A Novel Mental Health Curriculum Pilot for Pediatric Cardiology Fellows: Preparing the Subspecialist to Expand the Team.

Mental health (MH) is an important, yet understudied area of care for patients with congenital heart disease (CHD). Pediatric subspecialty fellows believe MH should be within their scope of practice, but few feel confident in their ability to appropriately screen, evaluate, manage, and make treatment referrals for youth with CHD and concurrent MH concerns. A 6-session, didactic-based curriculum was designed by an interprofessional team of experts. It was administered to pediatric cardiology fellows over 9 months during two academic years. Topics included the following: Introduction to MH and CHD, MH Screening and Clinic-Based Interventions, Psychopharmacology, Delirium, Trauma-Informed Care, and Adult CHD and Transitional Care. An investigator-designed survey was developed to assess fellow comfort with MH knowledge, screening, and treatment recommendation domains. Twenty-three fellows participated with 14 completing the pre-post survey (58% participation, 11/17 in year 1, 3/6 in year 2). Most participants were in their first year of training (9/14, 57%). Overall, fellow comfort with MH topics increased significantly (mean score 2.89 ± 0.46 vs. 3.4 ± 0.4, p = 0.0005), with improved comfort in the MH knowledge (p = 0.003) and treatment recommendation domains (p = 0.001). A didactic-based MH curriculum improves the comfort of pediatric cardiology fellows to address the MH concerns of their patients. The topics in this curriculum will continue to be refined over time and could be generalized to training programs, both nationally and across pediatric specialties, to improve MH care for high-risk populations.

A multimedia paediatric cardiology assessment tool for medical students and general paediatric trainees: development and validation.

BACKGROUND: Understanding how cardiovascular structure and physiology guide management is critically important in paediatric cardiology. However, few validated educational tools are available to assess trainee knowledge. To address this deficit, paediatric cardiologists and fellows from four institutions collaborated to develop a multimedia assessment tool for use with medical students and paediatric residents. This tool was developed in support of a novel 3-dimensional virtual reality curriculum created by our group. METHODS: Educational domains were identified, and questions were iteratively developed by a group of clinicians from multiple centres to assess understanding of key concepts. To evaluate content validity, content experts completed the assessment and reviewed items, rating item relevance to educational domains using a 4-point Likert scale. An item-level content validity index was calculated for each question, and a scale-level content validity index was calculated for the assessment tool, with scores of ≥0.78 and ≥0.90, respectively, representing excellent content validity. RESULTS: The mean content expert assessment score was 92% (range 88-97%). Two questions yielded ≤50% correct content expert answers. The item-level content validity index for 29 out of 32 questions was ≥0.78, and the scale-level content validity index was 0.92. Qualitative feedback included suggestions for future improvement. Questions with ≤50% content expert agreement and item-level content validity index scores <0.78 were removed, yielding a 27-question assessment tool. CONCLUSIONS: We describe a multi-centre effort to create and validate a multimedia assessment tool which may be implemented within paediatric trainee cardiology curricula. Future efforts may focus on content refinement and expansion to include additional educational domains.

Virtual reality curriculum increases paediatric residents' knowledge of CHDs.

OBJECTIVES: Virtual reality has emerged as a unique educational modality for medical trainees. However, incorporation of virtual reality curricula into formal training programmes has been limited. We describe a multi-centre effort to develop, implement, and evaluate the efficacy of a virtual reality curriculum for residents participating in paediatric cardiology rotations. METHODS: A virtual reality software program ("The Stanford Virtual Heart") was utilised. Users are placed "inside the heart" and explore non-traditional views of cardiac anatomy. Modules for six common congenital heart lesions were developed, including narrative scripts. A prospective case-control study was performed involving three large paediatric residency programmes. From July 2018 to June 2019, trainees participating in an outpatient cardiology rotation completed a 27-question, validated assessment tool. From July 2019 to February 2020, trainees completed the virtual reality curriculum and assessment tool during their cardiology rotation. Qualitative feedback on the virtual reality experience was also gathered. Intervention and control group performances were compared using univariate analyses. RESULTS: There were 80 trainees in the control group and 52 in the intervention group. Trainees in the intervention group achieved higher scores on the assessment (20.4 ± 2.9 versus 18.8 ± 3.8 out of 27 questions answered correctly, p = 0.01). Further analysis showed significant improvement in the intervention group for questions specifically testing visuospatial concepts. In total, 100% of users recommended integration of the programme into the residency curriculum. CONCLUSIONS: Virtual reality is an effective and well-received adjunct to clinical curricula for residents participating in paediatric cardiology rotations. Our results support continued virtual reality use and expansion to include other trainees.

Paediatric cardiology training: burnout, fulfilment, and fears.

BACKGROUND: Burnout is well characterised in physicians and residents but not in paediatric cardiology fellows, and few studies follow burnout longitudinally. Training-specific fears have been described in paediatric cardiology fellows but also have not been studied at multiple time points. This study aimed to measure burnout, training-specific fears, and professional fulfilment in paediatric cardiology fellows with the attention to time of year and year-of-training. METHODS: This survey-based study included the Professional Fulfillment Index and the Impact of Events Scale as well as an investigator-designed Fellow Fears Questionnaire. Surveys were distributed at three-time points during the academic year to paediatric cardiology fellows at a large Midwestern training programme. Fellow self-reported gender and year-of-training were collected. Descriptive analyses were performed. RESULTS: 10/17 (59%) of fellows completed all surveys; 60% were female, 40% in the first-year class, 40% in the second-year class, and 20% in the third-year class. At least half of the fellows reported burnout at each survey time point, with lower mean professional fulfilment scores. The second-year class, who rotate primarily in the cardiac ICU, had higher proportions of burnout than the other two classes. At least half of fellows reported that they "often" or "always" worried about not having enough clinical knowledge or skills and about work-life balance. CONCLUSIONS: Paediatric cardiology fellows exhibit high proportions of burnout and training-specific fears. Interventions to mitigate burnout should be targeted specifically to training needs, including during high-acuity rotations.

Establishing Entrustable Professional Activities in Pediatric Cardiac Critical Care.

OBJECTIVES: Define a set of entrustable professional activities for pediatric cardiac critical care that are recognized as the core activities of the subspecialty by a diverse group of pediatric cardiac critical care physicians and that can be broadly and consistently applied irrespective of training pathway. DESIGN: Mixed methods study with sequential integration of qualitative and quantitative data. SETTING: Structured telephone interviews of pediatric cardiac critical care medical directors at Pediatric Cardiac Critical Care Consortium centers followed by an electronic survey of pediatric cardiac critical care physician members of the Pediatric Cardiac Intensive Care Society from across the United States and internationally. SUBJECTS: Pediatric cardiac intensive care physicians. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Twenty-four of 26 eligible Pediatric Cardiac Critical Care Consortium medical directors participated in the interviews. Based on qualitative analyses of interview data, we identified an initial set of nine entrustable professional activities. Fifty-eight of 185 eligible physicians completed a subsequent survey asking them to rate their agreement with the entrustable professional activities. It showed consensus (> 80% agreement) with the entire initial set of entrustable professional activities, with greater than 96% agreement in most cases. The feedback from free-text survey responses was incorporated to generate a final set of entrustable professional activities. CONCLUSIONS: We generated a set of nine entrustable professional activities, which we believe can be broadly applied to any physician training in pediatric cardiac critical care, irrespective of individual training pathway. Next steps include incorporation of these entrustable professional activities into curriculum design and trainee assessment tools.

Native Aortic Root Thrombosis in Single-Ventricle Patients with Native-to-Neoaortic Anastomoses.

In single-ventricle patients with native-to-neoaortic anastomoses, the native aortic root serves as a conduit to the coronary arteries. Thrombosis of the native aortic root has been described only in small, limited reports. We described our center's experience with this rare adverse event. All single-ventricle patients who underwent native-to-neoaortic anastomosis from 2002 to 2017 were compiled from institutional databases. Chart review identified cases of native aortic root thrombosis. Of 467 patients, there were 9 (2%) cases of native aortic root thrombosis; all had hypoplastic left heart syndrome. Timing of thrombosis varied and occurred following each stage of single-ventricle palliation. For treatment, 8 patients received systemic anticoagulation and one patient did not receive any treatment. One patient also underwent percutaneous thrombectomy. Three patients (33.3%) died during the follow-up period. Among survivors, 4/6 had right ventricular systolic function that was mildly depressed or worse at a median follow-up of 8.3 years (interquartile range 1.3-10.8). Native aortic root thrombosis is a rare complication occurring at various stages of single-ventricle palliation. In this series, 6 of 9 patients (66.6%) survived; however, ventricular function was often compromised. No risk factors were identified to focus preventative therapies.

Not just vegetations: focal myocardial changes in patients with fungal infections.

BACKGROUND: Fungal endocarditis classically involves dense heterogenous vegetations. However, several patients with fungal infections were noted to have myocardial changes ranging from focal brightening to nodular thickening of chordae or papillary muscles. This study evaluates whether these findings are associated with fungal infections. METHODS: In a retrospective case-control study, paediatric inpatients with fungal infections (positive blood, urine, or catheter tip culture) in a 5-year period were matched 1:1 to inpatients without positive fungal cultures. Echocardiograms were scored on a 5-point scale by two independent readers for presence of myocardial brightenings, nodular thickenings, and vegetations. Clinical data were compared. RESULTS: Of 67 fungal cases, positive culture sites included blood (n = 44), vascular catheter tip (n = 7), and urine (n = 29); several had multiple positive sites. "Positive" echo findings (score ≥ 2+) were more frequent in the Fungal Group (33 versus 18%, p = 0.04). Fungal Group patients with "positive" versus "negative" echo findings had similar proportion of bacterial infections. Among fungal cases, those with "positive" echo findings had longer hospital length of stay than cases with "negative" echos (median 58 versus 40 days, p = 0.03) but no difference in intensive care unit admission, extracorporeal membranous oxygenation support, or mortality. CONCLUSIONS: Myocardial and papillary muscle brightening with nodular thickening on echocardiogram appear to be associated with fungal infections. There may be prognostic implications of these findings as patients with "positive" echo have longer length of stay. Further studies are needed to better understand the mechanism and temporal progression of these changes and determine the prognostic value of this scoring system.

Counseling Practices for Fetal Hypoplastic Left Heart Syndrome.

While counseling parents of a fetus diagnosed with hypoplastic left heart syndrome (HLHS), pediatric cardiologists play a critical role in shaping a family's expectations for the months and years to come. However, techniques for the most effective counseling practices have not been studied, and significant variation among physicians is likely present. Web-based survey of pediatric cardiologists that perform fetal echocardiography using snowball sampling. 201 physicians responded (61% male, 81% from academic centers, and 95% from the U.S.), with an average experience of 12 years. The majority of respondents (73%) typically received initial referrals for HLHS between 20 and 24 weeks of gestation. Most physicians counsel families alone (54%), while others counsel with a nurse (35%), social worker (12%), and/or maternal-fetal medicine colleague (15%). Termination of pregnancy was discussed by 79% of respondents, although 15% did not know their state's legal limit for termination. While initial counseling sessions routinely described the typical earlier ramifications of HLHS, many long-term sequelae of the disease were not commonly discussed. Content of counseling was affected by region of the country, but not by practice setting, experience, or fetal volume. Respondents identified multiple barriers that limited their counseling practices. Our data suggest that current counseling practices often fail to cover important information. Perceived barriers to a full discourse on long-term sequelae of HLHS are common and may lead to a disconnect between reality and a family's understanding of the natural history of palliated HLHS. Opportunities to improve counseling practices exist, and there may be benefits to gain from more formal training.

Hyperoxia Is Associated With Poor Outcomes in Pediatric Cardiac Patients Supported on Venoarterial Extracorporeal Membrane Oxygenation.

OBJECTIVES: Patients who require venoarterial extracorporeal membrane oxygenation because of cardiac failure frequently have supranormal blood oxygen tensions (hyperoxia). Recent studies have suggested worse outcomes in patients with hyperoxia after resuscitation from cardiac or respiratory arrests, presumably because of oxidative stress. There are limited data regarding the effect of hyperoxia on outcomes in pediatric patients on venoarterial extracorporeal membrane oxygenation. DESIGN: Retrospective chart review. SETTING: Pediatric cardiothoracic ICU. PATIENTS: Cardiac surgery patients less than 1 year old requiring venoarterial extracorporeal membrane oxygenation in the postoperative period from 2007 to 2013. MEASUREMENTS AND MAIN RESULTS: In 93 infants (median time on extracorporeal membrane oxygenation, 5 d), mortality at 30 days post surgery (primary outcome) was 38%. Using a receiver operating characteristic curve, a mean PaO2 of 193 mm Hg in the first 48 hours of extracorporeal membrane oxygenation was determined to have good discriminatory ability with regard to 30-day mortality. Univariate analysis identified a mean PaO2 greater than 193 mm Hg (p = 0.001), longer cardiopulmonary bypass times (p = 0.09), longer duration of extracorporeal membrane oxygenation (p < 0.0001), and higher extracorporeal membrane oxygenation pump flows (p = 0.052) as possible risk factors for 30-day mortality. In multivariable analysis controlling for the variables listed above, a mean PaO2 greater than 193 mm Hg remained an independent risk factor for mortality (p = 0.03). In addition, a mean PaO2 greater than 193 mm Hg was associated with the need for renal dialysis (p = 0.02) but not with neurologic injury (p = 0.41) during the hospitalization. CONCLUSIONS: In infants with congenital heart disease who are placed on venoarterial extracorporeal membrane oxygenation postoperatively, hyperoxia (defined as a mean PaO2 > 193 mm Hg in the first 48 hr of extracorporeal membrane oxygenation) was an independent risk factor for 30-day mortality after surgery. Future studies are needed to delineate the causative or associative role of hyperoxia with outcomes, especially in children with baseline cyanosis who may be more susceptible to the effects of oxidative stress.

Aortico-left ventricular tunnel and left ventricular non-compaction: a case series.

Aortico-left ventricular tunnel and left ventricular non-compaction are rare congenital cardiac anomalies with varied clinical presentations and sparsely described co-existence in the medical literature. Owing to the limited information about these diagnoses in tandem, we present four cases of aortico-left ventricular tunnel and left ventricular non-compaction from our institution and discuss the clinical presentation, management, and follow-up.

Biventricular Badness: Rare Images of Ebstein Anomaly of the Tricuspid Valve in a Patient with Hypoplastic Left Heart Syndrome.

We report a rare case of hypoplastic left heart syndrome coexisting in a patient with Ebstein anomaly of the tricuspid valve, which has previously been described only in pathological studies. A fetal echocardiogram at 27-weeks gestation showed severe aortic stenosis with evolving hypoplastic left heart syndrome, significant endocardial fibroelastosis, a dysplastic tricuspid valve with moderate regurgitation, right atrial and ventricular dilation, and signs of fetal congestive heart failure. Due to inadequate left heart size, the patient was not a candidate for fetal intervention for critical aortic stenosis, and repeat studies showed progression of the lesion through the pregnancy. The infant was delivered at 36-weeks gestation with signs of hydrops, and a postnatal echocardiogram confirmed hypoplastic left heart syndrome as well as severe Ebstein anomaly of the tricuspid valve. The infant did not survive to intervention.

Prospective Evaluation of High Titer Autoantibodies and Fetal Home Monitoring in the Detection of Atrioventricular Block Among Anti-SSA/Ro Pregnancies.

OBJECTIVE: This prospective study of pregnant patients, Surveillance To Prevent AV Block Likely to Occur Quickly (STOP BLOQ), addresses the impact of anti-SSA/Ro titers and utility of ambulatory monitoring in the detection of fetal second-degree atrioventricular block (AVB). METHODS: Women with anti-SSA/Ro autoantibodies by commercial testing were stratified into high and low anti-52-kD and/or 60-kD SSA/Ro titers applying at-risk thresholds defined by previous evaluation of AVB pregnancies. The high-titer group performed fetal heart rate and rhythm monitoring (FHRM) thrice daily and weekly/biweekly echocardiography from 17-26 weeks. Abnormal FHRM prompted urgent echocardiography to identify AVB. RESULTS: Anti-52-kD and/or 60-kD SSA/Ro met thresholds for monitoring in 261 of 413 participants (63%); for those, AVB frequency was 3.8%. No cases occurred with low titers. The incidence of AVB increased with higher levels, reaching 7.7% for those in the top quartile for anti-60-kD SSA/Ro, which increased to 27.3% in those with a previous child who had AVB. Based on levels from 15 participants with paired samples from both an AVB and a non-AVB pregnancy, healthy pregnancies were not explained by decreased titers. FHRM was considered abnormal in 45 of 30,920 recordings, 10 confirmed AVB by urgent echocardiogram, 7 being second-degree AVB, all <12 hours from normal FHRM and within another 0.75 to 4 hours to echocardiogram. The one participant with second/third-degree and two participants with third-degree AVB were diagnosed by urgent echocardiogram >17 to 72 hours from an FHRM. Surveillance echocardiograms detected no AVB when the preceding interval FHRM recordings were normal. CONCLUSION: High-titer antibodies are associated with an increased incidence of AVB. Anti-SSA/Ro titers remain stable over time and do not explain the discordant recurrence rates, suggesting that other factors are required. Fetal heart rate and rhythm (FHRM) with results confirmed by a pediatric cardiologist reliably detects conduction abnormalities, which may reduce the need for serial echocardiograms.

Representation of Women and Minority Faculty and Fellows in Academic Pediatric Cardiology Training Programs.

BACKGROUND: Studies have shown that diverse care teams optimize patient outcomes. Describing the current representation of women and minorities has been a critical step in improving diversity across several fields. OBJECTIVES: To address the lack of data specific to pediatric cardiology, the authors conducted a national survey. METHODS: U.S. academic pediatric cardiology programs with fellowship training programs were surveyed. Division directors were invited (July 2021 to September 2021) to complete an e-survey of program composition. Underrepresented minorities in medicine (URMM) were characterized using standard definitions. Descriptive analyses at the hospital, faculty, and fellow level were performed. RESULTS: Altogether, 52 of 61 programs (85%) completed the survey, representing 1,570 total faculty and 438 fellows, with a wide range in program size (7-109 faculty, 1-32 fellows). Although women comprise approximately 60% of faculty in pediatrics overall, they made up 55% of fellows and 45% of faculty in pediatric cardiology. Representation of women in leadership roles was notably less, including 39% of clinical subspecialty directors, 25% of endowed chairs, and 16% of division directors. URMM comprise approximately 35% of the U.S. population; however, they made up only 14% of pediatric cardiology fellows and 10% of faculty, with very few in leadership roles. CONCLUSIONS: These national data suggest a "leaky pipeline" for women in pediatric cardiology and very limited presence of URRM overall. Our findings can inform efforts to elucidate underlying mechanisms for persistent disparity and reduce barriers to improving diversity in the field.

Intermediate-term effects of intracardiac communications created noninvasively by therapeutic ultrasound (histotripsy) in a porcine model.

The authors have demonstrated that histotripsy (pulsed cavitational ultrasound) can create atrial septal defects and ventricular septal defects (VSDs) in an open-chest canine model transcutaneously through the intact chest of neonatal pigs. To assess the potential untoward effects of these applications, the clinical, systemic, and pathologic effects of histotripsy-induced intracardiac communications were analyzed. Six neonatal pigs received noninvasive ultrasound therapy to their ventricular septa, then were allowed to survive 1 month for evaluation of intermediate-term effects. The results were compared with those of six previous animals killed immediately and three others killed 2-3 days after the procedure. Brain magnetic resonance imaging (MRI) and an assessment of cardiac function were performed with long-term survivors, and pathologic specimens were obtained when the animals were killed. In all 15 animals, VSDs 2-6.5 mm wide were successfully created. No fatalities occurred, and all the animals thrived, achieving normal weight gain by the time they were killed. Brain MRI and lung pathology exhibited no evidence of thromboembolic events. No damage to intervening tissue was observed. Pathologic analysis showed demarcated damage to the ventricular septa. Flanking injury and hemorrhage observed acutely were resolved by 1 month, with tissue remodeling present. Transcutaneous histotripsy is a safe and effective technique for creating intracardiac communications noninvasively without intermediate-term untoward effects. With further refinement and development, histotripsy has the potential to become an effective tool for palliation of congenital heart disease.

Extended Reality International Grand Rounds: An Innovative Approach to Medical Education in the Pandemic Era.

PROBLEM: While bedside training has always presented its own unique challenges, the COVID-19 pandemic era has intensified barriers to suitable provider and trainee experiences for both patient care and medical education. APPROACH: This project introduced an innovative solution with the Extended Reality International Grand Rounds, a collaboration between the University of Michigan Center for Medical and Surgical Extended Reality and Imperial College London. Three complex cases were presented to trainees through a wireless, extended reality (XR) headset and augmented by holographic visual aids and expert commentary. This pilot rounding experience was performed through the first-person view of one clinician at the bedside. OUTCOMES: In 2020, 140 attendees participated in XR International Grand Rounds, and 82 (59%) and 61 (44%) completed pre- and postsurveys, respectively. Survey analysis showed that the majority of respondents (65, 79.3%) had very little to no baseline experience with XR technologies and nearly all (75, 91.5%) agreed that the development and implementation of XR curricula are important in medical training, indicating an unmet need. Nearly all respondents (59, 96.7%) found value in the ability to visualize patients' clinical findings in the XR rounding experience and 60 (98.4%) found value in the ability to visualize patient-specific imaging and test findings in an XR format. Limiting exposure to high-risk patients and care team members with this innovative format was believed to be important to 79 (96.3%) respondents at baseline and that perception was unchanged following the event. NEXT STEPS: This solution to a long-standing dilemma, newly stressed by a unique era in medicine, was a successful collaboration using state-of-the-art XR technology. Next steps will include introducing more advanced physical exam visualization and detection and comprehensive evaluation of the patient experience, as well as expanding the international experience in a format that is scalable to other interested institutions.

Resuscitating the Socratic Method: Student and Faculty Perspectives on Posing Probing Questions During Clinical Teaching.

PURPOSE: Teaching by way of asking questions is a time-honored practice that has taken on the negative connotation of "pimping" among medical students and has made some faculty hesitant to ask students questions during clerkship rotations. Yet, quantitative studies exploring student perspectives on this practice are limited. This study aimed to solicit student and faculty views and investigate faculty perceptions of students' preferences. METHOD: Students who completed their internal medicine clerkship during the 2017-2018 academic year (n = 165) and were from the 2020 graduating class and their supervising faculty (n = 144) at the University of Michigan Medical School were asked to complete a Likert response survey in April 2019. The survey solicited perspectives on questions probing medical knowledge posed to students by faculty. Surveys were constructed using an iterative process, and data were analyzed using t tests and linear regressions. RESULTS: A total of 140 (85%) students and 112 (78%) faculty participated. Of those, 125 (89%) students and 109 (97%) faculty agreed that probing questions are valuable for student education, but only 73 (65%) faculty perceived that students agreed with this statement (P < .001). In addition, 115 (82%) students preferred to be asked too many questions than none at all. Fifty-five (39%) students agreed that they feel humiliated when they answer a question incorrectly. However, only 7 (5%) students agreed that faculty ask questions to humiliate them, and only 20 (14%) preferred that faculty stop asking questions if they answer a question incorrectly. CONCLUSIONS: Students valued probing questions more than faculty perceived, which argues against a withdrawal from the Socratic teaching method in the clinical arena. The students' experience of humiliation when answering incorrectly requires further study and perhaps can be tempered by more explicit framing of the role of the questioning process.

Successful staged neonatal repair of tetralogy of Fallot with long-segment hypoplasia of the aorta.

We describe an extremely rare combination of tetralogy of Fallot (TOF), right-sided cervical aortic arch with long-segment hypoplasia, and other vascular anomalies. A two-stage surgical approach included aortic arch reconstruction followed by right ventricular muscle bundle division and ventricular septal defect closure a few weeks later. The initial clinical presentation, perioperative course, and imaging studies are presented along with a review of the relevant literature. This is the first report of successful neonatal repair of TOF with long-segment hypoplasia of the aorta.

Acquired von Willebrand Syndrome in an Infant With Coarctation of the Aorta and Williams Syndrome.

An infant with coarctation of the aorta and Williams syndrome was noted to have petechiae in cardiology clinic prior to planned surgical intervention. Workup revealed acquired von Willebrand syndrome secondary to the high shear force generated by the aortic coarctation. He was treated with intra- and postoperative Humate P; there were no postoperative bleeding complications. His acquired von Willebrand syndrome resolved postoperatively.

Emerging 3D technologies and applications within congenital heart disease: teach, predict, plan and guide.

3D visualization technologies have evolved to become a mainstay in the management of congenital heart disease (CHD) with a growing presence within multiple facets. Printed and virtual 3D models allow for a more comprehensive approach to educating trainees and care team members. Computational fluid dynamics can take 3D modeling to the next level, by predicting post-procedural outcomes and helping to determine surgical approach. 3D printing and extended reality are developing resources for pre-procedural planning and intra-procedural guidance with the potential to revolutionize decision-making and procedural success. Challenges still remain within existing technologies and their applications to the CHD field. Addressing these gaps, both by those within and outside of CHD, will transform education and patient care within our field.

Outcomes for patients with unbalanced atrioventricular septal defects.

Infants with an unbalanced atrioventricular septal defect (AVSD) frequently present with comorbidities that may have an impact on their medical course and outcome. This study aimed to assess outcomes and explore possible prognostic indicators for patients undergoing surgical palliation for an unbalanced AVSD. The medical records of all infants presenting to the authors' institution with an unbalanced AVSD over a 5-year period were retrospectively reviewed for assessment of outcomes and comorbidities. The study group consisted of 44 patients with an overall survival rate of 51% for the entire follow-up period. The majority of these patients (88%) underwent single-ventricle palliation, with an 83% rate of survival to initial hospital discharge and an overall long-term survival rate of 50%. The midterm outcome was significantly worse than that for a cohort of hypoplastic left heart syndrome patients undergoing single-ventricle palliation during the same period (P = 0.03). In addition, 30% of the patients required either repair or replacement of their systemic atrioventricular valve at initial palliation or during subsequent follow-up evaluation. Of the patients with an unbalanced AVSD, 75% had associated congenital anomalies. In conclusion, infants with an unbalanced AVSD are a high-risk population with diminished midterm survival compared with palliated patients who have more classic forms of hypoplastic heart syndromes. This may be due to the higher incidence of both severe atrioventricular valve regurgitation and important associated congenital anomalies.