Mortality and Heart Transplantation After Hybrid Palliation of Hypoplastic Left Heart Syndrome: A Systematic Review and Meta-Analysis.

BACKGROUND: Newborns with hypoplastic left heart syndrome (HLHS) who are considered at increased risk for death following Norwood/Sano surgery often undergo hybrid palliation (HP) as initial surgery. We aimed to compile the HP experience in HLHS and its variants and assess the rates of, and risk factors for, death and heart transplantation. METHODS: CINAHL, CINAHL PLUS, PubMed/MEDLINE, and SCOPUS were systematically searched for HP outcome studies of death or heart transplantation in HLHS between 1998 and 2022. Pooled incidence was estimated, and potential risk factors were identified using random-effects meta-analysis and reconstructed time-to-event data from Kaplan-Meier curves. RESULTS: Thirty-three publications were included in our review. Overall, of 1,162 patients 417 died and 57 underwent heart transplantation, resulting in a combined outcome of 40.7%, (474/1,162). There was a trend toward decreasing mortality risk across the stages of palliation. Pooled mortality between HP and comprehensive stage 2 palliation was 25%, after stage 2 up to Fontan palliation was 16%, and 6% post-Fontan. The incidence of death or heart transplantation was higher in high-risk patients-43% died and 10% received heart transplantation. CONCLUSION: Our systematic review and meta-analysis found high rates of death or heart transplantation in HP of HLHS patients between HP and Fontan surgeries. All patients should be closely followed during the initial interstage period, which is associated with the highest hazard. Prospective studies on appropriate patient selection, indications, and / or alternatives, as well as refining HP strategies for managing newborns with HLHS are needed to improve outcomes.

Review on clinician bias and its impact on racial and socioeconomic disparities in pediatric heart transplantation.

This expert review seeks to highlight implicit bias in health care, transplant medicine, and pediatric heart transplantation to focus attention on the role these biases may play in the racial/ethnic and socioeconomic disparities noted in pediatric heart transplantation. This review breaks down the transplant decision making process to highlight points at which implicit bias may affect outcomes and discuss how the science of human decision making may help understand these complex processes.

Cardiac allograft vasculopathy in pediatric heart transplant recipients does early-onset portend a worse prognosis?

PURPOSE: We sought to evaluate the association between timing of cardiac allograft vasculopathy (CAV) occurrence post-heart transplant (HT) with graft survival and progression of CAV severity in pediatric HT recipients. METHODS: Data from the Pediatric Heart Transplant Society for pediatric (<18 years old) HT recipients between 1993-2019 with available angiographic data were obtained (N = 5,075). The timing of CAV diagnosis (<3; 3-<5; 5-<10; and ≥10 years post-HT) and severity of disease at each assessment (CAV 1-3) was determined. Associations between CAV timing, graft survival, and CAV progression were evaluated using Kaplan-Meier survival curves, multivariable COX proportional hazard regression analyses, and competing risk analyses. RESULTS: Over a median follow-up period of 4.1 (IQR 1.3-8.3) years, CAV was identified in 17% (885/5,075), 28% (252/885) of which were early-onset CAV. Compared with late onset CAV ≥10 years post-HT, patients with early CAV were older at the time of transplant (8.3 ± 6.2 vs. 3.8 ± 4.8 years, p < .0001). While the five-year graft-survival in the ≥10-year group (79.2%, p = 0.03) was significantly higher than the <3, 3-<5, and 5-<10 years post-HT groups (65.0%-67%) (p = 0.03), overall post-CAV graft survival was not significantly different across the CAV time-points. CAV disease progression did not vary with CAV timing post-HT, with an overall five-year freedom from CAV ≥2 of 75.4% (73.1%-77.6%). CONCLUSION: Later onset CAV (≥10-years post-HT) was associated with improved five-year graft survival compared with CAV onset at earlier time-points, but similar and poor long-term outcomes. CAV timing post-HT was not associated with progression of CAV disease severity.

Contemporary Provider Management Practices and Attitudes Toward Referral for Advanced Heart Failure Therapies in Fontan Patients Across North America.

BACKGROUND: To date, no reports have described clinicians' management practices for patients with Fontan circulatory failure or their understanding of risk factors for mortality and transplant outcomes in these patients. METHODS AND RESULTS: A cross-sectional survey of caregivers across North America was conducted from February to September 2020. Responses were compared by primary specialty (heart failure/transplant vs non-heart failure/transplant), years of experience (early, mid, and late career), and Fontan center volume (low, medium, and high). Of 400 responses, the majority were from general cardiologists (111, 28%) followed by heart failure/transplant specialists (93, 23%). Although most agreed that patients with Fontan physiology will have signs/symptoms of heart failure (369 [93%]) and eventuate in heart transplant (286 [72%]), many disagreed (180 [45%]) that routine evaluation by a transplant cardiologist is needed without symptoms. Transplant providers were more likely than non-transplant providers to suggest referral for manifestations of Fontan circulatory failure such as protein-losing enteropathy, plastic bronchitis, liver fibrosis/cirrhosis, and worsening valve regurgitation. Non-transplant providers were more likely to suggest that protein-losing enteropathy, plastic bronchitis, and Fontan-associated liver disease lead to inferior outcomes after transplantation. Early career and transplant providers more favorably viewed ventricular assist device use for Fontan patients failing traditional heart failure therapy (P < .05 for all). CONCLUSIONS: There is significant variation in the management of Fontan patients, including heterogeneous timing of referral of such patients to the heart failure/transplant team, which may have implications for future outcomes.

Berlin Heart EXCOR and ACTION post-approval surveillance study report.

BACKGROUND: The Berlin Heart EXCOR Pediatric (EXCOR) ventricular assist device (VAD) was introduced in North America nearly 2 decades ago. The EXCOR was approved under Humanitarian Device Exemption status in 2011 and received post-market approval (PMA) in 2017 from Food and Drug Administration. Since the initial approval, the field of pediatric mechanical circulatory support has changed, specifically with regard to available devices, anticoagulation strategies, and the types of patients supported. This report summarizes the outcomes of patients supported with EXCOR from the Advanced Cardiac Therapies Improving Outcomes Network (ACTION) registry. These data were part of the PMA surveillance study (PSS) required by the Food and Drug Administration. METHODS: ACTION is a learning collaborative of over 40 pediatric heart failure programs worldwide, which collects data for all VAD implantations as one of its initiatives. All patients in North America with EXCOR implants reported to ACTION from 2018 to 2020 (n = 72) who had met an outcome were included in the EXCOR PSS group. This was compared with a historical, previously reported Berlin Heart EXCOR study group (Berlin Heart study [BHS] group, n = 320, 2007‒2014). RESULTS: Patients in the PSS group were younger, were smaller in weight/body surface area, were more likely to have congenital heart disease, and were less likely to receive a bi-VAD than those in the BHS group. Patients in the PSS group were less likely to be in Interagency Registry for Mechanically Assisted Circulatory Support Profile 1 and were supported for a longer duration. The primary anticoagulation therapy for 92% of patients in the PSS group was bivalirudin. Success, defined as being transplanted, being weaned for recovery, or being alive on a device at 180 days after implantation, was 86% in the PSS group compared with 76% in the BHS group. Incidence of stroke was reduced by 44% and the frequency of pump exchange by 40% in the PSS group compared with those in the BHS group. Similarly, all other adverse events, including major bleeding, were reduced in the PSS group. CONCLUSIONS: The PSS data, collected through ACTION, highlight the improvement in outcomes for patients supported with EXCOR compared with the outcomes in a historical cohort. These findings may be the result of changes in patient care practices over time and collaborative learning.

Fontan-associated plastic bronchitis waitlist and heart transplant outcomes: A PHTS analysis.

Plastic bronchitis is a rare post-Fontan complication with limited treatment options. Heart transplantation has evolved as a potential curative option, but outcomes have not been well-defined. This study aims to assess contemporary waitlist and post-transplant outcomes in patients with plastic bronchitis. All Fontan patients were identified in the PHTS database (2010 - 2018). Waitlist and post-transplant outcomes were compared between Fontan patients with and without plastic bronchitis. Competing outcomes and Kaplan-Meier analyses were used to assess the impact of plastic bronchitis on waitlist and post-transplant survival. A secondary analysis excluded those with PLE from the comparison cohort. Of 645 Fontan patients listed for heart transplant, 69 (11%) had plastic bronchitis. At listing, patients with plastic bronchitis were younger (8.9 vs 11.1 years, P = .02), but had few other differences in baseline characteristics. A fewer Fontan patients with plastic bronchitis were listed in the more recent era (46 [15.4%] in 2010-2014 vs 23 [6.6%] in 2015-2018, P < .01). Overall, there was no difference in waitlist (P = .30) or post-transplant (P = .66) survival for Fontan patients with and without plastic bronchitis. The results were similar after excluding patients with PLE. Contrary to prior reports, this relatively large series showed that plastic bronchitis did not have a negative impact on survival to or after heart transplantation in Fontan patients. Our study also found a 50% reduction in listing in the current era, which may indicate evolution in management of Fontan patients.

Variability in clinical decision-making for ventricular assist device implantation in pediatrics.

BACKGROUND: Minimal data exist on clinical decision-making in VAD implantation in pediatrics. This study aims to identify areas of consensus/variability among pediatric VAD physicians in determining eligibility and factors that guide decision-making. METHODS: An 88-item survey with clinical vignettes was sent to 132 pediatric HT cardiologists and surgeons at 37 centers. Summary statistics are presented for the variables assessed. RESULTS: Total respondents were 65 (72% cardiologists, 28% surgeons) whose centers implanted 1-5 (34%), 6-10 (40%), or >10 (26%) VADs in the past year. Consensus varied by patients' age, diagnosis, and Pedimacs profile. Highest agreement to offer VAD (97%) was a mechanically ventilated teenager with dilated cardiomyopathy. Patients stable on inotropes were less likely offered VAD (11%-25%). SV infant with Pedimacs profile 2 had the most varied responses: 37% offered VAD; estimated survival ranged from 15% to 90%. Variables considered for VAD eligibility included mild developmental delays (100% offered VAD), moderate-severe behavioral concerns (46%), cancer in remission >2 years (100%), active malignancy with good prognosis (68%) or uncertain prognosis (36%), and BMI >35 (74%) or <15 (69%). Most respondents (91%) would consider destination therapy VADs in pediatrics, though not currently feasible at 1/3 of centers. Factors with greatest influence on decision-making included HT candidacy, families' goals of care, and risks of complications. CONCLUSIONS: Significant variation exists among pediatric VAD physicians when determining VAD eligibility and estimating survival, which can lead to differences in access to emerging technologies across institutions. Further work is needed to understand and mitigate these differences.

Should Decision Making Be Shared in High-Risk Pediatric Heart Donation?

This article considers complexities of shared decision making in pediatric heart transplantation and suggests that decisions about pediatric heart transplantation should be shared between a clinical team and parents. This article also considers goals of shared decision making involving Public Health Service increased-risk donors and recommends policy changes to strengthen decision sharing.

Review of interactions between high-risk pediatric heart transplant recipients and marginal donors including utilization of risk score models.

BACKGROUND: Donor organ acceptance practices vary among pediatric heart transplant professionals. We sought to understand what is known about the interactions between the "high-risk" recipient and the "marginal donor," and how donor risk scores can impact this discussion. METHODS: A systematic review of published literature on pediatric HTx was undertaken with the assistance of a medical librarian. Two authors independently assessed search results, and papers were reviewed for inclusion. RESULTS: We found that there are a large number of individual factors, and clusters of factors, that have been used to label individual recipients "high-risk" and individual donors "marginal." The terms "high-risk recipient" and "marginal donor" have been used broadly in the literature making it virtually impossible to make comparisons between publications. In general, the data support that patients who could be easily agreed to be "sicker recipients" are at more risk compared to those who are clearly "healthier," albeit still "sick enough" to need transplantation. Given this variability in the literature, we were unable to define how being a "high-risk" recipient interplays with accepting a "marginal donor." Existing risk scores are described, but none were felt to adequately predict outcomes from factors available at the time of offer acceptance. CONCLUSIONS: We could not determine what makes a donor "marginal," a recipient "high-risk," or how these factors interplay within the specific recipient-donor pair to determine outcomes. Until there are better risk scores predicting outcomes at the time of organ acceptance, programs should continue to evaluate each organ and recipient individually.

ISHLT consensus statement on donor organ acceptability and management in pediatric heart transplantation.

The number of potential pediatric heart transplant recipients continues to exceed the number of donors, and consequently the waitlist mortality remains significant. Despite this, around 40% of all donated organs are not used and are discarded. This document (62 authors from 53 institutions in 17 countries) evaluates factors responsible for discarding donor hearts and makes recommendations regarding donor heart acceptance. The aim of this statement is to ensure that no usable donor heart is discarded, waitlist mortality is reduced, and post-transplant survival is not adversely impacted.

Pediatric Acute Myocarditis: Predicting Hemodynamic Compromise at Presentation to Health Care.

BACKGROUND: The clinical spectrum of pediatric acute myocarditis ranges from minimal symptoms with intact hemodynamics to rapid cardiovascular collapse and death. We sought to identify factors on initial presentation associated with subsequent hemodynamic compromise. METHODS: We performed a retrospective cohort study of patients with acute myocarditis at a freestanding pediatric hospital from 2007 to 2016. We defined 2 cohorts: high-acuity patients with hemodynamic compromise defined as requiring inotropic or vasoactive medications, cardiopulmonary resuscitation, extracorporeal membrane oxygenation, ventricular assist devices, or transplant or who died and low-acuity patients without these interventions. We collected the first recorded set of vital signs, symptoms, laboratory values, and chest radiograph, electrocardiogram, and echocardiography results. Univariate analysis was performed, and 2 multivariable logistic regression models were created to discriminate between cohorts. RESULTS: A total of 74 patients were included: 33 high acuity and 41 low acuity. There were significant differences in demographics, symptoms, and physical examination, laboratory, electrocardiogram, and echocardiography findings between high- and low-acuity cohorts. Multivariable logistic regression models were highly discriminate in predicting those in the high-acuity cohort. The first model included presence of tachycardia, tachypnea, creatinine, and cardiomegaly on chest radiograph (area under the curve = 0.913). The second model added the presence of pericardial effusion to the above variables (area under the curve = 0.964). CONCLUSIONS: Models based on factors available at initial presentation with acute myocarditis are predictive of subsequent hemodynamic compromise. If our results can be validated in a multicenter study, these models may help disposition patients with suspected acute myocarditis (with those who meet model criteria being admitted to centers capable of rapidly providing extracorporeal membrane oxygenation, ventricular assist devices, and heart transplant evaluation).

Variability in donor selection among pediatric heart transplant providers: Results from an international survey.

There is considerable variability in donor acceptance practices among adult heart transplant providers; however, pediatric data are lacking. The aim of this study was to assess donor acceptance practices among pediatric heart transplant professionals. The authors generated a survey to investigate clinicians' donor acceptance practices. This survey was distributed to all members of the ISHLT Pediatric Council in April 2018. A total of 130 providers responded from 17 different countries. There was a wide range of acceptable criteria for potential donors. These included optimal donor-to-recipient weight ratio (lower limit: 50%-150%, upper limit: 120%-350%), maximum donor age (25-75 years), and minimum acceptable left ventricular EF (30%-60%). Non-US centers demonstrated less restrictive donor selection criteria and were willing to accept older donors (50 vs 35 years, P < 0.001), greater size discrepancy (upper limit weight ratio 250% vs 200%, P = 0.009), and donors with a lower EF (45% vs 50%, P < 0.001). Recipient factors were most influential in the decision to accept marginal donors including recipients requiring ECMO support, ventilator support, and highly sensitized patients with a negative XM. However, programmatic factors impacted the decision to decline marginal donors including recent programmatic mortalities and concerns for programmatic restrictions from regulatory bodies. There is significant variation in donor acceptance practices among pediatric heart transplant professionals. Standardization of donor acceptance practices through the development of a consensus statement may help to improve donor utilization and reduce waitlist mortality.

Should Physicians Offer a Ventricular Assist Device to a Pediatric Oncology Patient With a Poor Prognosis?

A case is presented of a 10-year-old girl with refractory leukemia with poor prognosis and chemotherapy-induced heart failure. She is evaluated for a ventricular assist device (VAD), but the pediatric heart failure team views VAD as clinically inappropriate due to her active oncologic problems. This article examines ethical concerns that arise in deciding whether to offer and use this technology.

Variations in Criteria and Practices for Heart Transplantation Listing Among Pediatric Transplant Cardiologists.

Ethical issues in pediatric heart transplantation (Htx) include resource allocation, benefit, and burden assessment in high-risk recipients, and informed consent. Practice patterns and decision-making was investigated using an internet survey with 47-multiple choice items and vignette-based questions. Of 43 pediatric Htx cardiologists contacted, 28 (65%) responded. Respondents reported that an overall median 1-month survival of 73% (range 50-100%), 1-year survival of 70% (range 50-85%), 5-year survival of 50% (range 40-85%), and 10-year survival of 50% (range 25-85%) was adequate to offer Htx. Based on vignettes presented, 100% of those surveyed would offer Htx to a straightforward 12-year old with end-stage dilated cardiomyopathy and a 7-year old with hypoplastic left heart syndrome with protein losing enteropathy. Thirty percent of physicians would offer Htx to a patient status post a Fontan procedure with mutliple co-morbidities. Seventy-five percent of physicians would offer Htx despite proven non-adherence. Considerable variability exists in the practice patterns of pediatric heart transplant cardiologists with regards to decision-making while evaluating patients for listing. Disagreements among pediatric Htx cardiologists exist when there are concerns for non-adherence and associated multiple co-morbidities. Further work is needed to understand these variations and develop consensus for pediatric Htx organ allocation.

Fontan-associated protein-losing enteropathy and post‒heart transplant outcomes: A multicenter study.

BACKGROUND: The influence of Fontan-associated protein-losing enteropathy's (PLE) severity, duration, and treatment on heart transplant (HTx) outcomes is unknown. We hypothesized that long-standing PLE and PLE requiring more intensive therapy are associated with increased post-HTx mortality. METHODS: This 12-center, retrospective cohort study of post-Fontan patients with PLE referred for HTx from 2003 to 2015 involved collection of demographic, medical, surgical, and catheterization data, as well as PLE-specific data, including duration of disease, intensity/details of treatment, hospitalizations, and complications. Factors associated with waitlist and post-HTx outcomes and PLE resolution were sought. RESULTS: Eighty patients (median of 5 per center) were referred for HTx evaluation. Of 68 patients listed for HTx, 8 were removed due to deterioration, 4 died waiting, and 4 remain listed. In 52 patients undergoing HTx, post-HTx 1-month survival was 92% and 1-year survival was 83%. PLE-specific factors, including duration of PLE pre-HTx, pre-HTx hospitalizations, need for/frequency of albumin replacement, PLE therapies, and growth parameters had no association with post-HTx mortality. Immunosuppressant regimen was associated with mortality; standard mycophenolate mofetil immunotherapy was used in 95% of survivors compared with only 44% of non-survivors (p = 0.03). Rejection (53%) and infection (42%) post-HTx were common, but not associated with PLE-specific factors. PLE resolved completely in all but 1 HTx survivor at a median of 1 month (interquartile range 1 to 3 months); resolution was not affected by PLE-specific factors. CONCLUSIONS: PLE severity, duration, and treatment do not influence post-HTx outcome, but immunosuppressive regimen may have an impact on survival. PLE resolves in nearly all survivors.

Changes in renal function after left ventricular assist device placement in pediatric patients: A Pedimacs analysis.

BACKGROUND: Renal dysfunction (RD) is prevalent among pediatric patients with advanced heart failure. Data are limited regarding changes in renal function after left ventricular assist device (LVAD) placement in this population. METHODS: Pediatric LVAD recipients enrolled in the Pediatric Interagency Registry for Mechanical Circulatory Support (Pedimacs) between September 19, 2012 and June 30, 2016 were included. Longitudinal changes in renal function were analyzed for the entire cohort as well as subgroups stratified by patient and device characteristics. Logistic regression was used to attempt to identify factors associated with lack of improvement in renal function after LVAD placement. Post-LVAD outcomes were assessed using the Kaplan‒Meier method. RESULTS: Data from 247 patients from 39 centers were analyzed. Baseline RD (estimated glomerular filtration rate [eGFR] <90 ml/min/1.73 m2) was present in 150 (61%) patients. Overall, eGFR improved post-LVAD, peaking at 1 month post-implant. There was an inverse relationship between baseline eGFR and the degree of improvement at 1 month. Degree of improvement in eGFR at 1 month was not impacted by device type, age, Interagency Registry for Mechanically Assisted Circulatory Support (INTERMACS) profile, or diagnosis. Failure to normalize renal function at 1 week was correlated with persistent RD at 1 month. Post-implant outcomes did not differ among patients stratified by pre-implant renal function. CONCLUSIONS: Renal function improves post-LVAD placement in pediatric patients regardless of age, diagnosis, illness severity, or device type, with improvement most pronounced in patients with baseline RD. Identifying patients with irreversible renal dysfunction before LVAD placement remains difficult. Pre-LVAD renal function does not appear to impact survival to transplant.

Outcomes of children supported with devices labeled as "temporary" or short term: A report from the Pediatric Interagency Registry for Mechanical Circulatory Support.

BACKGROUND: Historically, the "temporary" or short-term ventricular assist device (VAD) was used only as a quick bridge to recovery for children with an acute process. In the current era, the devices that were originally used for temporary support are now being used to support children for longer durations and for a variety of indications. In this study we aimed to describe the overall use, patients' characteristics and outcomes of "temporary" VAD use in children. METHODS: The Pediatric Interagency Registry for Mechanical Circulatory Support (PediMACS) is a National Institutes of Health-supported national registry for United States Food and Drug Administration-approved VADs in patients <19 years of age at the time of VAD implantation (either durable or temporary VAD). Patients undergoing placement of a device classified as a temporary VAD between September 19, 2012 and June 30, 2016 were included. RESULTS: Temporary VADs were implanted in 63 patients at 20 centers, accounting for 19% of all pediatric VAD patients entered into PediMACS. The median age at implantation was 3.7 (range <1 day to 18) years. The underlying diseases were: congenital heart disease in 26 (41%), 20 of whom were classified as single ventricle; cardiomyopathy in 25 (40%); and myocarditis/rejection in 12 (19%). Patients were predominately Interagency Registry for Mechanically Assisted Circulatory Support (INTERMACS) Profile 1 (51%), and 10 patients (16%) had previously been supported with extracorporeal membrane oxygenation. Median duration of support was 15 (range <1 day to 227) days, with 41 patients (65%) being on support for ≥10 days. The most frequent adverse events were bleeding (29% of patients) and neurologic dysfunction (24% of patients). Overall, 71% (45) achieved a positive outcome (defined as bridge to recovery [30%], transplantation [17%], alive on device [2%] or transition to durable VAD [22%]). Eighty-eight percent (n = 22) of the cardiomyopathy patients and 60% (n = 12) of the single-ventricle patients achieved a favorable outcome. CONCLUSION: Devices historically classified as "temporary" pumps are being used not only as a short-term mechanical circulatory support strategy but also as a longer term support strategy. In this multi-institutional, high-acuity, complex patient cohort, the use of "temporary" VADs resulted in a positive outcome (bridge to transplant, recovery durable device or alive) in 71% of patients.

Preoperative Use of CT Angiography in Infants With Coarctation of the Aorta.

BACKGROUND: Computed tomographic angiography (CTA) and echocardiography (echo) are used preoperatively in coarctation of the aorta to define arch hypoplasia and great vessel branching. We sought to determine differences in quantitative measurements, as well as surgical utility, between modalities. METHODS: Infants (less than six months) with both CTA and echo prior to coarctation repair from 2004 to 2013 were included. Measurements were compared and correlated with surgical approach. Three surgeons reviewed de-identified images to predict approach and characterize utility. Computed tomographic angiography radiation dose was calculated. RESULTS: Thirty-three patients were included. No differences existed in arch measurements between echo and CTA ( z-score: -2.59 vs -2.43; P = .47). No differences between modalities were seen for thoracotomy ( z-score: -2.48 [echo] vs -2.31 [CTA]; P = .48) or sternotomy ( z-score: -3.13 [echo] vs -3.08 [CTA]; P = .84). Computed tomographic angiography delineated great vessel branching pattern in two patients with equivocal echo findings ( P = .60). Surgeons rated CTA as far more useful than echo in understanding arch hypoplasia and great vessel branching in cases where CTA was done to resolve anatomical questions that remain after echo evaluation. Two of three surgeons were more likely to choose the surgical approach taken based on CTA (surgeon A, P = .02; surgeon B, P = .01). Radiation dose averaged 2.5 (1.6) mSv and trended down from 2.9 mSv (1.8 mSv; n = 20) to 1.6 mSv (0.5 mSv; n = 7) ( P = .06) with new technology. CONCLUSION: Although CTA and echo measurements of the aorta do not differ, CTA better delineates branching and surgeons strongly prefer it for three-dimensional arch anatomy. We recommend CTA for patients with anomalous arch branching patterns, diffuse or complex hypoplasia, or unusual arch morphology not fully elucidated by echo.