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Item In Vitro Ultrasound Measurements of Powered and Unpowered Total Cavopulmonary Connection(Austin Publishing Group, 2014) Iliff, BP; Kerlo, AEM; Chen, J; Rodefeld, MD; Goergen, CJ; Department of Surgery, IU School of MedicineThree-staged Fontan palliation is performed on children suffering from single ventricle congenital heart disease. The series of surgical procedures reroutes blood from the vena cavae directly to the pulmonary arteries, creating a total cavopulmonary connection (TCPC). A viscous impeller pump (VIP) is currently being developed as a cavopulmonary assist device that can modestly augment cavopulmonary flow, reduce systemic venous pressure, and improve ventricular preload. This study used ultrasound to visualize complex flow patterns in powered and unpowered in vitro mock Fontan circulations. The idealized TCPC was modeled with a silicone mold and blood analog made of water and glycerol that was seeded with 10-μm glass beads. B-mode, color Doppler, and pulsed-wave Doppler images were used to visualize complex flow patterns in the idealized TCPC with (1) no VIP, (2) static VIP, and powered VIP rotation rates of (3) 500 and (4) 2,000 rotations per minute (RPM). Pulsed-wave Doppler data showed higher mean velocities and greater variance in the outlets relative to the larger inlets. The maximum inlet velocity ± SD increased from 10.9 ± 3.53 cm/s with no VIP to 15.9 ± 1.03 when the VIP was rotating at 2,000 RPM. Likewise, the maximum outlet velocity increased from 14.9 ± 11.2 cm/s to 18.9 ± 7.25 cm/s at 2,000 RPM. The faster mean velocities with the VIP rotating suggest that the pump augments cavopulmonary flow. The results of this study suggest that measuring complex flow patterns with ultrasound in vivo could be used clinically to optimize VIP positioning and rotation rate during and after implantation.Item Long Term Consequences of the Fontan Procedure and How to Manage Them(Elsevier, 2018) Kay, W. Aaron; Moe, Tabitha; Suter, Blair; Tennancour, Andrea; Chan, Alice; Krasuski, Richard A.; Zaidi, Ali N.; Medicine, School of MedicineIn 1971, Fontan and Baudet described a surgical technique for successful palliation of patients with tricuspid atresia. Subsequently, this technique has been applied to treat most forms of functional single ventricles and has become the current standard of care for long-term palliation of all patients with single ventricle congenital heart disease. Since 1971, the Fontan procedure has undergone several variations. These patients require lifelong management including a thorough knowledge of their anatomic substrate, hemodynamic status, management of rhythm and ventricular function along with multi organ evaluation. As these patients enter middle age, there is increasing awareness regarding the long-term complications and mortality. This review highlights the long-term outcomes of the Fontan procedure and management of late sequelae.Item Non-invasive cardiac output monitoring (NICOM) in adult congenital heart disease patients with Fontan palliation(Elsevier, 2021-12) Quain, Angela; Hoyer, Mark; Ephrem, Georges; Kay, W. Aaron; Medicine, School of MedicineRationale Fontan palliation for single ventricle malformations is an increasingly common reason for heart failure in the adult population. Cardiac output (CO) measurement in Fontan physiology is achieved by invasive cardiac catheterization (RHC). Noninvasive CO monitors using thoracic bioreactance (NICOM) have been validated in non-congenital patients but have not been studied in adult Fontan patients. Objective To compare RHC obtained values of CO using the Fick equation with those measured simultaneously by NICOM in a cohort of adults with Fontan palliation. Methods and results In nineteen patients undergoing routine outpatient RHC, we compared CO values as determined by Fick with those generated by the Starling SV NICOM device. Bland-Altman plots and intraclass correlation coefficients (ICCs) revealed internal consistency within NICOM measurements, however the agreement between RHC and NICOM for CO was poor (ICCs ∼ 0.40). We performed sub-analyses using two-sample T-tests and ICCs to determine if clinical cyanosis, acute desaturation, or Fontan pressure affected the difference observed between RHC and NICOM. Neither chronic hypoxia, acute desaturation, nor Fontan pressure measures were found to be associated with the observed difference between the RHC and NICOM measured CO. Discussion and conclusion Our study did not find a correlation between RHC and NICOM derived measures of CO in a cohort of Fontan patients, even in sub-analyses of confounders of Fontan physiology. We observed internal consistency within the device, which may open a role for monitoring of trends rather than absolute values in Fontan patients. Our study was limited due to small sample size.Item The Risk of Thromboembolic Complications in Fontan Patients with Atrial Flutter/fibrillation Treated with Electrical Cardioversion(Springer, 2016-10) Lin, Jiuann-Huey I.; Kean, Adam C.; Cordes, Timothy M.; Department of Pediatrics, IU School of MedicineAtrial flutter or fibrillation (AFF) remains a major chronic complication of the Fontan procedure. This complication further predisposes this patient population to thromboembolic events. However, the incidence of thromboembolic complications in Fontan patients with AFF prior to or acutely after electrical cardioversion is unknown. This study aimed to characterize the risk of post-cardioversion thromboembolic events in this population. We performed a retrospective medical record review of all patients with a history of Fontan operation treated with direct current cardioversion for AFF at Riley Children’s Hospital between June 1992 and March 2014. A total of 57 patients were identified and reviewed. A total of 216 episodes of AFF required electrical cardioversion. Patients were treated with anticoagulation/antiplatelet therapy in 86.1 % (N = 186) of AFF episodes. Right atrial or Fontan conduit clots were observed in 33 patients (57.9 %) with 61 episodes of AFF. Approximately half (49.2 %, N = 30) of these episodes were treated immediately with electrical cardioversion. Twenty-five of 33 (75.8 %) patients with intracardiac thrombi had an atriopulmonary Fontan. Five (15.2 %) patients with a lateral caval tunnel had clots in the Fontan conduit, and three (9.1 %) patients with right atrium to right ventricular outflow tract (RVOT) connections presented with right atrial mural thrombi. Nine of the 57 (15.8 %) patients had documented stroke, and three (5.3 %) patients had pulmonary emboli during follow-up, although none of these emboli were associated with electrical cardioversion. The risk of thrombus and thromboembolism associated with AFF is high in the Fontan population. However, the risk of thromboembolism associated with cardioversion in the setting of anticoagulation is very low.