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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.

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.

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.

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.

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: There are more than 1.4 million adult congenital heart disease (ACHD) patients estimated in the United States. As this population continues to grow, as will their utilization of the healthcare system. Objectives: This study aims to analyze trends in discharges of ACHD patients in the state of Texas from 2009 through 2018. Methods: This is a retrospective review of the Texas Inpatient Discharge Public Use File from 2009 through 2018. Discharges from acute care hospitals of patients ≥18 years of age with an International Classification of Diseases 9 or 10 diagnosis code consistent with a CHD diagnosis which could be categorized by the American Heart Association/American College of Cardiology lesion severity score (LSS). Isolated atrial septal defect and congenital aortic valve insufficiency diagnoses were excluded from the analysis. Results: A total of 10,515 ACHD discharges were identified. Annual discharges increased from 930 to 1,315, a 41% increase during the study period (p-trend

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.

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.

Background: Intraseptal anomalous aortic origin of a coronary artery is considered a benign condition. However, there have been case reports of patients with myocardial ischemia, arrhythmia, and sudden cardiac death. The purpose of this study was to determine the clinical presentation, myocardial perfusion on provocative stress testing, and management of children with anomalous aortic origin of a coronary artery with an intraseptal course in a prospective cohort. Methods: Patients with anomalous aortic origin of a coronary artery and intraseptal course were prospectively enrolled from December 2012 to May 2019, evaluated, and managed following a standardized algorithm. Myocardial perfusion was assessed using stress imaging. Fractional flow reserve was performed in patients with myocardial hypoperfusion on noninvasive testing. Exercise restriction, β-blockers, and surgical intervention were discussed with the families. Results: Eighteen patients (female 6, 33.3%), who presented with no symptoms (10, 55.6%), nonexertional (4, 22.2%), and exertional symptoms (4, 22.2%), were enrolled at a median age of 12.4 years (0.3–15.9). Perfusion imaging was performed in 14/18 (77.8%) and was abnormal in 7/14 (50%); fractional flow reserve was positive in 5/8 (62.5%). All 4 patients with exertional symptoms and 3/10 (30%) with no or nonexertional symptoms had myocardial hypoperfusion. Coronary artery bypass grafting was performed in a 4-year-old patient; β-blocker and exercise restriction were recommended in 4 patients not suitable for surgery. One patient had nonexertional chest pain and 17 were symptom-free at median follow-up of 2.5 years (0.2–7.1). Conclusions: Up to 50% of patients with intraseptal anomalous aortic origin of a coronary artery had inducible myocardial hypoperfusion during noninvasive provocative testing. Long-term follow-up is necessary to understand the natural history of this rare anomaly.

Background: Anomalous aortic origin of a coronary artery (CA) is the second leading cause of sudden cardiac death in young athletes. Management is controversial and longitudinal follow-up data are sparse. We aim to evaluate outcomes in a prospective study of anomalous aortic origin of CA patients following a standardized algorithm. Methods: Patients with anomalous aortic origin of a CA were followed prospectively from December 2012 to April 2017. All patients were evaluated following a standardized algorithm, and data were reviewed by a dedicated multidisciplinary team. Assessment of myocardial perfusion was performed using stress imaging. High-risk patients (high-risk anatomy—anomalous left CA from the opposite sinus, presence of intramurality, abnormal ostium—and symptoms or evidence of myocardial ischemia) were offered surgery or exercise restriction (if deemed high risk for surgical intervention). Univariate and multivariable analyses were used to determine predictors of high risk. Results: Of 201 patients evaluated, 163 met inclusion criteria: 116 anomalous right CA (71%), 25 anomalous left CA (15%), 17 single CA (10%), and 5 anomalous circumflex CA (3%). Patients presented as an incidental finding (n=80, 49%), with exertional (n=31, 21%) and nonexertional (n=32, 20%) symptoms and following sudden cardiac arrest/shock (n=5, 3%). Eighty-two patients (50.3%) were considered high risk. Predictors of high risk were older age at diagnosis, black race, intramural course, and exertional syncope. Most patients (82%) are allowed unrestrictive sports activities. Forty-seven patients had surgery (11 anomalous left CA and 36 anomalous right CA), 3 (6.4%) remained restricted from sports activities. All patients are alive at a median follow-up of 1.6 (interquartile range, 0.7–2.8) years. Conclusions: In this prospective cohort of patients with anomalous aortic origin of a CA, most have remained free of exercise restrictions. Development of a multidisciplinary team has allowed a consistent approach and may have implications in risk stratification and long-term prognosis.

Contemporary outcomes of complete atrioventricular septal defect (CAVSD) repair, particularly for defects with associated abnormalities, is unclear. The goal of this study is to report an all-inclusive experience of CAVSD repair using a consistent surgical approach.All patients undergoing CAVSD repair between 1995 and 2016 at our institution were included. Patients were divided into 2 groups: isolated and complex (tetralogy of Fallot, aortic arch repair, double outlet right ventricle, and total anomalous pulmonary venous return). Survival and reoperation were analyzed using log-rank test and Gray's test, respectively. Multivariable analysis was performed with Cox regression.Overall, 406 patients underwent repair: 350 (86%) isolated and 56 (14%) complex CAVSD (tetralogy of Fallot: 34, double outlet right ventricle: 7, aortic arch repair: 12, total anomalous pulmonary venous return: 3). Median age at repair was 5 months (range, 10 days to 16 years); 339 (84%) had trisomy 21. A 2-patch repair was used in 395 (97%) and the zone of apposition was completely closed in 305 (75%). Perioperative mortality was 2% and 4% in the isolated and complex groups, respectively. Perioperative mortality since 2006 was 0.9%. Median follow-up was 7 years. Overall 10-year survival and incidence of any reoperation were 92% and 11%, respectively. Complex anatomy was not a risk factor for mortality (p = 0.35), but it was for reoperation (hazard ratio [HR]: 2.6; p0.01). Risk factors for left atrioventricular valve reoperation were a second bypass run (HR: 2.7) and preoperative moderate or worse regurgitation (HR: 2.3).Mortality after CAVSD repair is low, yet reoperation remains a significant problem. Repair of complex CAVSD can be performed with similar mortality rates.

Off-pump coronary artery bypass grafting (OPCAB) may have advantages in the elderly. Although proven safe, it remains unclear whether OPCAB provides a short-term survival benefit in octogenarians. We sought to compare outcomes using propensity matching between OPCAB and conventional surgery in a statewide database. We identified all octogenarians (≥ 80 years) who underwent isolated coronary artery bypass grafting (CAB) at 10 different centers in the state of Maryland from July 2011 to June 2016. We separated patients into two groups: OPCAB and on-pump coronary artery bypass (ONCAB). Patients were assigned propensity scores with a semi-parsimonious logistic regression model and matched 1:1 by the nearest-neighbor principle. A revascularization ratio was determined between the number of distal grafts sewn and number of diseased coronaries (≥ 50% stenosis). In total, 926 octogenarians underwent isolated CAB (8.2% of all CAB): 798 (86%) had ONCAB and 128 (14%) had OPCAB. Propensity matching yielded 128 well-matched pairs. Operative mortality was similar between groups (OPCAB 5.5% vs ONCAB 3.1%, p = 0.364). Rates of complications were similar between groups. OPCAB patients had a lower revascularization ratio (0.92 vs 1.15, p

BackgroundAnomalous aortic origin of a coronary artery is the second leading cause of sudden cardiac arrest/death in young athletes in the United States of America. Limited data are available regarding family history in this patient population.MethodsPatients were evaluated prospectively from 12/2012 to 02/2017 in the Coronary Anomalies Program at Texas Children’s Hospital. Relevant family history included the presence of CHD, sudden cardiac arrest/death, arrhythmia/pacemaker use, cardiomyopathy, and atherosclerotic coronary artery disease before the age of 50 years. The presence of one or more of these in 1st- or 2nd-degree relatives was considered significant.ResultsOf 168 unrelated probands (171 patients total) included, 36 (21%) had significant family history involving 19 (53%) 1st-degree and 17 (47%) 2nd-degree relatives. Positive family history led to cardiology referral in nine (5%) patients and the presence of abnormal tests/symptoms in the remaining patients. Coronary anomalies in probands with positive family history were anomalous right (27), anomalous left (five), single right coronary artery (two), myocardial bridge (one), and anomalous circumflex coronary artery (one). Conditions present in their family members included sudden cardiac arrest/death (15, 42%), atherosclerotic coronary artery disease (14, 39%), cardiomyopathy (12, 33%), CHD (11, 31%), coronary anomalies (3, 8%), myocardial bridge (1, 3%), long-QT syndrome (2, 6%), and Wolff–Parkinson–White (1, 3%).ConclusionIn patients with anomalous aortic origin of a coronary artery and/or myocardial bridges, there appears to be familial clustering of cardiac diseases in approximately 20% of patients, half of these with early occurrence of sudden cardiac arrest/death in the family.

Background The purpose of this study was to determine the incidence and risk factors for the development and rate of progression of scoliosis and moderate/severe scoliosis in patients undergoing cardiothoracic operations (CTOs). Methods Included were patients aged younger than 12 years who underwent CTOs in 1995 to 2006 with a preoperative chest roentgenogram (CRG) and a CRG at least 8 years after CTOs. Scoliosis and moderate/severe scoliosis were defined as a Cobb angle of 10 degrees or more and an angle of 25 degrees or more or the need for surgical intervention, respectively. Risk factors were analyzed using nonparametric and parametric survival analyses. For patients that developed scoliosis, progression rate was analyzed using linear regression models for repeated measures using CRG at 6-month intervals. Results The study included 871 patients (380 girls [44%]). Median CRG follow-up was 11 years (interquartile range, 9 to 13 years). Overall 10-year incidence of scoliosis and moderate/severe scoliosis was 12% and 3%, respectively. Independent predictors for scoliosis included female sex (hazard ratio [HR], 1.7; 95% confidence interval [CI], 1.2 to 2.5), syndrome (HR, 1.9; 95% CI, 1.3 to 2.8), and isolated developmental delay (HR, 2.4; 95% CI, 1.4 to 4.2). For development of moderate/severe scoliosis, independent risk factors included female sex (HR, 2.8; 95% CI, 1.4 to 5.8), syndrome (HR, 3; 95% CI, 1.5 to 6.1), isolated developmental delay (HR, 3.1; 95% CI, 1 to 9.2]), and prematurity for neonates/infants (HR, 2.3; 95% CI, 1 to 5.2). Rate of angle progression was 0.17 times the current angle per year. Age, syndrome, and developmental delay were risk factors for angle progression. Conclusions Patients that undergo pediatric CTO, regardless of the operative approach, are at increased risk for development of scoliosis and moderate/severe scoliosis. Long-term follow-up of these patients is warranted, in particular for girls and patients with genetic syndromes or developmental delay.

Background Patients with end-stage cardiomyopathy due to cardiac sarcoidosis (CS) may be referred for mechanical circulatory support (MCS) and heart transplantation (HT). We describe outcomes of patients with CS undergoing HT, focusing on the use of MCS as a bridge to transplant (BTT). Methods Using the United Network for Organ Sharing Scientific Registry of Transplant Recipients, we identified all adult waitlisted patients and isolated HT recipients from 2006 to 2015. These were divided into those with and without CS and further divided into those who did or did not receive MCS as BTT. Outcomes included 1- and 5-year post-transplantation freedom from mortality and 5-year freedom from primary graft failure. Results Over the study period, 31,528 patients were listed for HT, 148 (0.4%) of whom had CS. Among the CS patients, 34 (23%) received MCS as BTT. 18,348 patients (58%) eventually underwent HT, including 67 (0.4%) with CS, 20 (30%) of whom had received BTT MCS. Compared with non-CS diagnoses, CS patients had similar 1-year (91% vs 90%; log rank P = .88) and 5-year (83% vs 77%; log rank P = .46) freedom from mortality. Survival was also similar between CS BTT and non-CS BTT groups at 1 year (89% vs 89%; log-rank P = .92) and 5 years (72% vs 75%; log-rank P = .77). Conclusions Survivals after HT were similar between CS and non-CS patients out to 5 years, and were also similar between CS and non-CS BTT cohorts. Both HT and BTT MCS should be considered in patients with CS.

Background Debate persists over the association between blood transfusions, especially those considered discretionary, and outcomes after cardiac operations. Using data from the Maryland Cardiac Surgery Quality Initiative, we sought to determine whether outcomes differed among coronary artery bypass grafting (CABG) patients receiving 1 U of red blood cells (RBCs) vs none. Methods We used a statewide database to review patients who underwent isolated CABG from July 1, 2011, to June 30, 2016, across 10 Maryland cardiac surgery centers. We included patients who received 1 U or fewer of RBCs from the time of the operation through discharge. Propensity scoring, using 20 variables to control for treatment effect, was performed among patients who did and did not receive a transfusion. These two groups were matched 1:1 to assess for differences in our primary outcomes: operative death, prolonged postoperative length of stay (>14 days), and a composite postoperative respiratory complication of pneumonia or reintubation, or both. Results Of 10,877 patients who underwent CABG, 6,124 (56%) received no RBCs (group 1) during their operative hospitalization, and 981 (9.0%) received 1 U of RBCs (group 2), including 345 of 981 patients (35%) who received a transfusion intraoperatively. Propensity score matching generated 937 well-matched pairs. Compared with group 2, propensity-matched analysis revealed significantly greater 30-day survival in group 1 (99% vs 98%, p = 0.02) and reduced incidence of prolonged length of stay (3.7% vs 4.0%, p Conclusions Our collaborative statewide analysis demonstrated that even 1 unit of blood was associated with significantly worse survival and longer length of stay after CABG. Multiinstitutional quality initiatives may seek to address discretionary transfusions and possess the potential to improve patient outcomes.

Objectives The purpose of this systematic review is to provide a comprehensive overview on the clinical course, perinatal outcome and effectiveness of antenatal management options for pericardial teratoma. Methods A comprehensive search including Ovid MEDLINE, Ovid EMBASE, and Scopus was conducted from inception to September 2016. All studies that reported the antenatal course of pericardial teratoma in singleton or twin gestations were considered eligible Standardized forms were used for data abstraction by two independent reviewers. Results Out of 217 screened abstracts, 59 studies reporting 67 fetuses with pericardial teratoma were included. 23 singleton fetuses and 3 fetuses in twin gestations underwent prenatal treatment and 20(76.9%) of them were hydropic at the time of intervention. Of those, 15/20(75%) had a favorable outcome. In the non-intervention group (n=41), 26(63.4%) developed hydrops and out of those, 8(30.8%) had a favorable outcome. Conclusion Antenatal fluid drainage and other antenatal techniques have been utilized in the treatment of intrapericardial teratoma. While most fetuses tolerated pericardiocentesis, the neonatal benefit of this procedure is still uncertain and outcomes of other interventions had variable success. Antenatal intervention for pericardial teratoma may be an option in specialized units, but given the maternal and fetal risks, needs careful consideration.

Background Anomalous aortic origin of a coronary artery (AAOCA) is the second leading cause of sudden cardiac death in young athletes in the USA. Long-term outcome data for these patients are lacking to date. There is insufficient knowledge on the best approach to these patients and they are managed in a nonuniform manner. Methods An online survey of 15 questions regarding management of AAOCA was sent out to 198 cardiac healthcare providers. The goal was to define gaps in knowledge to justify a dedicated scientific forum for discussion of AAOCA. Descriptive statistics were performed. Results A total of 91 providers (46%) completed the survey including pediatric cardiology subspecialists (40%), general pediatric cardiologists (24%), cardiovascular (CV) surgeons (22%), adult cardiologists (10%), nurse practitioners (8%), cardiology fellows (3%) and CV anesthesiologist (1%). Forty-eight percent had been practicing for over 15 years and 28% were in their first 5 years of practice. Fifty-two percent of the providers cared for adults and 93% cared for children/adolescents. Eighty-eight percent were affiliated with an academic institution. All but one provider practiced in the USA, 62% practiced in Texas. Half of participants (50%) were very comfortable managing AAOCA patients and 36% were somewhat comfortable. Providers utilized various imaging tests to confirm the anatomy including computed tomography angiography 88%, cardiac magnetic resonance imaging 70%, cardiac catheterization 60%, echocardiogram 12%, IVUS 2% and myocardial perfusion scan 1%. The majority felt comfortable in counseling the families and felt that depending on the type of lesion these patients should get surgical referral (85%) vs clinical follow up (67%) with exercise restriction (65%). Conclusion There is heterogeneity in the way AAOCA patients are currently evaluated and managed. A knowledge gap exists even with participants from academic institutions. Long-term data with a defined approach to management of these patients may help to improve outcomes and prevent unnecessary exercise restriction or surgery.

The Society of Thoracic Surgeons Congenital Heart Surgery Database is the largest congenital and pediatric cardiac surgical clinical data registry in the world. It is the platform for all activities of The Society of Thoracic Surgeons related to the analysis of outcomes and the improvement of quality in this subspecialty. This report summarizes current aggregate national outcomes in congenital and pediatric cardiac surgery and reviews related activities in the areas of quality measurement, performance improvement, and transparency. The reported data about aggregate national outcomes are exemplified by an analysis of 10 benchmark operations performed from January 2012 to December 2015. This analysis documents the overall aggregate operative mortality (interquartile range among all participating programs) for the following procedural groups: off-bypass coarctation repair, 1.3% (0.0% to 1.8%); ventricular septal defect repair, 0.6% (0.0% to 0.9%); tetralogy of Fallot repair, 1.1% (0.0% to 1.4%); complete atrioventricular canal repair, 3.0% (0.0% to 4.7%); arterial switch operation, 2.7% (0.0% to 4.1%); arterial switch operation and ventricular septal defect repair, 5.3% (0.0% to 6.7%); Glenn/hemi-Fontan, 2.5% (0.0% to 4.5%); Fontan operation, 1.2% (0.0% to 1.2%); truncus arteriosus repair, 9.4% (0.0% to 16.7%); and Norwood procedure, 15.7% (8.9% to 25.0%).

The evaluation of the vast majority of children with anomalous aortic origin of a coronary artery (AAOCA) and/or myocardial bridges is performed with non-invasive testing. However, a subset of these patients may benefit from invasive testing for risk stratification. All patients included in the Coronary Anomalies Program (CAP) at Texas Children's Hospital who underwent cardiac catheterization were included. Techniques included selective coronary angiograms (SCA), intravascular ultrasound (IVUS), and fractional flow reserve (FFR) measurements with provocative testing using adenosine and/or dobutamine infusions. Out of the 131 patients followed by the CAP between 12/12-4/16, 8 (6%) patients underwent 9 cath investigations at median age 13.1 (2.6-18.7) years and median weight 49.5 (11.4-142.7) kg. Six patients presented with cardiac signs/symptoms. Four patients had myocardial bridges of the left anterior descending (LAD) coronary artery, 2 patients had isolated AAOCA, and 2 patients had an anomalous left coronary artery (LCA) with an intramyocardial course of the LAD. SCA was performed in all patients. FFR was positive in 4/6 patients: IVUS showed >70% intraluminal narrowing in 3/5 patients. One patient had hemodynamic instability that reversed with catheter removal from the coronary ostium. Based on the catheterization data obtained, findings were reassuring in three patients, surgery was performed in three patients, and two patients are being medically managed/restricted from competitive sports. In our small cohort of patients, we demonstrated that IVUS and FFR can safely be performed in children and may help to risk stratify some patients with AAOCA and myocardial bridges.

Pediatric cardiac tumors are rare, with a prevalence as low as 0.0017 (autopsy studies). The majority of these tumors are primary, and approximately 90% are benign. Myxomas comprise 2-4% of the benign cardiac tumors in the pediatric population, and are most frequently found in the left atrium.1,2 Here we present a case of a myxoma in the right ventricle (RV) causing syncope after blood donation.

Pulmonary artery slings and vascular rings are very rare congenital anomalies. It is even rarer to have both anomalies in the same setting. We present a case of a toddler who was diagnosed with a left pulmonary artery sling and a vascular ring as part of the screening process for the VACTERL association – co-occurrence of vertebral, anorectal, cardiac, tracheoesophageal, renal, and limb malformations. He underwent a successful surgical repair via median sternotomy and on cardiopulmonary bypass with an uneventful postoperative course.

INTRODUCTION The right ventricular infundibular sparing approach (RVIS) to the repair of tetralogy of Fallot (TOF) avoids a full-thickness ventricular incision, typically utilized in the transinfundibular (TI) method. METHODS We performed a retrospective, age-matched cohort study of patients who underwent RVIS at Texas Children's Hospital or TI at Children's Hospital Medical Center in Nebraska and subsequently underwent cardiac magnetic resonance imaging (CMR). We compared right ventricular end-diastolic and systolic volumes indexed to body surface area (RVEDVi and RVESVi) and right ventricular ejection fraction (RVEF) as primary endpoints. Secondary endpoints were indexed left ventricular diastolic and systolic volume (LVEDVi and LVESVi), left ventricular ejection fraction (LVEF), right ventricular (RV) sinus ejection fraction (EF) and RV outflow tract EF (RVOT EF). RESULTS Seventy-nine patients were included in the analysis; 40 underwent RVIS and 39 underwent TI repair. None of the patients in the TI repair group had an initial palliation with a systemic to pulmonary arterial shunt compared to seven (18%) in the RVIS group (P

Background Prolonged ventilation after cardiac surgery affects survival and increases morbidity. Previous studies have focused on predicting this complication preoperatively; however, indicators of poor outcome in those requiring prolonged ventilation remain ill-defined. We sought to identify predictors of operative mortality in cardiac surgery patients who experience prolonged mechanical ventilation. Methods 1698 patients who underwent cardiac surgery (CAB, aortic valve replacement ± CAB, or mitral valve repair/replacement ± CAB) required prolonged postoperative mechanical ventilation (>24 hours) between 2012 to 2016 in a statewide consortium. Perioperative factors were evaluated to identify the association with operative mortality. Covariates were selected through bootstrap aggregation to fit multivariable logistic regression models. The relative strength of association was determined by the Wald chi-square statistic. Results Median patient age was 68 years [IQR 61 to 76], 38% (644/1,698) were female, median duration of mechanical ventilation was 65 hours [IQR 38 to 143], median STS predicted risk of mortality was 3.1% [IQR 1.4 to 6.9%], and 15.7% (266/1698) suffered operative mortality. Among preoperative and operative characteristics, patient age and intraoperative initiation of extracorporeal membrane oxygenation (ECMO) were the strongest correlates of operative mortality on the multivariate analysis. Among postoperative factors, cardiac arrest and renal failure requiring dialysis were the strongest predictors of risk-adjusted operative mortality. Type of operation or surgical center had no association to mortality after risk adjustment. Conclusion Prolonged ventilation following cardiac surgery is associated with a five-fold increase in operative mortality. In these patients, operative mortality is associated with older age, intraoperative initiation of ECMO, postoperative cardiac arrest, and renal failure requiring dialysis.

Background Heart transplantation is the mainstay of treatment for patients in end-stage heart failure. This study sought to contrast survival after transplantation with that of the general population to quantify standardized mortality rates using a nested case-control study design. Methods Control subjects were noninstitutionalized inhabitants of the United States identified through the National Longitudinal Mortality study. Case subjects were adults who underwent heart transplantation between 1990 and 2007 and identified through the Organ Procurement and Transplantation Network. Propensity-matching (5:1, nearest neighbor, caliper = 0.1) was utilized to identify suitable control subjects based on age, sex, race, and state of permanent residency. The primary study endpoint was 10-year survival. Results In all, 31,883 heart transplant recipients were matched to 159,415 noninstitutionalized residents of the United States. The 10-year survival of heart transplant recipients was 53%. The population expected mortality rate was 15.9 deaths per 100 person-years with an observed rate of 45.1 deaths per 100 person-years (standardized mortality rate [SMR] 2.84; 95% confidence interval, 2.82 to 2.87). The broadest gaps between observed and expected survival were evident in female (SMR 3.63), black (SMR 3.67), and Hispanic (SMR 4.12) recipients. Standardized mortality ratios declined over time (1990 to 1995, 3.09; 1996 to 2000, 2.90; 2001 to 2007, 2.58). The long-term standardized survival of older recipients was closest to that expected for their age. Conclusions Heart transplant recipients have considerable long-term survival and have a threefold higher standardized long-term mortality rate than that of the noninstitutionalized population. Long-term mortality rates have consistently declined over time and will likely continue to decrease.

Introduction Frailty is an important component of risk prognostication in transcatheter aortic valve replacement (TAVR). Objective markers of frailty, including sarcopenia, the modified Frailty Index (mFI), and albumin levels, have emerged, but little is known how such markers compare to each other in predicting outcomes after TAVR. We sought to define and compare these markers in predicting long-term outcomes after TAVR. Methods Patients who underwent TAVR at our institution from 2011 to 2016 were included. Indexed cross-sectional areas of the lumbosacral muscles on preoperative computed tomography scans were used to assess sarcopenia. Optimal cutoffs for sarcopenia were defined using a statistically validated method. mFI was calculated using an 11-point scale of clinical characteristics. The primary outcome was 2-year all-cause mortality. Adjusted survival analysis was used to analyze outcomes. Results A total of 381 patients were included in this study. Sarcopenia of the psoas muscles was associated with an increased risk of mortality on univariate (HR: 2.3, P = 0.01) and multivariate (HR: 2.5, P = 0.01) analysis. Sarcopenia of the paravertebral muscles was associated with increased risk of mortality only on univariate analysis (HR: 2.1, P = 0.03). Increased preoperative albumin levels were associated with decreased risk of mortality on univariate (HR: 0.3, P < 0.01) and multivariate analysis (HR: 0.3, P < 0.01). The (mFI) was not associated with mortality on univariate or multivariate analysis. Discussion Novel cutoffs for sarcopenia of the psoas muscles were determined and associated with decreased survival after TAVR. Sarcopenia and albumin levels may be better tools for risk prediction than mFI in TAVR.

Mortality after surgery for congenital heart disease (CHD) in Mexico is significantly higher than in high-income countries due to structural, medical, and financial factors. In Mexico, public hospitals have a large volume of patients but inadequate quality control systems, whereas private hospitals, although having higher quality control systems, have an insufficient number of patients to build programs of excellence. We describe the creation of a novel hybrid private–public program in Mexico that leverages the advantages of both sectors while establishing an integrated multidisciplinary unit that has allowed us to improve the quality of care for patients with CHD.

Background The utilization of multiorgan transplantation in cardiac transplantation has steadily increased over the past several years. We sought to characterize the trends and outcomes in simultaneous heart and other organ transplantation compared with heart transplantation alone. Methods The United Network for Organ Sharing database was queried for all adult patients (age ≥ 18 y) who underwent isolated heart transplantation or simultaneous heart-lung or heart-kidney transplantation from 1987-2016. Patients were stratified into 3 equal time intervals. Demographics and postoperative outcomes were compared. Results A total of 58,060 patients were identified with a distribution based on era. Dual organ recipients had more factors associated with increased operative risk including higher rates of diabetes, pulmonary hypertension, intensive care unit admissions, and dialysis prior to transplantation. Heart-lung and heart-kidney recipients had decreased 1-year survival compared with isolated heart recipients from 2007-2016. However, heart-kidney recipients had significantly increased 5-year post-transplantation survival compared with isolated heart recipients with impaired renal function. For isolated heart transplants and heart-lung transplants, 5-year survival rates improved over time, whereas 5-year survival for heart-kidney recipients did not improve with time. Conclusions We found a significantly increased 5-year survival rate for heart-kidney transplant recipients compared with isolated heart transplant recipients with renal impairment. Lack of improvement in 5-year postoperative outcomes for heart-kidney recipients in the setting of higher-risk pretransplant clinical characteristics suggests decreased selectivity regarding heart-kidney recipients. Continued scrutiny and evaluation of postoperative outcomes are required to ensure just and appropriate utilization of organs.

In recent years there has been a growing awareness of and focus on connective tissue diseases (CTDs) and associated cardiovascular pathology, particularly in children. These diseases are hereditary disorders of the connective tissues of the body. Many CTDs impose risk of structural heart defects, including patent ductus arteriosus, bicuspid aortic valve, coarctation of the aorta, and atrioventricular valve disease. Children with CTDs are prone to aortic dissection, aneurysm, and aortic rupture, and in fact, aortic dissection is the primary cause of morbidity and mortality in the most common CTDs. Advances in genetic analysis have had a significant impact on the identification and management of children with CTDs, providing a better understanding of their cause and phenotypes, improving management strategies, and offering insights into long-term prognosis. In this chapter we provide a description of the most common CTDs that affect the pediatric population and their associated cardiovascular pathology.

Background A descending thoracic aorta that traverses the midline is an uncommon cause of airway compression affecting the distal trachea and proximal main bronchi. Posterior aortopexy has had inconsistent results. Methods A retrospective review determined that, since 2004, 5 children have undergone descending aortic translocation at Texas Children's Hospital. The average age at the time of surgical treatment was 4.2 years, and all patients presented with recurring respiratory illness requiring hospitalization. All patients had preoperative imaging (4 patients with computed tomography scans and 1 with magnetic resonance imaging) confirming a compromised airway caused by a midline aorta, and 4 of the 5 patients had perioperative bronchoscopy. Three patients had a right-dominant double aortic arch. Descending aortic translocation was performed through a midline sternotomy with cardiopulmonary bypass and deep hypothermia. The proximal descending aorta was transected distal to the subclavian artery, brought up through the transverse sinus caudad to the tracheal carina and pulmonary artery, and anastomosed in an end-to-side fashion to the ascending aorta. Results Mean cardiopulmonary bypass was 144.8 ± 32.6 minutes, with an aortic cross-clamp time of 59 ± 40.9. Absence of perfusion to the descending thoracic aorta averaged 44.4 ± 13.7 minutes. Concomitant procedures were performed in 4 of the 5 patients. At a median follow-up of 26 months (range, 3 to 101 months), all patients had resolution of symptoms. Conclusions A midline descending aorta can cause compression of the tracheal carina and proximal bronchi, with debilitating symptoms. Translocation of the descending aorta is a reliable procedure that relieves the compression and results in long-term resolution of symptoms.

Background We describe survival in patients with Down syndrome (DS) with single-ventricle anatomy and palliation and characterize risk factors associated with mortality. Methods All patients with DS and single-ventricle anatomy documented in the electronic medical record at our institution from January 1, 1992, to May 1, 2014, were compared with patients with unbalanced atrioventricular septal defects and single-ventricle anatomy, without DS or heterotaxy, during the same period. Survival analysis was performed to evaluate factors associated with mortality, including the presence of DS. Results There were 28 patients with DS and 30 patients without DS. One-year survival with DS was 75% (95% CI: 55% to 87%); 5-year survival was 61% (95% CI: 40% to 76%). All DS deaths except one occurred before 2 years of age. One-year non-DS survival was 93% (95% CI: 76% to 98%); 5-year survival was 85% (95% CI: 64% to 94%). Factors associated with death by univariable analysis included DS ( p = 0.04), pulmonary vascular resistance (PVR) of at least 3 Wood units × meter 2 (WUm 2 ) in the first year of life ( p = 0.03), and moderate-to-severe atrioventricular valve regurgitation ( p = 0.1). In combined analysis, when accounting for PVR of at least 3 WUm 2 (hazard ratio [HR] 9.8, 95% CI: 1.1 to 83.5, p = 0.04), DS was not associated with increased mortality (HR 1.5, 95% CI: 0.3 to 7.8, p = 0.66). No patient with DS with PVR less than 3 WUm 2 died. Conclusions Children with DS and single-ventricle anatomy have excellent survival when PVR is less than 3 WUm 2 in the first year of life, with minimal mortality beyond 2 years of age. When accounting for PVR, DS alone is not associated with increased mortality in patients with single-ventricle anatomy.

Heart transplant recipients of traumatically brain-injured (TBI) donors have been reported to have inferior survival and increased rates of cardiac allograft vasculopathy in single-center studies. This study sought to examine the impact of TBI donors on outcomes after heart transplantation across all transplantation centers.We identified all adult heart transplants performed during 2007-2016 in the OPTN database. Recipients were dichotomized based on donor cause of death (TBI versus non-TBI), propensity-scored across 22 variables with known associations with mortality, and matched 1:1 without replacement. The primary endpoint was all-cause mortality. Secondary endpoints were conditional survival and rates of cardiac allograft vasculopathy.In total, 20,244 patients underwent heart transplantation. TBI was the primary cause of death in 53.4% of donors (10,816/20,244), and among TBI donors, blunt injury (59.6%; 6443/10,816) and gunshot wound (35%; 3781/10,816) were the most common mechanisms of injury. Propensity matching generated 6919 pairs (all absolute mean differences 0.07). Risk-adjusted survival was similar between recipients of TBI donors and non-TBI donors at 5 y (78.1% versus 77.5%, log-rank P = 0.34). Risk-adjusted survival conditional on 1-y survival was also similar at 5 y (86.2% versus 86.1%, log-rank P = 0.74). The 5-y risk-adjusted rates of cardiac allograft vasculopathy did not differ either (30.6% versus 30.4%; log-rank P = 0.78).In the largest analysis of TBI donors in heart transplantation, we found similar survival and rates of cardiac allograft vasculopathy to those who received hearts from non-TBI donors out to 5 y. These findings should allay concerns over continued transplantation with this unique donor population.

BackgroundA wide variety of surgical strategies are used in tetralogy of Fallot repair. We sought to describe the international contemporary practice patterns for surgical management of tetralogy of Fallot.MethodsSurgeons from 18 international paediatric cardiac surgery centres (representing over 1800 tetralogy of Fallot cases/year) completed a Research Electronic Data Capture-based survey. Participating countries include: China (4), India (2), Nepal (1), Korea (1), Indonesia (1), Saudi Arabia (3), Japan (1), Turkey (1), Australia (1), United States of America (2), and Canada (1). Summary measures were reported as means and counts (percentages). Responses were weighted based on case volume/centre.ResultsPrimary repair is the prevalent strategy (83%) with variation in age at elective repair (range). Approximately 47% of sites use patient age as a factor in determining the strategy, with age 90% of all trans-annular repairs.ConclusionsIn this cohort representing 11 countries, there is variation in tetralogy of Fallot surgical management with no consensus on standard of practice. A large international prospective cohort study would allow analysis of impact of underlying anatomy and repair strategy on early and late outcomes.

Background Left ventricular assist devices (LVADs) are an effective therapy in bridging patients with end-stage heart failure to heart transplantation. The aim of this study was to identify the role of race in survival of patients bridged to heart transplantation with a LVAD. Methods The United Network of Organ Sharing database was queried for all adult heart transplant recipients (age 18 years or older) who were bridged to transplantation with a LVAD from 2005 to 2018. Patients were stratified based on their race, with whites as the reference group. Demographic characteristics, 5-year survival, and graft failure after transplantation were assessed with χ2 test, analysis of variance, Kaplan-Meier survival analyses, log-rank tests, and Cox proportional hazards modeling or logistic regression modeling as appropriate. Results Patients (N = 6476) successfully bridged with a LVAD to heart transplantation were identified. There were 4263 whites, 1536 African Americans, 508 Hispanics, and 169 Asians. Compared with whites, African Americans had higher body mass indexes, were more likely to be women, pay with private insurance, and be working for income at the time of transplantation. African Americans were found to have increased odds of graft failure (odds ratio 1.27, P = .048) compared with whites. In addition, African Americans were found to have increased risk of mortality at 5 years (hazard ratio 1.26, P = .003). Conclusions The African American race is associated with increased rates of graft failure after transplantation and decreased 5-year survival compared with the white race. Given these findings, directed clinical attention may be warranted in African American patients bridged to heart transplantation with a LVAD.

There has not been a multicenter investigation to elucidate the prevalence and impact of congenital diaphragmatic hernia (CDH) on children undergoing cardiac surgery. We investigated the prevalence of CDH across congenital cardiac diagnostic and procedural groups and its impact on outcomes. The STS Congenital Heart Surgery Database was queried to identify children undergoing cardiac surgery who also had CDH. Baseline perioperative characteristics and postoperative outcomes were compared between groups. Subgroup analyses were performed based on case complexity as determined by STAT Categories. Overall 426 of 157,419 (0.27%) pediatric patients undergoing an index cardiac operation had a diagnosis of CDH including 89 neonates (0.25% of all neonatal index operations), 217 infants (0.39%), and 120 children (0.18%). The frequency of concomitant CDH varied across diagnostic groups and, in neonates and infants, was highest for tetralogy of Fallot (0.45%, n = 41), coarctation (0.39%, n = 27) and complete atrioventricular septal defects (0.31%, n = 19). For neonates and infants outcomes were generally worse in CDH vs no-CDH patients across the various procedural strata. For example, STAT ≥3 operative mortality was 34.4% vs 10.3% ( P0.001) for neonates, and 24.2% vs 5.3% ( P0.001) for infants with vs without CDH, respectively. In older children, outcomes disparities were less clear cut with no significant differences in mortality, and variable differences for complication and length of stay outcomes. CDH occurs in a small percentage of patients born with congenital heart disease. Concomitant diagnosis of CDH portends increased morbidity and mortality in infants and neonates undergoing cardiac surgery.

BACKGROUND Loeys-Dietz syndrome (LDS) is an autosomal dominant connective tissue disorder predisposing patients to aneurysm formation and arterial dissection. Aortic root replacement is often performed prophylactically and valve-sparing root replacement (VSRR) has become the procedure of choice. However, in these patients with connective tissue disorders, postoperative pseudoaneurysms may develop. METHODS All children with LDS undergoing VSRR at a single institution were retrospectively reviewed to identify patients who developed postoperative pseudoaneurysms. RESULTS Thirty-one children with LDS underwent VSRR; four of these developed pseudoaneurysms of their synthetic aortic root grafts requiring reoperation. These four children were reviewed to investigate the cause of pseudoaneurysm formation after VSRR. Each had severe subtypes of LDS. Each underwent reoperation for repair of their pseudoaneurysms and were found to have suffered pseudoaneurysms as a result of tearing of sutures from their reimplantation VSRR. CONCLUSIONS Pseudoaneurysms following aortic root replacement with VSRR can occur in children with severe subtypes of LDS. Long-term surveillance is required to detect these potentially life-threatening lesions.

Objective Valve-sparing root replacement is an attractive alternative to composite mechanical or biologic prostheses for aortic root aneurysms in children. Data on outcomes in pediatric patients are limited. We present our institutional experience with 100 consecutive pediatric valve-sparing aortic root procedures. Methods All children who underwent valve-sparing root replacement at our institution from May 1997 to August 2017 were identified, and echocardiographic and clinical data were reviewed. The primary end point was mortality, and secondary end points included complications, further interventions, and subsequent valvular dysfunction. Results Median age at operation was 13.6 years (interquartile range, 9.42-15.9); 51 patients (51%) had Marfan syndrome, and 39 patients (39%) had Loeys–Dietz syndrome. Mean preoperative maximum sinus diameter was 4.4 ± 0.71 cm (z score 7.3 [5.7-9.3]). Most patients (n = 80, 80%) underwent reimplantation procedures with a Valsalva graft. Four patients (4%) underwent David I reimplantation with a straight-tube graft, 13 patients (13%) underwent a Yacoub remodeling procedure, and 3 patients (3%) underwent a Florida sleeve procedure. Perioperative valve-sparing root replacement mortality was 2% (n = 2). Six patients required late reintervention for development of pseudoaneurysms. Eight patients underwent additional aortic surgery. Average time to reoperation was 7.23 ± 4.56 years. Of the 84 patients undergoing a reimplantation procedure, 5 (5.9%) underwent late valve replacement versus 5 (33.3%) of the 15 patients who received a remodeling procedure (P = .001). Conclusions Valve-sparing root replacement is a safe and effective option for children with aortic root aneurysms in children. The reimplantation procedure is preferred. Late aortic insufficiency and pseudoaneurysm formation remain late concerns.

Aberrant left-right patterning in the developing human embryo can lead to a broad spectrum of congenital malformations. The causes of most laterality defects are not known, with variants in established genes accounting for

Objective The authors sought to assess the relationship between low oxygen delivery (DO2) during cardiopulmonary bypass (CPB) and a neuron-specific biomarker of neurologic injury, ubiquitin C-terminal hydrolase L1 (UCH-L1). Design Retrospective analysis of patient charts and prospectively collected blood samples. Setting University-affiliated tertiary care hospital. Participants Adult patients undergoing cardiac surgery on CPB. Interventions Serum UCH-L1 levels were drawn at baseline and 6 and 24 hours after CPB cessation. DO2 was computed from perfusion records, with area-under-the-curve (AUC) computations performed to account for distance of DO2 excursions below predefined DO2 thresholds and the amount of time spent below them. Strokes were defined radiographically using computed tomography and magnetic resonance imaging. Measurements and Main Results Forty-three adults were included (median age 65 y, interquartile range 59-72). Three patients experienced strokes (imaged at 2, 7, and 8 d postoperatively). Most patients underwent isolated coronary artery bypass grafting (41%, 18 patients) or isolated aortic valve replacement (30%, 13). Median UCH-L1 levels differed from baseline to 6 and 24 hours after CPB (40, 232, and 166 pg/mL, respectively; p Conclusions This is the first study to demonstrate an independent association between DO2 during CPB and elevations of a brain injury biomarker; additional study is needed to clarify the clinical significance of these results.

Graft rejection is the most common factor that limits graft survival after transplantation. During infancy, the humoral immune system is partially suppressed and humoral rejection of a cardiac allograft has not been reported in the absence of risk factors such as prior transplantation, blood transfusions, ventricular assist devices, and elevation of panel reactive antibodies. We present a case of an infant with dilated cardiomyopathy who developed multiple episodes of acute humoral rejection after heart transplantation in the absence of risk factors.

Background Bilateral internal mammary artery (BIMA) grafting in diabetic patients undergoing coronary artery bypass grafting remains controversial. Our study compared morbidity and mortality between (1) diabetic and nondiabetic BIMA patients and (2) diabetic BIMA versus diabetic patients who underwent left internal mammary artery (LIMA) grafting only. Methods Patients who underwent isolated coronary artery bypass grafting from July 2011 to June 2016 at any of the 10 Maryland Cardiac Surgery Quality Initiative centers were propensity scored across 16 variables. Diabetic BIMA patients were matched 1:1 by nearest neighbor matching to nondiabetic BIMA patients and were separately matched 1:1 to diabetic LIMA patients. We calculated observed-to-expected (O/E) ratios for composite morbidity/mortality, operative mortality, unplanned reoperation, stroke, renal failure, prolonged ventilation, and deep sternal wound infection and compared ratios among matched populations. Results During the study period, 812 coronary artery bypass grafting patients received BIMA grafts, including 302 patients (37%) with diabetes. We matched 259 diabetic and nondiabetic BIMA patients. O/E ratios were higher in matched diabetic (versus nondiabetic) BIMA patients when comparing composite morbidity/mortality, reoperation, stroke, renal failure, and prolonged ventilation (all O/E ratios >1.0); however, the O/E ratio for operative mortality was higher in nondiabetic BIMA patients. We additionally matched 292 diabetic BIMA to diabetic LIMA patients. Diabetic BIMA patients had a higher O/E ratio for composite morbidity/mortality, operative mortality, stroke, renal failure, and prolonged ventilation. Conclusions In this statewide analysis, diabetic patients who received BIMA grafts (compared with diabetic patients with LIMA grafts or nondiabetic patients with BIMA grafts) had higher O/E ratios for composite morbidity/mortality as a result of higher O/E ratios for major complications.