Impact of new allocation policy on waitlist and transplant outcomes of adult congenital heart patients supported with ECMO.

BACKGROUND: The use of ECMO as a bridge to heart transplantation has been growing rapidly in all heart transplant recipients since the implementation of the new UNOS allocation policy; however, the impact on adult congenital heart disease (ACHD) patients is not known. METHODS: We analyzed the UNOS data (2015-2021) for ACHD patients supported with extracorporeal membrane oxygenation (ECMO) during the waitlist, before and after October 2018, to assess the impact on the waitlist and posttransplant outcomes. We compared the characteristics and outcomes of ACHD patients with or without ECMO use during the waitlist and pre- and postpolicy changes. RESULTS: A total of 23 821 patients underwent heart transplantation, and only 918 (4%) had ACHD. Out of all ACHD patients undergoing heart transplants, 6% of patients in the prepolicy era and 7.6% in the postpolicy era were on ECMO at the time of listing. Those on ECMO were younger and sicker compared to the rest of the ACHD cohort. Those on ECMO had similar profiles pre- and postpolicy change; however, there was a very significant decrease in the waitlist time [136 days (IQR 29-384) vs. 38 days (IQR 11-108), p = 0.01]. There was no difference in waitlist mortality; however, competing risk analyses showed a higher likelihood of transplantation (51% vs. 29%) and a lower likelihood of death or deterioration (31% vs. 42%) postpolicy change. Long-term outcomes posttransplant for those supported with ECMO compared to the non-ECMO cohort are similar for ACHD patients, although there was higher attrition in the first year for the ECMO cohort. CONCLUSION: The new allocation policy has resulted in shorter waitlist times and a higher likelihood of transplantation for ACHD patients supported by ECMO. However, the appropriate use of ECMO and the underuse of durable circulatory support devices in this population need further exploration.

A novel approach to retrograde autologous priming for infant, pediatric and adult populations undergoing congenital heart surgery.

INTRODUCTION: Retrograde Autologous Priming (RAP) of cardiopulmonary bypass (CPB) circuits is an effective way to reduce prime volume, commonly through the transfer of prime into separate reservoirs or circuit manipulation. We describe a simple and safe technique for RAP without the need for any circuit modifications or manipulations. METHODS: For this technique, a separate roller pump for ultrafiltration (UF) is used. After adequate heparinization and arterial cannulation, the UF pump is initiated slowly, removing prime through the effluent of the UF, replacing with the patient's blood from the aortic cannula. Once the arterial line and UF circuit are autologous primed, the arterial head displaces reservoir crystalloid toward the UF circuit at a flow rate equal to the UF pump, displacing the crystalloid prime with blood from the UF circuit, autologous priming the boot and oxygenator with blood, crystalloid again being removed by the effluent. After venous cannulation, the venous line prime is replaced with autologous blood, the crystalloid removed by the effluent of the UF circuit via the arterial head. During RAP, if the patient becomes hypovolemic, either autologous volume is transfused back to the patient, or CPB is initiated, without the need for circuitry modifications. RESULTS: The patient population in this sample consisted of 63 patients ranging between 6.1 kg and 115.6 kg. The smaller the patient, the less blood volume available for RAP and therefore the less prime volume able to be removed. Overall percent removal increases as our patients size increases compared to total circuit volume. CONCLUSION: This RAP technique is a safe and effective way to achieve a standardized asanguinous prime for many regardless of patient or circuit size in the absence of contraindications such as low starting hematocrit, emergency surgery or physiologic instability. Most importantly, this potentially reduces the amount of hemodilution patients see from CPB initiation and therefore the lowest nadir hematocrit and consequently the amount of required homologous blood products needed during surgery.

Normalizing Anti-Thrombin III for heparin management during routine cardiopulmonary bypass for congenital cardiothoracic surgery: A single institution practice review.

INTRODUCTION: Over the past decade, there has been an increase in the use of recombinant Anti-Thrombin III (AT-III) administration during neonatal and pediatric short- and long-term mechanical support for the replacement of acquired deficiencies. Recombinant AT-III (Thrombate) administration is an FDA licensed drug indicated primarily for patients with hereditary deficiency to treat and prevent thromboembolism and secondarily to prevent peri-operative and peri-partum thromboembolism. Herein we propose further use of Thrombate for primary AT-III deficiency of the newborn as well as for acquired dilution and consumption secondary to cardiopulmonary bypass (CPB). METHODOLOGY: All patients undergoing CPB obtain a preoperative AT-III level. Patients with identified deficiencies are normalized in the OR using recombinant AT-III as a patient load, in the CPB prime, or both. Patient baseline Heparin Dose Response (HDR) is assessed using the Heparin Management System (HMS) before being exposed to AT-III. If a patient load of AT-III is given, a second HDR is obtained and this AT-III Corrected HDR is used as the primary goal during CPB. Once CPB is initiated, an AT-III level is obtained with the first patient blood analysis. A subtherapeutic level results in an additional dose of AT-III. During the rewarm period, a final AT-III level is obtained and AT-III treated once again if subtherapeutic. A retrospective, matched analysis review of practice analyzing two groups, a Study Group (Repeat HDR, May 2022 onward) and Matched Group (Without Repeat HDR, July 2019 to April 2022), for age (D), weight (Kg) and operation was conducted. The focus of the study was to determine any change in heparin sensitivity identified post AT-III patient bolus load in the HDR (U/mL), Slope (U/mL/s), ACT (s), and total amount of heparin on CPB (U) and protamine (mg) used in each group. RESULTS: No significance was seen in Baseline AT-III (%), post heparin load HDR (U/mL), first CPB ACT (s), first CPB HDR (U/mL), or total CPB heparin (u/Kg) between the two groups. Statistical significance was seen in Baseline ACT (s), Baseline HDR (U/mL), Baseline Slope (U/mL/s), Post Heparin Load ACT (s), first CPB AT-III (%), and Protamine (mg/Kg) (p < .05). No statistical significance was seen in the Study Intragroup between pre versus post AT-III patient load baseline sample in ACT (s), however significance was seen in HDR (U/mL) and Slope (U/mL/s) (p < .05). CONCLUSION: Implementation of AT-III monitoring and therapy before and during CPB in conjunction with the HMS allows patients to maintain a steady state of anticoagulation with overall less need for excessive heparin replacement and potentially thrombin activation. The result is obtaining a steady state of anticoagulation, a reduced fluctuation in the heparin and ACT levels and a potential for lower co-morbidities associated with prolonged CPB times.

Hybrid First-stage Palliation and Other Strategies to Achieve Biventricular Repair in High-Risk Neonates With Complex Heart Anomalies and Aortic Arch Obstruction.

Medical and surgical advances have allowed single-stage total repair in neonates born with complex congenital heart anomalies and aortic arch obstruction. Nonetheless, total repair might be too complex or high risk in certain neonates with demographic, clinical or morphologic risk factors. Alternative management strategies might offer these neonates better outcomes with superior anatomic repair, shorter hospitalization, reduced morbidity, and improved survival. Alternative initial surgical strategies might include aortic arch repair and pulmonary artery band with or without cardiopulmonary bypass, extracardiac repair only and pulmonary artery band, Norwood operation, and hybrid first-stage palliation; all deferring complex biventricular intra-cardiac repair to later stage. The strategy choice should be personalized to each patient, taking into consideration the morphologic and clinical state, and the existent goals of care.

Interplay between donor and recipient factors impacts outcomes after pediatric heart transplantation: An analysis from the united network for organ sharing database.

BACKGROUND: Donor utilization rates continue to be low for pHT, however, efforts to expand the donor acceptance criteria have shown mixed results in single-institution studies in pediatric and adult transplantation. Purpose of this study is to assess impact of individual and cumulative donor risk factors on transplant outcomes as well as the interplay between donor and recipient risk factors as it relates to transplant outcomes. METHOD: We analyzed pHT UNOS data (2008-2018) to compare the recipient characteristics, donor characteristics, and outcomes based on donor ejection fraction of less than 50% (low EF) and or ischemic time of greater than 4 hours (prolonged IT). RESULTS: A total of 4345 pHT were performed of which 1309 (30.1%) were with prolonged IT and 122 (2.8%) in low EF. Additionally, 58 (1.3%) were performed with both low EF and prolonged IT (combined risk). Rest (2856 patients, 65.7%) was considered low risk. Recipients of combined risk were more likely to be younger, have post-surgical congenital heart disease, be on ECMO or ventilator but less likely on VAD (all P < .01) compared with any other group. Waitlist time was significantly lower for low EF (mean 39 days, 15-109) or combined risk group (36 days, range 15-80) compared with other groups (60 days, range 23-125) (P = .01). 1-year mortality was 8% in low-risk group, 12% in prolonged IT, 14% in reduced EF, and 28% in combined risk patients (P < .01). Number of treated rejections in one year were significantly higher in prolonged IT and combined risk group compared to other groups (P < .01). When stratified by recipient risk, there was no difference in outcomes for low risk, prolonged IT, or low EF groups; however, there were significant survival differences for high-risk recipient versus low-risk recipient in each donor group. CONCLUSION: Lower EF donors performed similar to prolonged IT donor, but were uncommonly used. Acceptance of risk was common in recipients deemed higher risk for waitlist mortality and led to shorter wait times. Caution should be used in accepting combined risk transplants. The recipient risk factors have significant impact on outcomes across all donor risk groups and further analysis will help balance the waitlist mortality with post-transplant outcomes.

Modified Microvascular Plug as a Flow Restrictor in Hypoplastic Left Heart Syndrome with Dysplastic Tricuspid and Pulmonary Valves.

While the Norwood operation is the most common palliative surgery for neonates with hypoplastic left heart syndrome (HLHS), initial hybrid strategy aiming to restrict pulmonary blood flow and maintain systemic output is necessary when Norwood is contraindicated or at high risk. The traditional mainstay of initial hybrid palliation is surgical pulmonary artery branch banding (PABB) plus interventional ductal stenting. We present a case of a transcatheter approach for pulmonary flow restriction (PFR) that was accomplished by modifying a Medtronic microvascular plug (MVP)™. The patient is a 2.4 kg neonate diagnosed with HLHS, dysplastic tricuspid and pulmonary valves with pulmonary stenosis, tricuspid stenosis, and regurgitation. He was not considered a candidate for surgical intervention. He started developing sequelae of unbalanced pulmonary and systemic blood flow; therefore, he underwent placement of transcatheter PFR as alternative to PABB. He underwent successful orthotopic heart transplant 104 days after index procedure. This case demonstrates the significant complexity that can occur in patients with HLHS and abnormal right sided valves. Additionally, it is another example that transcatheter branch pulmonary artery flow restriction can be a safe and feasible alternative to PABB as initial palliation strategy. It may benefit patients in whom primary Norwood procedure would have increased risks or who will undergo primary transplant.

The Evolution of an Adult Congenital Heart Surgery Program: The Emory System.

The Emory Adult Congenital Heart (Emory University, Atlanta, GA) program was founded in 2001. In 2004, the surgical component transitioned from a pediatric facility to an adult facility. The aim of this article is characterize the program as a whole, outline changes in the program, and discuss the challenges of the transition process. Between 2001 and 2015, changes in program structure and personnel were evaluated. There has been significant growth of the program between 2001 and 2015. There are currently 19 half-day clinics per week, with 2,700 clinic visits per year. There are six cardiologists, three congenital cardiac surgeons, two sonographers, one advanced practice provider, and one social worker dedicated to the program. There are Accreditation Council for Graduate Medical Education-accredited adult congenital cardiology and congenital cardiac surgery fellowships. One thousand forty-four operations were performed between 2001 and 2015. There were 828 open-heart operations, of which 581 (70%) were re-operations. Over the study period, the number of yearly operations increased from 30 to 119, and the mean age at surgery increased from 22 to 35 years. Over time, more of the operations were performed at the adult hospital: increasing from 3% in 2001 to 82% in 2015, and more of the operations were performed by congenital cardiac surgeons: 87% (114 of 131) before the 2004 transition to 97% (881 of 913) afterward. The Emory Adult Congenital Heart program has undergone significant growth and change, including transition of the surgical component from the pediatric to the adult facility. While numerous obstacles have been overcome and great progress has been made, additional challenges remain.

A 30-year experience with mixed-type total anomalous pulmonary venous connection: a word of caution.

BACKGROUND: Patients with total anomalous pulmonary venous connection can be problematic, particularly those with mixed-type pathology. We aimed to describe a cohort of patients with mixed-type anomalous drainage, highlighting the treatment challenges, and identifying risk factors for poor outcome. METHODS: We reviewed the clinical records of patients who underwent repair for mixed-type total anomalous pulmonary venous connection between 1986 and 2015. RESULTS: A total of 19 patients were identified. The median age and weight of patients at surgery were 18 days (with a range from 1 to 185) and 3.4 kg (with a range from 1.9 to 6.5), respectively. Venous anatomy included a combination of duplicate supracardiac (four), supracardiac and cardiac (11), and supracardiac and infracardiac (four) drainage. Out of 19 patients, six (32%) died within 30 days or the initial hospital stay; two additional patients died from progressive pulmonary vein stenosis at 72 and 201 days, respectively, resulting in 42% mortality within the 1st year. Follow-up data were available for 8/11 long-term survivors. The median follow-up period was 7.3 years (with a range from 1.8 to 15.7). Only one patient underwent re-intervention for recurrent pulmonary vein stenosis. For surgical mortality, no statistically significant risk factors were identified, although the risk trended to be higher (p⩽0.1) with lower age and weight, an infracardiac component, and prolonged cardiopulmonary bypass. For 1-year mortality, the risk became significant (p⩽0.05) with a lower weight (p=0.01), an infracardiac component (p=0.03), and prolonged cardiopulmonary bypass (p=0.04). CONCLUSION: The surgical and 1-year mortality in patients with mixed-type total anomalous pulmonary venous connection is high. On the other hand, among patients who survive past the 1st year, most have good outcomes without subsequent sequelae.

Reducing readmissions following paediatric cardiothoracic surgery: a quality improvement initiative.

BACKGROUND: We have previously identified risk factors for readmission following congenital heart surgery - Hispanic ethnicity, failure to thrive, and original hospital stay more than 10 days. As part of a quality initiative, changes were made to the discharge process in hopes of reducing the impact. All discharges were carried out with an interpreter, medications were delivered to the hospital before discharge, and phone calls were made to families within 72 hours following discharge. We hypothesised that these changes would decrease readmissions. METHODS: The current cohort of 635 patients underwent surgery in 2012. Demographic, preoperative, operative, and postoperative variables were evaluated. Univariate and multivariate risk factor analyses were performed. Comparisons were made between the initial (2009) and the current (2012) cohorts. RESULTS: There were 86 readmissions of 77 patients during 2012. Multivariate risk factors for readmission were risk adjustment for congenital heart surgery score and initial hospital stay >10 days. In comparing 2009 with 2012, the overall readmission rate was similar (10 versus 12%, p=0.27). Although there were slight decreases in the 2012 readmissions for those patients with Hispanic ethnicity (18 versus 16%, p=0.79), failure to thrive (23 versus 17%, p=0.49), and initial hospital stay >10 days (22 versus 20%, p=0.63), they were not statistically significant. CONCLUSIONS: Potential risk factors for readmission following paediatric cardiothoracic surgery have been identified. Although targeted modifications in discharge processes can be made, they may not reduce readmissions. Efforts should continue to identify modifiable factors that can reduce the negative impact of hospital readmissions.

Outcomes and hospital costs associated with the Norwood operation: beyond morbidity and mortality--CORRIGENDUM.

This paper contains an error in Table 4. The authors apologise for this error. In the last row of the Table, it states: Reimbursement (charges/collections)=46% It should actually be: Reimbursement (collections/ charges)=46%

Outcomes and hospital costs associated with the Norwood operation: beyond morbidity and mortality.

BACKGROUND: Although much is known about morbidity and mortality, there are limited data focussing on the financial aspect of the Norwood operation. Our objective is to characterise the hospitalisation and detail the hospital costs. METHODS: We retrospectively studied 86 newborns with hypoplastic left heart syndrome who underwent Norwood palliation between 2008 and 2012. Clinical and financial data were collected. Financial data have been reported for 2011-2012. RESULTS: At surgery, median age and weight of the patients were 4 days (range 1-13) and 3 kg (range 2-4.8), respectively. The median time from admission to surgery was 4 days (range 1-10), with the postoperative ICU stay and total length of stay at the hospital being 10 days (range 4-135) and 16 days (range 5-136), respectively. Discharge mortality was 14/86 (16%) patients. For patients operated on between 2011 and 2012 (n=40), median hospital costs, charges, and collections per patient were $117,021, $433,054, and $198,453, respectively, and mean hospital costs, charges, and collections per patient were $322,765, $1,109,500, and $511,271, respectively. A breakdown of total hospital costs (direct and indirect) by department showed that the top four areas of resource utilisation (excluding physician fees) were as follows: the cardiac ICU (35%), laboratory (12%), pharmacy (12%), and operating room (7%). Interestingly, point-of-care laboratory evaluations accounted for almost half of the laboratory total (5%). Extracorporeal membrane oxygenation, although only utilised in eight patients between 2011 and 2012, accounted for 7% of utilisation.General radiology only accounted for 2%, despite numerous radiographs. CONCLUSIONS: Limited data are available that detail the hospitalisation and costs associated with the Norwood operation. We hope that this analysis will identify areas for quality and value improvement from both system and patient perspectives.

Associated Factors and Impact of Persistent Renal Dysfunction in Pediatric Heart Transplantation.

BACKGROUND: We evaluated the impact of significant renal dysfunction (SRD) on listing and pediatric heart transplantation (PHT) outcomes. METHODS: The United Network of Organ Sharing registry was queried. Our cohort included 11,625 children listed for PHT (2000-2020). At listing, 1494 (13%) had SRD, defined as an estimated glomerular filtration rate of <45 mL/min/1.73 m2 and/or dialysis requirement. Characteristics of children with and without SRD were compared. SRD impact on outcomes was examined. Factors associated with waiting list mortality, persistent SRD at PHT, and post-PHT survival with and without simultaneous heart-kidney transplantation were assessed. RESULTS: Compared with children with an estimated glomerular filtration rate >45 mL/min/1.73 m2, those with SRD had higher waiting list death (37% vs 14%, P < .01) and lower transplantation rate (51% vs 71%, P < .01). On multivariable analysis, SRD was associated with waiting list death (hazard ratio, 3.016; P < .0001). Among 767 children with SRD who received PHT, 361 (47%) had persistent SRD at the time of PHT. On multivariable analysis, factors associated with persistent SRD were older age (odds ratio [OR], 1.147 per year; 95% CI, 1.046-1.258 per year; P = .0035), bilirubin (OR, 1.127 per 1-mg/dL; 95% CI, 1.061-1.197 per 1-mg/dL; P < .0001), dialysis (OR, 1.839; 95% CI, 1.017-3.326; P = .0115), mechanical ventilation (OR, 1.972; 95% CI, 1.336-2.911; P = .0006), extracorporeal membrane oxygenation (OR, 1.747; 95% CI, 1.074-2.842; P = .0247), and not using a ventricular assist device (VAD) (OR, 0.498 [VAD use]; 95% CI, 0.277-0.895 VAD use; P = .0198). Post-PHT survival was 72%, 70%, and 56% (P < .01) at 8 years for PHT alone with improved renal function, simultaneous heart-kidney transplantation (n = 69), and PHT alone with persistent SRD, respectively. CONCLUSIONS: SRD is associated with high waiting list death and decreased transplantation rate. Timely proper pre-PHT support with VAD could enhance kidney recovery. Simultaneous heart-kidney transplantation neutralized persistent SRD effect on survival and might be considered in high-risk patients such as those on dialysis, mechanical ventilation, or extracorporeal membrane oxygenation support.

Perioperative Morbidity and Outcomes in Pediatric Patients Transitioned From Extracorporeal Membrane Oxygenation to Ventricular Assist Device Support: A Study of the Society of Thoracic Surgeons Congenital Heart Surgery Database.

As a bridge to transplant strategy, children transitioned from extracorporeal membrane oxygenation (ECMO) to ventricular assist device (VAD) have higher waitlist mortality compared with those who receive de novo VAD. However, the contribution of the immediate perioperative period and differences in the two groups are not well studied. We performed a nested case-control study between children receiving de novo VAD (group 1) and those transitioned from ECMO to VAD (group 2) between 2014 and 2019 using The Society of Thoracic Surgeons (STS) database. A total of 735 children underwent VAD placement with 498 in group 1 and 237 in group 2. Patients in group 2 were significantly younger, smaller, and significantly sicker, were twice as likely to transition to biventricular VAD and need unplanned reoperations. Overall mortality was 16% for group 1 and 34% for group 2 ( p < 0.01). Regression analysis showed that ECMO use (odds ratio [OR], 2.17 [1.3-3.4]), ventilator need (OR, 2.2 [1.3-3.9]), and cardiogenic shock (OR, 1.8 [1.2-2.8]) were all independent preoperative predictors of VAD mortality while dialysis need (OR, 25.5 [8.6-75.3]), stroke (OR, 6.2 [3.1-12.6]), and bleeding (OR, 1.9 [1.1-3.4]) were independent postoperative predictors of VAD mortality within 30 days (all p < 0.05). The study demonstrated significant baseline differences between the two cohorts, warranting avoidance of comparison. Early elective VAD placement in this cohort of patients should be sought to avoid interim ECMO and high post-VAD mortality.

Outcomes of heart transplantation in children with previously palliated hypoplastic left heart syndrome.

OBJECTIVES: Paediatric heart transplantation in children who fail multistage palliation for hypoplastic left heart syndrome is associated with challenges related to immune, clinical or anatomic risk factors. We review current outcomes and risk factors for survival following heart transplantation in this challenging patient population. METHODS: The United Network for Organ Sharing transplantation database was merged with Paediatric Health Information System database to identify children who received heart transplantation following prior palliation for hypoplastic left heart syndrome. Multivariable Cox analysis of outcomes and factors affecting survival was performed. RESULTS: Our cohort included 849 children between 2009 and 2021. The median age was 1044 days (interquartile range 108-3535), and the median weight was 13 kg (interquartile range 7-26). Overall survival at 10 years following heart transplantation was 71%, with most of the death being perioperative. On multivariable analysis, risk factors for survival included Black race (hazard ratio = 1.630, P = 0.0253), blood type other than B (hazard ratio = 2.564, P = 0.0052) and male donor gender (hazard ratio = 1.367, P = 0.0483). Recipient age, the use of ventricular assist device or extracorporeal membrane oxygenation were not significantly associated with survival. Twenty-four patients underwent retransplantation, and 10-year freedom from retransplantation was 98%. Rejection before hospital discharge and within 1 year from transplantation was 20% and 24%, respectively, with infants having lower rejection rates. CONCLUSIONS: Compared with existing literature, the number of children with prior hypoplastic left heart syndrome palliation who receive heart transplantation has increased in the current era. Survival following transplantation in this patient population is acceptable. Most of the death is perioperative. Efforts to properly support these patients before transplantation might decrease early mortality and improve overall survival.

The Use of Impella 5.5 Reduces Pulmonary Vascular Resistance as Bridge to Heart Transplant in a Pediatric Patient.

This case report explores the successful use of the Impella 5.5 (Abiomed Inc., Danvers, MA) device in a 22 kg, 0.89 body surface area (BSA) pediatric patient with longstanding dilated cardiomyopathy and elevated pulmonary vascular resistance. The article details the device selection, management, and outcome demonstrating the feasibility and potential benefits of the Impella 5.5 as a device within the pediatric population.

Outcomes of heart transplants in children with heterotaxy syndrome.

OBJECTIVES: End-stage congenital heart disease (CHD) in children with heterotaxy syndrome might necessitate a heart transplant (HTx). An HTx in heterotaxy patients can be associated with several technical (e.g. redo, systemic/pulmonary-venous/situs anomalies, pulmonary artery reconstruction) and extra-cardiac (e.g. ciliary dyskinesia, infections, gastrointestinal) challenges. Our goal was to determine if heterotaxy syndrome is associated with increased early or late transplant risks. METHODS: The United Network for Organ Sharing transplant database was merged with the Paediatric Health Information System administrative database to identify children with heterotaxy who received an HTx. Characteristics and outcomes were compared between children with heterotaxy and contemporaneous non-heterotaxy congenital and non-congenital cardiomyopathy control groups. RESULTS: After we merged the databases, we divided our cohort of 1122 patients into 3 groups: the heterotaxy (n = 143), group the non-heterotaxy congenital (n = 428) group and the cardiomyopathy (n = 551) group. There were differences in the characteristics between the 3 groups, with the heterotaxy group being comparable to the non-heterotaxy congenital group. The waiting list duration was longer for the heterotaxy than for the non-heterotaxy congenital and cardiomyopathy groups (91 vs 63 vs 56 days, P < 0.001). Early post-transplant complications were similar for all groups except for operative mortality, which was 1% for the cardiomyopathy and 4% for the heterotaxy and non-heterotaxy congenital groups (P < 0.001). The post-transplant hospital stay was shorter for the cardiomyopathy (57 days) compared to the non-heterotaxy congenital (99 days) and heterotaxy (89 days) groups (P < 0.001). Whereas rejection prior to discharge was comparable between the heterotaxy and the CHD groups, it was higher at 1 year for the heterotaxy (22%) than for the non-heterotaxy congenital (19%) and cardiomyopathy (13%) groups (P < 0.001). Survival at 5 years was superior for the cardiomyopathy (87%) compared to the heterotaxy (69%) and non-heterotaxy congenital groups (78%) (P < 0.001). For the heterotaxy group, no risk factors affecting survival were identified on multivariable analysis. CONCLUSIONS: Regardless of the complexity, an HTx in selected children with heterotaxy is associated with good mid-term outcomes. Despite early results that are comparable to those of other patients with CHD, the increasing rejection rate at 1 year and the relatively accelerated attrition at mid-term warrant further follow-up. Due to database limitations in defining morphologic and surgical details, further work is warranted to delineate anatomical and surgical variables that could affect survival.

Trends and outcomes of heart transplantation in adults with congenital heart disease.

OBJECTIVES: Heart transplantation for adult congenital heart disease is complicated and associated with challenging pretransplant support, long waiting and high early post-transplant mortality. We explored if surgical and medical advances and allocation system changes have affected outcomes. METHODS: From United Network for Organ Sharing database, adults with congenital heart disease listed for heart transplantation were queried. To explore practice and outcome trends, patients were divided into 4 eras (eras 1-3: nearly 3 equal periods from 1992 to 2018, era 4: after 2018, corresponding with new allocation system). Univariate and multivariable analysis was performed to evaluate outcomes. RESULTS: A total of 2737 patients were listed. There was gradual increase in listed and transplanted patients, along with significant increase in use of mechanical support, simultaneous kidney and liver transplantation. While proportion of transplanted remained constant, there was decrease in proportion delisted/died after listing (P = 0.01) and waiting list duration (P = 0.01), especially in era 4. Thirty-day post-transplant mortality remains high; however, it has significantly improved starting era 3 (P = 0.01). Current survival at 1-year and 5-years is 85% and 65%, with improvement mainly related to decreased early death. On multivariable analysis, factors associated with survival were lower glomerular filtration rate (hazard ratio = 0.99, P = 0.042), bilirubin (hazard ratio = 1.17, P<0.001) and mechanical ventilation (hazard ratio = 2.3, P=0.004). CONCLUSIONS: Heart transplantation in adults with congenital heart disease is increasing, along with added complexity, higher usage of pretransplant mechanical support and simultaneous organ transplantation. Despite that, more complex patients do not experience worse outcomes. Early mortality improved but remains high. New donor allocation system allowed shorter waiting time and higher proportion transplanted without altering early mortality.