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ImportanceSingle ventricle CHD affects about 5 out of 100,000 newborns, resulting in complex anatomy often requiring multiple, staged palliative surgeries. Paediatricians are an essential part of the team that cares for children with single ventricle CHD. These patients often encounter their paediatrician first when a complication arises, so it is critical to ensure the paediatrician is knowledgeable of these issues to provide optimal care.ObservationsWe reviewed the subtypes of single ventricle heart disease and the various palliative surgeries these patients undergo. We then searched the literature to detail the general paediatrician’s approach to single ventricle patients at different stages of surgical palliation.Conclusions and relevanceSingle ventricle patients undergo staged palliation that drastically changes physiology after each intervention. Coordinated care between their paediatrician and cardiologist is requisite to provide excellent care. This review highlights what to expect when these patients are seen by their paediatrician for either well child visits or additional visits for parental or patient concern.

Background: Understanding the clinical course and short-term outcomes of suspected myocarditis after the coronavirus disease 2019 (COVID-19) vaccination has important public health implications in the decision to vaccinate youth. Methods: We retrospectively collected data on patients Results: We report on 139 adolescents and young adults with 140 episodes of suspected myocarditis (49 confirmed, 91 probable) at 26 centers. Most patients were male (n=126, 90.6%) and White (n=92, 66.2%); 29 (20.9%) were Hispanic; and the median age was 15.8 years (range, 12.1–20.3; interquartile range [IQR], 14.5–17.0). Suspected myocarditis occurred in 136 patients (97.8%) after the mRNA vaccine, with 131 (94.2%) after the Pfizer-BioNTech vaccine; 128 (91.4%) occurred after the second dose. Symptoms started at a median of 2 days (range, 0–22; IQR, 1–3) after vaccination. The most common symptom was chest pain (99.3%). Patients were treated with nonsteroidal anti-inflammatory drugs (81.3%), intravenous immunoglobulin (21.6%), glucocorticoids (21.6%), colchicine (7.9%), or no anti-inflammatory therapies (8.6%). Twenty-six patients (18.7%) were in the intensive care unit, 2 were treated with inotropic/vasoactive support, and none required extracorporeal membrane oxygenation or died. Median hospital stay was 2 days (range, 0–10; IQR, 2–3). All patients had elevated troponin I (n=111, 8.12 ng/mL; IQR, 3.50–15.90) or T (n=28, 0.61 ng/mL; IQR, 0.25–1.30); 69.8% had abnormal ECGs and arrhythmias (7 with nonsustained ventricular tachycardia); and 18.7% had left ventricular ejection fraction Conclusions: Most cases of suspected COVID-19 vaccine myocarditis occurring in persons

Importance:Paediatricians play an integral role in the lifelong care of children with CHD, many of whom will undergo cardiac surgery. There is a paucity of literature for the paediatrician regarding the post-operative care of such patients.Observations:The aim of this manuscript is to summarise essential principles and pertinent lesion-specific context for the care of patients who have undergone surgery or intervention resulting in a biventricular circulation.Conclusions and relevance:Familiarity with common issues following cardiac surgery or intervention, as well as key details regarding specific lesions and surgeries, will aid the paediatrician in providing optimal care for these patients.

Late diagnosis of critical congenital heart disease (CCHD) is associated with higher levels of morbidity and mortality in neonates. Nearly all states have passed laws mandating universal pulse oximetry screening (POxS) of newborns to improve early detection rates of CCHD. We performed a retrospective chart review of all transthoracic echocardiograms (TTEs) interpreted by our institution on patients between 0 and 30 days of life in the years 2010 (prior to POxS) and 2015 (after POxS). Between 2010 and 2015, the number of neonatal TTEs interpreted by our institution decreased by 18.2%. In 2015, there were 46 neonates diagnosed with CCHD with a 78% prenatal detection rate. There was only one case of a true-positive POxS. Our study demonstrated that the initiation of POxS coincided with a significant decrease in neonatal TTEs, suggesting universal POxS may impart reassurance to primary providers leading to a decrease in TTE utilization.

We sought to identify associations between prenatal care coordination (PNC) and outcomes in hypoplastic left heart syndrome (HLHS).We hypothesized that suboptimal PNC is associated with worse pre-operative status. HLHS patients from 2016 through 2019 were identified using a multicenter registry. Optimal PNC was defined as (1) a completed interdisciplinary conference and (2) closed-loop communication with the obstetric team. Associations between PNC and outcomes were identified.Of 1441 patients, 1242 (86%) had prenatal diagnosis. Among those with a prenatal diagnosis, PNC was achieved in only 845 (68%). Suboptimal PNC was associated with adverse events (50% vs 40%, p 0.001), inotrope need (19% vs 13%, p = 0.007), mechanical ventilation (22% vs 16%, p = 0.016), and parenteral feeding (60% vs 46%, p 0.001). African-American race and non-commercial insurance were associated with a lower likelihood of optimal PNC (p = 0.006 and p 0.001, respectively).Improving PNC and overcoming racial and socioeconomic barriers are important targets to improve HLHS perinatal care.

Objective: Children with critical congenital heart disease (CHD) often present in remote areas with life-threatening illness. Although performing complete pediatric echocardiograms requires significant training, remote locations necessitate sonographers with limited pediatric experience screen for CHD. The Sonographer University (SU) was designed to improve comfort in adult sonographers scanning children and recognizing CHD. Methods: The SU consisted of lectures, multimedia case studies, roundtable discussions, pathology stations, and hands-on scanning of 20 volunteer pediatric patients with either normal or diseased hearts. Topics included an overview of critical congenital heart lesions, crucial views for each defect, and pitfalls in imaging children. Results: Forty-four participants completed the SU seminar. In pretest assessment, approximately two-thirds said they were currently required to scan children. Participants’ comfort level sonographically scanning children and recognizing CHD improved substantially. A posttest showed that the course improved participants’ skill, knowledge, and confidence with pediatric studies. Conclusion: The SU seminar helped sonographers from remote areas to develop improved recognition of CHD, confidence in pediatric scanning, and methods for obtaining assistance with imaging a critically ill child.

Due to the COVID-19 pandemic, there has been an increased interest in telehealth as a means of providing care for children by a pediatric cardiologist. In this article, we provide an overview of telehealth utilization as an extension of current pediatric cardiology practices and provide some insight into the rapid shift made to quickly implement these telehealth services into our everyday practices due to COVID-19 personal distancing requirements. Our panel will review helpful tips into the selection of appropriate patient populations and specific cardiac diagnoses for telehealth that put patient and family safety concerns first. Numerous practical considerations in conducting a telehealth visit must be taken into account to ensure optimal use of this technology. The use of adapted staffing and billing models and expanded means of remote monitoring will aid in the incorporation of telehealth into more widespread pediatric cardiology practice. Future directions to sustain this platform include the refinement of telehealth care strategies, defining best practices, including telehealth in the fellowship curriculum and continuing advocacy for technology.

OBJECTIVES Critical pulmonary stenosis (PS) and pulmonary atresia with intact ventricular septum (PAIVS) require urgent neonatal intervention. Since PS may be more insidious than PAIVS during gestation, we hypothesized that neonates with PS would have lower rates of prenatal detection than PAIVS. METHODS We performed a retrospective chart review of all neonates who underwent diagnostic or interventional cardiac catheterization between 2000 and 2014 for critical PS or PAIVS. The rates of prenatal diagnosis were calculated for PS and PAIVS. Prenatal and postnatal echocardiographic data were reviewed. RESULTS 178 patients met inclusion criteria: 91 with critical PS and 87 with PAIVS. The prenatal diagnosis rate for critical PS was lower than for PAIVS at 37% (34/91) vs 60% (52/87) (P = .003). At the time of diagnosis at a median gestational age of 25 weeks, the median TV z-score for patients with critical PS was larger than in PAIVS (-0.15 vs -3.0 P = .004). CONCLUSION Critical PS had a lower prenatal detection rate than PAIVS, likely due to a relatively normal 4-chamber view at the time of routine second trimester screening in patients with PS. Color flow Doppler of the outflow tracts may improve detection, since outflow tracts may appear normal by 2D imaging.

Objective:Hypoplastic left heart syndrome is a single ventricle defect. While staged surgical palliative treatments have revolutionised care, patients with hypoplastic left heart syndrome continue to have significant morbidity and mortality. In 2017, the National Pediatric Cardiology Quality Improvement Collaborative recommended all single ventricle patients to receive a prenatal palliative care consult. This study aimed to elucidate provider perspectives on the implementation of prenatal palliative care consults for families expecting a child with hypoplastic left heart syndrome.Methods:An online survey was administered to obstetric and paediatric providers of relevant disciplines to assess their experience with palliative care involvement in hypoplastic left heart syndrome cases.Results:Nearly, all physicians (97%) and most registered nurses (79%) agreed that the initial palliative care consult for patients with hypoplastic left heart syndrome should occur during the prenatal period. Respondents also indicated that prenatal palliative care consults should also be offered in a variety of other CHD conditions. Participants believed positive aspects of this new referral protocol included an expanded support network for families, decreased family stress during the postnatal period, increased patient education about what to expect during the postnatal period, and continuity of care.Conclusion:Multidisciplinary healthcare professionals believe that prenatal palliative care consults provide a variety of benefits for patients and families with hypoplastic left heart syndrome. Additional, multi-centre research is necessary to evaluate whether prenatal palliative care consults should become standard of care for families expecting a child with a single ventricle defect.

Introduction: Congenitally corrected transposition of the great arteries (ccTGA) is rare with varied associated cardiac defects and rhythm abnormalities. We aimed to describe the natural history, associated anomalies and prenatal outcome in a cohort of prenatally diagnosed patients in which biventricular repair is anticipated. Methods: A retrospective cohort study was conducted via the Fetal Heart Society Research Collaborative. All fetuses with ccTGA encountered at 15 North American cardiac centers between 1/2004-7/2020 were identified. Fetuses with a hypoplastic ventricle precluding biventricular repair were excluded. Data is presented as median (interquartile range). Results: Inclusion criteria were met in 139 fetuses who were diagnosed with ccTGA at 24 (21-29) weeks. There was a family history of congenital heart disease in 12%. Maternal diabetes was present in 10%. Prenatal genetic testing in 50 pregnancies was normal. Excluding 14 fetuses with heterotaxy, extracardiac anomalies were observed in 9/125 (7.1%). Associated cardiac/extracardiac defects are detailed in table 1. Fetal atrioventricular block (AVB) was present in 17 fetuses (12%), diagnosed at a median of 26 (23-28), range 20-36 weeks. Two fetuses had SVT; 1 self-resolved and 1 during labor. Change occurred during follow-up in 34 fetuses, most commonly in severity of tricuspid regurgitation (6 improved, 4 worsened) and worsening of pulmonary stenosis (n=5). There were 112 live births, 17 terminations, 2 fetal deaths (FD) and 8 lost to follow-up. Both fetuses with FD had AVB, making the intrauterine mortality associated with fetal AVB 11.8% (2/17). Conclusions: This study represents the largest cohort to date of fetuses with ccTGA. Fetal ccTGA is associated with a spectrum of cardiac defects that may evolve, with extracardiac pathology occurring in 7.1%. AVB is found in 12% of fetuses and is a risk factor for FD. This data informs fetal counseling and can aid in delivery planning and perinatal care.

Introduction: Comprehensive prenatal care of congenital heart disease (CHD) relies on a multi-disciplinary approach that includes timely prenatal counseling and individualized care plans for the pregnancy, delivery and neonatal period. With the COVID-19 pandemic our fetal therapy clinic (FTC) approach to patient care was altered to accommodate restrictions, including the use of telehealth visits. We investigated whether counseling and access to care for fetal patients with CHD was negatively impacted by COVID-19. Hypothesis: Fetuses with CHD would experience a delay in timing of diagnosis and referral; parents would receive a shorter counseling session during the COVID-19 pandemic. Methods: Retrospective chart review of all fetal cardiology patients seen in our multidisciplinary FTC both before (5/1/19-12/31-19) and during the COVID-19 pandemic (5/1/20-12/31/20). Data collected included gestational age (GA) at referral, GA at first fetal echo, use of telehealth, total time counseled, number of sub-specialty consults, and total number of fetal cardiology visits. Results: A comparable number of dyads were seen before and during the pandemic (Table 1). GA at initial diagnosis was similar, but GA at referral and first visit was earlier during the pandemic. Additionally, families seen during the pandemic had longer counselling across all disciplines with significant differences in pediatric cardiology and neonatology. There was no difference in total number of consults or access to care based on insurance. Conclusions: While prenatal counseling and referral to FTC is multifaceted, we demonstrated that COVID-19 and alterations in care delivery did not in fact lead to delays in referrals for patients across a large geographical catchment. Moreover, with telemedicine we were able to ensure our families continued to receive multidisciplinary care and families were able to spend more time with our care team developing comprehensive individualized plans.

BACKGROUND Early detection of right ventricular dysfunction after transannular patch for tetralogy of Fallot (TOF-TAP) is essential for management. OBJECTIVES To evaluate echocardiographic metrics of ventricular function correlate with functional MRI measurements, in patients with TOF-TAP. METHODS A retrospective review of patients with TOF-TAP between 2007 and 2017 who had an echocardiogram and MRI within six months were analyzed. Systolic to diastolic ratio (SD ratio) was measured from the tricuspid regurgitation and adjusted for heart rate. Tricuspid Annular Plane Systolic Excursion (TAPSE), Fractional Area Change (FAC), and shortening fraction (SF) were additionally measured. Echocardiographic measurements were correlated with MRI assessment of right ventricular ejection fraction (RVEF), left ventricular ejection fraction (LVEF), right ventricular end-diastolic volume index (RVEDi), and right to left ventricle volume ratio (RV/LV). RESULTS Of the 53 patients (mean age 12.8 years) that met inclusion criteria, 45 (85%) had available TR jets for SD ratio analysis. The HR adjusted SD ratio negatively correlated with RVEF (r = -.359, P = .016), LVEF (r = -.317, P = .038) and positively with RV/LV EDV ratio (r = .347, P = .024). TAPSE, FAC, and SF measurements did not show significant correlation. CONCLUSION In patients with TOF-TAP, there is a moderate negative correlation between heart rate adjusted SD ratio and MRI metrics of ventricular function, suggesting that decreased filling time is a marker for reduced right ventricular function. The SD ratio may be a useful echocardiographic tool for serial evaluation of in this population.

Transient left ventricular (LV) dysfunction occurs in some infants born with critical pulmonary stenosis (PS) or membranous pulmonary atresia with intact ventricular septum (PAIVS) after pulmonary valve (PV) balloon dilation (BD). The cause for this is not well understood. We sought to characterize this LV dysfunction by investigating regional differences in this cohort using myocardial strain imaging. Patients who underwent neonatal (2 weeks age) PV BD for critical PS or PAIVS from Jan, 2007 to March, 2014 with echocardiographic images suitable for strain analysis were identified; infants with ≥moderate post-BD LV dysfunction (ejection fraction40%, n = 8) were matched 1:1 with controls who underwent PV BD but did not develop LV dysfunction. Longitudinal and circumferential global and segmental strain were analyzed before and after PV BD. For the 8 LV dysfunction cases, LV global longitudinal strain worsened after PV BD (-16% pre- vs -8% post-PV BD, p = 0.008) with similar impairment in global LV circumferential strain (-17% vs -8%, p = 0.008); there was no significant change in RV global or segmental longitudinal strain pre- vs post-PVBD. No significant pre/post-BD differences in global or circumferential strain were found in control pts. Segmental analysis of longitudinal and circumferential LV strain before and after balloon dilation in cases demonstrated decreased strain in all segments, but more pronounced and statistically significant in septal segments as compared with the free wall. In conclusion, transient LV dysfunction post-PV BD for critical PS/PAIVS is characterized by impaired global longitudinal and circumferential LV strain, with the most significant reductions in strain at the interventricular septum; longitudinal RV strain remains unchanged. These findings suggest that the mechanism of LV dysfunction post-PV BD is adverse ventricular-ventricular interactions specifically involving the interventricular septum.

To assess the accuracy of maternal understanding of fetal cardiac defects following initial fetal counseling.Pregnant women with a fetal diagnosis of congenital heart disease (CHD) were surveyed regarding understanding of their fetus's heart defect. The survey asked: (1) for a description of the heart condition; (2) how confident they were in the diagnosis; (3) whether their fetus would require heart surgery. Two fetal cardiologists evaluated the maternal qualitative description. Partners were excluded from the study.Fifty-one participants consented and 39 completed the survey. Mean age was 31 years, 60% had some college level or post-graduate education, 48% had Medicaid insurance, and 81% were Caucasian. More than three-quarters of participants, stated they had either "quite a bit" or "very much" understanding of their fetus's diagnosis. Maternal assessment matched the physician's assessment of accuracy with 77% (Fetal counseling is effective in conveying anatomy and the need for surgery; however, accuracy amongst women with lower levels of education and maternal confidence in understanding can be improved.

Background: Prenatal diagnosis of congenital heart disease (CHD) allows for complex decisions about care to be made in a timely manner and allows parents the opportunity to fully participate in those decisions. Counseling provides information to families regarding diagnosis, prognosis, management and treatment options, however, dynamics may vary from family to family. This study sought to investigate if the characteristics of fetal counseling …

Background: The current literature on neurodevelopment (ND) in pediatric cardiology patients has largely revolved around specific ND outcomes. Children with congenital heart defects (CHD) have a higher risk for acquiring developmental disabilities due to many factors including prenatal cerebral perfusion, cyanosis, and exposure to cardiopulmonary bypass. Routine developmental evaluations throughout childhood can increase the chances of identifying these deficits, allowing early intervention. We sought to …

Introduction: Timely prenatal diagnosis of congenital heart disease (CHD) allows for families to participate in complex decisions and plan for the care of their child. This study sought to investigate if the timing of the first fetal echocardiogram (FE) and the characteristics of fetal counseling were impacted by parental socioeconomic factors. Hypothesis: We hypothesized that the scheduling of initial FE would be delayed due to distance from hospital, rurality of maternal home and median income. Methods: We performed a retrospective chart review of all fetal patients referred to our institution from 1/1/17 to 12/31/18 with a diagnosis of CHD. We looked at the gestational age at the first FE, age of mother, zip code of residence, rurality index, distance from our hospital and maternal ethnicity. Counseling was evaluated based on documentation in the FE report regarding use of interpreter, time billed for counseling, individuals accompanying mother, and treatment option chosen. Results: 138 maternal-fetal dyads met inclusion criteria, 29 dyads had a diagnosis of single ventricle heart disease. The median gestational age (GA) at first FE was 24 weeks 4 days. The median income was $57,019 ($42,624-$83,695), and the median distance to the hospital was 51 miles (3.2-379.9miles). There was no difference in income, distance from hospital or rurality index and timing of first FE. There was no significant difference between maternal ethnicity and age of mother, GA at initial FE, number of follow-up FEs or family accompanying mother to the visit. There was no difference in maternal ethnicity and use of interpreter with time counseled. Patients who lived in rural areas did have increased counseling time (p Conclusions: Oregon comprises a heterogeneous population from a large geographical catchment. While prenatal counseling and family decision making is multifaceted we demonstrated that dyads were referred from across the state and received care in a uniformly timely manner, and once at our center received consistent counseling despite differences in parental socioeconomic factors.

Introduction: Prenatal diagnosis (PD) of congenital heart disease permits coordination of care to optimize postnatal outcomes. Using phase II registry data from the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC), we sought to identify associations between prenatal care coordination (PNC) and pre-operative status among infants with HLHS and variants intended for Stage I Norwood or hybrid palliation. Hypothesis: We hypothesized that suboptimal PNC would be associated with worse pre-operative status. Methods: The NPC-QIC database was queried for all instances of prenatally diagnosed HLHS and variants intended for Stage 1 Norwood or hybrid palliation from 2016 through 2019. Demographic and clinical data pertaining to the prenatal period through the first birthday were obtained. Optimal PNC was defined as (1) a completed interdisciplinary case conference prior to delivery and (2) a note in the medical record documenting closed-loop communication between the OB and cardiology teams. Associations between PNC and outcomes were identified. Results: Among 1441 registry patients with HLHS and variants, 1242 (86%) had PD. Among patients with PD, optimal PNC was achieved in 68%. Preoperatively, infants without optimal PNC were more likely to have any adverse event (50% vs 40%, p Conclusions: Among prenatally diagnosed infants with HLHS and variants, optimal PNC was associated with fewer preoperative risk factors. There also appear to be disparities in PD related to SES and in PNC related to race. Additional study is required to understand the complex interplay between PNC and clinical outcomes, as well as identify and address barriers to PD and optimal PNC.

Background/Objective: Historically, Trisomy 13 (T13) and Trisomy 18 (T18) have been considered “lethal” diagnoses with a limited life expectancy. Due to extensive comorbidities and perception of low quality of life (QOL), most T13 and T18 neonates have not been offered (or families have not pursued) neonatal intensive care or surgical interventions. With the observation of longer term survival in some T13 and T18 infants, in addition to changing societal attitudes toward people with disabilities, there is advocacy …

Background Children with congenital heart defects have a higher risk of acquiring developmental disabilities. Routine developmental evaluations throughout childhood increase the chances of identifying these deficits, prompting referral to Early Intervention services. Objectives We hypothesized that referral rates to Early Intervention and/or neurodevelopmental evaluation were low despite national guidelines. We also sought to investigate the relationship between type of congenital heart disease and results of neurodevelopmental evaluation. Methods A retrospective chart review was performed for congenital heart disease patients requiring cardiac surgery within their first year of life. Variables assessed included type of congenital heart disease, prenatal diagnosis, number of surgeries in the first year of life, and neurodevelopmental referral, evaluation, and quantitative assessment. Results 151 patients met inclusion criteria from 1/1/13 through 12/31/18. 64 (42%) were referred to neurodevelopmental services, of which 60 patients accessed services. Single ventricle patients had an increased rate of neurodevelopmental referral (88% vs 31, p Conclusions Only 42% of congenital heart disease patients were referred to neurodevelopmental services, despite known risks. Most congenital heart disease patients had low to average neurodevelopment scores. Excluding single ventricle patients, there was no difference between congenital heart disease type and neurodevelopmental outcomes. Single ventricle patients scored particularly low in cognition and gross motor. Ultimately, 67% of patients who underwent initial evaluation received additional Early Intervention services, demonstrating the significant importance of initial neurodevelopmental evaluation.

The objective of this study was to evaluate the utility of transthoracic echocardiography (TTE) in children with structurally normal hearts suspected of having infective endocarditis (IE). We hypothesized that the diagnostic yield of TTE is minimal in low-risk patients with normal hearts. We performed a retrospective chart review of TTEs performed for concern for endocarditis at a pediatric tertiary care referral center in Portland, Oregon. Three hundred patients met inclusion criteria (

OBJECTIVES Children with tetralogy of Fallot (TOF) require initial intervention at varying times reflecting a spectrum of obstruction to pulmonary blood flow. In utero, fetal patients with right sided obstructive lesions demonstrate increased middle cerebral artery (MCA) pulsatility index (PI). We sought to determine whether MCA flow patterns are associated with the degree of pulmonary obstruction and the consequential need for early surgical intervention (ESI). METHODS We performed a multi-center retrospective study evaluating patients with a prenatal diagnosis of TOF. All echocardiographic fetal studies performed from 16 weeks' gestational age through delivery were reviewed between 2001 and 2015 with subsequent postnatal medical chart review. RESULTS 82 patients met inclusion criteria. Patients who ultimately required ESI (n=30) demonstrated an increased MCA PI z-score (-0.68 vs -1.32, p=0.02) compared to those who did not (n=52). An elevated MCA PI in patients with TOF was associated with ESI after 24 weeks' gestational age (-0.36 vs -1.22, p=0.02). CONCLUSIONS Fetal patients with TOF demonstrate altered MCA flow that correlates with need for ESI. This effect is blunted earlier in gestation which may reflect cerebral autoregulation. MCA PI may be a beneficial tool in helping determine perinatal management and counseling for patients with TOF.

Background: Congenital heart disease (CHD) patients have a higher rate of developmental disorders and cognitive delay. Routine developmental evaluations throughout childhood can increase the chances of identifying these deficits. We sought to evaluate referral rates and neurodevelopmental (ND) results for specific cardiac lesions after CHD surgery. Design/Methods: A retrospective review was performed on CHD patients requiring cardiac surgery within the first year of life at Doernbecher Children’s Hospital from 1/1/13-12/31/18. A surgical database collected patients’ variables, …

IntroductionNew paediatric cardiology trainees are required to rapidly assimilate knowledge and gain clinical skills to which they have limited or no exposure during residency. The Pediatric Cardiology Fellowship Boot Camp (PCBC) at Boston Children’s Hospital was designed to provide incoming fellows with an intensive exposure to congenital cardiac pathology and a broad overview of major areas of paediatric cardiology practice.MethodsThe PCBC curriculum was designed by core faculty in cardiac pathology, echocardiography, electrophysiology, interventional cardiology, exercise physiology, and cardiac intensive care. Individual faculty contributed learning objectives, which were refined by fellowship directors and used to build a programme of didactics, hands-on/simulation-based activities, and self-guided learning opportunities.ResultsA total of 16 incoming fellows participated in the 4-week boot camp, with no concurrent clinical responsibilities, over 2 years. On the basis of pre- and post-PCBC surveys, 80% of trainees strongly agreed that they felt more prepared for clinical responsibilities, and a similar percentage felt that PCBC should be offered to future incoming fellows. Fellows showed significant increase in their confidence in all specific knowledge and skills related to the learning objectives. Fellows rated hands-on learning experiences and simulation-based exercises most highly.ConclusionsWe describe a novel 4-week-long boot camp designed to expose incoming paediatric cardiology fellows to the broad spectrum of knowledge and skills required for the practice of paediatric cardiology. The experience increased trainee confidence and sense of preparedness to begin fellowship-related responsibilities. Given that highly interactive activities were rated most highly, boot camps in paediatric cardiology should strongly emphasise these elements.

BackgroundIn 2003, work-hour regulations were implemented by the Accreditation Council for Graduate Medical Education. Much has been published regarding resident rest and quality of life as well as patient safety. There has been no examination on the effect of work-hour restrictions on academic productivity of fellows in training. Paediatric subspecialty fellows have a scholarly requirement mandated by the American Board of Pediatrics. We have examined the impact of work-hour restrictions on the scholarly productivity of paediatric cardiology fellows during their fellowship.MethodsWe conducted a literature search for all paediatric cardiology fellows between 1998 and 2007 at a single academic institution as first or senior authors on papers published during their 3-year fellowship and 3 years after completion of their categorical fellowship (n=63, 30 fellows before 2003 and 33 fellows after 2003). The numbers of first- or senior-author fellow publications before and after 2003 were compared. We also collected data on final paediatric cardiology subspecialty career choice.ResultsThere was no difference in the number of fellow first-author publications before and after 2003. Before work-hour restrictions, the mean number of publications per fellow was 2.1 (±2.2), and after work-hour restrictions it was 2.0 (±1.8), (p=0.89). By subspecialty career choice, fellows who select electrophysiology, preventative cardiology, and heart failure always published within the 6-year time period.ConclusionsSince the implementation of work-hour regulations, total number of fellow first-authored publications has not changed. The role of subspecialty choice may play a role in academic productivity of fellows in training.

Pulmonary valve (PV) balloon dilation (BD) is the primary therapy for infants born with critical pulmonary stenosis (PS) or membranous pulmonary atresia with intact ventricular septum (PAIVS). We observed left ventricular (LV) dysfunction in patients following BD and sought to determine its incidence, clinical course and associated risk factors. Clinical, echocardiographic and catheterization data for all patients who underwent neonatal (

Background: Coronary artery (CA) z-scores are commonly used for clinical decisions in Kawasaki disease (KD). We evaluated reliability in CA measurement, reproducibility of z-score calculation, and frequency with which different z-score formulas lead to divergent management strategies. Methods: We randomly selected 21 KD patients (pts) with ≥1 CA z-score 1.5-3 and all KD pts with ≥ 1 CA z-score 7-14 (n=20). Two echocardiographers measured LMCA, LAD and RCA. Inter- and intraobserver reliability were calculated. T-tests were used to compare CA z-score using 3 commonly used formulas (Boston, DC and Montreal). Results: Median age at KD echo was 1.2 y (0.2-11.5 y). Interobserver reliability was high for LAD (intraclass correlation [ICC] 0.970) and RCA (ICC 0.943) and lower for LMCA (ICC 0.725). Intraobserver reliability was also high for LAD and RCA (ICC 0.991 and 0.999) and lower for LMCA (ICC 0.946). Z-scores for the 3 formulas were similar at smaller CA size, i.e., z < 3, but varied markedly at larger CA dimensions (Figure). Z-scores for the same CA dimension calculated by each of the 3 formulas resulted in disparate classification of normal vs. mild dilation in 7/21 (22%) pts, and different guidance for anticoagulation based on CA z ≥10 in 10/20 (50%) pts. Conclusion: Although CA measurements have high inter- and intraobserver agreement, CA z-scores vary dramatically based on the z-score formula, particularly at larger CA dimensions. Discrepancies in CA z-score between calculators impacts not only the distinction between normal and mild dilatation, but most importantly, the recommendation of anticoagulation for pts with larger CA dimensions.

Concerns about medications influence breastfeeding decisions. Mothers may stop breastfeeding when they take medications. After the distribution of Thomas Hale's Medications and Mother's Milk (MMM) (Hale Publishing, Amarillo, TX, 2006) by the Rhode Island Department of Health (Providence, RI) in November 2006 to Rhode Island pharmacies, we investigated, during the summer of 2007, what strategies and resources pharmacists were using to identify breastfeeding women and guide medication recommendations.Copies of MMM were sent to 47 pharmacies in Rhode Island. Subsequently, one pharmacist at each site completed a confidential, 10-question written survey.The survey response rate was 92%. More than half (58%) of the pharmacists surveyed never asked women if they were breastfeeding. Most (85%) of pharmacists reported feeling somewhat or very comfortable giving advice to breastfeeding women. All but one pharmacist who had received MMM reported using the reference at least monthly. More than half (52%) of the pharmacists reported using the Physician's Desk Reference (Thomson PDR, Montvale, NJ).Pharmacists need a consistent approach to identify breastfeeding women and access to reliable, continuously updated resources to guide their advice about medication use to breastfeeding women. Physicians and pharmacists should collaborate to prevent medication use from being a barrier to breastfeeding.