Methylprednisolone for Heart Surgery in Infants - A Randomized, Controlled Trial.

BACKGROUND: Although perioperative prophylactic glucocorticoids have been used for decades, whether they improve outcomes in infants after heart surgery with cardiopulmonary bypass is unknown. METHODS: We conducted a multicenter, prospective, randomized, placebo-controlled, registry-based trial involving infants (<1 year of age) undergoing heart surgery with cardiopulmonary bypass at 24 sites participating in the Society of Thoracic Surgeons Congenital Heart Surgery Database. Registry data were used in the evaluation of outcomes. The infants were randomly assigned to receive prophylactic methylprednisolone (30 mg per kilogram of body weight) or placebo, which was administered into the cardiopulmonary-bypass pump-priming fluid. The primary end point was a ranked composite of death, heart transplantation, or any of 13 major complications. Patients without any of these events were assigned a ranked outcome based on postoperative length of stay. In the primary analysis, the ranked outcomes were compared between the trial groups with the use of odds ratios adjusted for prespecified risk factors. Secondary analyses included an unadjusted odds ratio, a win ratio, and safety outcomes. RESULTS: A total of 1263 infants underwent randomization, of whom 1200 received either methylprednisolone (599 infants) or placebo (601 infants). The likelihood of a worse outcome did not differ significantly between the methylprednisolone group and the placebo group (adjusted odds ratio, 0.86; 95% confidence interval [CI], 0.71 to 1.05; P = 0.14). Secondary analyses (unadjusted for risk factors) showed an odds ratio for a worse outcome of 0.82 (95% CI, 0.67 to 1.00) and a win ratio of 1.15 (95% CI, 1.00 to 1.32) in the methylprednisolone group as compared with the placebo group, findings suggestive of a benefit with methylprednisolone; however, patients in the methylprednisolone group were more likely than those in the placebo group to receive postoperative insulin for hyperglycemia (19.0% vs. 6.7%, P<0.001). CONCLUSIONS: Among infants undergoing surgery with cardiopulmonary bypass, prophylactic use of methylprednisolone did not significantly reduce the likelihood of a worse outcome in an adjusted analysis and was associated with postoperative development of hyperglycemia warranting insulin in a higher percentage of infants than placebo. (Funded by the National Center for Advancing Translational Sciences and others; STRESS ClinicalTrials.gov number, NCT03229538.).

Intravenous Dabigatran Provides Adequate Anticoagulation for Cardiopulmonary Bypass Using a Rabbit Model.

BACKGROUND: Heparin anticoagulation has been used successfully for cardiopulmonary bypass (CPB). However, an alternative anticoagulant approach is desirable due to the cases of heparin-induced thrombocytopenia. Dabigatran provides anticoagulation for an in vitro model of simulated CPB. The current analysis tests the hypothesis that dabigatran provides sufficient anticoagulation for CPB in intact rabbits. METHODS: Nonlinear mixed effects models were used to estimate dabigatran parameters for a two-compartment pharmacokinetic model in 10 New Zealand White rabbits. A dabigatran infusion designed to maintain a plasma concentration of 90 µg/ml was run throughout CPB based on the pharmacokinetics. Animals were subjected to sternotomy and anticoagulated with IV dabigatran (six animals) or heparin (four animals). Rabbits were cannulated centrally using the right atrium and ascending aorta and CPB was maintained for 120 min. Measurement of activated clotting time, thromboelastometric reaction time, and blood gases were performed during CPB. Then, the animals were euthanized, and the brain and one kidney were removed for histology. Sections of the arterial filters were inspected using electron microscopy. RESULTS: The observed dabigatran concentrations during CPB were greater than the target concentration, ranging from 137 ± 40 µg/ml at 5 min of CPB to 428 ± 150 µg/ml at 60 min, and 295 ± 35 µg/ml at 120 min. All rabbits completed 2 h of CPB without visible thrombosis. In the two groups, reaction time values were elevated, reaching 10,262 ± 4,198 s (dabigatran group) and 354 ± 141 s (heparin group) at 120 min of CPB. Brains and kidneys showed no evidence of thrombosis or ultrastructural damage. Sections of the arterial line filter showed minimal or no fibrin. There was no significant difference in outcomes between dabigatran- and heparin-treated animals. CONCLUSIONS: In this first-use, proof-of-concept study, the authors have shown that dabigatran provides acceptable anticoagulation similar to heparin to prevent thrombosis using a rabbit CPB model.

Pulmonary Venous Gradients in Healthy Controls and Following the Repair of Total Anomalous Pulmonary Venous Return.

The percent of children who can achieve a normal and physiologic pulmonary venous gradient and flow following the repair of Total Anomalous Pulmonary Venous Return (TAPVR) is not known. Pulmonary venous confluence gradients from infants with supra-, infra-, or mixed TAPVR, repaired using a direct anastomotic connection were measured. Data from age, weight, and gender-matched controls established the normal pulmonary venous gradient range (0.30-0.94 mmHg). TAPVR subjects were divided into three groups: (I) pulmonary venous gradient < 2 × normal with multiphasic flow (II) pulmonary venous gradient > 2 × normal with multiphasic flow, and (III) pulmonary venous gradient > 2 × normal with monophasic flow. From 63 children following TAPVR repair and 63 matched controls, pulmonary venous gradients were significantly lower [0.5 mmHg (IQR:0.4, 0.6) vs 1.6 mmHg (IQR:1.0, 2.4); p < 0.001], and multiphasic flow more frequent (100 vs. 84.1%; p = 0.001) within the control group. There were 38 children (60.3%) in group I, 15 (23.8%) in group II, and 10 (15.8%) in group III. Children in Group I were significantly older at the time of repair, had shorter cardiopulmonary bypass times, and did not utilize deep hypothermic circulatory arrest (DHCA). Multivariate analysis confirmed that avoiding DHCA [Odds Ratio 0.931 (0.913,0.994; p = 0.002)] and shorter cardiopulmonary bypass times [Odds Ratio 0.962 (0.861,0.968; p = 0.02)] during repair were associated with the lowest pulmonary venous gradients and multiphasic flow. Following TAPVR repair with a direct anastomosis, the majority of children can achieve a gradient two times normal or less with multiphasic pulmonary venous flow.

Using Serum Cystatin C to Predict Acute Kidney Injury Following Infant Cardiac Surgery.

Acute kidney injury (AKI) following cardiopulmonary bypass (CPB) is associated with increased morbidity and mortality. Serum Cystatin C (CysC) is a novel biomarker synthesized by all nucleated cells that may act as an early indicator of AKI following infant CPB. Prospective observational study of infants (< 1 year) requiring CPB during cardiac surgery. CysC was measured at baseline and 12, 24, 48, and 72 h following CPB initiation. Each post-op percent difference in CysC (e.g. %CysC12h) from baseline was calculated. Clinical variables along with urine output (UOP) and serum creatinine (SCr) were followed. Subjects were divided into two groups: AKI and non-AKI based upon the Kidney Disease Improving Global Outcomes (KDIGO) classification. AKI occurred in 41.9% (18) of the 43 infants enrolled. Patient demographics and baseline CysC levels were similar between groups. CysC levels were 0.97 ± 0.28 mg/L over the study period, and directly correlated with SCr (R = 0.71, p < 0.0001). Although absolute CysC levels were not significant between groups, the %CysC12h was significantly greater in the AKI group (AKI: - 16% ± 22% vs. Non-AKI - 28% ± 9% mg/L; p = 0.003). However, multivariate analysis demonstrated that a lower UOP (Odds Ratio:0.298; 95% CI 0.073, 0.850; p = 0.02) but not %CysC12h was independently associated with AKI. Despite a significant difference in the %CysC12h, only UOP was independently associated with AKI. Larger studies of a more homogenous population are needed to understand these results and to explore the variability in this biomarker seen across institutions.

Mitochondrial ATP Synthase Tetramer Disassembly following Blood-Based or del Nido Cardioplegia during Neonatal Cardiac Surgery.

Conservation of mitochondrial adenosine triphosphate (ATP) synthase proteins during ischemia is critical to preserve ATP supply and ventricular function. Following myocardial ischemia in adults, higher order ATP synthase tetramer proteins disassemble into simpler monomer units, reducing the efficiency of ATP production. However, it is unknown if myocardial ischemia following the use of cardioplegia results in tetramer disassembly in neonates, and whether it can be mitigated by cardioplegia if it does occur. We investigated myocardial ATP synthase tetramer disassembly in both a neonatal lamb cardiac surgery model and in neonatal children requiring cardiac surgery for the repair of congenital heart disease. Neonatal lambs (Ovis aries) were placed on cardiopulmonary bypass (CPB) and underwent cardioplegic arrest using a single dose of 30 mL/kg antegrade blood-based potassium cardioplegia (n = 4) or a single dose of 30 mL/kg antegrade del Nido cardioplegia (n = 6). Right ventricular biopsies were taken at baseline on CPB (n = 10) and after approximately 60 minutes of cardioplegic arrest before the cross clamp was released (n = 10). Human right ventricular biopsies (n = 3) were taken following 40.0 ± 23.1 minutes of ischemia after a single dose of antegrade blood-based cardioplegia. Protein complexes were separated on clear native gels and the tetramer to monomer ratio quantified. From the neonatal lamb model regardless of the cardioplegia strategy, the tetramer:monomer ratio decreased significantly during ischemia from baseline measurements (.6 ± .2 vs. .5 ± .1; p = .03). The del Nido solution better preserved the tetramer:monomer ratio when compared to the blood-based cardioplegia (Blood .4 ± .1 vs. del Nido .5 ± .1; p = .05). The tetramer:monomer ratio following the use of blood-based cardioplegia in humans aligned with the lamb data (tetramer:monomer .5 ± .2). These initial results suggest that despite cardioprotection, ischemia during neonatal cardiac surgery results in tetramer disassembly which may be limited when using the del Nido solution.

In Vitro and In Vivo Comparison of Hemoglobin and Electrolytes Following the Collection of Cell Saver Blood Washed with Either Normal Saline or Plasma-Lyte A.

Cell saver blood is typically washed with normal saline (NS); however, recent studies have reported decreased red blood cell hemolysis and increased platelet function when a more physiologic washing solution, such as Plasma-Lyte A (PL-A) is used. We evaluated the in vitro and in vivo effects of NS compared to PL-A as washing solutions for cell saver blood in pediatric cardiac surgery. Cell saver blood was re-infused for up to 24 hours post-collection. Laboratory and clinical data were collected from infants receiving cell saver washed with either NS (n = 20) or PL-A (n = 21). Compositions of the cell saver blood were compared between groups at 5 in vitro time points and in vivo patient blood at 24 hours post-bypass. Although there were differences in in vitro laboratory values between groups; 24 hours post-bypass, in vivo results were similar. Our data supports 24-hour reinfusion of cell saver washed with either NS versus PL-A in pediatric cardiac surgery patients, and provides data on the differences in cell saver composition to guide future studies.

Damus-Kaye-Stansel procedure avoids coronary obstruction in complex D-transposition.

Although all coronary anatomy in D-transposition of the great arteries may be "switchable," there are reports in the literature of early and late coronary obstruction following the arterial switch operation. The Damus-Kaye-Stansel procedure does not risk injury to the coronary arteries, and unlike the atrial switch, commits the left ventricle to the systemic circulation. We present a series of four neonates over 22 years with D-transposition of the great arteries and a concomitant coronary artery anomaly precluding arterial switch that were repaired using a Damus-Kaye-Stansel procedure and right ventricle to pulmonary artery conduit.

Transfer of Neonates with Critical Congenital Heart Disease Within a Regionalized Network.

Regionalization of pediatric cardiac surgical care varies between and within states. In most geographic regions, at least some neonates with critical heart disease are transferred from their birth hospital to a different hospital for surgery. The impact of neonatal transfer for surgery, particularly over a considerable distance (>10 miles), has been largely unexplored. We sought to examine the impact of transferring neonates for cardiac surgery. We queried the New York State Cardiac Surgery database (2005-2014) from a single institution to identify neonates born within the cardiac surgery center and those transferred for surgery. Outcomes were compared between groups, with subgroup analysis of neonates with single ventricle anatomy. 113 surgical neonates were born at the cardiac surgery center, and 268 were transferred to the cardiac surgery center. Median transfer distance was 91 (IQR 73, 94) miles. Age at operation and the need for preoperative ventilation were significantly lower in neonates born at the cardiac surgery center. In addition, single ventricle anatomy was more prevalent among those born at the cardiac surgery center (48.7 vs. 31.3%; p = 0.001). However, postoperative outcomes were the same-30-day survival was similar across groups (birth: 89% vs. transfer: 90%; p = 0.7), and for those with single ventricle palliation (birth: 81% vs. transfer: 81%; p = 0.9). Within our regionalized network, we found no difference in 30-day survival between neonates either born or transferred to a cardiac surgery center, which supports the use of a regionalized network of hospitals to the care of children with congenital heart disease.

A modified LVAD technique to augment caval and pulmonary arterial blood flow in the "failing Fontan" circulation.

BACKGROUND: There are minimal circulatory support options for patients with a failing Fontan. The Heartmate II (HMII) left ventricular assist device (Thoratec, Bedford, MA) in its packaged state cannot augment caval/pulmonary arterial blood flow. AIM: We hypothesized that a modified HMII pump could augment caval and pulmonary arterial blood flow. METHODS: A bifurcated ringed Gore-Tex graft (W. L. Gore & Associates, Flagstaff, AZ) was sewn to the HMII inflow, and the outflow graft transected and tapered from 16 mm to 8 mm in diameter. In three sheep, the inflow and outflow grafts were anastomosed end-to-side to both cava and the pulmonary artery. RESULTS: Following baseline measurements, the pump speed was increased to 8000 revolutions per minute (RPMs). Compared to baseline, at 8000 RPMs, there were no significant differences in mean arterial, central venous, or pulmonary arterial pressure. However, there was a significant decrease in right ventricular diastolic diameter (3.1 ± 0.1 vs. 1.8 ± 0.2 cm, R = 0.6, p = 0.02) and similarly a decrease in pulmonary arterial pulse pressure (8.5 ± 2.1 vs. 2.1 ± 2.9 mmHg, p = 0.01). As pump speed increased, there was a corresponding increase in pump flow and power, with a decrease in pulsatility index. CONCLUSIONS: These findings suggest that the HMII may be modified to provide caval/pulmonary circulatory support for the failing Fontan circulation.

Dominant frequency increase rate predicts transition from paroxysmal to long-term persistent atrial fibrillation.

BACKGROUND: Little is known about the mechanisms underlying the transition from paroxysmal to persistent atrial fibrillation (AF). In an ovine model of long-standing persistent AF we tested the hypothesis that the rate of electric and structural remodeling, assessed by dominant frequency (DF) changes, determines the time at which AF becomes persistent. METHODS AND RESULTS: Self-sustained AF was induced by atrial tachypacing. Seven sheep were euthanized 11.5±2.3 days after the transition to persistent AF and without reversal to sinus rhythm; 7 sheep were euthanized after 341.3±16.7 days of long-standing persistent AF. Seven sham-operated animals were in sinus rhythm for 1 year. DF was monitored continuously in each group. Real-time polymerase chain reaction, Western blotting, patch clamping, and histological analyses were used to determine the changes in functional ion channel expression and structural remodeling. Atrial dilatation, mitral valve regurgitation, myocyte hypertrophy, and atrial fibrosis occurred progressively and became statistically significant after the transition to persistent AF, with no evidence for left ventricular dysfunction. DF increased progressively during the paroxysmal-to-persistent AF transition and stabilized when AF became persistent. Importantly, the rate of DF increase correlated strongly with the time to persistent AF. Significant action potential duration abbreviation, secondary to functional ion channel protein expression changes (CaV1.2, NaV1.5, and KV4.2 decrease; Kir2.3 increase), was already present at the transition and persisted for 1 year of follow up. CONCLUSIONS: In the sheep model of long-standing persistent AF, the rate of DF increase predicts the time at which AF stabilizes and becomes persistent, reflecting changes in action potential duration and densities of sodium, L-type calcium, and inward rectifier currents.

Effect of Dexmedetomidine on Incidence of Hypertension Following Repair of Coarctation of the Aorta.

OBJECTIVE: Recent literature suggests a potential role for dexmedetomidine in reducing the incidence and severity of hypertension following repair of coarctation of the aorta (CoA). The primary aim of this study was to assess the association between dexmedetomidine use and the incidence of hypertension following repair of CoA in pediatric patients. METHODS: This was a single-center, retrospective cohort study in patients younger than 19 years who underwent surgical repair of CoA between January 1, 2016, and September 30, 2021. Patients were divided into 2 groups: dexmedetomidine initiation within the first 3 hours after surgery or no dexmedetomidine. The primary outcome was incidence of hypertension within the first 4 to 24 hours after repair. Secondary outcomes included the incidence of hypotension and bradycardia. RESULTS: A total of 80 patients were included, 25 (31.25%) received dexmedetomidine. Median age at the time of procedure was 26 days (IQR, 13-241) in the dexmedetomidine group and 14 days (IQR, 8-53) in the no dexmedetomidine group (p = 0.014). The primary outcome of hypertension was met in 7 patients (28%) in the dexmedetomidine group and 12 patients (21.8%) in the no dexmedetomidine group, p = 0.547. The only variable found to be associated with the incidence of hypertension was age greater than 30 days at the time of procedure. More patients who received dexmedetomidine experienced bradycardia. There was no difference in the incidence of hypotension. CONCLUSIONS: There was no association between the use of dexmedetomidine and the incidence of -hypertension following repair of CoA in pediatric patients.

Impact of Social Determinants of Health on Predictive Models for Outcomes After Congenital Heart Surgery.

BACKGROUND: Despite documented associations between social determinants of health and outcomes post-congenital heart surgery, clinical risk models typically exclude these factors. OBJECTIVES: The study sought to characterize associations between social determinants and operative and longitudinal mortality as well as assess impacts on risk model performance. METHODS: Demographic and clinical data were obtained for all congenital heart surgeries (2006-2021) from locally held Congenital Heart Surgery Collaborative for Longitudinal Outcomes and Utilization of Resources Society of Thoracic Surgeons Congenital Heart Surgery Database data. Neighborhood-level American Community Survey and composite sociodemographic measures were linked by zip code. Model prediction, discrimination, and impact on quality assessment were assessed before and after inclusion of social determinants in models based on the 2020 Society of Thoracic Surgeons Congenital Heart Surgery Database Mortality Risk Model. RESULTS: Of 14,173 total index operations across New York State, 12,321 cases, representing 10,271 patients at 8 centers, had zip codes for linkage. A total of 327 (2.7%) patients died in the hospital or before 30 days, and 314 children died by December 31, 2021 (total n = 641; 6.2%). Multiple measures of social determinants of health explained as much or more variability in operative and longitudinal mortality than clinical comorbidities or prior cardiac surgery. Inclusion of social determinants minimally improved models' predictive performance (operative: 0.834-0.844; longitudinal 0.808-0.811), but significantly improved model discrimination; 10.0% more survivors and 4.8% more mortalities were appropriately risk classified with inclusion. Wide variation in reclassification was observed by site, resulting in changes in the center performance classification category for 2 of 8 centers. CONCLUSIONS: Although indiscriminate inclusion of social determinants in clinical risk modeling can conceal inequities, thoughtful consideration can help centers understand their performance across populations and guide efforts to improve health equity.

Decreased incidence of supravalvar pulmonary stenosis after arterial switch operation.

BACKGROUND: Supravalvar pulmonary stenosis (SVPS) is frequently observed after arterial switch. Traditionally the coronary arteries are removed from the neopulmonic root by excising the entire sinus of Valsalva. As a result, reconstruction of the neopulmonic root requires a pericardial patch encompassing two-thirds of the anastomosis between the neopulmonic root and pulmonary artery. We present a technique where the coronary arteries are removed as limited buttons of sinus tissue, leaving the transected edge of the neopulmonic root intact. We hypothesize that maintaining native arterial tissue in the anastomosis between the neopulmonic root and the pulmonary artery bifurcation reduces postoperative SVPS. METHODS AND RESULTS: We performed a retrospective review of neonates with D-transposition of the great arteries undergoing arterial switch procedure from 1996 to 2009. Charts were reviewed, and clinical outcomes recorded for each patient. Most recent echocardiograms were evaluated for right ventricular outflow tract obstruction. A total of 120 patients received arterial switch using this technique. There was 99% survival and no injuries to the coronary arteries regardless of anatomy. Total follow-up was 564 patient-years. Mean follow-up at last clinical visit was 66 ± 46 months. Evaluation of the most recent outpatient echocardiogram revealed an average peak instantaneous gradient across the neopulmonic root of 22.5 ± 5 mm Hg. Only 7 (5%) patients required reintervention (balloon dilation, n=5; surgery, n=2). CONCLUSIONS: Our technique of removing the coronary arteries as limited buttons, and anastomosis of the pulmonary artery using only native arterial tissue provides excellent midterm results with minimal SVPS.

Transfusion of cell saver salvaged blood in neonates and infants undergoing open heart surgery significantly reduces RBC and coagulant product transfusions and donor exposures: results of a prospective, randomized, clinical trial.

OBJECTIVE: To evaluate whether transfusion of cell saver salvaged, stored at the bedside for up to 24 hrs, would decrease the number of postoperative allogeneic RBC transfusions and donor exposures, and possibly improve clinical outcomes. DESIGN: Prospective, randomized, controlled, clinical trial. SETTING: Pediatric cardiac intensive care unit. PATIENTS: Infants weighing less than 20 kg (n = 106) presenting for cardiac surgery with cardiopulmonary bypass. INTERVENTIONS: Subjects were randomized to a cell saver transfusion group where cell saver blood was available for transfusion up to 24 hrs after collection, or to a control group. Cell saver subjects received cell saver blood for volume replacement and/or RBC transfusions. Control subjects received crystalloid or albumin for volume replacement and RBCs for anemia. Blood product transfusions, donor exposures, and clinical outcomes were compared between groups. MEASUREMENTS AND MAIN RESULTS: Children randomized to the cell saver group had significantly fewer RBC transfusions (cell saver: 0.19 ± 0.44 vs. control: 0.75 ± 1.2; p = 0.003) and coagulant product transfusions in the first 48 hrs post-op (cell saver: 0.09 ± 0.45 vs. control: 0.62 ± 1.4; p = 0.013), and significantly fewer donor exposures (cell saver: 0.60 ± 1.4 vs. control: 2.3 ± 4.8; p = 0.019). This difference persisted over the first week post-op, but did not reach statistical significance (cell saver: 0.64 ± 1.24 vs. control: 1.1 ± 1.4; p = 0.07). There were no significant clinical outcome differences. CONCLUSION: Cell saver blood can be safely stored at the bedside for immediate transfusion for 24 hrs after collection. Administration of cell saver blood significantly reduces the number of RBC and coagulant product transfusions and donor exposures in the immediate postoperative period. Reduction of blood product transfusions has the potential to reduce transfusion-associated complications and decrease postoperative morbidity. Larger studies are needed to determine whether this transfusion strategy will improve clinical outcomes.

Long-Term Health Care Utilization After Cardiac Surgery in Children Covered Under Medicaid.

BACKGROUND: Understanding the longitudinal burden of health care expenditures and utilization after pediatric cardiac surgery is needed to counsel families, improve care, and reduce outcome inequities. OBJECTIVES: The purpose of this study was to describe and identify predictors of health care expenditures and utilization for Medicaid-insured pediatric cardiac surgical patients. METHODS: All Medicaid enrolled children age <18 years undergoing cardiac surgery in the New York State CHS-COLOUR database, from 2006 to 2019, were followed in Medicaid claims data through 2019. A matched cohort of children without cardiac surgical disease was identified as comparators. Expenditures and inpatient, primary care, subspecialist, and emergency department utilization were modeled using log-linear and Poisson regression models to assess associations between patient characteristics and outcomes. RESULTS: In 5,241 New York Medicaid-enrolled children, longitudinal health care expenditures and utilization for cardiac surgical patients exceeded noncardiac surgical comparators (cardiac surgical children: $15,500 ± $62,000 per month in year 1 and $1,600 ± $9,100 per month in year 5 vs noncardiac surgical children: $700 ± $6,600 per month in year 1 and $300 ± $2,200 per month in year 5). Children after cardiac surgery spent 52.9 days in hospitals and doctors' offices in the first postoperative year and 90.5 days over 5 years. Being Hispanic, compared with non-Hispanic White, was associated with having more emergency department visits, inpatient admissions, and subspecialist visits in years 2 to 5, but fewer primary care visits and greater 5-year mortality. CONCLUSIONS: Children after cardiac surgery have significant longitudinal health care needs, even among those with less severe cardiac disease. Health care utilization differed by race/ethnicity, although mechanisms driving disparities should be investigated further.

Chronic Medication Burden After Cardiac Surgery for Pediatric Medicaid Beneficiaries.

BACKGROUND: Congenital heart defects are the most common and resource-intensive birth defects. As children with congenital heart defects increasingly survive beyond early childhood, it is imperative to understand longitudinal disease burden. OBJECTIVES: The purpose of this study was to examine chronic outpatient prescription medication use and expenditures for New York State pediatric Medicaid enrollees, comparing children who undergo cardiac surgery (cardiac enrollees) and the general pediatric population. METHODS: This was a retrospective cohort study of all Medicaid enrollees age <18 years using the New York State Congenital Heart Surgery Collaborative for Longitudinal Outcomes and Utilization of Resources database (2006-2019). Primary outcomes were total chronic medications per person-year, enrollees per 100 person-years using ≥1 and ≥3 medications, and medication expenditures per person-year. We described and compared outcomes between cardiac enrollees and the general pediatric population. Among cardiac enrollees, multivariable regression examined associations between outcomes and clinical characteristics. RESULTS: We included 5,459 unique children (32,131 person-years) who underwent cardiac surgery and 4.5 million children (22 million person-years) who did not. More than 4 in 10 children who underwent cardiac surgery used ≥1 chronic medication compared with approximately 1 in 10 children who did not have cardiac surgery. Medication expenditures were 10 times higher per person-year for cardiac compared with noncardiac enrollees. Among cardiac enrollees, disease severity was associated with chronic medication use; use was highest among infants; however, nearly one-half of adolescents used ≥1 chronic medication. CONCLUSIONS: Children who undergo cardiac surgery experience high medication burden that persists throughout childhood. Understanding chronic medication use can inform clinicians (both pediatricians and subspecialists) and policymakers, and ultimately the value of care for this medically complex population.

Transseptal catheter decompression of the left ventricle during extracorporeal membrane oxygenation.

Veno-arterial extracorporeal membrane oxygenation (VA-ECMO) can result in left-ventricular distension and the development of pulmonary edema. We present the case of a 13-year-old girl who presented with cardiogenic shock. VA-ECMO was initiated, but after 6 days, severe left-ventricular distension resulted in decreased VA-ECMO flows. With guidance by bedside transesophageal echocardiography, a percutaneous atrial transseptal cannula was placed and connected to the venous circuit, thus decompressing the left ventricle. The patient improved, was weaned from VA-ECMO 5 days later, and was discharged from the hospital. Bedside transseptal catheter insertion is an effective method of left-ventricular decompression.

A modified approach in the repair of type I and II truncus arteriosus to promote branch pulmonary arterial growth and limit early reoperation.

Objective: Maintaining adequate branch pulmonary arterial growth is critical in preventing early (<3 years) right ventricular outflow tract reoperation after the repair of truncus arteriosus. We hypothesized that a modified truncus arteriosus repair keeping the branch pulmonary arteries in situ would promote branch pulmonary arterial growth and limit early right ventricular outflow tract reoperation. Methods: For infants requiring repair for type I and II truncus arteriosus, the truncal root was septated through a hockey stick incision keeping the branch pulmonary arteries in situ, the ventricular septal defect was closed, and a short aortic homograft was used to reconstruct the right ventricular outflow tract. Echocardiograms measured preoperative and follow-up branch pulmonary artery diameter. Results: Between 1998 and 2020, 41 infants were repaired using the modified approach (type I, 28; type II, 13). With a median follow-up of 11.6 (interquartile range, 3.1-15.5) years, there was no significant change between preoperative left pulmonary artery and right pulmonary artery Z-scores and their corresponding follow-up measurement (left pulmonary artery: 0.97, interquartile range, 0.6-1.6 vs left pulmonary artery: 1.4, interquartile range, -0.3 to 1.9) (right pulmonary artery: 0.6, interquartile range, -0.4 to 1.7 vs right pulmonary artery: 0.3 interquartile range, 0.5-0.9). Only 7.3% (n = 2) of follow-up right pulmonary artery Z-scores were less than 2.5 Z-scores below preoperative measurements. Four children (9.8%) required early right ventricular outflow tract reoperation. On multivariable analysis, larger conduit Z-scores were associated with greater time to right ventricular outflow tract reoperation (hazard ratio, 0.55, confidence interval, 0.307-0.984; P = .043). Conclusions: Maintaining the branch pulmonary arteries in situ at initial truncus arteriosus repair allows for branch pulmonary arterial growth, limiting early right ventricular outflow tract reoperation.

Enoxaparin Reduces Catheter-associated Venous Thrombosis After Infant Cardiac Surgery.

BACKGROUND: Central venous catheter (CVC) related venous thrombosis (VT) after pediatric cardiac surgery increases morbidity and mortality. Although VT prevention using low-dose anticoagulation therapy has proven ineffective, anticoagulation therapy using high-dose enoxaparin to achieve a therapeutic anti-Xa level has not been studied. We hypothesized that high-dose enoxaparin would reduce VT after pediatric cardiac surgery. METHODS: Enoxaparin was administered to infants aged less than 150 days when postoperative CVC duration was anticipated to extend beyond 5 days. The primary outcome was the rate of VT, reexploration for bleeding, and postoperative red blood cell transfusions per 1000 CVC days. RESULTS: From 2012 to 2019, 157 infants were treated with enoxaparin. Infants were divided into two groups: (1) subtherapeutic (n = 51), in which therapeutic anti-Xa level (0.5 to 1.0 IU/mL) was not achieved; and (2) therapeutic (n = 106), in which therapeutic anti-Xa level was achieved. Baseline demographics demonstrated a lower age at operation in the therapeutic group. The subtherapeutic group had a higher VT rate per 1000 CVC days (8.2) compared with the therapeutic group (2.6; P = .005). Reexploration for bleeding was similar between groups. The number of postoperative red blood cell transfusions per 1000 CVC days was significantly greater in the subtherapeutic group (109.4 vs 81.6; P = .008). Multivariate analysis demonstrated that higher median anti-Xa levels reduced the risk of VT (odds ratio 0.02; 95% confidence interval, 0.001 to 0.63; P = .02). CONCLUSIONS: These data suggest that enoxaparin treatment resulting in a therapeutic anti-Xa level reduces postoperative CVC-associated VT without increasing bleeding complications.

Factors Associated With an Abnormal Blood Pressure Response During Exercise After Coarctation Repair.

BACKGROUND: Although resting blood pressures following aortic arch repair or the extended end-to-end anastomosis (EEA) repair for coarctation can be physiologic, factors associated with an abnormal blood pressure response after exercise are unknown. We measured blood pressure gradients following exercise in children who had undergone previous repair in accordance with a surgical selection algorithm and sought to identify factors associated with an abnormal blood pressure response. METHODS: In accordance with our practice's surgical algorithm for repair of coarctation, infants were stratified to aortic arch repair when the distal transverse arch-to-left carotid artery ratio (DTA:LCA) ≤ 1.0, or when a brachiocephalic trunk or intra-cardiac lesion requiring repair was present. A thoracotomy and EEA were otherwise used. A follow-up exercise stress test (EST) measured the arm:leg blood pressure gradient after exercise, and a gradient ≥ 20 mm Hg was defined as an abnormal blood pressure response. RESULTS: Thirty-seven infants who had previously undergone coarctation repair (aortic arch repair-19, EEA-18) completed an EST at 12.3 ± 2.2 years of age. Thirteen (35%) children (aortic arch repair-5, EEA-8; p = .3) exhibited an abnormal blood pressure response. Factors associated with an abnormal blood pressure response included: smaller DTA:LCA ratios prior to repair (1.0 ± .2 vs. 1.2 ± .3; p = .04) and greater body weight at the time of EST (57.5 ± 19.1 vs. 40.9 ± 15.6 kg; p = .03). CONCLUSION: An abnormal blood pressure response following exercise is associated with smaller DTA:LCA ratios at the time of repair and increased weight during follow-up suggesting that patients with these factors warrant close observation.