INTRAOPERATIVE MANAGEMENT OF A NEWBORN WITH COR TRIATRIUM DEXTRUM: A CHALLENGING CASE

Abstract

Introduction Cor triatrium dextrum is an extremely rare cardiac condition with an incidence of Methods CASE REPORT: Informed parental consent was obtained to present this case. A 20-day-old term infant weighing 2.8 kg was admitted for progressive cyanosis and difficulties to thrive. At physical examination the newborn had a peripheral oxygen saturation of 75%. Preoperative chest X-ray showed a clog-shaped heart. Preoperative NT pro-BNP was 2311 ng/L. Repeated transthoracic echocardiography confirmed the diagnosis of cor triatrium dextrum and a R-to-L shunt through a patent foramen ovale. Distal pulmonary arteries were small for age. Surgery was scheduled. At the induction of anesthesia specific attention was paid to maintain normovolemia and a blood pressure and heart rate as compared to baseline values. Induction of anesthesia was performed with 2 mg Ketamine, 0.2 mg midazolam and 2 µg sufentanil. Tracheal intubation was facilitated with cisatracurium. Anesthesia was maintained with 1% Sevoflurane and a continuous infusion of sufentanil. Intraoperative transesophageal echocardiography (TEE) confirmed the diagnosis. The TEE 4 chamber view showed a membrane within the RA (Figure 1A) with a spinnaker movement in diastole when it was bulging into the TV (Figure 2). Laterally, there was an opening within the membrane permitting turbulent blood flow from the RA through the TV (Figure 1B). Resection of the membrane was uneventful and the foramen ovale was closed. Weaning from cardiopulmonary bypass (CPB) was facilitated with a continuous infusion of milrinone. Results Figures 1A and 1B: Intraoperative TEE view of the membrane. Discussion Cor triatrium dextrum being an extremely rare CHD, anesthesiologists may not be familiar with its clinical presentations. Depending on the degree of the RA obstruction, poor right ventricle (RV) filling and compromised pulmonary blood flow may occur. Neonatal cyanosis may appear due to streaming of the blood from the RA across the atrial septum to the left atrium. Intraoperative actions need to be taken in order to increase RV filling. In this case the opening from the RA to the RV resulted in turbulent flow but intraoperative hemodynamic optimization increased this forward flow and prevented any oxygen saturation concentrations LEARNING POINTS 1. Anesthesiologists need to be aware of intraoperative hemodynamic instability and cyanosis due to the obstructive membrane within RA in case of cor triatrium dextrum. 2. Pre CPB TEE should carefully evaluate the TV that may mimick an Ebstein anomaly as the membrane may bulge into the TV.