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Background: Pericardial effusion (PCE) is a significant complication after pediatric cardiac surgery. This study investigates PCE development after the arterial switch operation (ASO) and its short-term and longitudinal impacts. Methods: A retrospective review of the Pediatric Health Information System database. Patients with dextro-transposition of the great arteries who underwent ASO from January 1, 2004, to March 31, 2022, were identified. Patients with and without PCE were analyzed with descriptive, univariate, and multivariable regression statistics. Results: There were 4896 patients identified with 300 (6.1%) diagnosed with PCE. Thirty-five (11.7%) with PCE underwent pericardiocentesis. There were no differences in background demographics or concomitant procedures between those who developed PCE and those who did not. Patients who developed PCE more frequently had acute renal failure (N = 56 (18.7%) vs N = 603(13.1%), P = .006), pleural effusions (N = 46 (15.3%) vs N = 441 (9.6%), P = .001), mechanical circulatory support (N = 26 (8.7%) vs N = 199 (4.3%), P

Objective: To perform a statewide characteristics and outcomes analysis of the Trisomy 18 (T18) population and explore the potential impact of associated congenital heart disease (CHD) and congenital heart surgery. Study Design: Retrospective review of the Texas Hospital Inpatient Discharge Public Use Data File between 2009 and 2019, analysing discharges of patients with T18 identified using ICD-9/10 codes. Discharges were linked to analyse patients. Demographic characteristics and available outcomes were evaluated. The population was divided into groups for comparison: patients with no documentation of CHD (T18NoCHD), patients with CHD without congenital heart surgery (T18CHD), and patients who underwent congenital heart surgery (T18CHS). Results: One thousand one hundred fifty-six eligible patients were identified: 443 (38%) T18NoCHD, 669 (58%) T18CHD, and 44 (4%) T18CHS. T18CHS had a lower proportion of Hispanic patients (n = 9 (20.45%)) compared to T18CHD (n = 315 (47.09%)), and T18NoCHD (n = 219 (49.44%)) (p < 0.001 for both). Patients with Medicare/Medicaid insurance had a 0.42 odds ratio (95%CI: 0.20–0.86, p = 0.020) of undergoing congenital heart surgery compared to private insurance. T18CHS had a higher median total days in-hospital (47.5 [IQR: 12.25–113.25] vs. 9 [IQR: 3–24] and 2 [IQR: 1–5], p < 0.001); and a higher median number of admissions (n = 2 [IQR: 1–4]) vs. 1 [IQR: 1–2] and 1 [IQR: 1–1], (p < 0.001 for both). However, the post-operative median number of admissions for T18CHS was 0 [IQR: 0–2]. After the first month of life, T18CHS had freedom from in-hospital mortality similar to T18NoCHD and superior to T18CHD. Conclusions: Short-term outcomes for T18CHS patients are encouraging, suggesting a freedom from in-hospital mortality that resembles the T18NoCHD. The highlighted socio-economic differences between the groups warrant further investigation. Development of a prospective registry for T18 patients should be a priority for better understanding of longer-term outcomes.

Introduction: With advances in care, an increasing number of individuals with single-ventricle CHD are surviving into adulthood. Partners of individuals with chronic illness have unique experiences and challenges. The goal of this pilot qualitative research study was to explore the lived experiences of partners of individuals with single-ventricle CHD. Methods: Partners of patients ≥18 years with single-ventricle CHD were recruited and participated in Experience Group sessions and 1:1 interviews. Experience Group sessions are lightly moderated groups that bring together individuals with similar circumstances to discuss their lived experiences, centreing them as the experts. Formal inductive qualitative coding was performed to identify salient themes. Results: Six partners of patients participated. Of these, four were males and four were married; all were partners of someone of the opposite sex. Themes identified included uncertainty about their partners’ future health and mortality, becoming a lay CHD specialist, balancing multiple roles, and providing positivity and optimism. Over time, they took on a role as advocates for their partners and as repositories of medical history to help navigate the health system. Despite the uncertainties, participants described championing positivity and optimism for the future. Conclusions: In this first-of-its-kind pilot study, partners of individuals with single-ventricle CHD expressed unique challenges and experiences in their lives. There is a tacit need to design strategies to help partners cope with those challenges. Further larger-scale research is required to better understand the experiences of this unique population.

Patients after heart transplantation are at increased risk for malignancy secondary to immunosuppression and oncogenic viral infections. Most common among children is posttransplant lymphoproliferative disorder (PTLD), occurring in 5% to 10% of patients. We used a national database to examine the incidence and risk factors for posttransplant malignancy.The United Network for Organ Sharing database was queried for pediatric (18 years) heart transplant recipients from October 1987 through November 2019. Freedom from malignancy after transplant was assessed with Kaplan-Meier analysis. Cox regression was performed to generate hazard ratios (HRs) and 95% CIs for risk of malignancy development.Of 8581 pediatric heart transplant recipients, malignancy developed in 8.1% over median follow-up time of 6.3 years, with PTLD compromising 86.4% of the diagnosed cancers. The incidence of PTLD development was 1.3% at 1 year and 4.5% at 5 years. Older age at the time of transplant was protective against the development of malignancy (HR, 0.98; 95% CI, 0.96-0.99; P.001), whereas a history of previous malignancy (HR, 1.9; 95% CI, 1.2-3.0; P = .007) and Ebstein-Barr virus (EBV) recipient-donor mismatch (HR, 1.7; 95% CI, 1.3-2.2; P.001) increased the risk. Induction therapy, used in 78.9% of the cohort, did not increase malignancy risk (P = .355) nor did use of maintenance tacrolimus (P = .912).PTLD occurred after 7% of pediatric heart transplants, with risk increased by younger age and EBV mismatch, highlighting the importance of PTLD monitoring in EBV-seronegative recipients. Induction therapy, used in most of the pediatric heart transplants, does not seem to increase posttransplant malignancy nor does tacrolimus, the most commonly used calcineurin inhibitor.

Currently, surgical repair of tetralogy of Fallot (TOF) is associated with an 1.1% 30-day mortality rate. Those with junctional ectopic tachycardia (JET) and restrictive right ventricular physiology have poorer outcomes. Routine postoperative adrenergic or inodilator therapy has been reported, while beta-blockade following cardiopulmonary bypass has not. This study evaluated routine perioperative treatment with esmolol in infants undergoing TOF repair.Retrospective chart review of the perioperative course following TOF repair.Single-center case series describing perioperative management of TOF in a cardiac ICU.This study reviewed all patients less than 18 months old who underwent TOF repair, excluding cases of TOF with absent pulmonary valve or atrioventricular septal defect, at our institution from June 2018 to April 2021.This review investigates the hemodynamic effects of esmolol following cardiopulmonary bypass for TOF repair.Preoperative clinical characteristics and perioperative course were extracted from the medical record. Descriptive statistics were used. Twenty-six patients receiving perioperative esmolol after TOF repair were identified and included. Postoperative hemodynamic parameters were within a narrow range with minimal vasoactive support in most patients. Three of 26 patients experienced JET, and one of 26 of whom had a brief cardiac arrest. Median and interquartile range (IQR) for hospital and postoperative length of stay was 7 days (IQR, 6-9 d) and 6 days (IQR, 5-8 d), respectively. There were no 30-day or 1-year mortalities.In this infant cohort, our experience is that the routine use of postoperative esmolol is associated with good cardiac output with minimal requirement for vasoactive support in most patients. We believe optimal postoperative management of infant TOF repair requires a meticulous multidisciplinary approach, which in our experience is enhanced with routine postoperative esmolol treatment.

Background Red blood cell transfusions (pRBCTx) are given to many patients after congenital heart surgery to augment hemodynamics, but our current understanding is limited by hourly vital sign recordings. The goal of this study was to analyze hemodynamic parameters surrounding pRBCTx with high-fidelity, real-time telemetry monitoring.Methods This is a retrospective review of patients after the arterial switch operation receiving post-operative pRBCTx from 07/15/2020-07/15/2021. Continuous, 1-Hz vital sign data were analyzed in five-minute intervals up to six hours before, four hours during, and six hours after pRBCTx—up to 57,600 data points per pRBCTx. Oxygen delivery was assessed using pre- and post-pRBCTx laboratory data, hourly renal and cerebral near-infrared spectroscopy, sedation medication doses, and vasoactive-inotropic scores.Results Six patients, median age 8.5[IQR:5–22] days and weight 3.1[IQR:2.8–3.2]kg, underwent the arterial switch operation. There were 10 pRBCTx administered with a median dose of 10[IQR:10–15]mL/kg over 169[IQR:110–190]min; at median post-operative hour 36[IQR:10–40]. There was an increase in systolic and mean arterial blood pressures by 5-12.5% at three hours after pRBCTx, but returned to baseline at six hours. There were no changes in heart rate or oxygen saturations. Renal spectroscopy increased by 6.2% after pRBCTx. There were no changes in ventilation, sedation, vasoactive support, or laboratory variables related to oxygen delivery.Conclusions pRBCTx given to arterial switch operation patients increases arterial blood pressures three hours post-pRBCTx without evidence of sustained effects at six hours. High-fidelity real-time data can be used to better assess hemodynamic parameters after congenital heart surgery and provide nuanced, patient-specific care.

Background The lifetime journey of patients with single‐ventricle congenital heart disease is characterized by long‐term challenges that are incompletely understood and still unfolding. Health care redesign requires a thorough understanding of this journey to create and implement solutions that improve outcomes. This study maps the lifetime journey of individuals with single‐ventricle congenital heart disease and their families, identifies the most meaningful outcomes to them, and defines significant challenges in the journey. Methods and Results This qualitative research study involved experience group sessions and 1:1 interviews of patients, parents, siblings, partners, and stakeholders. Journey maps were created. The most meaningful outcomes to patients and parents and significant gaps in care were identified across the life journey. A total of 142 participants from 79 families and 28 stakeholders were included. Lifelong and life‐stage specific journey maps were created. The most meaningful outcomes to patients and parents were identified and categorized using a “ capability (doing the things in life you want to), comfort (experience of physical/emotional pain/distress), and calm (experiencing health care with the least impact on daily life) ” framework. Gaps in care were identified and classified into areas of ineffective communication, lack of seamless transitions, lack of comprehensive support, structural deficiencies, and insufficient education. Conclusions There are significant gaps in care during the lifelong journey of individuals with single‐ventricle congenital heart disease and their families. A thorough understanding of this journey is a critical first step in developing initiatives to redesign care around their needs and priorities. This approach can be used for people with other forms of congenital heart disease and other chronic conditions. Registration URL: https://www.clinicaltrials.gov ; Unique identifier: NCT04613934.

Introduction: Hypoplastic Left Heart Syndrome accounts for a significant proportion of CHD morbidity and mortality, despite improvements in care and improved survival. This study evaluates number of, reasons for, and trends in discharges of patients with hypoplastic left heart syndrome over 11 years in Texas. Methods: The Texas Inpatient Discharge Dataset Public Use File captures almost all discharges in Texas and was reviewed from 2009 to 2019. Discharges of patients ≥5 years of age and diagnosis codes for Hypoplastic Left Heart Syndrome were included. The admitting and principle diagnoses were categorised and all discharges were evaluated for procedures performed. Descriptive and univariate statistical analyses were performed. Results: A total of 1024 discharges were identified with a 16.9% annual increase over the study period. Median length of stay was 4 [IQR: 2–8] and there were 17 (1.7%) in-hospital mortalities with no differences across age groups. Seven (17.1%) discharges of patients 25+ years were uninsured, higher than other age groups (p < 0.001). The most common admitting diagnosis was CHD and 224 (21.9%) of discharges included a procedure, including 23 heart transplants. Discharges occurred from 67 different hospitals with 4 (6.0%) representing 71.4% of all discharges. Conclusions: Discharges of Hypoplastic Left Heart Syndrome have increased rapidly, particularly in the older age groups and were spread over a large number of hospitals. Further work is needed to understand the interplay between Hypoplastic Left Heart Syndrome and other conditions and care experiences that occur within the general population, which will become more common as this population ages and grows.

Previous single-center studies of diaphragm paralysis (DP) after pediatric cardiac surgery report incidence of 0.3% to 12.8% and associate DP with respiratory complications, prolonged ventilation and length of stay, and mortality. To better define incidence and associations between DP and various procedures and outcomes, we performed a multicenter study.The Society of Thoracic Surgeons Congenital Heart Surgery Database was queried to identify children who experienced DP after cardiac surgery (2010-2018; 126 centers). Baseline characteristics and postoperative outcomes were compared between patients with and without DP as well as between patients who underwent plication and those who did not. Associations between center volume and center rates of DP and use of plication were also explored.A total of 2214 of 191,463 (1.2%) patients experienced DP. Postoperative DP portended worse outcomes, including mortality (5.6% vs 3.5%; P.001), major morbidity (37.2% vs 10.7%; P.001), tracheostomy (7.1% vs 0.9%; P.001), prolonged mechanical ventilation (38.0% vs 7.8%; P.001), and 30-day readmission (22.0% vs 10.6%; P.001). A total of 1105 of 2214 (49.9%) patients with DP underwent plication. Patients who underwent plication were younger, were smaller, had more risk factors, and underwent more complex surgeries. Plication rates varied widely across centers. There was no correlation between center volume and center risk-adjusted rates of DP (r = .05, P = .5), nor frequency of plication (r = .08, P = .4).DP complicating pediatric heart surgery is rare but portends significantly worse outcomes. One-half of patients underwent plication. Center-level risk-adjusted rates of DP and plication are not associated with case volume. Significant variability in plication practices suggests a target for quality improvement.

Background Survival after heart transplantation is typically reported only in terms of overall survival. Conditional survival may provide prognostic information for patients after surviving a given period. This study sought to provide an analysis of conditional survival in heart transplantation. Methods Data from 29,000 patients who underwent heart transplantation between 2002 and 2016 were analyzed from the Organ Procurement and Transplantation Network database, and 5-year conditional survival rates were calculated according to age, sex, race, renal function, and hepatic function at transplantation. Results As time from transplantation increased from 0 to 5 years, the 5-year observed conditional survival changed from 74% to 82% for ages younger than 40 years, 79% to 82% for ages 40 to 49, 79% to 78% for ages 50 to 60, and 75% to 70% for ages older than 60 at transplantation. Conditional survival peaked at 1 and 2 years after transplantation for most subgroups. In recipients younger than 40 years, men had slightly higher conditional survival than women (absolute difference, 3%-4%). In recipients older than 60 years, women had slightly higher conditional survival (absolute difference, 1%-4%). Black recipients had lower survival than white and Hispanic recipients for nearly all time points. Recipients younger than 40 years with the worst renal (65% to 88%) and hepatic function (66% to 83%) at transplantation experienced the largest increase in conditional survival. Conclusions The conditional survival of patients who undergo heart transplantation changes substantially over time. The largest increases in conditional survival are in young patients with impaired renal and hepatic function. Conditional survival can provide more accurate prognostic information for heart recipients who survive a given period after transplantation.

Background Revascularization after lower extremity bypass failure poses many challenges. Despite nearly 7 decades of experience with lower extremity revascularization, there is little data on the success of redo bypass particularly when autogenous conduit is utilized. The purpose of this study is to review outcomes of redo infrainguinal bypass constructed solely of autogenous vein. Methods All patients who underwent redo infrainguinal bypass at a single institution by a single surgeon were retrospectively reviewed. Bypasses were categorized into 3 groups: femoral-popliteal, femoral-distal, and popliteal-distal bypasses. Since the repeat bypasses were all done for limb salvage, freedom from above or below knee amputation (FFA) was primary outcome, which was defined as the number of days from redo bypass to subsequent amputation or the most recent follow-up. Results From 2006 to 2016, 100 limbs underwent redo bypass. Fifty-nine (59.0%) limbs had undergone one previous bypass while 41 (41.0%) had undergone 2 or more. The redo configurations consisted of 23 (23.0%) femoral-popliteal, 70 (70.0%) femoral-distal, and 7 (7.0%) popliteal-distal bypasses. Ninety-seven (97.0%) underwent redo using autologous vein grafts including 41 (95.5%) of those who had 2 or more previous bypasses. The 3 patients who ultimately underwent prosthetic bypass had bilateral great and small saphenous veins and bilateral basilic and cephalic veins previously harvested. Nine (9.0%) limbs were subsequently amputated: 2 (2.0%) above knee and 7 (7.0%) below knee amputations. Of these, all had had 2 or more previous bypasses and 2 of the 3 patients who ultimately received prosthetic bypasses were in this group. In patients with one previous bypass, FFA was 775 days (IQR: 213–1,626 days). In patients with 2 or more previous bypasses, FFA was 263 days (IQR: 106–1,148 days). No patients with femoral-popliteal bypasses suffered amputation while 7 (10.0%) of the femoral-distal and 2 (28.6%) of the popliteal-distal bypasses suffered subsequent amputations (P = 0.067). Conclusions Redo infrainguinal bypass is effective in salvaging threatened lower extremities. Furthermore, once a patient is deemed a bypass candidate, revascularization with autologous vein can be achieved. A significant FFA rate is achieved with redo bypass, although patients with more distal disease are harder to salvage.

Background This single-institution study assessed the midterm outcomes of patients undergoing complete vascular ring (CVR) repair and the need for reintervention. Methods The study included all patients who underwent surgical repair of an isolated CVR from 1996 to 2018 at our institution. Patients who underwent concomitant intracardiac repair were excluded. Data analysis included demographics, type of anomaly, other congenital heart disease, clinical symptomatology, operative technique, perioperative outcomes, reoperation rates, and mortality. Results CVR repair through open thoracotomy was performed in 148 patients (80 boys [54%]), median age, 1.04 years (interquartile range, 0.4-5.2 years), and median weight, 12.8 kg (interquartile range, 7.5-26.5 kg). The cohort included 72 patients with double aortic arch (DAA), 69 with right aortic arch (RAA) with aberrant left subclavian artery and left ligamentum arteriosum (LLA), 5 with RAA with left descending aorta and LLA, and 2 with RAA with mirror-image branching and LLA. There was 1 outpatient perioperative death (0.7%) 15 days postoperatively. Perioperative complications occurred in 20 patients (14%): 18 (12%) with chylothorax (3 required reintervention), 1 pneumothorax, and 1 vocal cord paresis. Two of 36 patients (5.5%) without primary diverticulum resection required reoperation and subclavian reimplantation at 3 and 4 years, and 1 patient required aortic translocation 9 years later for persistent symptoms. Conclusions Freedom from reoperation after CVR repair was 93% at 5 years and 86% at 10 years. A small proportion of patients who do not undergo diverticulum resection and aberrant left subclavian artery reimplantation at the time of CVR repair will require reintervention in the future.

Background The use of left ventricular assist devices (LVADs) as a bridge to heart transplantation has increased rapidly over the last 2 decades. We aim to explore the effect of pretransplant systemic and device-related complications on posttransplant survival for patients bridged with LVADs. Materials and methods The United Network of Organ Sharing (Organ Procurement and Transplantation Network) database was queried for all adult heart transplant recipients (aged ≥ 18 y) transplanted from April 1, 2015, to June 31, 2018. Device-related complications included thrombosis, device infection, device malfunction, life-threatening arrhythmia, and other device complications. Systemic complications included a new dialysis need or ventilator dependence between the time of listing and transplantation, transfusion, or systemic infection requiring treatment with intravenous antibiotics within 2 wk of transplantation. Results A total of 2131 patients were identified as requiring LVAD support before transplantation. LVAD patients had high rates of preoperative systemic complications (53%) and high rates of device-related complications (42.7% experienced at least one device-related complication). Kaplan–Meier analysis revealed a significantly decreased 1-y survival for LVAD patients bridged to transplantation who experienced a pretransplant systemic complication (P = 0.041). Interestingly, preoperative device-related complications had no effect on 1-y posttransplantation survival (P = 0.93). Multivariate Cox modeling revealed that systemic complications were associated with a significantly increased risk of posttransplant mortality for LVAD patients (hazard ratio 1.45; P = 0.033). Conclusions Recipients who suffered a systemic complication while awaiting heart transplantation experienced higher short-term mortality rates. Device-related complications do not appear to impact posttransplantation outcomes.

We aimed to describe the short- and long-term outcomes of patients after an arterial switch operation (ASO) at a single institution during a 23-year period. A retrospective chart review of all patients18 months of age who underwent an ASO between January 1995 and March 2018 at Texas Children's Hospital, Houston, TX was performed. Primary endpoints include mortality and reintervention. Perioperative mortality was defined as mortality occurring in-hospital and/or30 days after surgery. Survival and freedom-from-reintervention were analyzed using Kaplan-Meier method, log-rank tests, and Cox regression models. The cohort included 394 patients. Diagnoses included 204 patients (52%) with intact ventricular septum, 137 (35%) with a ventricular septal defect, 17 (4%) with a ventricular septal defect and left ventricular outflow tract obstruction (LVOTO), and 36 (9%) with Taussig-Bing anomaly. Median age at surgery was 8 days (range: 1 day to 17 months) and median weight was 3.4 (range: 0.8-12.0) kg. Overall perioperative mortality was 1.3% (n = 5), 0.3% (n = 1) since 1999. Overall survival at 5, 10, and 15 years was 98.2%, 97.8%, and 97.8%, respectively. Perioperative morality was associated with prematurity (P = 0.012),2.5 kg (P0.001), and longer circulatory arrest (P = 0.024) after univariate analysis. Reintervention was associated with a longer cross-clamp time (P0.001),2.5 kg (P = 0.009), LVOTO resection (P = 0.047), and genetic syndrome (P= 0.011) after multivariable analysis. Current ASO expectations should include a perioperative mortality risk of1% and good long-term survival. Reinterventions are more frequent in patients2.5 kg, concomitant LVOTO resection, a genetic syndrome, and longer cross-clamp time.

Background At the recent 6th World Symposium on Pulmonary Hypertension (PH), the definition of PH was redefined to include lower pulmonary artery pressures in the setting of elevated pulmonary vascular resistance (PVR). However, the relevance of this change to subjects with PH due to left-heart disease as well as the preoperative assessment of heart transplant (HT) recipients is unknown. Methods The United Network for Organ Sharing database was queried to identify adult recipients who underwent primary HT from 1996 to 2015. Recipients were subdivided into those with mean pulmonary artery pressure (mPAP) Results Over the study period, 32,465 patients underwent HT, including 12,257 (38%) with mPAP Conclusions Elevated PVR remains associated with a significant increase in the hazard for 30-day mortality after cardiac transplantation, even in the setting of lower pulmonary artery pressures. These data support the validity of the new definition of pulmonary hypertension.

Introduction: With advances in care, an increasing number of individuals with single-ventricle congenital heart disease (SVCHD) are surviving into adulthood. Partners of individuals with chronic illness have unique experiences and challenges. This pilot qualitative research study explores the lived experiences of partners of individuals with SVCHD. Methods: Partners of SVCHD patients ≥18 years were recruited and participated in Experience Group (EG) sessions and 1:1 interviews. EG sessions are lightly moderated groups that bring together individuals with similar experiences and place them as the experts to discuss lived experiences. Formal qualitative coding was performed to identify salient themes. Results: A total of 6 partners, 4 male, 4 married and all partners of someone of the opposite sex, participated. Themes identified included uncertainty of their partners future health and mortality, becoming a lay SVCHD specialist, balancing multiple roles, and providing positivity and optimism.(Table) Participants expressed a steep learning curve initially, which transforms into a strong understanding of the condition. Over time they took on the advocacy role as a repository of medical history and navigating the health system, which is often a role of parents of younger SVCHD patients. They struggled with taking on the role of primary medical caregiver of their partner while managing greater responsibility at home, and changes to their role as a spouse and a parent. Despite the uncertainties, participants described many facets of championing positivity and optimism for the future for their partner. Conclusions: In this first of its kind pilot study, partners of individuals with SVCHD expressed unique challenges and experiences in their lives. There is a tacit need to design strategies to help partners of SVCHD patients cope with those challenges. Further larger-scale research is required to better understand the experiences of this unique population.

Introduction: With advances in care, survival into adulthood of single-ventricle (SV) palliation is more frequent. Fertility and family planning in this setting is complex. In the general population, maternal mortality is 0.02%, post-partum hemorrhage (PPH) is 3.1% and pre-eclampsia is 7.5%. There are limited data on outcomes of pregnancies of women with SV physiology. This study reviews deliveries of SV mothers in Texas over the past 14 years. Methods: This is a retrospective review of the Texas Inpatient Discharge Dataset, an administrative dataset containing most hospital discharges. All discharges of females≥15 years old between 1/1/2006-12/31/2019 were reviewed. ICD-9/10 diagnosis codes consistent with Hypoplastic Left Heart Syndrome (HLHS), Double Inlet Ventricle (DIV)/Common Ventricle (CV), and Hypoplastic Right Heart Syndrome (HRHS)/Tricuspid Atresia (TA) were included. Pregnancy-related discharges were identified by ICD-9/10 codes. Descriptive and univariate statistics were utilized. Results: A total of 56 pregnancy-related discharges were identified, with 40 (71.4%) deliveries (Table). One (2.5%) was a twin gestation and 4(10%) had fetal demise. Number of annual deliveries increased from 0 in 2006 to 8 in 2019. Median gestational age was 37[IQR: 33.25-38] weeks, 7 (17.5%) had fetal anomalies and 7(17.5%) had poor fetal growth. Twelve (30%) underwent C-Section. Median length of stay was 3.5[2-5] days, 3(7.5%) mothers had PPH, 2(5%) required ventilator support, and there were no in-hospital mortalities. Conclusions: As the number of patients with SV surviving into adulthood increases, accurate counseling on risks of pregnancy is required. While these data cannot evaluate the outcomes for the delivered baby, they suggest favorable short-term outcomes for mothers with no in-hospital mortalities. Further research is needed to both understand outcomes for the delivered babies as well as potential longer-term sequelae for mothers.

Heart failure is an increasingly recognized later stage manifestation of arrhythmogenic right ventricular cardiomyopathy (ARVC) that can require heart transplantation (HT) to appropriately treat. We aimed to study contemporary ARVC HT outcomes in a national registry.The United Network for Organ Sharing registry was queried for HT recipients from 1/1994 through 2/2020. ARVC patients were compared with non-ARVC dilated, restrictive, and hypertrophic cardiomyopathy HT patients (HT for ischaemic and valvular disease was excluded from analysis). Post-HT survival was assessed using Kaplan-Meier estimates. A total of 189 of 252 (75%) waitlisted ARVC patients (median age 48 years, 65% male) underwent HT, representing 0.3% of the total 65 559 HT during the study time period. Annual frequency of HT for ARVC increased significantly over time. ARVC patients had less diabetes (5% vs. 17%, P 0.001), less cigarette use (15% vs. 23%, P 0.001), lower pulmonary artery and pulmonary capillary wedge pressures, and lower cardiac output than the 33 659 non-ARVC patients (P 0.001). Ventricular assist device use was significantly lower in ARVC patients (8% vs. 32%, P 0.001); 1 and 5 year post-HT survival was 97% and 93% for ARVC vs. 95% and 82% for non-ARVC HT recipients (P 0.001). On adjusted multivariable Cox regression, ARVC had decreased risk of post-HT death compared with non-ARVC aetiologies (hazard ratio 0.48, 95% confidence interval 0.28-0.82, P = 0.008). Patients with ARVC also had lower risk of death or graft failure than non-ARVC patients (hazard ratio 0.51, 95% confidence interval 0.32-0.81, P = 0.004).In the largest series of HT in ARVC, we found that HT is increasingly performed in ARVC, with higher survival compared with other cardiomyopathy aetiologies. The right ventricular predominant pathophysiology may require unique considerations for heart failure management, including HT.

Arrhythmias account for 55 per 100,000 patient evaluations in pediatric emergency departments. Most arrhythmias in children are amenable to medical management or cardioversion. Rarely, arrhythmias lead to significant hemodynamic instability requiring extracorporeal membrane oxygenation (ECMO) support. This study seeks to evaluate children under 1 year of age with a structurally normal heart requiring ECMO for an arrhythmia. This is a retrospective review of the Extracorporeal Life Support Organization Registry. All patients less than 1 year of age between 2009 and 2019 with a diagnosis of arrhythmia and without a diagnosis of structural heart malformation were included. Demographics, clinical characteristics, and outcomes were assessed with descriptive statistics and univariate and multivariable analyses. A total of 140 eligible patients were identified from the dataset. The most common arrhythmia was supraventricular tachycardia (SVT) in 70 (50%) patients. ECMO complications occurred in 106 (76.3%) patients and survival to discharge was achieved in 120 (85.7%) patients. In-hospital mortality was associated with neuromuscular blockade prior to ECMO [aOR 10.0 (95% CI 2.95-41.56), p 0.001], neurologic ECMO complication [aOR 28.1 (95% CI 6.6-155.1), p 0.001], and race with white race being protective [aOR 0.13, (95% CI 0.02-0.21), p = 0.002]. Similar survival and complication rates were found in subgroup analysis of SVT arrhythmias alone. Arrhythmias necessitating ECMO support in infants without structural congenital heart disease is a rare occurrence. However, survival to hospital discharge is favorable at greater than 85%. Given the favorable survival, earlier and more aggressive utilization of ECMO may result in improved outcomes.

Anomalous aortic origin of a coronary artery is a congenital condition associated with sudden cardiac death. There are no current recommendations for the acute management and urgency for this patient population. This manuscript describes and discusses two patients who presented with an acute coronary event and needed emergent intervention despite initial clinical stabilization and improvement.

Bartonella endocarditis can be an elusive diagnosis. The clinical manifestations can vary and, at times, include multiorgan involvement. This case report describes 2 patients who presented with multiorgan failure, cerebral mycotic aneurysms, and valvular endocarditis secondary to Bartonella infection. The complex diagnosis, decision making, and surgical management are described.

Background The Fontan procedure is the accepted standard for single-ventricle palliation. The goal of this study was to determine short- and midterm outcomes of patients undergoing a Fontan operation at a single institution and to identify contemporary risk factors for acute and chronic failure. Methods All patients undergoing a Fontan operation between 1995 and 2016 were included. Failure was defined as death, transplantation, Fontan takedown or revision, fenestration creation or enlargement, plastic bronchitis, protein-losing enteropathy, or major perioperative reintervention. Multivariable logistic and Cox regression models were used to identify risk factors for acute (perioperative) and chronic failure (after hospital discharge or 30 days postoperatively, or both). Results The cohort included 610 patients. Median age at surgery was 4 years. Median follow-up was 6.8 years. Trends showed increasing use of extracardiac conduits, nonfenestrated Fontan, and extubation in the operating room. Perioperative mortality was 0.5% (n = 3). Transplant-free survival at 5, 10, and 15 years was 97%, 94%, and 92%, respectively; freedom from failure was 91%, 89%, and 87%, respectively. Extubation in the operating room was associated with lower risk of acute failure (odds ratio, 0.30; 95% confidence interval [CI], 0.11 to 0.87). Independent risk factors for chronic failure included genetic syndrome (hazard ratio [HR], 2.54; 95% CI, 1.11 to 5.83), ventricular dysfunction (HR, 3.86; 95% CI, 1.81 to 8.24), cardiopulmonary bypass time in 30-minute intervals (HR, 1.242; 95% CI, 1.100 to 1.402), and persistent pleural effusions (HR, 4.26; 95% CI, 2.25 to 8.07). Moderate or severe atrioventricular valve regurgitation (HR, 2.61; 95% CI, 1.13 to 6.02) and cardiopulmonary bypass time (HR, 1.22; 95% CI, 1.03 to 1.45) were associated with reduced long-term transplant-free survival. Conclusions Contemporary midterm outcomes for Fontan patients are reassuring. Lifelong follow-up is mandatory to determine long-term outcomes and need for additional surgery as patients reach adulthood.

The use left ventricular assist devices (LVAD) as a bridge-to-transplant (BTT) has become a common modality to treat end-stage heart failure. We sought to examine the impact of BTT on long-term survival and quality of life after heart transplant. The population was all adult patients undergoing isolated heart transplantation in the United States between 2007 and 2017. Inclusion criteria covered BTT patients with a LVAD (only Heartmate II [HMII] or HeartWare Ventricular Assist System [HVAD]) and compared these with patients undergoing de novo heart transplantation. Our primary end-point was survival at 1, 2, and 5 years. Secondary end-points were functional status, return to work, and rates of hospital readmission and graft rejection. Unconditional and conditional survival was estimated with the Kaplan-Meier method. The independent influence of BTT on risk-adjusted mortality was determined using Cox proportional hazards models. In this period, 5,584 patients were bridged with an LVAD and 12,295 underwent de novo transplantation. Unconditional survival was 2% higher in de novo patients at 1, 2, and 5 years. After risk adjustment, BTT was associated with increased mortality at each time point. Unadjusted 5 year survival, conditional on 90 day survival, was similar between groups (82.6% vs. 83.4%; p = 0.15). Functional status, return to work, and unadjusted rates of hospital readmission and graft rejection were similar at 1, 2, 5 years. Bridge-to-transplant with LVADs provides excellent survival and similar quality of life to that of patients undergoing de novo heart transplantation. Bridge-to-transplant patients experience a slightly higher mortality rate within 90 days of transplantation.

BACKGROUND The impact of center volume on heart transplantation is widely recognized and serves as a benchmark for certification and reimbursement. STUDY AIMS Study sociodemographic variables associated with access to high-volume centers and substantiate the importance of extending access to underserved populations. METHODS This study focused on adults undergoing heart transplantation between 2006 and 2015. Centers were clustered into terciles (>25, 14-25, or

Spinal cord ischemia (SCI) is among the most devastating complications of thoracic endovascular aortic repair (TEVAR). Spinal fluid drainage has been proposed as a viable means to reduce SCI, but few data exist to support its routine use. This study investigated the association of preoperative spinal fluid drainage with the risk of SCI after TEVAR.The Vascular Quality Initiative TEVAR module was queried for adult patients (≥18 years) undergoing TEVAR (coverage of zones 0-5) between September 2014 and March 2018 (inclusive). Patients with preoperative spinal malperfusion, aortic rupture on presentation, and connective tissue disorders were excluded. One-to-one propensity matching was used to balance patients on 44 separate dimensions by the nearest neighbor principle to compare those with vs those without preoperative spinal drainage. The primary end point was SCI still present at the time of discharge. Secondary outcomes were 30-day mortality and prolonged intensive care unit stay (7 days).Among 4287 patients who underwent TEVAR (mean age, 67.1 [standard deviation, 13.7] years; 1665 [38.8%] women and 2622 [61.2%] men), 2076 had a spinal drain placed. Propensity matching yielded 1292 pairs with adequate covariate balance (all 44 absolute standardized differences 0.1). In the 2584 propensity-matched patients, spinal drain placement was associated with a reduced risk of SCI (1.5% vs 2.5%; risk-adjusted odds ratio [OR], 0.47; 95% confidence interval [CI], 0.24-0.89; P = .02). The rates of 30-day mortality (4.5% vs 5.0%; risk-adjusted OR, 0.67; 95% CI, 0.44-1.01; P = .05) and prolonged intensive care unit stay (7.0% vs 5.7%; risk-adjusted OR, 1.10; 95% CI, 0.84-1.45; P = .48) did not differ on the basis of spinal drain placement. The crossover rate was 10% (127/1292), and those with postoperative drain placement had a 20% (26/127) SCI rate on discharge.Among patients undergoing thoracic and thoracoabdominal endovascular aortic repair, preoperative placement of a spinal drain, compared with no drain, was associated with reduced risk of SCI. Cerebrospinal fluid drainage as a rescue measure does not provide the same protection offered by routine preoperative placement. Further investigation, including randomized controlled trials, is needed to more definitively determine the role for spinal drainage in TEVAR.

Left ventricular assist devices are increasingly used as bridge-to-transplantation in eligible patients. The HeartMate 3 (HM3; Abbott Laboratories, Abbot Park, IL) is the latest device to obtain US Food and Drug Administration approval as bridge-to-transplantation. This study examines early outcomes of transplant recipients after HM3 in comparison with recipients bridged with the HeartMate 2 (HM2; Abbott Laboratories) and HeartWare Ventricular Assist System (HVAD; Medtronic, Minneapolis, MN) devices.Using the Organ Procurement and Transplantation Network database, we identified all adult patients who were slated for bridge-to-transplantation with a continuous-flow left ventricular assist devices (HM2, HVAD, or HM3) between April 1, 2015 and January 31, 2018. The primary endpoint was all-cause mortality 6 months after transplantation. The independent influence of the bridging device on outcomes was determined using Cox proportional hazard models.Patients (N = 1,978) were successfully bridged to transplantation with the HM2 (n = 881), HVAD (n = 920), or HM3 (n = 177) device. Six-month mortality rates were similar across these devices (HM2, 5.9%; HVAD, 7.7%; HM3, 4.7%; log-rank p = 0.30). On average HM2 patients were on a left ventricular assist device for 2 months longer (p0.01). The HVAD had the lowest rate of device exchange before transplant (p = 0.01). The HM3 had no events of pump thrombosis (p0.01). HVAD patients had the lowest rate of device malfunction before to transplant (p0.01). Panel reactive antibodies at the time of transplantation were lower for HM3 patients (p0.01); however rates of graft rejection at 6 months were not different (p = 0.25).The HM3 device provides excellent early outcomes as a bridge to transplantation and may be associated with a reduction in comorbidities. Longer follow-up is needed to better define differences between durable left ventricular assist devices.

Background/Purpose Elective ambulatory surgical care traditionally involves three independent visits. Single-Visit Surgery (SVS) is an alternative surgical model that consolidates care into one visit. Evaluation of the effect of this novel program on hospital operations is limited. The objective of this study was to analyze SVS from an institutional perspective. Methods We retrospectively reviewed patients scheduled for SVS at a freestanding children’s hospital between January 2016 and August 2017. Data collected included clinic “no show” rates, operating room (OR) utilization, reimbursement rates, and postoperative visits. Results There were 89 patients scheduled for SVS, of which 63% (n = 56) were male, and the median age was 6 years [IQR, 4–9]. The SVS clinic “no show” rate was 2% (n = 2) compared to the pediatric surgery clinic “no show” rate of 11% (p = 0.01). The SVS OR block utilization rate was 90%. Payment was received from third-party payors for 92% of consultations and 100% of operative procedures without securing prior authorization. Postoperatively 25% (n = 17) of patients presented to clinic for follow-up, and one child presented to the emergency department for vomiting. There were no hospital admissions. Conclusion Single-Visit Surgery is an alternative model of ambulatory surgical care that improves institutional efficiency while also enhancing the patient experience. Type of study Retrospective cohort review Level of evidence III

Background: Given the shortage of donor organs in pediatric heart transplantation (HTx), pretransplant risk stratification may assist in organ allocation and recipient optimization. We sought to construct a scoring system to preoperatively stratify a patient’s risk of one-year mortality after HTx. Methods: The United Network for Organ Sharing database was queried for pediatric (Results: A total of 5,700 patients underwent HTx; one-year mortality was 10.7%. There was a similar distribution of variables between derivation (n = 4,560) and validation (n = 1,140) cohorts. Of the 12 covariates included in the final model, nine were allotted point values. The low-risk (score 0-9), intermediate-risk (10-20), and high-risk (>20) groups had a 5.18%, 10%, and 28% risk of one-year mortality ( P < .001), respectively. Both intermediate-risk (OR = 2.46, 95% confidence interval [95% CI]: 1.93-3.15; P < .001) and high-risk (OR = 9.24, 95% CI: 6.92-12.35; P < .001) scores were associated with an increased risk of one-year mortality when compared to the low-risk group. Conclusions: The Children’s Heart Assessment Tool for Transplantation score represents a pediatric-specific, recipient-based system to predict one-year mortality after HTx. Its use could assist providers in identification of patients at highest risk of poor outcomes and may aid in pretransplant optimization of these children.

Objective Although the morbidity associated with red blood cell transfusion in cardiac surgery has been well described, the impacts of platelet transfusion are less clearly understood. Given the conflicting results of prior studies, we sought to investigate the impact of platelet transfusion on outcomes after cardiac surgery across institutions in Maryland. Methods Using a multiinstitutional statewide database created by the Maryland Cardiac Surgery Quality Initiative, we retrospectively analyzed data from 10,478 patients undergoing isolated coronary artery bypass across 10 centers. Platelet transfusion practices were compared between institutions. Multivariate logistic regression model was used to analyze the association between platelet transfusion and 30-day mortality and postoperative complications. Results Rates of platelet transfusion varied between institutions from 4.4% to 24.7% ( P < 0.001), a difference that remained statistically significant in propensity score–matched cohorts. Among patients on preoperative antiplatelet therapy, transfusion rates varied from 8.5% to 46.4% ( P < 0.001). There was no statistically significant relationship between case volume and transfusion rates ( P = 0.815). In multivariate logistic regression, platelet transfusion was associated with increased risk of 30-day mortality (OR 2.43, P = 0.008), postoperative pneumonia (OR 2.21, P = 0.004), prolonged intubation (OR 2.05, P < 0.001), and readmission (OR 1.43, P = 0.039). Conclusions Significant variation existed in platelet transfusion rates between institutions, even after controlling for various risk factors. This variation may be associated with increased mortality and length of stay. Further study is warranted to better understand risks associated with platelet transfusion. Standardizing practice may help reduce risk and conserve resources.

OBJECTIVES Acute thoracic aortic dissection and rupture (TADR) has an incidence of 5–7 per 100 000-person years. Today, most children with congenital heart disease (CHD) survive to become adults with congenital heart disease (ACHD). This study evaluates TADR in patients with ACHD in a large, hospitalized patient population over 11 years to evaluate the incidence, risk factors and outcomes associated with TADR. METHODS This was a retrospective review of the Texas Inpatient Discharge Data Set from 1 January 2009 to 31 December 2019. All non-trauma discharges of patients ≥18 years were included. ACHD discharges were identified by International Classification of Diseases, 9th edition (ICD-9)/10 diagnosis codes. TADR were identified using 2 definitions: TADR1 is an ICD-9/10 code for TADR, and TADR2 is TADR1 with an ICD-9/10 procedure code for aortic intervention. Descriptive, univariate and logistic regression statistics were used. RESULTS A total of 22 154 664 eligible discharges were identified, of which 12 584 (0.06%) were TADR1 and a subgroup of 5699 (0.03%) were TADR2. CHD was more prevalent in TADR1 (0.2% vs 0.05%; P CONCLUSIONS ACHD discharges had a higher frequency of TADR versus the general population (0.9–1.2 vs 0.3–0.6 per 1000 discharges). There is an indication that CHD confers an increased adjusted odds of TADR. As the ACHD population continues to grow in number as well as age, it will be important to continue to assess the risk of TADR from CHD and how traditional risk factors impact this risk.

The survival of congenital heart disease (CHD) patients with single-ventricle (SV) physiology has markedly increased as a result of advances in operative techniques and postsurgical management. Nonetheless, these patients remain highly susceptible to end-stage heart failure requiring cardiac replacement therapies at early ages. Given a worldwide shortage of transplantable organs, mechanical circulatory support (MCS) represents an alternative treatment option. The significant heterogeneity of the SV population presents unique indications for MCS that have begun to be evaluated. This case study describes a 12-year-old female with heterotaxy syndrome and an SV condition, previously palliated with a Fontan operation at another institution. The patient was placed on veno-arterial (VA) extracorporeal membrane oxygenation (ECMO) during prolonged cardiopulmonary resuscitation, and later underwent HeartWare ventricular assist device (HVAD) implantation as a bridge to transplantation (BTT). A novel method was chosen to optimize careful de-airing of the heart through a minimized cardiopulmonary bypass (CPB) setup, during full ECMO support and surgical insertion of the HeartWare. The ascending aorta was vented proximal to the HVAD outflow graft anastomosis through a minimized CPB circuit at

Background:The rate of bleeding complications following arterial switch operation is too low to independently justify a prospective randomised study for benefit from recombinant factor VIIa. We aimed to evaluate factor VIIa in a pilot study.Methods:We performed a retrospective cohort study of patients undergoing arterial switch operation from 2012 to 2017. Nearest-neighbour propensity score matching on age, gender, weight, and associated cardiac defects was used to match 27 controls not receiving recombinant factor VIIa to 30 patients receiving recombinant factor VIIa. Fisher’s exact test was performed to compare categorical variables. Wilcoxon’s rank-sum test was used to compare continuous variables between cohorts.Results:Post-operative thrombotic complications were not associated with factor VIIa administration (Odds Ratio (OR) 0.28, 95% CI 0.005–3.77, p = 0.336), nor was factor VIIa administration associated with any re-explorations for bleeding. No intraoperative transfusion volumes were different between the recombinant factor VIIa cohort and controls. Post-operative prothrombin time (10.8 [10.3–12.3] versus 15.9 [15.1–17.2], p < 0.001) and international normalised ratio (0.8 [0.73–0.90] versus 1.3 [1.2–1.4], p < 0.001]) were lower in recombinant factor VIIa cohort relative to controls.Conclusions:In spite of a higher post-bypass packed red blood cell transfusion requirement, patients receiving recombinant factor VIIa had a similar incidence of bleeding post-operatively. With no difference in thrombotic complications, and with improved post-operative laboratory haemostasis, a prospective randomised study is warranted to evaluate recombinant factor VIIa.

Introduction: Contemporary experience suggests that congenital heart surgery (CHS) outcomes are optimized with an integrated, focused, and multidisciplinary program. In July 2018, our institution transitioned to an integrated Heart Center (IHC) model with new dedicated surgical, cardiac intensive care, anesthesiology, perfusion, and nursing teams. Multidisciplinary protocols and quality processes were implemented. This transition provides an unusual opportunity to evaluate the impact of an IHC model on outcomes after CHS. Methods: Patients who underwent index CHS 10/16-3/20 were included. The cohort was divided into 2 groups: historical (first 21 mo) and IHC (second 21 mo). Cases were evaluated using STAT category, mortality, and other outcomes as defined by Society of Thoracic Surgeons criteria. Descriptive statistics, univariate analyses, and multivariable regression models were used. Results: The cohort included 627 patients (historical: 230, IHC: 396, 72% increase). Numbers of STAT 4 and 5 cases increased 174% (31 to 85) and 900% (1 to 10), respectively. Neonatal cases increased 100%, infants 138%, and adults 622%. Despite an increase in complexity, perioperative mortality decreased from 3.5% to 0.5% (p=0.007) (Table). Median postoperative length of stay (LOS) increased from 5[4-10] to 7 [4-18] days (p Conclusions: Implementation of an IHC model improves perioperative survival after CHS, coupled with an increase in volumes and complexity. CHS care should be delivered as part of a dedicated and multidisciplinary IHC model.

A 21-year old woman with a history of Turner syndrome presented with a diastolic heart murmur, dizziness, dyspnea, and intermittent chest pain. Preoperative imaging revealed a fistula from the right sinus of Valsalva into the right atrium. Turner syndrome is associated with both aortopathy and congenital heart malformations. Acquired sinus of Valsalva fistula is a rare disorder, and this report describes its presence in association with Turner syndrome.

Background:We aimed to describe our experience with patients with sickle cell trait (SCT) and undergoing surgery on cardiopulmonary bypass (CPB).Methods:Data on all patients with SCT or sickle-α thalassemia who underwent surgery on CPB were collected (1996-2017).Results:Overall, 46 patients were included, 37 (80%) had SCT and 9 (20%) had sickle-α thalassemia. A total of 4 (9%) developed a potential sickle cell–related complication. Patients with sickle cell-related complications were significantly older (median 14 years vs 14 months, P = .037) and heavier (median 54 kg vs 9 kg, P = .041). Complications occurred, although without statistical significance, in patients who underwent longer median CPB times (249 minutes vs 137 minutes, P = .069), lower median temperature (31.7 °C vs 33.3 °C, P = .094), and a higher percentage underwent deep hypothermic circulatory arrest (50% vs 7%, P = .053). A total of 30 (65%) patients underwent exchange transfusion (ET) pre-bypass. Patients who underwent ET were significantly older (median 4 years vs 7 months, P = .003) and heavier (median 16 kg vs 6 kg, P = .015) than patients who did not undergo ET. The incidence of complications was comparable between patients who underwent ET (10%) and those who did not (6%).Conclusions:In this retrospective, single-center study, it has been shown that cardiac surgery requiring CPB in patients with SCT and sickle-α thalassemia had a low risk for sickle cell-associated complications. In this cohort of patients, older age, longer CPB times, lower median temperature, and the utilization of deep hypothermic circulatory arrest appear to play an important role in the development of complications.