Extracorporeal membrane oxygenation support after the Fontan operation

ObjectiveExtracorporeal membrane oxygenation has been used to support children with cardiac failure after the Fontan operation. Mortality is high, and causes of mortality remain unclear. We evaluated the in-hospital mortality and factors associated with mortality in these patients.MethodsExtracorporeal Life Support Organization registry data on patients requiring extracorporeal membrane oxygenation after the Fontan operation from 1987 to 2009 were retrospectively analyzed. Demographics and extracorporeal membrane oxygenation data were compared for survivors and nonsurvivors. A multivariable logistic regression model was used to identify factors associated with mortality.ResultsOf 230 patients, 81 (35%) survived to hospital discharge. Cardiopulmonary resuscitation was more frequent (34% vs 17%, P = .04), and median fraction of inspired oxygen concentration was higher (1 [confidence interval, 0.9–1.0] vs 0.9 [confidence interval, 0.8–1.0], P = .03) before extracorporeal membrane oxygenation in nonsurvivors compared with survivors. Extracorporeal membrane oxygenation duration and incidence of complications, including surgical bleeding, neurologic injury, renal failure, inotrope use on extracorporeal membrane oxygenation, and bloodstream infection, were higher in nonsurvivors compared with survivors (P < .05 for all). In a multivariable model, neurologic injury (odds ratio, 5.18; 95% confidence interval, 1.97–13.61), surgical bleeding (odds ratio, 2.36; 95% confidence interval, 1.22–4.56), and renal failure (odds ratio, 2.81; 95% confidence interval, 1.41–5.59) increased mortality. Extracorporeal membrane oxygenation duration of more than 65 hours to 119 hours (odds ratio, 0.33; 95% confidence interval, 0.14–0.76) was associated with decreased mortality.ConclusionsCardiac failure requiring extracorporeal membrane oxygenation after the Fontan operation is associated with high mortality. Complications during extracorporeal membrane oxygenation support increase mortality odds. Prompt correction of surgical bleeding when possible may improve survival

Aspirin unresponsiveness predicts thrombosis in high-risk pediatric patients after cardiac surgery

ObjectiveThrombosis occurs in up to 26% of patients with congenital heart disease after cardiac surgery and is associated with increased morbidity and mortality. Aspirin is commonly administered to reduce the risk of thrombosis, yet aspirin responsiveness is rarely assessed. In this study, we hypothesize that inadequate response to aspirin is associated with increased risk of thrombosis after selected congenital cardiac procedures considered to be high risk for thrombosis.MethodsPatients undergoing high-risk congenital cardiac surgery who received postoperative aspirin (N = 95) were studied. Response to aspirin was determined using the VerifyNow system several days after administration. Patients were monitored prospectively for 30 days for the development of a thrombosis event and the relationship between aspirin unresponsiveness and a thrombosis event was determined by the Fisher exact test.ResultsRate of aspirin unresponsiveness (≥550 aspirin reaction units [ARU]) was 10 of 95 (10.5%) and was highest in patients weighing less than 5 kg given 20.25 mg/d of aspirin. Thrombosis events occurred in 7 patients (7.4%). Thrombosis was observed in 6 of 10 (60%) patients who were unresponsive to aspirin, compared with 1 of 85 (1.2%) patients who were responsive to aspirin (P < .001). In 2 patients who were unresponsive to the initial aspirin dose, an increase in dose resulted in an adequate therapeutic aspirin response (ARU < 550), suggesting insufficiency rather than true resistance in a subset of patients.ConclusionsPostoperative thrombosis is associated with aspirin unresponsiveness in this patient population. In high-risk patients, monitoring of aspirin therapy and consideration of dose adjustment or alternative agents for unresponsive patients may be justified and warrants further investigation in a prospective trial

Survival and Clinical Course at Fontan After Stage One Palliation With Either a Modified Blalock-Taussig Shunt or a Right Ventricle to Pulmonary Artery Conduit

ObjectivesWe sought to determine whether the type of shunt used at stage one palliation (S1P) affected the survival and the perioperative course through Fontan completion.BackgroundAlthough improved surgical and interstage survival have been demonstrated with the use of the right ventricle to pulmonary artery (RV-PA) conduit compared with a modified Blalock-Taussig shunt (BTS) at S1P, it is unknown whether this effect will be observed in long-term follow-up.MethodsAll patients who underwent a S1P during 2002 and 2003 (n = 80) at our institution were included for analysis. Patients were followed until death or June 1, 2007. Perioperative variables at Fontan completion were recorded.ResultsFor the entire cohort, cumulative survival for those who underwent a RV-PA conduit (n = 34) was 79.4% at 3 years compared with 65.8% in the modified BTS group (n = 46) (log-rank = 0.31). At Fontan (n = 44), when compared with those who had received a modified BTS, those who had a RV-PA conduit placed at S1P had no difference in the median duration of ventilation (21 h [range 10 to 96 h] vs. 26.5 h [range 7 to 204 h], p = 0.09) or hospital stay (9 days [range 5 to 29 days] vs. 10 days [range 6 to 48 days], p = 0.89), although length of stay in the intensive care unit was shorter (2 days [range 0 to 6 days] vs. 4 days [range 1 to 25 days], p = 0.01). Sixty-seven percent of the RV-PA conduit group had at least one PA intervention 3 years after S1P compared with 42.8% in the modified BTS group (log-rank = 0.11).ConclusionsNonstatistically significant trends toward improved cumulative survival and increased PA interventions were demonstrated in patients who had a RV-PA conduit placed at S1P. Longitudinal follow-up of larger groups of randomized patients is required to determine the influence of the RV-PA conduit on long-term outcomes

A Monte Carlo simulation approach to optimizing capacity in a high-volume congenital heart pediatric surgical center

Importance: Elective surgeries are primarily scheduled according to surgeon availability with less consideration of patients' postoperative cardiac intensive care unit (CICU) length of stay. Furthermore, the CICU census can exhibit a high rate of variation in which the CICU is operating at over-capacity, resulting in admission delays and cancellations; or under-capacity, resulting in underutilized labor and overhead expenditures. Objective: To identify strategies to reduce variation in CICU occupancy levels and avoid late patient surgery cancellation. Design: Monte Carlo simulation study of the daily and weekly CICU census at Boston Children's Hospital Heart Center. Data on all surgical admissions to and discharges from the CICU at Boston Children's Hospital between September 1, 2009 and November 2019 were included to obtain the distribution of length of stay for the simulation study. The available data allows us to model realistic length of stay samples that include short and extended lengths of stay. Main Outcomes: Annual number of patient surgical cancellations and change in average daily census. Results: We demonstrate that the models of strategic scheduling would result in up to 57% reduction in patient surgical cancellations, increase the historically low Monday census and decrease the historically higher late-mid-week (Wednesday and Thursday) censuses in our center. Conclusions and Relevance: Use of strategic scheduling may improve surgical capacity and reduce the number of annual cancellations. The reduction of peaks and valleys in the weekly census corresponds to a reduction of underutilization and overutilization of the system

Bivalirudin Anticoagulation for a Pediatric Patient with Heparin-Induced Thrombocytopenia and Thrombosis Requiring Cardiopulmonary Bypass for Ventricular Assist Device Placement

The direct thrombin inhibitor bivalirudin is an option for anticoagulation in patients with heparin induced thrombocytopenia (HIT) requiring cardiopulmonary bypass (CPB). There are a limited number of reports of pediatric patients in which bivalirudin has been used for anticoagulation for CPB. We present the case of an 11 year old male with acute onset heart failure secondary to idiopathic dilated cardiomyopathy that developed heparin induced thrombocytopenia with thrombosis (HITT). The patient was anticoagulated in the operating room with bivalirudin and placed on CPB for insertion of a HeartWare® Ventricular Assist Device (Heartware®). Modified techniques were utilized. This included use of the Terumo CDI 500 (Terumo Cardiovascular Systems, Inc.) in-line blood gas monitor which contains a heparin coated arterial shunt sensor. We flushed this sensor with buffered saline preoperatively and noted no significant decrease in platelet count postoperatively. The patient was successfully placed on the ventricular assist device and was subsequently listed for heart transplantation

Resection of hepatic tumors with central venous and right atrial extension using cardiopulmonary bypass

Background: Pediatric liver tumors occasionally present with vascular invasion. Standard anatomic resections usually result in complete resection when tumor is intra-hepatic; however, direct tumor extension into the inferior vena cava (IVC) and right atrium (RA) requires additional considerations. Here we present our surgical approach to resection of primary liver tumors directly extending into the IVC and RA. Methods: A retrospective analysis of patients undergoing hepatic resection of primary liver tumors with direct extension into the IVC and RA from 1/2013-4/2015 was performed. Results: Three patients were identified with tumors arising from the left hepatic lobe, extending into the suprahepatic IVC and RA. Two underwent in-situ parenchymal division, followed by cardiopulmonary bypass (CPB) for en-bloc resection of the intra-atrial tumor. The third had a congested right lobe due to venous obstruction by tumor, thereby requiring total hepatectomy, en-bloc intra-caval tumor excision, ex-situ left hemihepatectomy, and auto-transplantation of the right lobe. All patients required partial IVC or RA resection and reconstruction. Postoperatively, one patient died from metastatic disease at 5 months and two patients were alive after 50 months and 33 months. Conclusion: Cardiopulmonary bypass provides a safe, controlled approach for en-bloc resection of hepatic tumors extending into the IVC and RA. This minimizes the risk of tumor fracture or spillage, allowing for complete gross resection