 4.3 kg female with biliary atresia status post (s/p) Kasai at three months of age, and a large secundum atrial septal defect (ASD) presenting for liver transplant. Prior studies discussing pediatric liver transplants in patients with congenital heart disease recommend cardiac surgery before liver transplant, but this patient presented for urgent transplantation due to her liver failure and so she could not tolerate open heart surgery with cardiac bypass. We describe the preoperative evaluation and perioperative monitoring and anesthetic management of this complex patient with congenital heart disease and liver failure.
Case
This is the case of an 8 month old 4.3 kg female with biliary atresia s/p kasai at 13 weeks of age who developed failure of her kasai two months prior to admission and as a result developed hepatic failure (bilirubin of 8.8), fat soluble vitamin deficiency, and severe malnutrition requiring a nasogastric tube. Her medical history was also notable for situs ambiguus, large ASD with right ventricular dilatation but normal function, ventricular septal defect with spontaneous closure, and interrupted inferior vena cava (IVC) with azygous continuation. She presented with jaundice, bilateral scleral icterus, a distended but nontender abdomen with abdominal ascites and hepatosplenomegaly. Because of her interrupted IVC and the size of the ASD, she was not a candidate for percutaneous ASD closure. In a multidisciplinary meeting, it was decided that the patient should have liver transplantation before undergoing ASD closure. Because of possible esophageal varices due to portal hypertension, transesophageal echocardiography could not be used during the surgery. Instead, precordial doppler was used to monitor for the presence of any air in the heart.
Intraoperatively, in order to maintain pulmonary vascular resistance (PVR) and systemic vascular resistance (SVR), FiO2 remained slightly higher than room air, ventilatory parameters were vigilantly adjusted, and fluids were administered to prevent any hypotension (decreased SVR) or hypoperfusion. The patient was started on a norepinephrine drip at 0.03 mcg/kg/hr in the pre-anhepatic phase, and over the course of the case received 50 ml/kg of albumin 5%. Frequent arterial blood gases were obtained, and thromboelastogram was performed for close monitoring and to make adjustments in the anesthetic management.
The transplant was successful and liver function normalized within a few days. The patient failed extubation two times in the postoperative period, which was found by otolaryngology to be due to the abutment of a prominent pulmonary artery on the left main bronchus. Multidisciplinary discussion in the postoperative period recommended closing the ASD, as the enlargement may have been due to pulmonary overcirculation, and would be the only way to facilitate the removal of the tracheostomy.
Conclusion
Although liver transplants have been performed successfully with limited morbidity in those with an ASD, the safe anesthetic that was utilized has not been described. Close attention was paid to the central venous pressure, as well as electrolytes and acid-base physiology to minimize any changes in SVR and PVR.
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