Propranolol Therapy in Tetralogy of Fallot: Treating the Echocardiogram or Treating the Patient.

Hypercyanotic spells are one of the defining clinical features of Tetralogy of Fallot (TOF). Limited data exist on peak Doppler right ventricular outflow tract (RVOT) gradient as a risk factor for the development of hypercyanotic spells, frequency of prophylactic use of propranolol based on peak RVOT gradient, and its impact on preventing the occurrence of hypercyanotic spells. We aimed to quantify peak RVOT gradients as measured on transthoracic echocardiography in infants with unrepaired TOF and assess for correlation with clinical symptoms of hypercyanotic spells. We also assessed the frequency of pre-operative use of propranolol, indication for medication initiation, and occurrence of hypercyanotic spells with or without propranolol use. Retrospective analysis was performed on patients at our institution who were born between February 1, 2011 and May 31, 2023. Patients were excluded if they were maintained on prostaglandin infusion or underwent palliative shunt placement or balloon valvuloplasty prior to complete surgical repair. Demographics, occurrence of hypercyanotic spells, propranolol use, peripheral oxygen saturation, age at surgical repair, and peak RVOT gradient at the time of propranolol initiation were collected from the electronic medical record. If no propranolol use was recorded, the single highest maximum RVOT gradient prior to surgery was collected. 203 patients were identified, of which 92 patients were included in analysis. Thirty-six (39%) patients received propranolol and 19% of patients developed hypercyanotic spells prior to surgery. Patients with higher peak RVOT gradients were more likely to be started on propranolol even in the absence of overt symptoms, and they also demonstrated more systemic desaturation. Additionally, peak RVOT gradient was found to be a poor predictor for the development of hypercyanotic spells. Wide clinical variation exists in the prophylactic use of propranolol for prevention of hypercyanotic spells. Peak RVOT gradient is not a reliable tool for prophylactic propranolol initiation to prevent hypercyanotic spells.

15-Year Analysis of Surgical Approaches and Outcomes for Coarctation in 132 Neonates and Infants.

A variety of surgical approaches exist to treat aortic coarctation in neonates and infants. Our institutional approach is designed to match the surgical approach to the individual anatomy of the patient. The objective of this study is to evaluate operative characteristics and outcomes of all neonates and infants who underwent surgical repair of coarctation of the aorta or hypoplastic aortic arch at University of Florida from 2006 to 2021, inclusive, either in isolation or with concomitant repair of atrial septal defect (ASD) and/or ventricular septal defect (VSD). A retrospective review was performed of 132 patients aged 0-1 year who underwent surgical repair of aortic coarctation or hypoplastic aortic arch between 2006 and 2021, inclusive, either in isolation or with concomitant repair of ASD and/or VSD. Patients were divided into two groups based on the surgical approach: Group 1 = Median Sternotomy and Group 2 = Left Lateral Thoracotomy. Continuous variables are presented as median (minimum-maximum); categorical variables are presented as N (%). The most common operative technique in Group 1 was end-to-side reconstruction with ligation of the aortic isthmus. The most common operative technique in Group 2 was extended end-to-end repair. Operative Mortality was one patient (1/132 = 0.76%). Transcatheter intervention for recurrent coarctation was performed in seven patients (7/132 = 5.3%). Surgical re-intervention for recurrent coarctation was performed in three patients (3/132 = 2.3%). From these data, one can conclude that a strategy of matching the surgical approach to the anatomy of neonates and infants who underwent surgical repair of aortic coarctation or hypoplastic aortic arch, either in isolation or with concomitant repair of ASD and/or VSD, is associated with less than 1% Operative Mortality and less than 3% recurrent coarctation requiring reoperation.

The safe addition of nitric oxide to the sweep gas of the extracorporeal membrane oxygenation circuit in a pediatric cardiac intensive care unit.

Background: Nitric Oxide (NO) is a naturally occurring modulator of inflammation found in the human body. Several studies in the pediatric cardiothoracic surgery literature have demonstrated some beneficial clinical effects when NO is added to the sweep gas of the cardiopulmonary bypass circuit.Purpose: Our primary aim was to determine the safety of incorporating nitric oxide into the oxygenator sweep gas of the extracorporeal membrane oxygenation (ECMO) circuit. Secondarily, we looked at important clinical outcomes, such as survival, blood product utilization, and common complications related to ECMO.Methods: We performed a single center, retrospective review of all patients at our institution who received ECMO between January 1, 2017 and March 31, 2023. We began additing NO to the ECMO sweep gas in 2019. Results: There were no instances of clinically significant methemoglobinemia with the addition of NO to the sweep gas (0% vs 0%, p = 1). The median daily methemoglobin level was higher in those who received NO via the sweep gas when compared to those who did not (1.6 vs 1.1, p = <0.001). Conclusions: The addition of NO to the sweep gas of the ECMO circuit is safe.

Elevated Aortic Stiffness after Pediatric Heart Transplantation.

In adults, arterial stiffness has been linked to the development of target end-organ damage, thought to be related to abnormal transmission of pulse pressure. Increased arterial stiffness and endothelial dysfunction have been hypothesized to contribute to the development of microvascular dysfunction and coronary allograft vasculopathy (CAV), an important comorbidity after heart transplantation. However, little data exists regarding arterial stiffness in pediatric heart transplantation and its influence on development of coronary allograft vasculopathy is not well understood. We sought to assess aortic stiffness and distensibility in pediatric post-heart transplant patients. A prospective, observational study analyzing the ascending (donor tissue) and descending aorta (recipient tissue) using transthoracic echocardiographic M-mode measurements in patients aged < 21 years was conducted. Descending and ascending aorta M-modes were obtained from the subcostal long axis view, and the parasternal long axis view 3-5mm above the sinotubular junction, respectively. Two independent reviewers averaged measurements over 2-3 cardiac cycles, and Aortic Distensibility (AD) and Aortic Stiffness Index (ASI) were calculated using previously validated methods. We recruited 39 heart transplant (HT) patients and 47 healthy controls. Median end diastolic dimension of the ascending aorta (donor tissue) was significantly larger in the transplant group than the control group (1.92 cm vs. 1.74 cm, p = 0.01). Ascending aortic distensibility in post-transplant patients was significantly lower than in the control group (4.87 vs. 10.53, p < 0.001). Ascending aortic stiffness index was higher in the transplant patients compared to the controls (4.63 vs. 2.21, p < 0.001). There is evidence of altered ascending aortic distensibility and stiffness parameters in post-heart transplant patients. Further studies are required to assess its influence on complications like development of coronary artery vasculopathy.

Risk factors associated with post-transplant superior caval vein stenosis in paediatric heart transplantation.

Superior caval vein stenosis is a known complication following paediatric heart transplantation. Herein, we sought to assess the incidence of superior caval vein stenosis and need for intervention in a single centre paediatric heart transplantation programme. A retrospective review was performed to identify variables associated with superior caval vein stenosis and need for intervention. Patients were identified based on angiographic and echocardiographic signs of superior caval vein stenosis. Of 204 paediatric heart transplantation recipients, 49 (24.0%) had evidence of superior caval vein stenosis with no need for catheter intervention and 12 (5.9%) had superior caval vein stenosis requiring catheter intervention. Overall, patients with superior caval vein stenosis with and without intervention had more cavopulmonary anastomosis (41.7%; 20.4%), pre-transplant superior caval vein procedures (41.7%; 28.6%), and bicaval approach (100.0%; 98.0%), compared to the group with no stenosis (11.9% and p = 0.015, 12.6% and p = 0.004, 73.4% and p < 0.001, respectively). Smaller recipients and donors were more likely to need intervention. Intervention was also seen more frequently in recipients who were younger at diagnosis (4.7 years) compared to non-intervention (13.3 years; p = 0.040). Re-intervention was required in 16.7% patients (n = 2) and was not associated with any complications.

Postoperative vasoactive inotropic score is predictive of outcomes in pediatric heart transplantation.

Vasoactive inotrope score (VIS) is scarcely studied in pediatric orthotopic heart transplantation (pOHT). We conducted a retrospective review of pOHT (<21 years) recipients. Max VIS and mean VIS were calculated at 0-24 and 24-48 hours post-pOHT. Patients were divided into groups based on ISHLT guidelines: high (>10) and low (≤10). In our group (n = 104), patients with high max and mean VIS groups at 0-24 and 24-48 hours had longer bypass times (high: >130 minutes; low: <108 minutes; P < .05) and high max and mean VIS groups at 0-24 hours had longer ischemic times (high: >215 minutes; low: <192 minutes; P < .05). Patients with high max and mean VIS at 0-24 and 24-48 hours had longer hospital stay, ventilation, inotrope duration, more cardiac events, and acute kidney injury postoperatively (P < .05). High max VIS at 24-48 hours and high mean VIS at 24-48 hours had higher 3-year mortality (P = .04; P = .02). Multivariate analysis confirmed the association of VIS with short-term outcomes. However, VIS was not identified as an independent predictor of mortality. The ROC curve exhibits 10 as the ideal cutoff with area under the curve >0.8 for primary graft dysfunction (PGD).

Safety of a 2-Day Antibiotic Regimen After Delayed Chest Closure Post Pediatric Cardiac Surgery.

BACKGROUND: There is no consensus for the length of prophylactic antibiotics after delayed chest closure (DCC) postcardiac surgery in pediatrics. In September 2014, our institution's pediatric cardiac intensive care unit changed the policy on length of prophylactic antibiotics after DCC from 5 days (control) to 2 days (study group). The objective of the study was to determine whether a 2-day course of antibiotics is as effective as a 5-day course in preventing blood stream and sternal wound infections in pediatric DCC. METHODS: Retrospective and prospective study. Primary end points included incidence of sternal wound infections and positive sternal imaging for infection. Surrogate markers of infection were collected at 4 time points. RESULTS: During the study period, 139 patients had DCC postcardiac surgery of which 110 patients were included for analysis, 54 patients in the control and 56 in the study group. There was no difference in total number of positive wound cultures/chest computed tomography (CT) findings (4/54 [7.5%] control vs 5/56 [8.9%] study group, P = .3), positive blood cultures (P = .586), median postsurgical length of stay (P = .4), or readmissions within 30 days postsurgery (P = .6). All secondary end points were similar in both groups except peak heart rate between weeks 2 and 4 (P = .041). CONCLUSION: Two days of prophylactic antibiotics is not inferior to 5 days of prophylactic antibiotics after DCC following pediatric cardiac surgery.

Thenar Muscle Oxygen Saturation Using Vascular Occlusion Test: A Novel Technique to Study Microcirculatory Abnormalities in Pediatric Heart Failure Patients.

Heart failure (HF) is associated with microcirculatory changes secondary to neuro-humoral imbalance, vascular stiffness and increased sympathetic tone. Near Infra-Red Spectroscopy (NIRS) derived Thenar muscle tissue oxygenation levels (StO2) can provide an estimate of the functional status of microcirculation. There is a paucity of literature regarding evaluation of microcirculation in pediatric subjects with HF. We hypothesized that microcirculation and oxygen saturation dynamics as assessed by Thenar StO2 levels using vascular occlusion test (VOT) would be altered in HF subjects and that these changes may correlate with the severity of heart failure. We prospectively enrolled 60 pediatric subjects (29 healthy control, 31 HF). Baseline StO2 levels were measured using InSpectra™ StO2 probe placed over the Thenar eminence of right hand, followed by a VOT for 3 min, during which the changes in StO2 levels during the occlusion phase and post occlusion phase were recorded. Baseline Thenar StO2 levels (72 ± 8 vs 76 ± 5, p = 0.02) and time to baseline StO2 in seconds (150 ± 70 vs 200 ± 70, p = 0.007) were significantly lower in HF group compared to healthy control (HC). In addition, HF patients had a significantly lower trough StO2 (37 ± 9 vs 42 ± 11%, p = 0.04) and peak StO2 compared to HC (87 ± 8 vs 91 ± 5%, p = 0.01). However, there was no difference in the rate of desaturation, rate of resaturation or time to peak StO2 levels in between the 2 groups. Significant correlation was present between baseline Thenar StO2 levels and NYU Pediatric Heart Failure Index Score (NYU-PHFI) (p = 0.003). This study is the first to report an objective assessment of microcirculation and Thenar tissue oxygen dynamics in pediatric subjects with HF in comparison with HC. Our study suggests altered microcirculation and oxygenation patterns in these subjects as well as correlation with a validated pediatric heart failure clinical score. Large-scale prospective studies are needed to further study the utility of this novel technology in HF subjects.

A single centre experience with an evolving approach for the repair of coarctation of the aorta.

BACKGROUND: Isolated coarctation of the aorta can be repaired by either lateral thoracotomy or sternotomy approach with end-to-end anastomosis. Most commonly, neonates with coarctation of the aorta also have hypoplasia of the arch, requiring median sternotomy and extended end-to-side anastomosis with arch augmentation. The aim of this study was to describe our experience as the institution adopted the median sternotomy approach for repair, by reviewing complications, mortality, and reintervention. METHODS: Retrospective chart review of 66 patients aged 0-1 year who had arch repair performed by a single surgeon over an 8-year period was performed. Median age at surgery was 22 days (4-232) and median weight was 3.08 kg (1.25-8.0). Forty-one (62%) patients underwent median sternotomy. RESULTS: There was 1 death from a noncardiac cause. Eighteen per cent of our patients were ≤2.5 kg. Vocal cord paresis occurred in 16% of patients under 2.5 kg and 9.5% of patients 2.5 kg or above at the time of surgery. Hypertension at 6-month follow-up was greater in patients under 2.5 kg (44%) than patients 2.5 kg or above (15%). Total surgical reintervention rate was 6%. For patients above 2.5 kg, the surgical reintervention rate was 5.4% and for patients below 2.5 kg, the surgical reintervention rate was 8.3%. CONCLUSION: We concluded that for neonates with coarctation of the aorta and hypoplastic arch, median sternotomy is a safe surgical approach with low morbidity and mortality with the possible advantage of reduced surgical re-intervention and mortality in the population below 2.5 kg.

Effect of prenatal diagnosis on hospital costs in complete transposition of the great arteries.

OBJECTIVE: Prenatal diagnosis of congenital heart disease (CHD) is associated with improved clinical outcomes, yet its impact on the cost of hospitalization is not well described. We hypothesized that prenatal diagnosis of complete transposition of the great arteries (d-TGA) results in lower total hospital costs compared with postnatal diagnosis. METHODS: Retrospective analysis of infants with d-TGA repaired at our center from July 2006 to 2014. Total charges from initial hospitalization until discharge were converted to costs using the cost-to-charge ratio and then converted into 2016 dollars using the consumer price index. A direct cost comparison from the hospital perspective was performed between groups. A secondary analysis included the cost of prenatal diagnosis. RESULTS: Thirty-three infants with d-TGA were identified; 8 with and 25 without prenatal diagnosis. There was no difference in baseline characteristics. Mean direct cost of hospitalization was higher in infants without prenatal diagnosis ($108 014 ± $51 305 vs $88 305 ± $22 896, P = .31). On secondary analysis, the cost of prenatal diagnosis was negligible compared with total hospital cost. CONCLUSIONS: Total cost of initial hospitalization was higher for infants without prenatal diagnosis of d-TGA. Prenatal diagnosis not only improves clinical outcomes but may also be cost saving in the current era of increasing health care costs.

Maternal hyperoxygenation: A potential therapy for congenital heart disease in the fetuses? A systematic review of the current literature.

OBJECTIVES: To assess efficacy, safety, outcomes, and intrauterine complications following maternal hyperoxygenation (MH) therapy in fetuses with congenital heart disease (CHD). METHODS: A systematic review was performed following an electronic search of databases. Articles were published before January 1, 2017, in an English-language and non-English-language journals (with English translations), and included human fetuses and expectant mothers with a fetal diagnosis of CHD who received MH. RESULTS: Ninety-six articles were identified; 72 were excluded and 24 full-text articles were reviewed. Only 9 articles met inclusion criteria and were analyzed. A total of 270 fetuses underwent MH therapy: 169 had CHD, and 101 had normal heart anatomies. Seven studies used fetal echocardiography, while 2 studies used cardiac magnetic resonance imaging (CMR). The mean gestational age at therapy was 33.4 weeks (26-38 weeks). Majority of MH protocols used 100% FiO2 with non-rebreather face mask at 8 L of flow, achieving 60%-70% FiO2 , or maternal PaO2 goal of 250 mm Hg. No significant adverse events were reported. Four studies reported increased size of the hypoplastic cardiac structures after MH. Three studies utilized acute MH to risk stratify hypoplastic left heart syndrome fetuses. Two studies assessed acute MH in the setting of CMR. CONCLUSION: The current evidence for MH therapy suggests an increase in pulmonary blood flow, and venous return, ductal flow, and heart dimensions in fetuses. MH has potential as a diagnostic and therapeutic tool in fetuses with CHD. Further randomized controlled trials are needed to ascertain whether MH therapy provides improved outcomes on fetuses with certain types of CHD.

ß1-receptor polymorphisms and junctional ectopic tachycardia in children after cardiac surgery.

Junctional ectopic tachycardia (JET) is a potentially life-threatening postoperative arrhythmia in children with specific congenital heart defects and can contribute significantly to postoperative morbidity for at-risk populations. In adults, ß1-adrenergic receptor (ADRB1) and ß2-adrenergic receptor (ADRB2) genotypes have been associated with increased risk for arrhythmias. However, their association with arrhythmia risk in children is unknown. We aimed to test associations between ADRB1 and ADRB2 genotypes and postoperative JET in patients with congenital heart defects. Children who underwent cardiac surgery were genotyped for the ADRB1 p.Ser49Gly (rs1801252; c.145A>G), p.Arg389Gly (rs1801253; c.1165C>G), ADRB2 p.Arg16Gly (rs1042713; c.46A>G), and p.Glu27Gln (rs1042714; c.79G>C) polymorphisms. The occurrence of postoperative JET was assessed via cardiologist-interpreted electrocardiograms. Genotype associations with JET were analyzed via logistic regression, adjusted for clinical variables associated with JET, with separate analysis in patients not on a ß-blocker. Of the 343 children included (median age 8 months, 53% boys, 69% European ancestry), 45 (13%) developed JET. The Arg389Arg genotype was not significantly associated with JET in the overall population (odds ratio [OR] = 1.96, 95% confidence interval [CI] = 0.96-4.03, p = 0.064), but was nominally associated in patients not taking a ß-blocker (n = 324, OR = 2.25, 95% CI = 1.05-4.80. p = 0.034). None of the other variants were associated with JET. These data suggest that the ADRB1 Arg389Arg genotype may predict risk for JET following cardiac surgery in pediatric patients in the absence of ß-blockade. Whether treatment with a ß-blocker ameliorates this association requires further research.

A Comprehensive Approach to the Management of Patients With HLHS and Related Malformations: An Analysis of 83 Patients (2015-2021).

Background: Some patients with hypoplastic left heart syndrome (HLHS) and HLHS-related malformations with ductal-dependent systemic circulation are extremely high-risk for Norwood palliation. We report our comprehensive approach to the management of these patients designed to maximize survival and optimize the utilization of donor hearts. Methods: We reviewed our entire current single center experience with 83 neonates and infants with HLHS and HLHS-related malformations (2015-2021). Standard-risk patients (n = 62) underwent initial Norwood (Stage 1) palliation. High-risk patients with risk factors other than major cardiac risk factors (n = 9) underwent initial Hybrid Stage 1 palliation, consisting of application of bilateral pulmonary bands, stent placement in the patent arterial duct, and atrial septectomy if needed. High-risk patients with major cardiac risk factors (n = 9) were bridged to transplantation with initial combined Hybrid Stage 1 palliation and pulsatile ventricular assist device (VAD) insertion (HYBRID + VAD). Three patients were bridged to transplantation with prostaglandin. Results: Overall survival at 1 year = 90.4% (75/83). Operative Mortality for standard-risk patients undergoing initial Norwood (Stage 1) Operation was 2/62 (3.2%). Of 60 survivors: 57 underwent Glenn, 2 underwent biventricular repair, and 1 underwent cardiac transplantation. Operative Mortality for high-risk patients with risk factors other than major cardiac risk factors undergoing initial Hybrid Stage 1 palliation without VAD was 0/9: 4 underwent transplantation, 1 awaits transplantation, 3 underwent Comprehensive Stage 2 (with 1 death), and 1 underwent biventricular repair. Of 9 HYBRID + VAD patients, 6 (67%) underwent successful cardiac transplantation and are alive today and 3 (33%) died while awaiting transplantation on VAD. Median length of VAD support was 134 days (mean = 134, range = 56-226). Conclusion: A comprehensive approach to the management of patients with HLHS or HLHS-related malformations is associated with Operative Mortality after Norwood of 2/62 = 3.2% and a one-year survival of 75/83 = 90.4%. A subset of 9/83 patients (11%) were stabilized with HYBRID + VAD while awaiting transplantation. VAD facilitates survival on the waiting list during prolonged wait times.

Chest compression by two-thumb encircling method generates higher carotid artery blood flow in swine infant model of cardiac arrest.

OBJECTIVE: Two-Thumb(TT) technique provides superior quality chest compressions compared with Two-Finger(TF) in an instrumented infant manikin. Whether this translates to differences in blood flow, such as carotid arterial blood flow(CABF), has not been evaluated. We hypothesized that TT-CPR generates higher CABF and Coronary Perfusion Pressure(CPP) compared with TF-CPR in a neonatal swine cardiac arrest model. METHODS: Twelve anesthetized & ventilated piglets were randomized after 3 min of untreated VF to receive either TT-CPR or TF-CPR by PALS certified rescuers delivering a compression rate of 100/min. The primary outcome, CABF, was measured using an ultrasound transonic flow probe placed on the left carotid artery. CPP was calculated and end-tidal CO2(ETCO2) was measured during CPR. Data(mean ± SD) were analyzed and p-value ≤0.05 was considered statistically significant. RESULTS: Carotid artery blood flow (% of baseline) was higher in TT-CPR (66.2 ± 35.4%) than in the TF-CPR (27.5 ± 10.6%) group, p = 0.013. Mean CPP (mm Hg) during three minutes of chest compression for TT-CPR was 12.5 ± 15.8 vs. 6.5 ± 6.7 in TF-CPR, p = 0.41 and ETCO2 (mm Hg) was 29.0 ± 7.4 in TT-CPR vs. 20.7 ± 5.8 in TF-CPR group, p = 0.055. CONCLUSION: TT-CPR achieved more than twice the CABF compared with TF-CPR in a piglet cardiac arrest model. Although CPP and ETCO2 were higher during TT-CPR, these parameters did not reach statistical significance. This study provides direct evidence of increased blood flow in infant swine using TT-CPR and further supports that TT chest compression is the preferred method for CPR in infants.

Risk Factors for Seizures and Epilepsy in Children With Congenital Heart Disease.

OBJECTIVES: To identify potential risk factors for pre- and postoperative seizures and epilepsy in children with congenital heart disease. METHODS: Retrospective cohort study of neonates and infants <3 months of age with congenital heart disease who underwent cardiopulmonary bypass from November 24, 2006, until June 1, 2015. Children with seizures were classified based on time of occurrence into early preoperative, early postoperative, and late postoperative. Children with recurring seizures 30 days after cardiac surgery met criteria for epilepsy. RESULTS: 247 patients completed follow-up; 2.4% had seizures early preoperation and 1.6% early postoperation. Late postoperative epilepsy occurred in 5.3% of the cohort. The majority of seizures in the late postoperative epilepsy group started after 1 year of age (mean 1.53 years, range = 0.18-4.7 years). One of the 13 patients with epilepsy had a seizure during their intensive care unit hospitalization. Potential risk factors for seizures included brain injury (P < .001), high-risk surgery (Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery score ≥3, P = .024), and low birth weight (P < .04). Infants with stroke were more likely to develop epilepsy (P = .04). Presence of seizures was associated with increased length of stay (P < .001). CONCLUSIONS: Our study suggests an association between children with congenital heart disease diagnosed with stroke in the neonatal/infancy period and the development of epilepsy. These children may not have prior early pre- and postoperative seizures. Risk factors for seizures include brain injury, high-risk surgery, and lower birth weight. Seizures were associated with an increased length of stay but did not necessarily lead to subsequent epilepsy.

Using aptamers evolved from cell-SELEX to engineer a molecular delivery platform.

We report a chemically modified construct of the Sgc8 aptamer, selected against CEM cells, conjugated to an activator platform for stimulated release of molecules at the tumor surface using DNA template assisted functional group transfer reactions (DTGTR).

Impact of 3D Printouts in Optimizing Surgical Results for Complex Congenital Heart Disease.

Planning corrective and palliative surgery for patients who have complex congenital heart disease often relies on the assessment of cardiac anatomy using two-dimensional noninvasive cardiac imaging modalities (echocardiography, cardiac magnetic resonance imaging, and computed tomography scan). Advances in cardiac noninvasive imaging now include the use of three-dimensional (3D) reconstruction tools that produce 3D images and 3D printouts. There is scant evidence available in the literature as to what effect the availability of 3D printouts of complex congenital heart defects has on surgical outcomes. Surgical outcomes of study subjects with a 3D cardiac printout available and their paired control subject without a 3D cardiac printout available were compared. We found a trend toward shorter surgical times in the study group who had the benefit of 3D models, but no statistical significance was found for bypass time, cross-clamp time, total time, length of stay, or respiratory support. These preliminary results support the proposal that 3D modeling be made readily available to congenital cardiac surgery teams, for use in patients with the most complex congenital heart disease.

Severe Direct Hyperbilirubinemia as a Consequence of Right Heart Failure in Congenital Heart Disease.

Adult literature documents increased cholestasis in right heart failure yet is poorly documented in the pediatric population. We describe three infants with congenital heart disease who developed significantly elevated direct bilirubin levels of 43, 23, and 12 mg/dL, respectively, in the absence of hepatic dysfunction. The common hemodynamic pathophysiology in these infants is right heart dysfunction with moderate to severe tricuspid regurgitation in the setting of low perfusion state. Right heart dysfunction in infants can result in severe conjugated bilirubin, likely as a consequence of venous congestion and can be used as an indirect marker of right heart dynamics.

End-organ recovery post-ventricular assist device can prognosticate survival.

BACKGROUND: This study examines our institutional ventricular assist devices (VADs) experience over two decades to understand trends towards predictors of mortality. METHODS: Retrospective study of patients aged 0-21years supported with a VAD from January 1996 to May 2015. Patient data was examined pre and post-VAD implant among survivors and non-survivors. RESULTS: Thirty-six patients identified (8 supported by Thoratec® VAD and 28 supported by EXCOR Berlin Heart®). Patient's diagnosis included dilated cardiomyopathy (DCM) (n=19,53%), congenital heart disease (CHD) (n=12,33%), and other (n=5,14%). Median age and body surface area (BSA) were 1.0years[0-7years] and 0.41[0.24-0.92], respectively. Survival to discharge was 75% with no deaths with DCM. The survival rate for patients with CHD was 42%. Univariate analysis showed diagnosis of CHD, smaller BSA and respiratory failure post-implant (Intermacs criteria) as risk factors for mortality. Median duration of VAD support was lower in non-survivors, 14 vs 63days (p=0.03). Renal function at time of transplant or death was normal/pRIFLE Risk category in 20(74%) of survivors and 2(22%) of non-survivors (p=0.06). Post-implant, peak total bilirubin in the first week trended lower in survivors (p=0.06). CONCLUSIONS: Persistent end-organ impairment in the first 2weeks after VAD placement could be a useful prognostic marker for survival to transplant.