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Improvement in congenital heart disease (CHD) outcomes has created a growing population of adolescents and young adults with unique health needs that require thoughtful transition planning and eventual transfer of care to an adult provider. Often, poor health literacy and limited resources can lead to interrupted care, which places them at risk for adverse health-related consequences. In 2019, the Wisconsin Adult Congenital Heart Disease transition program partnered with Stanford Virtual Heart (SVH), a virtual reality (VR) platform, to allow young adult patients to learn about their CHD in a clinic-based setting. We completed a single-center pilot study to evaluate these patients’ experience and perceptions to using VR during their transition education. At an initial transition visit, we used an immediate post-VR experience survey, scored using Likert scales of 1–5 (1 = strongly disagree, 5 = strongly agree). Twenty-two patients (13 males) between the ages of 16 and 19 participated. Lesions included pulmonary stenosis, Tetralogy of Fallot, atrial and ventricular septal defect, coarctation, aortic stenosis, hypoplastic left heart syndrome, and patent ductus arteriosus. Likert averages were 4.7 for finding VR helped with understanding their heart lesion, 4.6 for finding VR helped with understanding their heart surgery, 4.7 for enjoying the VR heart simulation, and 4.6 for finding that it was a good use of time. This study demonstrates that adolescents enjoyed using SVH and found it helpful. Clinical implementation shows promise as a plausible adjunct tool for transition education.

Introduction and hypothesisThis study is aimed at evaluating the readability and quality of Wikipedia articles on pelvic floor disorders (PFD) and comparing their content with International Urogynecological Association patient education leaflets.MethodsReadability was assessed using six different readability scales, including the Simple Measure of Gobbledygook (SMOG) Index, which is considered superior for scoring healthcare information. Quality was assessed by three female pelvic medicine and reconstructive surgery fellows using the modified DISCERN instrument. DISCERN is validated to evaluate the quality of written consumer health information; it was subsequently modified by health education researchers to enable the evaluation of Wikipedia articles.ResultsWe evaluated 30 Wikipedia articles that correlated with 29 International Urogynecological Association leaflets. The mean SMOG score of the Wikipedia articles was 12.0 ± 2.1 (12th-grade reading level) whereas the mean SMOG score of the International Urological Association (IUGA) leaflets was 3.4 ± 0.3 (third-grade reading level, p < 0.001). The mean modified DISCERN score of the Wikipedia articles was 34.43 ± 5.90 (moderate quality); however, the mean modified DISCERN score of the IUGA literature was 45.02 ± 1.36 (good quality, p < 0.001).ConclusionsWikipedia articles on PFD are neither readable nor reliable: they require a 12th-grade-level education for comprehension and are merely rated moderate in quality. In comparison, IUGA leaflets require a third-grade education for comprehension and are rated good in quality. Urogynecological providers should provide appropriate health education materials to patients, as Wikipedia is both a popular and sometimes inaccurate resource for patients.

Introduction and hypothesisGuidelines vary on antibiotic prophylaxis for onabotulinumtoxinA (Botox) treatment for overactive bladder (OAB). Our primary objective was to determine whether any prophylactic regimen is more effective in preventing urinary tract infection (UTI) after Botox. The secondary objective was to identify prophylactic practice patterns among female pelvic medicine and reconstructive surgery (FPMRS) providers of different training backgrounds as well as general urologists.MethodsThis was a secondary analysis of a retrospective cohort study on urinary retention after Botox injection in women with and without diabetes mellitus and OAB. Women > 18 years old who underwent Botox injection for OAB between January 2013 and September 2018 were included. Exclusion criteria were history of urinary retention and neuromuscular bladder dysfunction.ResultsA total of 565 patients were included. Two hundred eighty (49.6%) were treated by OB-GYN FPMRS, 209 (37.0%) by urology FPMRS and 76 (13.5%) by general urologists. The majority (92.9%) received antibiotic prophylaxis: 44.4% received intravenous (IV) only, 8.9% received oral (PO) only, and 39.7% received combination IV and PO prophylaxis. Urology FPMRS used antibiotic prophylaxis less frequently (p = 0.003). Within 3 months, 171 patients developed UTI (30.4%). There was no difference in post-procedural UTI for any antibiotic regimen compared to no prophylaxis. No route of antibiotic administration was superior at preventing UTI.ConclusionsIn this cohort, no route of antibiotic administration was more effective in the prevention of UTI. Antibiotic prophylaxis did not lower the rate of post-procedural UTI compared to no antibiotics.

Hypoplastic left heart syndrome (HLHS) is a severe congenital heart disease (CHD) with complex genetic inheritance. HLHS segregates with other left ventricular outflow tract (LVOT) malformations in families, and can present as either an isolated phenotype or as a feature of a larger genetic disorder. The multifactorial etiology of HLHS makes it difficult to interpret the clinical significance of genetic variants. Specific genes have been implicated in HLHS, including rare, predicted damaging MYH6 variants that are present in >10% of HLHS patients, and which have been shown to be associated with decreased transplant-free survival in our previous studies. MYH6 (α-myosin heavy chain, α-MHC) variants have been reported in HLHS and numerous other CHDs, including LVOT malformations, and may provide a genetic link to these disorders. In this paper, we outline the MYH6 variants that have been identified, discuss how bioinformatic and functional studies can inform clinical decision making, and highlight the importance of genetic testing in HLHS.

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. 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. 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. 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.

Objectives:We investigated the efficacy and complication profile of intranasal dexmedetomidine for transthoracic echocardiography sedation in patients with single ventricle physiology and shunt-dependent pulmonary blood flow during the high-risk interstage period.Methods:A single-centre, retrospective review identified interstage infants who received dexmedetomidine for echocardiography sedation. Baseline and procedural vitals were reported. Significant adverse events related to sedation were defined as an escalation in care or need for any additional/increased inotropic support to maintain pre-procedural haemodynamics. Minor adverse events were defined as changes from baseline haemodynamics that resolved without intervention. To assess whether sedation was adequate, echocardiogram reports were reviewed for completeness.Results:From September to December 2020, five interstage patients (age 29–69 days) were sedated with 3 mcg/kg intranasal dexmedetomidine. The median sedation onset time and duration time was 24 minutes (range 12–43 minutes) and 60 minutes (range 33–60 minutes), respectively. Sedation was deemed adequate in all patients as complete echocardiograms were accomplished without a rescue dose. When compared to baseline, three (60%) patients had a >10% reduction in heart rate, one (20%) patient had a >10% reduction in oxygen saturations, and one (20%) patient had a >30% decrease in blood pressure. Amongst all patients, no significant complications occurred and haemodynamic changes from baseline did not result in need for intervention or interruption of study.Conclusions:Intranasal dexmedetomidine may be a reasonable option for echocardiography sedation in infants with shunt-dependent single ventricle heart disease, and further investigation is warranted to ensure efficacy and safety in an outpatient setting.

Aortopulmonary collaterals (APCs) develop universally, but to varying degrees, in patients with single ventricle congenital heart disease (CHD). Despite their ubiquitous presence, APCs remain poorly understood. We sought to evaluate the association between APC burden and common non-invasive clinical variables. We conducted a single center, retrospective study of patients with single ventricle CHD and previous Glenn palliation who underwent pre-Fontan cardiac magnetic resonance (CMR) imaging from 3/2018 to 3/2021. CMR was used to quantify APC flow, which was normalized to aortic (APC/Q Ao ) and pulmonary vein (APC/Q PV ) blood flow. Univariate, multivariable, and classification and regression tree (CART) analyses were done to investigate the potential relationship between CMR-quantified APC burden and clinical variables. A total of 29 patients were included, all of whom had increased APC flow (APC/Q Ao : 26.9, [22.0, 39.1]%; APC/Q PV : 39.4 [33.3, 46.9]%), but to varying degrees (APC/Q Ao : range 11.9–44.4%; APC/Q PV : range 17.7–60.0%). Pulmonary artery size (Nakata index, at pre-Fontan CMR) was the only variable associated with APC flow on multivariable analysis (APC/Q Ao : p  = 0.020, R 2  = 0.19; APC/Q PV : p  = 0.0006, R 2  = 0.36) and was the most important variable associated with APC burden identified by CART analysis (size inversely related to APC flow). APC flow is universally increased but highly variable in patients with single ventricle CHD and Glenn circulation. Small branch pulmonary artery size is a key factor associated with increased APC burden; however, the pathogenesis of APCs is likely multifactorial. Further research is needed to better understand APC pathogenesis, including predisposing and mitigating factors.

Endogenous prostacyclin stimulates pulmonary vasodilation and inhibits platelet aggregation. For the synthetic analog treprostinil, used in the treatment of pulmonary hypertension (PH), conflicting, anecdotal evidence exists regarding its effects on clinically relevant platelet function. This study investigated whether treprostinil therapy results in inhibition of platelet aggregation in pediatric PH patients. This is a single institution, prospective, cohort study. Pediatric patients ≤18 years of age on medical therapy for PH underwent platelet function testing by light transmission aggregometry with U‐46619—a stable analog of endoperoxide prostaglandin H2, exhibiting properties similar to thromboxane A2 (TXA2). Results were compared for those on continuous treprostinil therapy (TRE) versus those on other, non‐prostacyclin therapies (non‐TRE). Thirty‐five patients were enrolled: 18 in the TRE group and 17 in the non‐TRE group. There was no difference in platelet aggregation abnormalities between the two groups: 44% (n = 8) in the TRE group and 41% (n = 7) in the non‐TRE group were abnormal. Furthermore, subgroup analysis showed no difference based on treprostinil dosing. This study demonstrated similar, moderately high rates of abnormal platelet aggregation in pediatric PH patients on continuous treprostinil therapy compared to those on other, non‐prostacyclin therapies. The high rate of abnormal platelet aggregation in the entire cohort, however, warrants follow‐up study to identify a potential inherent risk in this population.

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. 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. 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. 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.

Introduction and hypothesisWe hypothesize that there will be improvement in a novice learners’ confidence and skill level with sacrospinous ligament fixation (SSLF) following a pelvic model-based simulation.MethodsWe performed a single-blinded randomized controlled trial with obstetrics and gynecology residents who were novices at SSLF. The residents were randomly assigned to two groups. The control group received a lecture on the SSLF procedure and anatomy, whereas the intervention group received the same lecture in addition to a pelvic model-based simulation session taught by urogynecologists. The residents’ knowledge of SSLF anatomy and confidence level with the procedure were measured via assessments administered before and after the educational interventions. Their technical skills were objectively assessed by one of two fellowship-trained urogynecologists who were blinded to their group allocation.ResultsA total of 28 residents were recruited with 14 residents in each group and equal distribution of junior and senior trainees. None of the residents had previously performed the SSLF procedure. There was no difference in anatomical knowledge between the two groups. The intervention group showed a greater increase in their average confidence score compared with the control group: 4.0 ± 1.4 (95% CI 3.1–4.8) versus 2.6 ± 1.6 (95% CI 1.7–3.4) respectively, with p = 0.02. The intervention group also showed better objective scores in specific technical skills, such as instrument handling (p < 0.001), instrument movement/motion (p < 0.001), and speed (p = 0.01).ConclusionOur results demonstrate that inclusion of a pelvic model simulation significantly improves confidence and certain technical skills of novice trainees in performing SSLF.