Impact of burnout and professional fulfillment on intent to leave among pediatric physicians: The findings of a quality improvement initiative.

BACKGROUND AND OBJECTIVES: Physician burnout is rampant, and physician retention is increasingly hard. It is unclear how burnout impacts intent to leave an organization. We sought to determine how physician burnout and professional fulfillment impact pediatric physicians' intent to leave (ITL) an organization. DESIGN AND METHODS: We performed 120, 1:1 semi-structured interviews of our pediatric faculty and used the themes therefrom to develop a Likert-scale based, 22-question battery of their current work experience. We created a faculty climate survey by combining those questions with a standardized instrument that assesses burnout and professional fulfillment. We surveyed pediatric and pediatric-affiliated (e.g. pediatric surgery, pediatric psychiatry, etc.) physicians between November 2 and December 9, 2022. We used standard statistical methods to analyze the data. An alpha-level of 0.05 was used to determine significance. RESULTS: A total of 142 respondents completed the survey, 129 (91%) were Department of Pediatrics faculty. Burnout was present in 41% (58/142) of respondents, whereas 30% (42/142) were professionally fulfilled. There was an inverse relationship between professional fulfillment and ITL, p < 0.001 for the trend. Among those who were not professionally fulfilled, the odds ratio of ITL in the next three years was 3.826 [95% CI 1.575-9.291], p = 0.003. There was a direct relationship between burnout and ITL, p < 0.001 for the trend. CONCLUSIONS: Among pediatric physicians, professional fulfillment is strongly, inversely related with ITL in the next three years. Similarly, burnout is directly related with ITL. These data suggest a lack of professional fulfillment and high burnout are strong predictors of pediatric physician turnover.

Intensive Care Unit Analgosedation After Cardiac Surgery in Children with Williams Syndrome : a Matched Case-Control Study.

OBJECTIVE: Cardiovascular abnormalities are common in patients with Williams syndrome and frequently require surgical intervention necessitating analgesia and sedation in a population with a unique neuropsychiatric profile, potentially increasing the risk of adverse cardiac events during the perioperative period. Despite this risk, the overall postoperative analgosedative requirements in patients with WS in the cardiac intensive care unit have not yet been investigated. Our primary aim was to examine the analgosedative requirement in patients with WS after cardiac surgery compared to a control group. Our secondary aim was to compare the frequency of major ACE and mortality between the two groups. DESIGN: Matched case-control study. SETTING: Pediatric CICU at a Tertiary Children's Hospital. PATIENTS: Patients with WS and age-matched controls who underwent cardiac surgery and were admitted to the CICU after cardiac surgery between July 2014 and January 2021. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Postoperative outcomes and total doses of analgosedative medications were collected in the first six days after surgery for the study groups. Median age was 29.8 (12.4-70.8) months for WS and 23.5 (11.2-42.3) months for controls. Across all study intervals (48 h and first 6 postoperative days), there were no differences between groups in total doses of morphine equivalents (5.0 mg/kg vs 5.6 mg/kg, p = 0.7 and 8.2 mg/kg vs 10.0 mg/kg, p = 0.7), midazolam equivalents (1.8 mg/kg vs 1.5 mg/kg, p = 0.4 and 3.4 mg/kg vs 3.8 mg/kg, p = 0.4), or dexmedetomidine (20.5 mcg/kg vs 24.4 mcg/kg, p = 0.5 and 42.3 mcg/kg vs 39.1 mcg/kg, p = 0.3). There was no difference in frequency of major ACE or mortality. CONCLUSIONS: Patients with WS received similar analgosedative medication doses compared with controls. There was no significant difference in the frequency of major ACE (including cardiac arrest, extracorporeal membrane oxygenation, and surgical re-intervention) or mortality between the two groups, though these findings must be interpreted with caution. Further investigation is necessary to elucidate the adequacy of pain/sedation control, factors that might affect analgosedative needs in this unique population, and the impact on clinical outcomes.

Surgical repair of peripheral pulmonary artery stenosis: A 2-decade experience with 145 patients.

BACKGROUND: Peripheral pulmonary artery stenosis (PPAS) is a relatively rare form of congenital heart disease often associated with Williams syndrome, Alagille syndrome, and elastin arteriopathy. This disease is characterized by stenoses at nearly all lobar and segmental ostia and results in systemic-level right ventricular pressures. The current study summarizes our experience with the surgical treatment of PPAS. METHODS: This was a retrospective review of 145 patients who underwent surgical repair of PPAS. This included 43 patients with Williams syndrome, 39 with Alagille syndrome, and 21 with elastin arteriopathy. Other diagnoses include tetralogy of Fallot with PPAS (n = 21), truncus arteriosus (n = 5), transposition (n = 3), double-outlet right ventricle (n = 2), arterial tortuosity syndrome (n = 3), and other (n = 8). RESULTS: The median preoperative right ventricle to aortic peak systolic pressure ratio was 1.01 (range, 0.50-1.60) which was reduced to 0.30 (range, 0.17-0.60) postoperatively. The median number of ostial repairs was 17 (range, 6-34) and median duration of cardiopulmonary bypass was 398 minutes (range, 92-844). There were 3 in-hospital deaths (2.1%). The median duration of follow-up was 26 months (range, 1-220) with 4 late deaths (2.9%). Eighty-two patients have subsequently undergone catheterization and 74 had a pressure ratio <0.50. CONCLUSIONS: The surgical treatment of PPAS resulted in a 70% reduction in right ventricular pressures. At 3 years, freedom from death was 94% and 90% of those evaluated maintained low pressures. These results suggest that the surgical treatment of PPAS is highly effective in most patients.

Impact of liver dysfunction on outcomes in children with Alagille syndrome undergoing congenital heart surgery.

OBJECTIVES: Children with Alagille syndrome often have complex forms of congenital heart defects with the majority having peripheral pulmonary artery stenosis (PPAS) and pulmonary valve atresia (PA) or pulmonary valve stenosis. Children with Alagille syndrome also have variable amounts of liver dysfunction. The purpose of this study was to evaluate the impact of liver dysfunction on outcomes in children with Alagille syndrome undergoing congenital heart surgery. METHODS: This was a retrospective review of 69 patients with Alagille syndrome who underwent congenital heart surgery at our institution. The underlying diagnoses included PPAS (n = 29), tetralogy of Fallot with PPAS (n = 14), tetralogy with PA (n = 3), PA with ventricular septal defect and major aortopulmonary collateral arteries (n = 21) and one each with D-transposition and supravalvar aortic stenosis. RESULTS: The median age at surgery was 16 months (range 0-228 months). Procedures performed included PPAS repair (n = 43), tetralogy with PA repair (n = 3), unifocalization procedures (n = 21) and other (n = 2). Forty-two (61%) patients had mild or no liver dysfunction, while 26 (38%) had moderate or severe liver dysfunction. The median cardiopulmonary bypass time was 345 min (341 with liver dysfunction, 345 without liver dysfunction). There were a total of 8 operative (12%) deaths and 3 late (4%) deaths. Six operative and 2 late deaths occurred in patients with liver dysfunction (combined 30.7%) versus 2 operative and 1 late death (combined 7.1%) for patients without liver dysfunction (P < 0.05). CONCLUSIONS: These results suggest that liver dysfunction has a profound impact on survival in children with Alagille syndrome undergoing congenital heart surgery.

Differential newborn DNA methylation among individuals with complex congenital heart defects and childhood lymphoma.

BACKGROUND: There is emerging evidence that children with complex congenital heart defects (CHDs) are at increased risk for childhood lymphoma, but the mechanisms underlying this association are unclear. Thus, we sought to evaluate the role of DNA methylation patterns on "CHD-lymphoma" associations. METHODS: From >3 million live births (1988-2004) in California registry linkages, we obtained newborn dried bloodspots from eight children with CHD-lymphoma through the California BioBank. We performed case-control epigenome-wide association analyses (EWAS) using two comparison groups with reciprocal discovery and validation to identify differential methylation associated with CHD-lymphoma. RESULTS: After correction for multiple testing at the discovery and validation stages, individuals with CHD-lymphoma had differential newborn methylation at six sites relative to two comparison groups. Our top finding was significant in both EWAS and indicates PPFIA1 cg25574765 was hypomethylated among individuals with CHD-lymphoma (mean beta = 0.04) relative to both unaffected individuals (mean beta = 0.93, p = 1.5 × 10-12 ) and individuals with complex CHD (mean beta = 0.95, p = 3.8 × 10-8 ). PPFIA1 encodes a ubiquitously expressed liprin protein in one of the most commonly amplified regions in many cancers (11q13). Further, cg25574765 is a proposed marker of pre-eclampsia, a maternal CHD risk factor that has not been fully evaluated for lymphoma risk in offspring, and the tumor microenvironment that may drive immune cell malignancies. CONCLUSIONS: We identified associations between molecular changes present in the genome at birth and risk of childhood lymphoma among those with CHD. Our findings also highlight novel perinatal exposures that may underlie methylation changes in CHD predisposing to lymphoma.

The number of postoperative surgical or diagnostic procedures following congenital heart surgery correlates with both mortality and hospital length of stay.

BACKGROUND: Outcomes for congenital heart disease have dramatically improved over the past several decades. However, there are patients who encounter intraoperative or postoperative complications and ultimately do not survive. It was our hypothesis that the number of postoperative procedures (including surgical and unplanned diagnostic procedures) would correlate with hospital length of stay and operative mortality. METHODS: This was a retrospective review of 938 consecutive patients undergoing congenital heart surgery at a single institution over a 2-year timeframe. The number of postoperative surgical and unplanned diagnostic procedures were counted and the impact on hospital length of stay and mortality was assessed. RESULTS: 581 of the 938 (62%) patients had zero postoperative diagnostic or surgical procedures. These patients had a median length of stay of 6 days with a single operative mortality (0.2%). 357 of the 938 (38%) patients had one or more postoperative diagnostic or surgical procedures. These patients had a total of 1586 postoperative procedures. There was a significant correlation between the number of postoperative procedures and both hospital length of stay and mortality (p < .001). Patients who required 10 or more postoperative procedures had a median hospital length of stay of 89 days and had a 50% mortality. There were no survivors in patients who had 15 or more postoperative procedures. CONCLUSIONS: The data demonstrate that the number of postoperative procedures was highly correlated with both hospital length of stay and mortality.

Prevalence and Outcomes of Primary Left Ventricular Dysfunction in Marfan Syndrome.

Even in the absence of significant valvular disease, patients with Marfan syndrome (MFS) have evidence of impaired left ventricular (LV) performance, suggestive of a primary cardiomyopathy. However, the true prevalence and long-term outcomes of this disease process remain largely unknown. We performed a retrospective analysis of all adult patients with confirmed MFS followed at Stanford Health Care. Those with significant valvular regurgitation, coronary artery disease, or previous cardiac surgery were excluded. LV systolic dysfunction was defined as a LV ejection fraction (LVEF) <55% on transthoracic echocardiography. A total of 753 patients with confirmed MFS were followed up over a median duration of 8 years (interquartile range 4 to 13). Of those, 241 patients (53% women, 71% White) met inclusion criteria and comprised the study cohort. LV systolic dysfunction was present in 30 patients (12%), with a median age of onset of 25 years (interquartile range 19 to 37), median EF of 52% (interquartile range 48 to 54), and evidence of clinical heart failure (New York Heart Association functional class ≥II) in 10% of patients. LV systolic dysfunction was more common in patients with larger aortic root diameters (≥4.0 cm: Odds ratio = 4.5, 95% confidence interval = 1.2 to 17.1) but was not associated with other cardiovascular manifestations of MFS or traditional atherosclerotic risk factors. In conclusion, apart from significant valvular pathology, LV systolic dysfunction was prevalent in MFS from a young age, suggestive of a primary cardiomyopathy. LV dysfunction was typically mild and subclinical and occurred more commonly in patients with more pronounced aortopathies.

Surgical Repair of Supravalvar Aortic Stenosis in Association With Transverse and Proximal Descending Aortic Abnormalities.

BACKGROUND: Supravalvar aortic stenosis (SVAS) may be an isolated defect of the proximal ascending aorta. However, more severe cases have extension of the arteriopathy into the transverse and proximal descending aorta. The purpose of this study was to review our experience with SVAS with and without aortic arch arteriopathy. METHODS: This was a retrospective review of 58 patients who underwent surgical repair of SVAS. The median age at repair was 18 months. A total of 37 patients had Williams syndrome. A total of 31 (53%) patients had associated peripheral pulmonary artery stenosis and 23 (39%) had coronary artery ostial stenosis (CAOS). RESULTS: A total of 37 of 58 (64%) patients had surgical repair of SVAS without the need for arch intervention while 21 (36%) patients had repair of the distal aortic arch. There were 3 (5.2%) operative deaths, 2 of whom had aortic arch involvement and one without arch involvement. There were 2 deaths after discharge from the hospital. Patients who needed arch surgery were more likely to have severe arch gradients compared to those without arch involvement (71% vs 30%, P < .05), were more likely to undergo concomitant procedures for peripheral pulmonary artery stenosis or CAOS (90% vs 62%, P < .05), and to have Williams syndrome (86% vs 51%, P < .05). CONCLUSIONS: More than one-third of patients who had SVAS repair at our institution had procedures directed at the transverse or proximal descending aorta. Patients with arch involvement had more severe arch obstruction, required more concomitant procedures, and were more likely to have Williams syndrome.

Virtual Transcatheter Interventions for Peripheral Pulmonary Artery Stenosis in Williams and Alagille Syndromes.

Background Despite favorable outcomes of surgical pulmonary artery (PA) reconstruction, isolated proximal stenting of the central PAs is common clinical practice for patients with peripheral PA stenosis in association with Williams and Alagille syndromes. Given the technical challenges of PA reconstruction and the morbidities associated with transcatheter interventions, the hemodynamic consequences of all treatment strategies must be rigorously assessed. Our study aims to model, assess, and predict hemodynamic outcomes of transcatheter interventions in these patients. Methods and Results Isolated proximal and "extensive" interventions (stenting and/or balloon angioplasty of proximal and lobar vessels) were performed in silico on 6 patient-specific PA models. Autoregulatory adaptation of the cardiac output and downstream arterial resistance was modeled in response to intervention-induced hemodynamic perturbations. Postintervention computational fluid dynamics predictions were validated in 2 stented patients and quantitatively assessed in 4 surgical patients. Our computational methods accurately predicted postinterventional PA pressures, the primary indicators of success for treatment of peripheral PA stenosis. Proximal and extensive treatment achieved median reductions of 14% and 40% in main PA systolic pressure, 27% and 56% in pulmonary vascular resistance, and 10% and 45% in right ventricular stroke work, respectively. Conclusions In patients with Williams and Alagille syndromes, extensive transcatheter intervention is required to sufficiently reduce PA pressures and right ventricular stroke work. Transcatheter therapy was shown to be ineffective for long-segment stenosis and pales hemodynamically in comparison with published outcomes of surgical reconstruction. Regardless of the chosen strategy, a virtual treatment planning platform could identify lesions most critical for optimizing right ventricular afterload.

Postoperative Acute Kidney Injury in Williams Syndrome Compared With Matched Controls.

OBJECTIVES: Cardiovascular manifestations occur in over 80% of Williams syndrome (WS) patients and are the leading cause of morbidity and mortality. One-third of patients require cardiovascular surgery. Renal artery stenosis (RAS) is common in WS. No studies have assessed postoperative cardiac surgery-related acute kidney injury (CS-AKI) in WS. Our objectives were to assess if WS patients have higher risk of CS-AKI postoperatively than matched controls and if RAS could contribute to CS-AKI. DESIGN: This was a retrospective study of all patients with WS who underwent cardiac surgery at our center from 2010 to 2020. The WS study cohort was compared with a group of controls matched for age, sex, weight, and surgical procedure. SETTING: Patients underwent cardiac surgery and postoperative care at Lucile Packard Children's Hospital Stanford. PATIENTS: There were 27 WS patients and 43 controls (31% vs 42% female; p = 0.36). Median age was 1.8 years (interquartile range [IQR], 0.7-3.8 yr) for WS and 1.7 years (IQR, 0.8-3.1 yr) for controls. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Postoperative hemodynamics, vasopressor, total volume input, diuretic administration, and urine output were collected in the first 72 hours. Laboratory studies were collected at 8-hour intervals. Multivariable analysis identified predictors of CS-AKI.Controlled for renal perfusion pressure (RPP) and vasoactive inotrope score (VIS), compared with controls, the odds ratio (OR) of CS-AKI in WS was 4.2 (95% CI, 1.1-16; p = 0.034). Higher RPP at postoperative hours 9-16 was associated with decreased OR of CS-AKI (0.88 [0.8-0.96]; p = 0.004). Increased VIS at hour 6 was associated with an increased OR of CS-AKI (1.47 [1.14-1.9]; p = 0.003). Younger age was associated with an increased OR of CS-AKI (1.9 [1.13-3.17]; p = 0.015). CONCLUSIONS: The OR of CS-AKI is increased in pediatric patients with WS compared with controls. CS-AKI was associated with VIS at the sixth postoperative hour. Increases in RPP and mean arterial pressure were associated with decreased odds of CS-AKI.

Midterm fate of unifocalized major aortopulmonary collateral arteries in patients with retroesophageal major aortopulmonary collateral arteries.

BACKGROUND: Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (MAPCAs) is a relatively rare and complex form of congenital heart disease. Unifocalization of MAPCAs has been advocated by some groups for the treatment of this condition. The purpose of this study was to assess the midterm fate of unifocalized MAPCAs in a cohort of patients with retroesophageal MAPCAs. METHODS: This was a retrospective review of 37 patients who underwent a unifocalization procedure. All patients in this study had 1 or more retroesophageal MAPCAs, and detailed mapping of the MAPCAs was made based on a combination of the cardiac catheterization and surgical findings. RESULTS: The 37 patients had a total of 166 MAPCAs, or 4.5 MAPCAs per patient. One hundred twenty-nine (78%) MAPCAs were unifocalized, whereas 37 (22%) were ligated because they were dual supply. Median follow-up was 69 months. At follow-up cardiac catheterization, evaluation of the 129 unifocalized MAPCAs demonstrated that 123 (95%) had antegrade flow, whereas 6 were occluded. For the 123 MAPCAs with antegrade flow, 97 (80%) were widely patent, whereas 26 were stenotic. Thirteen of the 37 patients have subsequently undergone reintervention on MAPCAs that were determined to be stenotic following unifocalization. Seven of these patients had mild disease and had complete resolution with balloon (n = 5) or surgical revision (n = 2). Six patients with moderate or severe disease underwent surgical revision with confirmed resolution in 4 of 6. CONCLUSIONS: The data demonstrate that the majority of unifocalized MAPCAs remain widely patent following unifocalization. However, one-quarter of unifocalized MAPCAs develop stenoses or occlusion. These results suggest the fate for most unifocalized MAPCAs is favorable but highlight the need for close vigilance.

The Sinotubular Junction-to-Aortic Annulus Ratio as a Determinant of Supravalvar Aortic Stenosis Severity.

Supravalvar aortic stenosis (SVAS) severity guides management, including decisions for surgery. Physiologic and technical factors limit the determination of SVAS severity by Doppler echocardiography and cardiac catheterization in Williams syndrome (WS). We hypothesized SVAS severity could be determined by the sinotubular junction-to-aortic annulus ratio (STJ:An). We reviewed all preintervention echocardiograms in patients with WS with SVAS cared for at our center. We measured STJ, An, peak and mean Doppler gradients, and calculated STJ:An. We created 2 mean gradient prediction models. Model 1 used the simplified Bernoulli's equation, and model 2 used computational fluid dynamics (CFD). We compared STJ:An to Doppler-derived and CFD gradients. We reviewed catheterization gradients and the waveforms and analyzed gradient variability. We analyzed 168 echocardiograms in 54 children (58% male, median age at scan 1.2 years, interquartile range [IQR] 0.5 to 3.6, median echocardiograms 2, IQR 1 to 4). Median SVAS peak Doppler gradient was 24 mm Hg (IQR 14 to 46.5). Median SVAS mean Doppler gradient was 11 mm Hg (IQR 6 to 21). Median STJ:An was 0.76 (IQR 0.63 to 0.84). Model 1 underpredicted clinical gradients. Model 2 correlated well with STJ:An through all severity ranges and demonstrated increased pressure recovery distance with decreased STJ:An. The median potential variability in catheterization-derived gradients in a given patient was 14.5 mm Hg (IQR 7.5 to 19.3). SVAS severity in WS can be accurately assessed using STJ:An. CFD predicts clinical data well through all SVAS severity levels. STJ:An is independent of physiologic state and has fewer technical limitations than Doppler echocardiography and catheterization. STJ:An could augment traditional methods in guiding surgical management decisions.

Impact of Modified Anesthesia Management for Pediatric Patients With Williams Syndrome.

OBJECTIVE: This study compared the percent change in systolic blood pressure and the incidence of adverse cardiac events (ACEs; defined as cardiac arrest, cardiopulmonary resuscitation, arrhythmias, or ST-segment changes) during anesthesia induction in patients with Williams syndrome (WS) before and after implementation of a perioperative management strategy. DESIGN: Retrospective observational cohort study. SETTING: Single quaternary academic referral center. PARTICIPANTS: The authors reviewed the records of all children with WS at the authors' institution who underwent general anesthesia for cardiac catheterization, diagnostic imaging, or any type of surgery between November 2008 and August 2019. The authors identified 142 patients with WS, 48 of whom underwent 118 general anesthesia administrations. A historic group (HG) was compared with the intervention group (IG). INTERVENTIONS: Change in perioperative management (three-stage risk stratification: preoperative intravenous hydration, intravenous anesthesia induction, and early use of vasoactives). MEASUREMENTS AND MAIN RESULTS: The authors determined event rates within 60 minutes of anesthesia induction. Standardized mean difference (SMD) was calculated (SMD >0.2 suggests clinically meaningful difference). Sixty-seven general anesthesia encounters were recorded in the HG (mean age, 4.8 years; mean weight, 16.3 kg) and 51 in the IG (mean age, 6.0 years; mean weight, 18.2 kg). The change in systolic blood pressure was -17.5% (-30.0, -5.0) in the HG versus -9% (-18.0, 5.0) in the IG (p = 0.015; SMD = 0.419), and the incidence of ACEs was 6% in the HG and 2% in the IG (p = 0.542; SMD = 0.207). CONCLUSIONS: Preoperative risk stratification, preoperative intravenous hydration, intravenous induction, and early use of continuous vasoactives resulted in greater hemodynamic stability, with a 2% incidence of ACEs.

The Outcomes of Surgical Separation in Thoracopagus Twins with Conjoined Hearts: An Analysis of the Literature.

We sought to perform a comprehensive review of reported cases of conjoined thoracopagus twins with cardiovascular conjunction to determine the overall survival after separation and factors that might be associated with survival. We performed a systematic review of cases of thoracopagus twins with conjoined cardiovascular systems reported in the English medical literature using Embase, PubMed, Web of Science, and Scopus. We employed standard statistical methods to analyze differences among groups. We identified 102 unique cases of thoracopagus twins (69% female) with some degree of cardiovascular conjunction who had undergone surgical separation. We identified 6 distinct types of cardiovascular union. Median age was 47 days (IQR 12, 120). Survival to at least hospital discharge occurred in 51% (105/204). Median age at separation was lower in emergent (9 days, IQR 1, 25) versus elective cases (93 days, IQR 49, 180) (p < 0.0001). Survival to hospital discharge was higher in those who underwent elective separation (70%, 93/132 total children versus 17%, 12/72) (p < 0.0001). Survival was associated with the type of cardiovascular union (p < 0.0001). The separation of thoracopagus twins with cardiovascular conjunction is higher than expected. Increased survival is associated with elective separation, older age, and shared pericardium only. These findings suggest survival could be improved with better delineation of degree of union and delayed separation.

Tele-Clinic Visits in Pediatric Patients with Marfan Syndrome Using Parentally Acquired Echocardiography.

OBJECTIVE: To test feasibility of tele-clinic visits using parentally acquired vital signs and focused echocardiographic images in patients with Marfan syndrome. STUDY DESIGN: We included patients with Marfan syndrome aged 5-19 years followed in our clinic. We excluded patients with Marfan syndrome and history of previous aortic root (AoR) surgery, cardiomyopathy, arrhythmia, or AoR ≥4.5 cm. We trained parents in-person to acquire focused echocardiographic images on their children using a hand-held device as well as how to use a stadiometer, scale, blood pressure (BP) machine, and a digital stethoscope. Before tele-clinic visits, parents obtained the echocardiographic images and vital signs. We compared tele-clinic and on-site clinic visit data. Parental and clinic echocardiograms were independently analyzed. RESULTS: Fifteen patient/parent pairs completed tele-clinic visits, conducted at a median of 7.0 (IQR 3.0-9.9) months from the in-person training session. Parents took a median of 70 (IQR 60-150) minutes to obtain the height, weight, heart rate, BP, cardiac sounds, and echocardiographic images before tele-clinic visits. Systolic BP was greater on-site than at home (median +13 mm Hg, P = .014). Height, weight, diastolic BP, heart rate, and AoR measurements were similar. CONCLUSIONS: This study provides information for implementing tele-clinic visits using parentally acquired vital signs and echocardiographic images in patients with Marfan syndrome. The results show that tele-clinic visits are feasible and that parents were able to obtain focused echocardiographic images on their children. TRIAL REGISTRATION: ClinicalTrials.gov: NCT03581682.

A validated model for prediction of survival to 6 months in patients with trisomy 13 and 18.

Congenital heart disease is exceedingly prevalent in trisomy 13 and 18. Improved survival following congenital heart surgery has been reported, however, mortality remains significantly elevated. Utilizing inpatient data on trisomy 13 and 18 from the 2003-2016 Pediatric Health Information System database, a survival model was developed and validated using data from the California Perinatal Quality Care Collaborative and the California Office of Statewide Health Planning and Development. The study cohort included 1,761 infants with trisomy 13 and 18. Two models predicting survival to 6 months of age were developed and tested. The initial model performed excellently, with a c-statistic of 0.87 and a c-statistic of 0.76 in the validation cohort. After excluding procedures performed on the day of death, the revised model's c-statistic was 0.76. Certain variables, including cardiac surgery, gastrostomy, parenteral nutrition, and mechanical ventilation, are predictive of survival to 6 months of age. This study presents a model, which potentially can inform decision-making regarding congenital heart surgery.

Surgical repair of coronary artery ostial stenosis in patients with Williams and elastin arteriopathy syndromes.

OBJECTIVE: Patients with Williams and elastin arteriopathy syndromes often have similar cardiac phenotypes characterized by supravalvar aortic stenosis (SVAS), peripheral pulmonary artery stenosis (PPAS), and coronary artery ostial stenosis (CAOS). SVAS and/or PPAS result in a marked increase in myocardial oxygen demand, whereas CAOS may limit myocardial oxygen supply. This combination predisposes to myocardial ischemic events and sudden cardiac arrest. The purpose of this study was to review our experience with the surgical repair of CAOS in patients with Williams and elastin arteriopathy syndromes. METHODS: This was a retrospective review of 16 patients with Williams (n = 11) or elastin arteriopathy (n = 5) who underwent surgical repair of CAOS as a concomitant procedure with SVAS and/or PPAS repair. Eleven patients had moderate or severe SVAS, and 10 had PPAS. The median age at surgery was 9 months (range, 3-108 months). RESULTS: Seven patients had repair of the left main and right coronary ostia, 6 the left main, and 3 the right coronary ostium. Median duration of aortic crossclamp was 56 minutes and cardiopulmonary bypass time was 454 minutes. The median SVAS gradient decreased from 70 to 12 mm Hg and pulmonary artery pressure decreased from 120 to 30 mm Hg. There was 1 operative mortality (6%). The remaining 15 patients were discharged from the hospital and are alive at a median of 17 months' follow-up. CONCLUSIONS: The data demonstrate that patients with Williams and elastin arteriopathy syndromes presenting with SVAS and/or PPAS plus CAOS can undergo successful repair of all hemodynamic issues simultaneously.

Implementation of a statewide, multisite fetal tele-echocardiography program: evaluation of more than 1100 fetuses over 9 years.

OBJECTIVES: We sought to describe the implementation and effectiveness of a statewide fetal tele-echocardiography program serving a resource-limited population. STUDY DESIGN: In 2009, our heart center established six satellite clinics for fetal tele-echocardiography around the state. We retrospectively reviewed all fetal tele-echocardiograms performed through 2018. Yearly statewide prenatal detection rates of operable congenital heart disease were queried from the Society of Thoracic Surgeons database. RESULT: In 1164 fetuses, fetal tele-echocardiography identified all types of congenital heart disease, with a sensitivity of 74% and specificity of 97%. For the detection of ductal-dependent congenital heart disease, fetal tele-echocardiography was 100% sensitive and specific. Between 2009 and 2018, annual statewide prenatal detection rates of congenital heart disease requiring heart surgery in the first 6 months of life rose by 159% (17-44%; R2 = 0.88, p < 0.01). CONCLUSIONS: The present study provides a framework for an effective, large-scale fetal tele-echocardiography program.

Pulmonary Artery Reconstruction After Failed Pulmonary Artery Stents.

BACKGROUND: Pulmonary artery stents are widely deployed in patients with stenoses in the branch pulmonary arteries. However, stents do not address more peripheral sites of stenosis and invariably develop in-stent restenosis. The purpose of this study was to review our experience with pulmonary artery reconstruction after failed pulmonary artery stents. METHODS: This was a retrospective study of 56 patients who underwent pulmonary artery reconstruction after failed pulmonary artery stents. These patients had undergone a median of 2 (range, 0-5) previous surgical procedures and 2 (range, 1-4) pulmonary artery stents. RESULTS: The median age at stent surgery was 5 (range, 0.3-23.6) years. The majority of stents (79%) were completely removed and patch augmented. The minority of stents (21%) were felt to be unremovable and thus were split longitudinally and reconstructed using a pulmonary artery homograft. There was 1 (1.8%) operative mortality. The mean pulmonary artery-to-aortic pressure ratio decreased from a preoperative value of 0.91 ± 0.21 to a postoperative value of 0.31 ± 0.07 (P < .001). The median hospital length of stay was 10 days. The median duration of follow-up was 1.8 years. There has been no midterm mortality. Six patients have undergone balloon dilation postoperatively for residual pulmonary artery stenosis. CONCLUSIONS: Pulmonary artery reconstruction resulted in a significant decrease in pulmonary artery-to-aortic pressure ratios. The subsequent need for reintervention on the pulmonary arteries has been relatively low (11% to date). These results suggest that patients with pulmonary artery stents can be successfully treated with surgical reconstruction.