Intraoperative and Postoperative Hemodynamic Predictors of Acute Kidney Injury in Pediatric Heart Transplant Recipients.

Acute kidney injury (AKI) is common after pediatric heart transplantation (HT) and is associated with inferior patient outcomes. Hemodynamic risk factors for pediatric heart transplant recipients who experience AKI are not well described. We performed a retrospective review of 99 pediatric heart transplant patients at Lucile Packard Children's Hospital Stanford from January 1, 2015, to December 31, 2019, in which clinical and demographic characteristics, intraoperative perfusion data, and hemodynamic measurements in the first 48 postoperative hours were analyzed as risk factors for severe AKI (Kidney Disease: Improving Global Outcomes [KDIGO] stage ≥ 2). Univariate analysis was conducted using Fisher's exact test, Chi-square test, and the Wilcoxon rank-sum test, as appropriate. Multivariable analysis was conducted using logistic regression. Thirty-five patients (35%) experienced severe AKI which was associated with lower intraoperative cardiac index ( p = 0.001), higher hematocrit ( p < 0.001), lower body temperature ( p < 0.001), lower renal near-infrared spectroscopy ( p = 0.001), lower postoperative mean arterial blood pressure (MAP: p = 0.001), and higher central venous pressure (CVP; p < 0.001). In multivariable analysis, postoperative CVP >12 mm Hg (odds ratio [OR] = 4.27; 95% confidence interval [CI]: 1.48-12.3, p = 0.007) and MAP <65 mm Hg (OR = 4.9; 95% CI: 1.07-22.5, p = 0.04) were associated with early severe AKI. Children with severe AKI experienced longer ventilator, intensive care, and posttransplant hospital days and inferior survival ( p = 0.01). Lower MAP and higher CVP are associated with severe AKI in pediatric HT recipients. Patients, who experienced AKI, experienced increased intensive care unit (ICU) morbidity and inferior survival. These data may guide the development of perioperative renal protective management strategies to reduce AKI incidence and improve patient outcomes.

Left ventricular strain, arch angulation, and velocity-time integral ratio improve performance of a clinical pathway for fetal diagnosis of neonatal coarctation of the aorta.

INTRODUCTION: Neonatal presentation of coarctation of the aorta (CoA) is a potentially life-threatening condition that is difficult to diagnose in fetal life. We therefore sought to validate and compare novel metrics that may add diagnostic value for fetal coarctation of the aorta, including the diastolic to systolic aortic isthmus VTI ratio (VTId:VTIs), ascending aorta to descending aorta angle (AAo-DAo), transverse aorta to descending aorta angle (TAo-DAo), and LV longitudinal strain (LVS). Then to evaluate whether these novel metrics improve specificity to identify fetuses at the highest risk for postnatal coarctation of the aorta (CoA) without compromising sensitivity. METHODS: Retrospective cohort study of fetuses followed a prospective clinical pathway and previously classified as mild, moderate, or high-risk for CoA based on standard fetal echo metrics. Novel metrics were retrospectively measured in a blinded manner. RESULTS: Among fetuses with prenatal concern for coarctation of the aorta, VTId:VTIs, AAo-DAo angle, TAo-DAo angle, and LVS were significantly different between surgical and non-surgical cases (p <0.01 for all variables). In the subgroup of moderate- and high-risk fetuses, the standard high-risk criteria (flow reversal at the foramen ovale or aortic arch) did not discriminate effectively between surgical and non-surgical cases. VTId:VTIs, AAo-Dao angle, Tao-DAo angle, and LVS all demonstrated greater discrimination than standard high-risk criteria, with specificity of 100% and PPV (positive predictive value) of 78-100%. CONCLUSIONS: The incorporation of novel metrics added diagnostic value to our clinical pathway for fetal CoA with higher specificity than the previous high-risk criteria. The incorporation of these metrics into the evaluation of fetuses at moderate- or high-risk for surgical CoA may improve prenatal counseling, allow for more consistent surgical planning, and ultimately optimize hospital resource allocation.

Results of the Double Switch Operation in Patients Who Previously Underwent Left Ventricular Retraining.

BACKGROUND: Congenitally corrected transposition of the great arteries (CC-TGA) is a complex form of congenital heart disease that has numerous subtypes. While most patients with CC-TGA have a large ventricular septal defect (VSD) and pulmonary stenosis, there are some patients who have either no VSD or a highly restrictive VSD. These patients will require left ventricular (LV) retraining prior to double switch. The purpose of this study was to review our experience with the double switch procedure in patients who had previously undergone LV retraining. METHODS: This was a retrospective review of a single institution experience with the double switch procedure in patients who had undergone LV retraining (2002-present). RESULTS: Forty-five patients underwent double switch following LV retraining. Of these, 39 had an arterial switch with hemi-Mustard/bidirectional Glenn and six had a Senning. The median cross-clamp time was 135 min (range 71-272) and median bypass time was 202 min (range 140-430 min). Median hospital length of stay was eight days (range 4-108). There were no in-hospital deaths. Median duration of follow-up was 30 months (range 0-175). One patient subsequently underwent heart transplantation and died 65 months following double switch. At follow-up, 41 of the 44 survivors (93%) have normal or low normal LV function and 40 of the 44 survivors (91%) have no or trace mitral regurgitation. CONCLUSIONS: The data demonstrate early and mid-term survival of 100% and 97%. Ninety-three percent had preserved LV function. These results suggest that patients with CC-TGA who undergo LV retraining and double switch can have excellent clinical outcomes.

Electrocardiograms Do Not Detect Myocardial Ischemia in Patients With Williams Syndrome and Nonsyndromic Elastin Arteriopathy With Coronary Artery Stenosis.

Coronary artery stenosis (CAS) may affect up to 27% of patients with Williams syndrome (WS), which may lead to myocardial ischemia. Patients with WS face a 25- to 100-fold greater risk of sudden cardiac death, frequently linked to anesthesia. Assessing CAS requires either imaging while under general anesthesia or intraoperative assessment, with the latter considered the gold standard. Our study aimed to identify electrocardiogram (ECG) markers of myocardial ischemia in patients with WS or nonsyndromic elastin arteriopathy and documented CAS. We retrospectively reviewed patients with WS/elastin arteriopathy who underwent supravalvar aortic stenosis surgery and CAS assessment from January 1, 2006 to April 30, 2021. A pediatric electrophysiologist, not aware of the patients' CAS status, reviewed their preoperative ECGs for markers of ischemia. We assessed associations of study parameters using Wilcoxon rank-sum and Fisher's exact tests. Of 34 patients, 62% were male, with a median age of 20 months (interquartile range: 8 to 34). CAS was present in 62% (21 of 34), 76% of whom (16 of 21) were male. There were no ECG indicators of myocardial ischemia in patients with CAS. In conclusion, CAS was present in >1/2 the children with WS/elastin arteriopathy who underwent repair of supravalvar aortic stenosis. CAS in WS/nonsyndromic elastin arteriopathy does not appear to exhibit typical ECG-detectable myocardial ischemia. ECGs are not a useful screening tool for CAS in WS/elastin arteriopathy. Given the high anesthesia-related cardiac arrest risk, other noninvasive indicators of CAS are needed.

Using Clinical Decision Support Systems to Decrease Intravenous Acetaminophen Use: Implementation and Lessons Learned.

BACKGROUND: Clinical decision support systems (CDSS) can enhance medical decision-making by providing targeted information to providers. While they have the potential to improve quality of care and reduce costs, they are not universally effective and can lead to unintended harm. OBJECTIVES: To describe the implementation of an unsuccessful interruptive CDSS that aimed to promote appropriate use of intravenous (IV) acetaminophen at an academic pediatric hospital, with an emphasis on lessons learned. METHODS: Quality improvement methodology was used to study the effect of an interruptive CDSS, which set a mandatory expiry time of 24 hours for all IV acetaminophen orders. This CDSS was implemented on April 5, 2021. The primary outcome measure was number of IV acetaminophen administrations per 1,000 patient days, measured pre- and postimplementation. Process measures were the number of IV acetaminophen orders placed per 1,000 patient days. Balancing measures were collected via survey data and included provider and nursing acceptability and unintended consequences of the CDSS. RESULTS: There was no special cause variation in hospital-wide IV acetaminophen administrations and orders after CDSS implementation, nor when the CDSS was removed. A total of 88 participants completed the survey. Nearly half (40/88) of respondents reported negative issues with the CDSS, with the majority stating that this affected patient care (39/40). Respondents cited delays in patient care and reduced efficiency as the most common negative effects. CONCLUSION: This study underscores the significance of monitoring CDSS implementations and including end user acceptability as an outcome measure. Teams should be prepared to modify or remove CDSS that do not achieve their intended goal or are associated with low end user acceptability. CDSS holds promise for improving clinical practice, but careful implementation and ongoing evaluation are crucial for maximizing their benefits and minimizing potential harm.

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.

Development of an Equity, Diversity, and Inclusion Committee for a collaborative quality improvement network: Pediatric Cardiac Critical Care Consortium (PC4) Equity, Diversity and Inclusion (EDI) Committee: white paper 2023.

Racial and ethnic disparities are well described in paediatric cardiac critical care outcomes. However, understanding the mechanisms behind these outcomes and implementing interventions to reduce and eliminate disparities remain a gap in the field of paediatric cardiac critical care. The Pediatric Cardiac Critical Care Consortium (PC4) established the Equity, Diversity, and Inclusion (EDI) Committee in 2020 to promote an equity lens to its aim of improving paediatric cardiac critical care quality and outcomes across North America. The PC4 EDI Committee is working to increase research, quality improvement, and programming efforts to work towards health equity. It also aims to promote health equity considerations in PC4 research. In addition to a focus on patient outcomes and research, the committee aims to increase the inclusion of Black, Indigenous, and People of Color (BIPOC) members in the PC4 collaborative. The following manuscript outlines the development, structure, and aims of the PC4 EDI Committee and describes an analysis of social determinants of health in published PC4 research.

Racial and Ethnic Disparities in Cardiac Reintervention After Pediatric Cardiac Surgical Procedures.

BACKGROUND: Children undergoing cardiac surgical procedures may require postoperative surgical or catheter-based reintervention before discharge. We examined racial/ethnic variations in reintervention and associated in-hospital death. METHODS: Children undergoing cardiac surgical procedures from 2004 to 2015 were identified in the Pediatric Health Information Systems (PHIS) database. Regression analysis measured associations between race/ethnicity, in-hospital death, and postoperative cardiac surgical or catheter-based reintervention (surgical/catheter reintervention). RESULTS: Of 124,263 patients, 8265 (6.7%) had a surgical/catheter reintervention. Black patients had fewer reinterventions (5.9% vs 6.7%) and higher in-hospital mortality (3.9% vs 2.7%, P < .01) than White patients. After adjusting for sociodemographic and illness severity indicators, Black patients remained less likely to receive surgical/catheter reintervention (adjusted hazard ratio [aHR], 0.89; 95% CI, 0.82-0.98) despite having similar risk of death after reintervention (adjusted odds ratio, 1.17; 95% CI, 0.98-1.41) compared with White patients. The risk of death without surgical/catheter reintervention was also higher for Black (aHR, 1.26; 95% CI, 1.08-1.47) and other race/ethnicity (aHR, 1.33; 95% CI, 1.13-1.57) patients than for White patients. Similar trends were demonstrated when mechanical circulatory support and cardiac transplantation were included as reinterventions. CONCLUSIONS: Patients of Black and other race/ethnicity undergoing pediatric cardiac surgical procedures are more likely to die without postoperative cardiac reintervention than White patients. Black patients are also less likely to receive reintervention despite no significant difference in mortality with reintervention. Further studies should evaluate etiologies and methods of addressing these disparities.

Arrhythmias in Williams Syndrome.

Williams syndrome (WS) is a congenital, multisystem disorder in which 80% of patients have cardiovascular abnormalities. Sudden cardiac death occurs 25 to 100 times more often in WS than in the general population, and cardiac repolarization is abnormal in WS. We sought to determine the prevalence of primary arrhythmias in patients with WS and whether QTc prolongation impacts arrhythmia risk. We retrospectively reviewed all patients with WS with ambulatory electrocardiogram (ECG) monitoring at our institution between October 2017 and January 2022. The primary outcome was the presence of arrhythmia. Predictors pre-determined for analysis included premature ventricular and atrial complex burden (%), degree of QTc change with varying heart rates, intervals and rhythm on 12-lead ECG, age, gender, symptomatology, and clinical and surgical history. A total of 74 patients (55% female, median age 8 years (3, 13) underwent 108 ambulatory monitors. Arrhythmias were present in 9 patients (12%). Within this group of 9 patients, 18/24 serial monitors were abnormal, and 3/9 patients (33%) had >1 arrhythmia type. Older age (p = 0.002) and symptoms (syncope, p = 0.005) were associated with arrhythmias. Arrhythmia was not associated with the degree of structural heart disease. Atrial tachycardia was the most identified arrhythmia (n = 6; 67% of patients with arrhythmias and 8% of the total cohort). The QTc abnormally increased with higher heart rates in all groups. There was a higher number of premature ventricular and atrial complexes per hour in patients with arrhythmias. In conclusion, atrial arrhythmias were the most common arrhythmia in patients with WS and routine ambulatory ECG and intermittent rhythm monitoring are indicated in WS, particularly given the high risk of sudden cardiac death in WS.

Analysis of risk factors associated with extracorporeal membrane oxygenation after surgical repair of peripheral pulmonary artery stenoses.

Objective: Acute lung injury is a known complication of pulmonary artery reconstruction for peripheral pulmonary artery stenosis. Severe cases may require support with extracorporeal membrane oxygenation. The purpose of this study was to evaluate the characteristics of patients requiring extracorporeal membrane oxygenation after pulmonary artery reconstruction. Methods: This was a retrospective study of 150 patients who underwent surgical repair of peripheral pulmonary artery stenosis at our institution from 2002 to 2022. Underlying diagnoses included Williams syndrome (n = 44), Alagille syndrome (n = 43), elastin arteriopathy (n = 21), tetralogy of Fallot (n = 21), and other (n = 21). Characteristics of patients who required extracorporeal membrane oxygenation were compared with those who did not require extracorporeal membrane oxygenation. Results: Eleven of the 150 patients undergoing pulmonary artery reconstruction (7.3%) required postoperative extracorporeal membrane oxygenation support (10 for acute lung injury and 1 for cardiac insufficiency). Four patients receiving extracorporeal membrane oxygenation had Williams syndrome, 3 patients had Alagille, and 4 patients had tetralogy of Fallot. Patients requiring extracorporeal membrane oxygenation had a higher preoperative right ventricle to aortic peak systolic pressure ratios (mean 1.14 vs 0.95), greater number of pulmonary artery ostial interventions (median, 23 vs 17), and longer duration of cardiopulmonary bypass (median, 597 vs 400 minutes). There were 3 in-hospital deaths (2.0%), 2 of whom required postoperative extracorporeal membrane oxygenation support. Conclusions: The data demonstrate multiple differences between patients who did and did not require extracorporeal membrane oxygenation after surgical repair of peripheral pulmonary artery stenosis. These results suggest that the preoperative extent of disease may predispose to the development of acute lung injury requiring extracorporeal membrane oxygenation support.

Williams Syndrome: Supravalvar Aortic, Aortic Arch, Coronary and Pulmonary Arteries: Is Comprehensive Repair Advisable and Achievable?

Williams syndrome, and various elastin protein mediated arteriopathies, presents a clinical challenge to pediatric cardiovascular specialists. In the severest phenotypes, multilevel obstruction to the systemic and pulmonic arterial systems result in biventricular dysfunction which can be imminently life-threatening. As a longstanding, quaternary referral center for complex pulmonary arteriopathies and pediatric connective tissue disease, Stanford Medicine Children's Health has developed a sizeable experience managing these patients. This manuscript is a summary of our current strategies, with a focus on our surgical techniques, peri-procedural considerations on timing and staging of various interventions, and long-term results.

Team Communication and Expectations Following Pediatric Cardiac Surgery: A Multi-Disciplinary Survey.

Patients and families desire an accurate understanding of the expected recovery following congenital cardiac surgery. Variation in knowledge and expectations within the care team may be under-recognized and impact communication and care delivery. Our objective was to assess knowledge of common postoperative milestones and perceived efficacy of communication with patients and families and within the care team. An 18-question survey measuring knowledge of expected milestones for recovery after four index operations and team communication in the postoperative period was distributed electronically to multidisciplinary care team members at 16 academic pediatric heart centers. Answers were compared to local median data for each respondent's heart center to assess accuracy and stratified by heart center role and years of experience. We obtained 874 responses with broad representation of disciplines. More than half of all respondent predictions (55.3%) did not match their local median data. Percent matching did not vary by care team role but improved with increasing experience (35.8% < 2 years vs. 46.4% > 10 years, p = 0.2133). Of all respondents, 62.7% expressed confidence discussing the anticipated postoperative course, 78.6% denoted confidence discussing postoperative complications, and 55.3% conveyed that not all members of their care team share a common expectation for typical postoperative recovery. Most respondents (94.6%) stated that increased knowledge of local data would positively impact communication. Confidence in communication exceeded accuracy in predicting the timing of postoperative milestones. Important variation in knowledge and expectations for postoperative recovery in pediatric cardiac surgery exists and may impact communication and clinical effectiveness.

Impact of Abnormal Potassium on Arrhythmia Risk During Pediatric Digoxin Therapy.

Digoxin is used in children with heart failure and tachyarrhythmia. Its use in patients with single ventricle anatomy has increased following evidence of improved interstage survival after the Norwood procedure. Digoxin has a narrow therapeutic window and may alter serum potassium balance, inducing arrhythmias. We hypothesized digoxin use in the setting of abnormal serum potassium levels is associated with arrhythmias. We reviewed all patients ≤ 18 years who received digoxin while admitted at our institution from 2014 to 2021. Admissions < 2 nights were excluded. We compared patients with a hemodynamically significant arrhythmia to those without. We performed adjusted mixed-effects logistic regression with arrhythmia as the outcome variable and potassium status as the predictor variable; adjusting for weight, route of digoxin administration, digoxin indication, serum creatinine, and number of interacting drugs prescribed. Abnormal potassium was defined as serum levels < 3.5 mmol/L or > 6.0 mmol/L. There were 268 encounters in 171 patients. Potassium levels were abnormal in 75.5% of patients who experienced an arrhythmia during digoxin administration, compared to 42.6% who did not (p < 0.001). Odds of arrhythmia was 138% higher in patients with abnormal potassium receiving digoxin (AOR = 2.38, 95% CI 1.07-5.29, p = 0.03). Receiving intravenous digoxin was also associated with a 7.35 odds of cardiac arrhythmia (AOR 7.35, p = 0.006, 95% CI 1.79-30.26). Odds of arrhythmia is increased during digoxin administration when pediatric patients have abnormal potassium levels. Vigilant attention to potassium levels is essential to prevent adverse outcomes during digoxin therapy.

Criteria for Early Pacemaker Implantation in Patients With Postoperative Heart Block After Congenital Heart Surgery.

BACKGROUND: Guidelines recommend observation for atrioventricular node recovery until postoperative days (POD) 7 to 10 before permanent pacemaker placement (PPM) in patients with heart block after congenital cardiac surgery. To aid in surgical decision-making for early PPM, we established criteria to identify patients at high risk of requiring PPM. METHODS: We reviewed all cases of second degree and complete heart block (CHB) on POD 0 from August 2009 through December 2018. A decision tree model was trained to predict the need for PPM amongst patients with persistent CHB and prospectively validated from January 2019 through March 2021. Separate models were developed for all patients on POD 0 and those without recovery by POD 4. RESULTS: Of the 139 patients with postoperative heart block, 68 required PPM. PPM was associated with older age (3.2 versus 1.0 years; P=0.018) and persistent CHB on POD 0 (versus intermittent CHB or second degree heart block; 87% versus 58%; P=0.001). Median days [IQR] to atrioventricular node recovery was 2 [0-5] and PPM was 9 [6-11]. Of the 100 cases of persistent CHB (21 in the validation cohort), 59 (59%) required PPM. A decision tree model identified 4 risk factors for PPM in patients with persistent CHB: (1) aortic valve replacement, subaortic stenosis repair, or Konno procedure; (2) ventricular L-looping; (3) atrioventricular valve replacement; (4) and absence of preoperative antiarrhythmic agent (in POD 0 model only). The POD 4 model specificity was 0.89 [0.67-0.99] and positive predictive value was 0.94 [95% CI 0.81-0.98], which was stable in prospective validation (positive predictive value 1.0). CONCLUSIONS: A data-driven analysis led to actionable criteria to identify patients requiring PPM. Patients with left ventricular outflow tract surgery, atrioventricular valve replacement, or ventricular L-Looping could be considered for PPM on POD 4 to reduce risks of temporary pacing and improve care efficiency.

Importance of Formula-Specific Centile Thresholds for Evaluation of Heart Rate-Corrected QT Prolongation in Williams Syndrome.

Patients with Williams syndrome (WS) have a 25- to 100-fold higher risk of sudden death and prolonged heart rate-corrected QT (QTc). A recent study using the Fridericia formula for QT correction suggested that prolongation is principally an issue of heart rate. We used multiple published heart rate correction formulas to reevaluate the prevalence of QTc prolongation in our original dataset from our 2010 study at the Children's Hospital of Philadelphia. The ninety-eighth centile for QTc and corrected JT Interval (JTc) of the control population for each formula were used to set the threshold for prolongation. Prevalence comparison was done with Fisher's exact test. Predictors of longer QTc/JTc were assessed using linear regression models adjusting for age, gender, and heart rate. Adjusted odds of QTc/JTc prolongation were evaluated with conditional logistic regression models matched based on age and heart rate. There were 482 electrocardiograms from 188 patients with WS and 1,522 from normal controls. Patients with WS were younger, with higher heart rates and shorter RR and QRS intervals. WS was associated with longer QTc/JTc compared with controls. There were higher odds of prolonged QTc/JTc in patients with WS than controls using both Bazett and Fridericia formulas. In conclusion, this study confirms the higher prevalence of QTc prolongation in WS compared with controls and highlights the importance of setting appropriate formula-specific upper thresholds for QTc prolongation for accurate diagnosis.

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.

A clinical scoring system for early onset (neonatal) Marfan syndrome.

PURPOSE: This study aimed to develop objective diagnostic criteria for early onset Marfan syndrome (eoMFS) to facilitate early diagnosis and timely interventions. METHODS: On the basis of an extensive literature review and the responses from a survey distributed among providers with expertise in the diagnosis and management of eoMFS, we developed an age-based, diagnostic scoring system encompassing 10 features common to eoMFS (9 clinical + 1 laboratory) and divided them into cardiac, systemic, and FBN1 (on the basis of the location of the pathogenic FBN1 variant) scores. RESULTS: In total, 77 individuals with eoMFS (13 newly reported) and 49 individuals diagnosed with classical Marfan syndrome during early childhood were used to validate the criteria. Median cardiac (8 vs 0, P < .001), systemic (11 vs 3, P < .001), FBN1 (5 vs 0, P < .001), and total (23 vs 4, P < .001) scores were significantly higher in individuals with eoMFS than in those without. A proposed clinical score (cardiac + systemic) cutoff of ≥14 points showed excellent sensitivity (100%), specificity (92%), and reliability (correctly classified = 94%). CONCLUSION: Distinct from classical Marfan syndrome in phenotype and morbidity, eoMFS can be diagnosed clinically using an objective scoring system encompassing the typical physical features and cardiac disease manifestations. Although genetic testing can be suggestive of eoMFS, genetic testing alone is insufficient for diagnosis.

Impact of a clinical pathway on acute kidney injury in patients undergoing heart transplant.

BACKGROUND: To evaluate the impact of a clinical pathway on the incidence and severity of acute kidney injury in patients undergoing heart transplant. METHODS: This was a 2.5-year retrospective evaluation using 3 years of historical controls within a cardiac intensive care unit in an academic children's hospital. Patients undergoing heart transplant between May 27, 2014, and April 5, 2017 (pre-pathway) and May 1, 2017, and November 30, 2019 (pathway) were included. The clinical pathway focused on supporting renal perfusion through hemodynamic management, avoiding or delaying nephrotoxic medications, and providing pharmacoprophylaxis against AKI. RESULTS: There were 57 consecutive patients included. There was an unadjusted 20% reduction in incidence of any acute kidney injury (p = .05) and a 17% reduction in Stage 2/3 acute kidney injury (p = .09). In multivariable adjusted analysis, avoidance of Stage 2/3 acute kidney injury was independently associated with the clinical pathway era (AOR -1.3 [95% CI -2.5 to -0.2]; p = .03), achieving a central venous pressure of or less than 12 mmHg (AOR -1.3 [95% CI -2.4 to -0.2]; p = .03) and mean arterial pressure above 60 mmHg (AOR -1.6 [95% CI -3.1 to -0.01]; p = .05) in the first 48 h post-transplant, and older age at transplant (AOR - 0.2 [95% CI -0.2 to -0.06]; p = .002). CONCLUSIONS: This report describes a renal protection clinical pathway associated with a reduction in perioperative acute kidney injury in patients undergoing heart transplant and highlights the importance of normalizing perioperative central venous pressure and mean arterial blood pressure to support optimal renal perfusion.

A Fetal Risk Stratification Pathway for Neonatal Aortic Coarctation Reduces Medical Exposure.

OBJECTIVE: To test the hypothesis that a fetal stratification pathway will effectively discriminate between infants at different levels of risk for surgical coarctation and reduce unnecessary medicalization. STUDY DESIGN: We performed a pre-post nonrandomized study in which we prospectively assigned fetuses with prenatal concern for coarctation to 1 of 3 risk categories and implemented a clinical pathway for postnatal management. Postnatal clinical outcomes were compared with those in a historical control group that were not triaged based on the pathway. RESULTS: The study cohort comprised 109 fetuses, including 57 treated along the fetal coarctation pathway and 52 historical controls. Among mild-risk fetuses, 3% underwent surgical coarctation repair (0% of those without additional heart defects), compared with 27% of moderate-risk and 63% of high-risk fetuses. The combined fetal aortic, mitral, and isthmus z-score best discriminated which infants underwent surgery (area under the curve = 0.78; 95% CI, 0.66-0.91). Compared with historical controls, infants triaged according to the fetal coarctation pathway had fewer delivery location changes (76% vs 55%; P = .025) and less umbilical venous catheter placement (74% vs 51%; P = .046). Trends toward shorter intensive care unit stay, hospital stay, and time to enteral feeding did not reach statistical significance. CONCLUSIONS: A stratified risk-assignment pathway effectively identifies a group of fetuses with a low rate of surgical coarctation and reduces unnecessary medicalization in infants who do not undergo aortic surgery. Incorporation of novel measurements or imaging techniques may improve the specificity of high-risk criteria.

Target-Based Care: An Intervention to Reduce Variation in Postoperative Length of Stay.

OBJECTIVES: To derive care targets and evaluate the impact of displaying them at the point of care on postoperative length of stay (LOS). STUDY DESIGN: A prospective cohort study using 2 years of historical controls within a freestanding, academic children's hospital. Patients undergoing benchmark cardiac surgery between May 4, 2014, and August 15, 2016 (preintervention) and September 6, 2016, to September 30, 2018 (postintervention) were included. The intervention consisted of displaying at the point of care targets for the timing of extubation, transfer from the intensive care unit (ICU), and hospital discharge. Family satisfaction, reintubation, and readmission rates were tracked. RESULTS: The postintervention cohort consisted of 219 consecutive patients. There was a reduction in variation for ICU (difference in SD -2.56, P < .01) and total LOS (difference in SD -2.84, P < .001). Patients stayed on average 0.97 fewer days (P < .001) in the ICU (median -1.01 [IQR -2.15, -0.39]), 0.7 fewer days (P < .001) on mechanical ventilation (median -0.54 [IQR -0.77, -0.50]), and 1.18 fewer days (P < .001) for the total LOS (median -2.25 [IQR -3.69, -0.15]). Log-transformed multivariable linear regression demonstrated the intervention to be associated with shorter ICU LOS (ß coefficient -0.19, SE 0.059, P < .001), total postoperative LOS (ß coefficient -0.12, SE 0.052, P = .02), and ventilator duration (ß coefficient -0.21, SE 0.048, P < .001). Balancing metrics did not differ after the intervention. CONCLUSIONS: Target-based care is a simple, novel intervention associated with reduced variation in LOS and absolute LOS across a diverse spectrum of complex cardiac surgeries.