"Fontan Conduit Stent-Angioplasty and Progression of Fontan-Associated Liver Disease".

Patients with Fontan circulation insidiously develop congestive hepatopathy related to chronically reduced cardiac output and central venous hypertension, also known as Fontan-associated liver disease (FALD). Fontan pathway obstruction is increasingly detected and may accelerate FALD. The impact of conduit stent angioplasty on FALD is unknown. Retrospective, single-center review of patients with Fontan circulation who underwent conduit stent angioplasty at cardiac catheterization over 5-year period. Demographics and cardiac histories were reviewed. Labs, liver ultrasound elastography, echocardiogram, hemodynamic and angiographic data at catheterization were recorded pre- and post-stent angioplasty. Primary outcome was change in hepatic function via MELD-XI scores and liver stiffness (kPa), with secondary outcomes of ventricular function, BNP, and repeat catheterization hemodynamics. 33 patients underwent Fontan conduit stent angioplasty, 19.3 ± 7.0 years from Fontan operation. Original conduit diameter was 19.1 ± 1.9 mm. Prior to angioplasty, conduit size was reduced to a cross-sectional area 132 (91, 173) mm2 and increased to 314 (255, 363) mm2 post-stent. Subjects' baseline median MELD-XI of 11 (9, 12) increased to 12 (9, 13) at 19 ± 15.5 months post-angioplasty (n = 22, p = 0.053). There was no significant change in liver stiffness at 12.1 ± 8.9 months post-angioplasty (n = 15, p = 0.13). Median total bilirubin significantly increased (1.4 [0.9, 1.8]), from baseline 1.1 [0.7, 1.5], p = 0.04), as did median BNP (41 [0, 148] from baseline 34 [15, 79]; p = 0.02). There were no significant changes in ventricular function or repeat invasive hemodynamics (n = 8 subjects). Mid-term follow-up of Fontan subjects post-conduit stent angioplasty did not show improvements in non-invasive markers of FALD.

Pre-operative Imaging of Critical Coarctation of the Aorta: Impact on Surgical Approach and Outcomes.

Computed tomographic angiography (CTA) has been increasingly used for the evaluation of infants with aortic arch hypoplasia and coarctation of the aorta. The goals of this study were to compare echocardiographic and CTA findings in critical coarctation of the aorta, to evaluate each modality's influence on surgical approach for repair and determine if pre-operative measurements or surgical approach are associated with residual lesions/re-interventions. This was a single-center retrospective cohort study that included 85 neonates and infants who underwent repair of coarctation/arch hypoplasia by three months of age. Two groups were compared: patients with pre-operative echocardiograms only and patients with both echocardiogram and CTA evaluations. 44 (52%) patients received an echocardiogram and CTA, and 41 (48%) patients received an echocardiogram only. Patients in the CTA + echo group had smaller mitral valve and ascending aorta measurements (p = 0.01). When comparing CTA to echocardiogram measurements, the aortic valve annulus, ascending aorta, proximal and distal transverse arch, and isthmus were smaller on echo (p < 0.01). A smaller aortic valve annulus and aortic root as well as thoracotomy approach were associated with residual gradients/re-intervention (p < 0.01). Our study found that patients who underwent CTA preoperatively had smaller left-sided structures. Aortic measurements were smaller on echocardiogram when compared to CTA. Smaller left-sided structures proximal to the aortic arch and thoracotomy predicted the development of residual lesions/re-intervention. CTA is useful in the surgical planning for neonates with arch hypoplasia/coarctation and may help risk stratify for residual lesions/re-intervention.

Association of Digoxin Use With Transplant-Free Interstage Survival in Infants Palliated With a Stage 1 Hybrid Procedure.

Background Digoxin prescription in patients with single-ventricle physiology after stage 1 palliation is associated with reduced interstage death. Prior literature has primarily included patients having undergone the Norwood procedure. We sought to determine if digoxin prescription at discharge in infants following hybrid stage 1 palliation was associated with improved transplant-free interstage survival. Methods and Results A retrospective multicenter cohort analysis was conducted using data from the National Pediatric Cardiology Quality Improvement Collaborative registry data from 2008 to 2021. Infants with functional single ventricles and aortic arch obstruction discharged home after the hybrid stage 1 palliation hospitalization were included. Patients were excluded if they had supraventricular tachycardia or conversion to Norwood operation. The primary outcome was transplant-free survival. Multivariable logistic regression analysis including a propensity score for digoxin use identified associations between digoxin use and interstage death or transplant. Of 259 included infants from 45 sites, 158 (61%) had hypoplastic left heart syndrome. Forty-nine percent had a gestational age ≤38 weeks, 18% had a birth weight <2.5 kg, and 58% had a preoperative risk factor. Of the 259 subjects, 129 (50%) were discharged on digoxin. Interstage death or transplant occurred in 30 (23%) patients in the no-digoxin group compared with 18 (14%) in the digoxin group (P=0.06). With multivariate analysis, discharge digoxin prescription was associated with a lower risk of interstage death or transplant (adjusted odds ratio, 0.48 [95% CI, 0.24-0.93]; P=0.03). Conclusions In infants with single-ventricle physiology who underwent hybrid stage 1 palliation, digoxin prescription at hospital discharge was associated with improved interstage transplant-free survival.

Factors Associated With Mortality and Adverse Outcomes After Truncus Arteriosus Repair.

BACKGROUND: Truncus arteriosus repair is associated with higher morbidity and mortality compared with many other congenital heart operations. We sought to determine factors associated with mortality and adverse outcomes in infants undergoing truncus arteriosus repair. METHODS: We used the Pediatric Health Information System Database to identify infants aged < 90 days who underwent truncus arteriosus repair from 2004 to 2019. The primary outcome was hospital mortality. Secondary outcomes were prolonged postoperative length of stay (>30 days) and hospital readmission within 90 days. Multivariable logistic regression models were used to identify associated factors for adverse outcomes. RESULTS: A total of 1645 subjects were included. Hospital mortality occurred in 164 (10%). Factors independently associated with mortality included birth weight < 3 kg, admit age < 48 hours, truncal valve surgery, cardiac arrest, extracorporeal membrane oxygenation, acute kidney injury, cardiac catheterization, tracheostomy, and earlier era. Prolonged postoperative length of stay occurred in 508 patients (31%). Factors independently associated with prolonged postoperative length of stay included prematurity, DiGeorge syndrome, admit age < 48 hours, later surgical era, acute kidney injury, infection, cardiac catheterization, vocal cord paralysis, tracheostomy, and gastrostomy. Readmission within 90 days occurred in 511 of 1481 surviving patients (34%). DiGeorge syndrome, cleft lip/palate, cardiac catheterization, and extracorporeal membrane oxygenation were factors independently associated with hospital readmission. CONCLUSIONS: We identified multiple factors associated with hospital mortality and adverse outcomes in infants undergoing truncus arteriosus repair. This information is useful for quality improvement initiatives, perioperative counseling, and discharge planning.

Outcomes of Gastrostomy and Tracheostomy in Infants Undergoing Truncus Arteriosus Repair: Database Study Using the Pediatric Health Information System.

OBJECTIVES: We sought to determine the prevalence of and factors associated with gastrostomy tube placement and tracheostomy in infants undergoing truncus arteriosus repair, and associations between these procedures and outcome. DESIGN: Retrospective cohort study. SETTING: Pediatric Health Information System database. PATIENTS: Infants less than 90 days old who underwent truncus arteriosus repair from 2004 to 2019. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Multivariable logistic regression models were used to identify factors associated with gastrostomy tube and tracheostomy placement and to identify associations between these procedures and hospital mortality and prolonged postoperative length of stay (LOS; > 30 d). Of 1,645 subjects, gastrostomy tube was performed in 196 (11.9%) and tracheostomy in 56 (3.4%). Factors independently associated with gastrostomy tube placement were DiGeorge syndrome, congenital airway anomaly, admission age less than or equal to 2 days, vocal cord paralysis, cardiac catheterization, infection, and failure to thrive. Factors independently associated with tracheostomy congenital airway anomaly, truncal valve surgery, and cardiac catheterization. Gastrostomy tube was independently associated with prolonged postoperative LOS (odds ratio [OR], 12.10; 95% CI, 7.37-19.86). Hospital mortality occurred in 17 of 56 patients (30.4%) who underwent tracheostomy versus 147 of 1,589 patients (9.3%) who did not ( p < 0.001), and median postoperative LOS was 148 days in patients who underwent tracheostomy versus 18 days in those who did not ( p < 0.001). Tracheostomy was independently associated with mortality (OR, 3.11; 95% CI, 1.43-6.77) and prolonged postoperative LOS (OR, 9.85; 95% CI, 2.16-44.80). CONCLUSIONS: In infants undergoing truncus arteriosus repair, tracheostomy is associated with greater odds of mortality; while gastrostomy and tracheostomy are strongly associated with greater odds of prolonged postoperative LOS.

Preoperative Computed Tomographic Angiography Can Predict Need for Branch Pulmonary Artery Intervention in Patients With Ductal-Dependent Pulmonary Blood Flow.

BACKGROUND: Neonates with ductal-dependent pulmonary blood flow (DD-PBF) are at risk for pulmonary artery (PA) stenosis. The objective of this study was to identify preoperative cardiovascular computed tomography angiography (CTA) measures that are associated with the need for branch PA intervention. METHODS: We identified neonates with DD-PBF who underwent preoperative CTA at our center and were followed for 24 months. The primary outcome was requiring intervention for branch PA stenosis at the initial or subsequent procedure. Patients were divided into three groups: 1) No PA intervention, 2) Initial PA intervention, and 3) Remote PA intervention. Measurements of the branch PAs and patent ductus arteriosus (PDA) were made prospectively. RESULTS: Forty patients were included, 7 (18%) did not receive a PA intervention, 23 (58%) were in the initial PA intervention group, and 10 (25%) were in the remote PA intervention group. The distance from PA bifurcation to the largest diameter of the PA that receives the PDA showed a difference between the no-intervention group versus the initial and remote intervention groups (0.8 mm [IQR 0.7, 2.0], 8.2 mm [IQR 1.9, 13.7], 8.5 mm [IQR 6.5, 11.1], respectively, P = .02). The receiver operating characteristic curve showed a distance >2.2 mm had a sensitivity = 91% and specificity = 86% in predicting the need for PA intervention. CONCLUSION: The distance from the PA bifurcation to the largest diameter of the branch PA that accepts the PDA on preoperative CTA is highly predictive of the need for initial or remote PA intervention in this group. Preoperative CTA should be considered for risk stratification in neonates undergoing intervention for DD-PBF.

Association Between Birth Weight Z-Scores and Early Outcomes Following Truncus Arteriosus Repair.

In infants undergoing truncus arteriosus (TA) repair, we sought to determine associations between fetal growth restrictions as measured by birth weight Z-score and early outcomes. We utilized the Pediatric Health Information System (PHIS) database to identify infants < 90 days old who underwent TA repair from 2004 to 2019. The primary exposure variable was birth weight Z-score, calculated based on gestational age at birth, gender, and birth weight. The primary outcome was postoperative hospital mortality. Secondary outcomes included major complications, prolonged postoperative length of hospital stay (LOS; > 30 days), and hospital readmission within 1 year. Generalized estimating equation (GEE) models were used to identify adjusted associations between birth weight Z-score, small for gestational age (SGA) status, and mortality and included were 1039 subjects. Median birth weight was 2960 g, gestational age at birth was 38 weeks, and birth weight Z-score was - 0.47. SGA was present in 21% of subjects. Hospital mortality occurred in 104 patients (10%). By multivariable analysis, lower birth weight Z-score was associated with higher hospital mortality [for each unit decrease in birth weight Z-score below - 1.0, adjusted OR 1.71 (95% CI 1.10-4.25)]. SGA status was associated with increased hospital mortality (adjusted OR 2.17; 95% CI 1.39-3.40). Birth weight Z-scores and SGA status were not significantly associated with occurrence of cardiac arrest, ECMO use, gastrostomy tube placement, tracheostomy, seizures, infection, prolonged postoperative LOS, or hospital readmission. In infants undergoing TA repair, lower birth weight Z-scores and SGA status were strongly associated with increased hospital mortality.

Timing of surgical repair and resource utilisation in infants with complete atrioventricular septal defect.

INTRODUCTION: Variation exists in the timing of surgery for balanced complete atrioventricular septal defect repair. We sought to explore associations between timing of repair and resource utilisation and clinical outcomes in the first year of life. METHODS: In this retrospective single-centre cohort study, we included patients who underwent complete atrioventricular septal defect repair between 2005 and 2019. Patients with left or right ventricular outflow tract obstruction and major non-cardiac comorbidities (except trisomy 21) were excluded. The primary outcome was days alive and out of the hospital in the first year of life. RESULTS: Included were 79 infants, divided into tertiles based on age at surgery (1st = 46 to 137 days, 2nd = 140 - 176 days, 3rd = 178 - 316 days). There were no significant differences among age tertiles for days alive and out of the hospital in the first year of life by univariable analysis (tertile 1, median 351 days; tertile 2, 348 days; tertile 3, 354 days; p = 0.22). No patients died. Fewer post-operative ICU days were used in the oldest tertile relative to the youngest, but days of mechanical ventilation and hospitalisation were similar. Clinical outcomes after repair and resource utilisation in the first year of life were similar for unplanned cardiac reinterventions, outpatient cardiology clinic visits, and weight-for-age z-score at 1 year. CONCLUSIONS: Age at complete atrioventricular septal defect repair is not associated with important differences in clinical outcomes or resource utilisation in the first year of life.

Factors associated with caregiver adherence to mobile health interstage home monitoring in infants with single ventricle or biventricular shunt-dependent heart disease.

Mobile health technology is an emerging tool in interstage home monitoring for infants with single ventricle heart disease or biventricular shunt-dependent defects. This study sought to describe adherence to mobile health monitoring and identify factors and outcomes associated with adherence to mobile health monitoring. This was a retrospective, single-institution study of infants who were followed in a mobile health-based interstage home monitoring programme between February 2016 and October 2020. The analysis included 105 infants and subjects were grouped by frequency of adherence to mobile health monitoring. Within the study cohort, 16 (15.2%) had 0% adherence, 25 (23.8%) had <50% adherence, and 64 (61.0%) had >50% adherence. The adherent groups had a higher percentage of infants who were male (p = 0.02), white race (p < 0.01), non-Hispanic or non-Latinx ethnicity (p < 0.01) and had mothers with primary English fluency (p < 0.01), married marital status (p < 0.01), and a prenatal diagnosis of faetal cardiac disease (p = 0.03). Adherent groups also had a higher percentage of infants with non-Medicaid primary insurance (p < 0.01) and residence in a neighbourhood with a higher median household income (p < 0.04). Frequency of adherence was not associated with interstage mortality, unplanned cardiac reinterventions, or hospital readmissions. Impact of mobile health interstage home monitoring on caregiver stress as well as use of multi-language, low literacy, affordable mobile health options for interstage home monitoring warrant further investigation.

QRS fragmentation and prolongation as predictors of exercise capacity in patients after Fontan palliation.

INTRODUCTION: Patients with Fontan anatomy are at increased risk for exercise intolerance and early morbidity and mortality. QRS complex fragmentation (fQRS) and prolongation have been studied in multiple heart diseases, but their clinical importance is unknown in the Fontan population. METHODS: A retrospective cross-sectional study was performed. ECGs were evaluated for QRS prolongation (>98 percentile for age) and fQRS (≥3 R-waves/notches in the R/S complex [more than two in RBBB] in ≥2 contiguous leads). The primary outcome measures were CPET performance. RESULTS: Total 90 patients (median age 18 years, 57% male, 59% RV dominant) were included; 13% had fQRS and 31% had prolonged QRS. Demographically, patients with fQRS or prolonged QRS were like those without. Peak VO2 (64% vs. 63%, p .45), VE/VCO2 slope (85% vs. 88%, p = .74), and O2 pulse (149% vs. 129%, p = .83) were similar in the fQRS group versus those without. Upon multi-variable regression, body mass index (ß = -0.38, p < .01) and QRS duration (ß = -0.29, p < .01) were independently associated with % predicted VO2; fQRS was not. Lower cardiac index (2.2 vs. 2.8 L/min/m2 , p = .03) and higher ventricular end-diastolic pressure (13 vs. 10 mmHg, p = .02) was seen with fQRS. CONCLUSIONS: QRS fragmentation is present in patients with Fontan physiology. fQRS showed no association with CPET performance but was related to invasive hemodynamic markers of ventricular performance. QRS duration may be a better predictor of exercise function following Fontan.

Diastolic Dysfunction With Preserved Ejection Fraction After the Fontan Procedure.

Background Heart failure phenotyping in single-ventricle Fontan patients is challenging, particularly in patients with normal ejection fraction (EF). The objective of this study was to identify Fontan patients with abnormal diastolic function, who are high risk for heart failure with preserved ejection fraction (HFpEF), and characterize their cardiac mechanics, exercise function, and functional health status. Methods and Results Data were obtained from the Pediatric Heart Network Fontan Cross-sectional Study database. EF was considered abnormal if <50%. Diastolic function was defined as abnormal if the diastolic pressure:volume quotient (lateral E:e'/end-diastolic volume) was >90th percentile (≥0.26 mL-1). Patients were divided into: controls=normal EF and diastolic function; systolic dysfunction (SD) = abnormal EF with normal diastolic function; diastolic dysfunction (DD) = normal EF with abnormal diastolic pressure:volume quotient. Exercise function was quantified as percent predicted peak VO2. Physical Functioning Summary Score (FSS) was reported from the Child Health Questionnaire. A total of 239 patients were included, 177 (74%) control, 36 (15%) SD, and 26 (11%) DD. Median age was 12.2 (5.4) years. Arterial elastance, a measure of arterial stiffness, was higher in DD (3.6±1.1 mm Hg/mL) compared with controls (2.5±0.8 mm Hg/mL), P<0.01. DD patients had lower predicted peak VO2 compared with controls (52% [20] versus 67% [23], P<0.01). Physical FSS was lower in DD (45±13) and SD (44±13) compared with controls (50±7), P<0.01. Conclusions Fontan patients with abnormal diastolic function and normal EF have decreased exercise tolerance, decreased functional health status, and elevated arterial stiffness. Identification of patients at high risk for HFpEF is feasible and should be considered when evaluating Fontan patients.

Calcium Chloride Infusions are Not Associated with Improved Outcomes in Neonates Undergoing Cardiac Operations.

Neonatal cardiac performance is dependent on calcium delivery to the myocardium. Little is known about the use and impact of calcium chloride infusions in neonates who undergo cardiac surgery. We hypothesized that the use of calcium chloride infusions would decrease the doses required of traditional inotropic and vasoactive medications by supporting cardiac output in this patient population. We performed a single-institution, retrospective, cohort study. All neonates (≤ 30 days old) undergoing cardiac surgery from 06/01/2015 through 12/31/2018 were included. Patients were divided into two groups: those who received postoperative calcium chloride infusions (calcium group) and those who did not (control group). The primary outcome was the occurrence of a maximum Vasoactive Inotropic Score (VIS) > 15 in the first 24 h following surgery. One hundred and thirty-five patients met inclusion criteria. Sixty-six patients received postoperative calcium infusions and 69 patients did not. Gestational age, weight at surgery, age at surgery, surgical complexity and cardiopulmonary bypass times were similar between groups. Forty-two (70%) patients receiving calcium had a postoperative maximum VIS > 15 compared with 38 (55%) patients not on a calcium infusion (p = 0.08). There were no differences in postoperative length of ventilation, time to enteral feeding, hospital LOS, or operative mortality between groups. Calcium chloride infusions in neonates who underwent cardiac surgery did not decrease exposure to other inotropic and vasoactive agents in the first 24 post-operative hours or improve patient outcomes.

Preoperative in-hospital mortality in neonates with critical CHD.

OBJECTIVE: Data regarding preoperative mortality in neonates with critical CHD are sparse and would aid patient care and family counselling. The objective of this study was to utilise a multicentre administrative dataset to report the rate of and identify risk factors for preoperative in-hospital mortality in neonates with critical CHD across US centres. STUDY DESIGN: The Pediatric Health Information System database was utilised to search for newborns ≤30 days old, born 1 January 2009 to 30 June 2018, with an ICD-9/10 code for d-transposition of the great arteries, truncus arteriosus, interrupted aortic arch, or hypoplastic left heart syndrome. Preoperative in-hospital mortality was defined as patients who died prior to discharge without an ICD code for cardiac surgery or interventional catheterisation. RESULTS: Overall preoperative mortality rate was at least 5.4% (690/12,739) and varied across diagnoses (d-TGA 2.9%, TA 8.3%, IAA 5.5%, and HLHS 7.3%) and centres (0-20.5%). In multivariable analysis, risk factors associated with preoperative mortality included preterm delivery (<37 weeks) (OR 2.3, 95% CI: 1.8-2.9; p < 0.01), low birth weight (<2.5 kg) (OR 3.8, 95% CI: 3.0-4.7; p < 0.01), and genetic abnormality (OR 1.6, 95% CI: 1.2-2.2; p < 0.01). Centre average surgical volume was not a significant risk factor. CONCLUSION: Approximately 1 in 20 neonates with critical CHD suffered preoperative in-hospital mortality, and rates varied across diagnoses and centres. Better understanding of the factors that drive the variation (e.g. patient factors, preoperative care models, surgical timing) could help identify patient care improvement opportunities and inform conversations with families.

Changes in circumferential strain can differentiate pediatric heart transplant recipients with and without graft rejection.

BACKGROUND: Routine surveillance protocols rely heavily on endomyocardial biopsy (EMB) for detection of rejection in pediatric heart transplant recipients. More sensitive echocardiographic tools to assess rejection may help limit the number of EMBs. This study compared changes in left ventricular (LV) strain in patients who had rejection versus those who did not. METHODS: A single center retrospective review was conducted between 2013 and 2020. Patients were categorized based on rejection history. Echocardiograms were evaluated at the time of 2 consecutive EMBs; in the rejection group, the second echocardiogram was collected at the time of a rejection episode. Conventional measures of LV function and speckle-tracking echocardiography-derived longitudinal (LS) and circumferential strain (CS) were measured. RESULTS: 17 patients were in the non-rejection group and 17 were in the rejection group (30 total rejection episodes). The rejection group was older at the time of transplant (12.5 vs. 1.3 years, p = .01). A decline in CS was seen in the rejection group at the second echocardiogram [-18.5 (IQR -21.5, -14.6) to -15.7 (IQR -19.8, -13.2)] while CS improved in the non-rejection group [-20.8 (IQR -23.9, -17.8) to -23.9 (IQR -24.9, -20.1)]. This difference in change reached significance (p = .02). A similar pattern was seen in LS that neared significance (p = .06). There was no significant difference in ejection fraction change (p = .24). CONCLUSIONS: Patients in the non-rejection group displayed improvement in CS between echocardiograms while patients in the rejection group showed subsequent decline. Worsening of LV CS may help identify acute rejection in the early post-transplant period.

Improved outcomes after implementation of a specialized pediatric cardiac rapid response team.

INTRODUCTION: The efficacy of a specialized pediatric cardiac rapid response team is unknown. We hypothesized that a specialized cardiac rapid response team would facilitate team-wide communication between the cardiac stepdown unit and cardiac intensive care unit (ICU) teams and improve patient care. MATERIALS AND METHODS: A specialized pediatric cardiac rapid response team was implemented in June 2015. All pediatric cardiac rapid response team activations and outcomes from implementation through December 2018 were reviewed. Cardiac arrests and unplanned transfers to the cardiac ICU were indexed to 1000 patient-days to account for inpatient volume trends and evaluated over time. RESULTS: There were 202 cardiac rapid response team activations in 108 unique patients during the study period. After implementation of the pediatric cardiac rapid response team, unplanned transfers from the cardiac stepdown unit to the cardiac ICU decreased from 16.8 to 7.1 transfers per 1000 patient days (p = 0.012). The stepdown unit cardiac arrest rate decreased from 1.2 to 0.0 arrests per 1000 patient-days (p = 0.015). There was one death on the cardiac stepdown unit in the 5 years since the implementation of the cardiac rapid response team, compared to four deaths in the previous 5 years. CONCLUSIONS: A reduction in unplanned cardiac ICU transfers, cardiac arrests, and mortality on the cardiac stepdown unit has been observed since the implementation of a specialized pediatric cardiac rapid response team. A specialized cardiac rapid response team may improve communication and empower the interdisciplinary care team to escalate care for patients experiencing clinical decline.

Multi-institution assessment of the use and risk of cardiovascular computed tomography in pediatric patients with congenital heart disease.

BACKGROUND: Cardiac computed tomography (CT) is increasingly used in pediatric patients with congenital heart disease (CHD). Variability of practice and of comprehensive diagnostic risk across institutions is not known. METHODS: Four centers prospectively enrolled consecutive pediatric CHD patients <18 years of age undergoing cardiac CT from January 6, 2017 to 1/30/2020. Patient characteristics, cardiac CT data and comprehensive diagnostic risk were compared by age and institutions. Risk categories included sedation and anesthesia use, vascular access, contrast exposure, cardiovascular medication, adverse events (AEs), and estimated radiation dose. RESULTS: Cardiac CT was performed in 1045 pediatric patients at a median (interquartile range, IQR) age of 1.7 years (0.3, 11.0). The most common indications were arterial abnormalities, suspected coronary artery anomalies, functionally single ventricle heart disease, and tetralogy of Fallot/pulmonary atresia. Sedation was used in 8% and anesthesia in 11% of patients. Peripheral vascular access was utilized for 93%. Median contrast volume was 2 â€‹ml/kg. Beta blockers were administered in 11% of cases and nitroglycerin in 2% of cases. The median (IQR) total procedural dose length product (DLP) was 20 â€‹mGy∗cm (10, 50). Sedation, vascular access, contrast exposure, use of cardiovascular medications and radiation dose estimates varied significantly by institution and age (p â€‹< â€‹0.001). Seven minor adverse events (0.7%) and no major adverse events were reported. CONCLUSION: Cardiac CT for CHD is safe in pediatric patients when appropriate CT technology and expertise are available. Scans can be acquired at relatively low radiation exposure with few minor adverse events.

Infantile dilated cardiomyopathy due to congenital left main coronary ostial stenosis.

Although a rare form of congenital heart disease, anomalies of the coronary arteries can present as heart failure in infants. The most common lesion is an anomalous left coronary artery arising from the pulmonary artery, but other abnormalities can present similarly. This case is an infant who is found to have left coronary ostial stenosis causing dilated cardiomyopathy.

Novel Echocardiography-Derived Left Ventricular Stiffness Index in Low-Flow Versus Normal-Flow Severe Aortic Stenosis with Preserved Left Ventricular Ejection Fraction.

Background Paradoxical low-flow (LF) severe aortic stenosis (AS) with preserved left ventricular ejection fraction (LVEF) may have poorer prognosis than normal-flow (NF) AS, though its pathophysiology remained unclear. In particular, LV stiffness has not been compared between LF vs NF. We used a novel echocardiography-derived index of LV stiffness to compare between these groups. Consecutive patients with medically-managed isolated severe AS (aortic valve area < 1 cm2) and preserved LVEF (>50%) were studied. Echocardiographic LV stiffness index was measured by a method previously validated against cardiac catheterization. We compared LF (stroke volume index, SVI < 35 ml/m2) and NF severe AS. Of the 352 patients, 121 (34%) were LF. Both LF and NF groups had similar demographics, valve areas and indices. Compared to NF, LF severe AS had higher LV stiffness indices (>0.11 ml-1 OR 3.067, 95% CI 1.825-5.128, p < 0.001). Increased LV stiffness was associated with concentric remodelling and more severe diastolic dysfunction, especially in LF AS. An LV stiffness index of > 0.11 ml-1 was independently associated with increased mortality, after adjusting for age, clinical and echocardiographic parameters (HR 2.283 95% CI 1.318-3.968, p = 0.003). Non-invasive echocardiographic-derived index of LV stiffness may be important in LF AS. Increased LV stiffness was related to LV concentric remodelling and diastolic dysfunction, and associated with poorer clinical outcomes in medically-managed AS.