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Various techniques and electrode catheters have been developed for effective and safe temporary transvenous cardiac pacing. In neonates and children, lead dislodgment and myocardial perforation have been attendant risks with passive fixation leads. We report our experience with the recently introduced Medtronic 6416 active fixation bipolar lead in a small group of children. Between January 2000 and February 2002, 4 children (2 boys, 2 girls) underwent temporary transvenous pacing with the Medtronic 6416 lead. The median age at implantation was 4.25 years (range: 5 days-13 years), median weight was 14.5 kg (range: 2.5 kg-54 kg) and the median duration of temporary pacing was 19.5 days (range: 5-38 days). The indication for temporary pacing was AV block (n = 2), sinus arrest (n = 1) and sinus node dysfunction (n = 1). The acute pacing threshold was <1.5 mA with no significant rise in the follow-up period. The venous access for lead placement was femoral vein (n = 2), internal jugular vein (n = 1), and subclavian vein (n = 1). There were no complications related to implantation or in the follow-up period. The Medtronic 6416 lead appears to be a safe and effective tool in temporary transvenous pacing in neonates and children. [ABSTRACT FROM AUTHOR]

The primary objective of this study was to determine whether or not hemodynamic parameters and laboratory values at the time of admission to the pediatric cardiac intensive care unit after the Norwood operation were associated with a composite outcome of either need for extracorporeal membrane oxygenation or inpatient mortality. This was a single-center retrospective study of infants with functionally univentricular hearts admitted to intensive care after the Norwood procedure from January 2011 to January 2020. Data were obtained at a single point (after a Norwood procedure) and then compared between two subsets of patients based on the presence or not of the composite outcome of interest. In univariate and multiple regression analyses, a series of receiver operator curves were generated to assess the relationship between the variables of interest and the composite outcome. Eight (7.6%) experienced the composite outcome out of a total of 104 patients. Those who experienced the composite endpoint had significantly higher oxygen extraction ratio (0.43 vs. 0.31, p = 0.01), lower systemic blood flow (2.5 L/min versus 3.1 L/min, p = 0.01), and higher systemic vascular resistance (20.2 indexed woods units versus 14.8 indexed woods units, p = 0.01). Those with systemic blood flow of less than 2.5 L/min/m 2 had a 17% risk of experiencing the composite endpoint AUC = 0.79. Those with systemic vascular resistance of greater than 19 indexed woods units had a 22% risk of experiencing the composite endpoint AUC 0.80. Systemic blood flow and systemic vascular resistance are independently associated with this composite outcome., (© 2022. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

We report a rare case of hereditary hemorrhagic telangiectasia (HHT) in a 4-month-old female infant with findings of child abuse. She presented with poor feeding, vomiting, and irritability after a short fall from the bed. Initial evaluation found subdural hematomas, persistent hypoxia, failure to thrive, a frenulum tear, facial lacerations, and bruising. The patient was admitted, and an extensive workup led to the diagnosis of brain and pulmonary arteriovenous malformations and finally the diagnosis of HHT. The subdural hematomas, cutaneous injuries, and oral injury were highly suspicious for child abuse and were reported to Child Protective Services and law enforcement for investigation simultaneous to the medical work-up. Her hospital course was complicated by progressive hypoxemia with radiographic evidence of several large pulmonary arteriovenous malformations, for which she underwent successful embolization. Her head injury was indeterminate for physical abuse in the setting of a medical condition predisposing to intracranial hemorrhage. A few weeks later, she was readmitted with repeat abusive injuries in the form of femur fractures. This case demonstrates the unique diagnostic dilemma when 2 diagnoses are occurring simultaneously-HHT and child abuse-and showcases the importance of a detailed family history, genetic testing, strong multidisciplinary collaboration with a holistic approach and medically informed Child Protective Services systems to ensure accurate diagnoses and safe disposition., Competing Interests: The authors declare no conflicts of interest., (© 2024 The Author(s).)

Background: There are a variety of approaches to biventricular repair in neonates and infants with adequately sized ventricles and left-sided obstruction in the presence of a ventricular septal defect. Those who undergo this in a staged manner initially undergo a Norwood procedure followed by a ventricular septal defect closure such that the neo-aorta is entirely committed to the left ventricle and placement of a right ventricular to pulmonary artery conduit (Yasui operation). This study aimed to determine clinical and haemodynamic factors upon paediatric cardiac ICU admission immediately after the two-stage Yasui operation that was associated with post-operative length of stay., Methods: This was a retrospective review of patients who underwent the Yasui procedure after the initial Norwood operation between 1 January 2011 and 31 December 2020. Patients with complete data on admission were identified and analysed using Bayesian regression analysis., Results: A total of 15 patients were included. The median age was 9.0 months and post-operative length of stay was 6days. Bayesian regression analysis demonstrated that age, weight, heart rate, mean arterial blood pressure, central venous pressure, pulse oximetry, cerebral near infrared spectroscopy, renal near infrared spectroscopy, pH, pCO2, ionised calcium, and serum lactate were all associated with post-operative length of stay., Conclusion: Discrete clinical and haemodynamic factors upon paediatric cardiac ICU admission after staged Yasui completion are associated with post-operative length of stay. Clinical target ranges can be developed and seem consistent with the notion that greater systemic oxygen delivery is associated with lower post-operative length of stay.

Hereditary hemorrhagic telangiectasia (HHT), also known as Osler-Weber-Rendu disease, is a rare disorder with a case prevalence as high as one in 5000, causing arteriovenous malformations in multiple organ systems. HHT is familial with autosomal dominant inheritance, with genetic testing allowing confirmation of the diagnosis in asymptomatic kindreds. Common clinical manifestations are epistaxis and intestinal lesions causing anemia and requiring transfusions. Pulmonary vascular malformations predispose to ischemic stroke and brain abscess and may cause dyspnea and cardiac failure. Brain vascular malformations can cause hemorrhagic stroke and seizures. Rarely, liver arteriovenous malformations can cause hepatic failure. A form of HHT can cause juvenile polyposis syndrome and colon cancer. Specialists in multiple fields may be called to care for one or more aspects of HHT, but few are familiar with evidence-based guidelines for HHT management or see a sufficient number of patients to gain experience with the unique characteristics of the disease. Primary care physicians and specialists are often unaware of the important manifestations of HHT in multiple systems and the thresholds for their screening and appropriate management. To improve familiarity, experience, and coordinated multisystem care for patients with HHT, the Cure HHT Foundation, which advocates for patients and families with this disease, has accredited 29 centers in North America with designated specialists for the evaluation and care of patients with HHT. Team assembly and current screening and management protocols are described as a model for evidence-based, multidisciplinary care in this disease.

Despite the controversy, sodium bicarbonate is a commonly used medication in critically ill patients of all ages. There is a lack of data on the acute impact on hemodynamic parameters, biomarker indicators of cardiac output, and changes in vasoinotropic support after sodium bicarbonate therapy. In our retrospective study on children with biventricular circulation in pediatric cardiac intensive care unit receiving bicarbonate therapy: we analyzed its effects on arterial blood gases, heart rate, blood pressure (BP), central venous pressures (CVP), cerebral and renal near-infrared spectroscopy (NIRS), changes in vasoinotropic and ventilator changes before and after sodium bicarbonate administration. Thirty-one administrations of sodium bicarbonate in 23 patients with congenital heart disease without residual shunts were analyzed. The average age was 15.4 months, weight 7.7 kg, and the average bicarbonate dose was 1 meq/kg. There was an increase in arterial pH from 7.24 to 7.30 (p = 0.14) and bicarbonate changed from 18 to 20 mEq/L (p = 0.23). No clinically significant changes were found in the following parameters: heart rate (141 ± 20.1 to 136 ± 19), systolic BP (84 ± 17 to 86 ± 14 mmHg), diastolic BP (48 ± 12 to 49 ± 12 mmHg), cerebral NIRS (64 ± 12 to 65 ± 12), renal NIRS (80 ± 10 to 81 ± 7), CVP (9 ± 3 to 10 ± 4 mmHg), paCO 2 (45 ± 26 to 42 ± 7 mmHg), paO 2 (143 ± 78 to 127 ± 59 mmHg), serum lactate (2.2 ± 2.7 to 3.6 ± 3.8 mmol/L), and vasoinotropic score (7.5 ± 5.0 to 7.7 ± 4.7). Outside of a change in serum pH and bicarbonate levels no other significant changes were noted after sodium bicarbonate administration in children with congenital heart disease with fully septated, biventricular circulation. There was no improvement in systemic oxygen delivery., (© 2022. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

A patent foramen ovale (PFO) is a frequent incidental finding during echocardiography in otherwise healthy children. In most healthy children with a diagnosis of isolated incidental PFO, no further follow-up or intervention is necessary. In some children, PFO is associated with certain clinical syndromes such as cryptogenic stroke, decompression sickness, migraine, and platypnea-orthodeoxia syndrome. This review discusses PFO anatomy, diagnostic imaging, PFO-associated clinical situations, management options, and the role of PFO in certain congenital heart disease. This review also highlights the current deficiency of pediatric data guiding management of these uncommon but important PFO-associated conditions. Future multicenter randomized controlled studies are necessary to guide the management of these unique and challenging PFO-associated conditions., Competing Interests: Seshadri Balaji serves as a consultant for yoR labs and Milestone Pharmaceuticals., (Copyright: © 2022 Annals of Pediatric Cardiology.)

Background: Patients with end-stage heart failure possess many attributes that place them at risk for prolonged mechanical ventilation (MV). However, there are only limited data on MV support among children after ventricular assist device (VAD) implantation. We report the duration of MV after VAD placement, indications for respiratory support in the postimplantation period, and associated patient factors., Methods: This single-center retrospective study included 43 consecutive children (aged <18 years) with end-stage heart failure who were supported with a VAD as a bridge to transplantation from January 2005 to December 2011. Multivariable analysis was performed using the multiple Poisson regression model for the duration of MV., Results: Overall, 33% (n = 14) remained on MV until heart transplant or death. Of those requiring pre-VAD extracorporeal membrane oxygenation (ECMO) support, 63% (n = 12 of 19) remained on MV until heart transplant or death compared with 8% (n = 2 of 24) among those not on ECMO before VAD (p < 0.001). Patients with moderate or severe mitral regurgitation while on VAD support had 1.7-times more MV days compared with those with none or trivial on-VAD mitral regurgitation. In addition, previous support on ECMO, those with moderate or severe tricuspid regurgitation, and those with only left VAD implants had an increased risk of prolonged MV., Conclusions: Our results suggest that VAD recipients previously supported on ECMO, those with moderate or severe mitral regurgitation, moderate or severe tricuspid regurgitation, and those with only left VAD implants had an increased risk of prolonged MV. Future studies in larger cohorts are necessary to confirm the findings from this single-institutional experience., (Copyright © 2015 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Hyperfibrinogenemia, which can create a procoagulant milieu, is frequently observed in patients supported with the Berlin EXCOR (Berlin Heart GmbH, Berlin, Germany) ventricular assist device (VAD). We began initiating corticosteroids in patients with systemic inflammatory response syndrome (SIRS) episodes to mitigate hyperfibrinogenemia. We set forth to describe the impact of corticosteroids on the hyperfibrinogenemic state in our institutional experience., Methods: Retrospective data was collected on 44 consecutive patients implanted with the Berlin EXCOR VAD from April 15, 2005 through May 6, 2013. Pertinent information was abstracted from the electronic medical record. The reduction of C-reactive protein (CRP) and fibrinogen levels among days from corticosteroid treatment were described. Infections and insulin use were reported based on whether patients received steroids and if steroids were given for SIRS., Results: Over the initial 44 Berlin EXCOR VAD implantations, 14 patients were treated with 21 courses of corticosteroids for SIRS episodes as identified by clinical features and rise in CRP. Treatment with corticosteroids reduced fibrinogen levels by day 2 to a statistically significant degree (p = 0.008). No difference in hyperglycemia or infections occurred among patients receiving corticosteroids for SIRS., Conclusions: Treatment with corticosteroids can potentially mitigate the SIRS response among children supported on the Berlin EXCOR VAD. In patients who received corticosteroids to mitigate inflammation, there was no increase in infections or hyperglycemia requiring insulin administration compared with patients who did not receive steroids., (Copyright © 2015 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Discrepancy between clinical and autopsy diagnosis in children supported on extracorporeal membrane oxygenation (ECMO) has not been previously described. To assess the utility of autopsy examination in children supported on ECMO and assess discrepancies between premortem and postmortem diagnosis in these patients. Retrospective chart review. General pediatric and cardiac intensive care units (ICUs) in a tertiary children's hospital. The hospital's ECMO database was queried for patients supported on ECMO from 2000 through 2010 who died and underwent autopsy examination. Fifty-four autopsies were performed in 139 nonsurvivors (28%) who required ECMO support in the pediatric and cardiac ICU. Major discrepancies between premortem and postmortem diagnoses were found in 29 patients (53.7%). The commonest missed diagnosis was myocardial infarction that occurred in 16 patients, followed by adrenal hemorrhage in three patients. Five patients with a cardiac diagnosis had both major (type 1 discrepancy) and minor (type 2 discrepancy) discrepancies. Surgical complications were noted in four postmortem study with three of them being class 1 discrepancy. We report significant discrepancy between autopsy and clinical findings among ECMO-supported pediatric patients. Our findings underscore the need for enhanced premorbid surveillance in patients supported on ECMO.

Airway abnormalities are frequently associated with congenital heart disease in infants and children. Respiratory distress in these children frequently requires prolonged respiratory support. Wide-detector dynamic pulmonary computed tomography angiography (DP-CTA) is a non-invasive technique that completely evaluates vascular and airway abnormalities during a single breathe. Our purpose was to evaluate the efficacy of DP-CTA to provide unique actionable information for patient care in newborns and infants with congenital heart disease and persistent respiratory distress. 23 infants with congenital heart disease and persistent respiratory distress underwent DP-CTA. All were intubated at the time of the examinations. The most common cardiac anomalies were tetralogy of Fallot (6) and hypoplastic left heart syndrome variants (5). The most common cardiac surgeries prior to DP-CTA were Norwood (4) and hybrid (3) procedures. The protocol for DP-CTA for intubated infants is four gantry rotations in 1.4 s after intravenous contrast injection. 3D and multiplanar reconstruction with cine loops were created for combined cardiopulmonary imaging. Tracheobronchomalacia was present in 17 children. Lobar bronchomalacia was identified in six children. Branch pulmonary artery stenosis was the most common vascular finding (10 children). Medical management was changed or a surgical procedure performed based on the information resulting from DP-CTA in 16 of the 23 patients (70 %). DP-CTA is non-invasive, fast and provides unique information for the management of infants with congenital heart disease and persistent respiratory distress. DP-CTA is uniquely suited for comprehensive and simultaneous evaluation of airway and vascular abnormalities in infants.

Antithrombin III (ATIII) is used during extracorporeal membrane oxygenation (ECMO) based on physiologic rationale and studies during cardiopulmonary bypass. In February 2008, our institution began using ATIII as replacement for low ATIII activity (<70%) in patients supported with ECMO. We hypothesized that ATIII supplementation would reduce heparin infusion rates, increase unfractionated heparin anti-Xa levels, and prolong ECMO circuit life. Data from 40 consecutive patients (45 deployments) requiring ECMO support for >72 hours with venoarterial ECMO from January 1, 2007, through December 31, 2008, were collected. Antithrombin III concentrate was administered for ATIII activity <70% at the discretion of the attending physician. The primary outcome was whether the heparin infusion rate was reduced by 10% or more as a result of ATIII administration. No difference in heparin infusion rate (p = 0.245) as a result of ATIII administration was observed. Anti-Xa levels were lower before ATIII administration (p< 0.001) and were increased after ATIII administration (p < 0.001). There was an increased frequency of circuit failure in ATIII treatment group compared with nontreatment group (p = 0.018). Neither heparin responsiveness nor circuit life was enhanced by daily ATIII supplementation for activity <70%. Future studies are warranted to evaluate the effectiveness of antithrombin replacement.

Background: Cerebrovascular events (CVEs) are common among children supported with the Berlin EXCOR (Berlin Heart GmbH, Berlin, Germany) ventricular assist device (VAD). Given the high incidence of CVEs associated with this device, we sought to describe our institutional experience in incrementally reducing CVEs in children supported with the Berlin EXCOR VAD., Methods: We collected pertinent data on 39 consecutive patients who underwent Berlin EXCOR VAD implantation at a single center. Frequency of CVEs was described in risk per implantation, per day, and in reference to the time of therapeutic anticoagulation. Risk factors were analyzed for association with CVEs., Results: Of the initial 39 Berlin EXCOR VAD implantations, 16 CVEs occurred in 12 patients. The incidence of CVEs decreased with institutional experience per patient (R(2) = 0.6909, p = 0.007) and per patient-day (R(2) = 0.8051, p = 0.002). CVEs occurred more frequently before therapeutic anticoagulation targets were achieved (4.1%/day) compared with after therapeutic anticoagulation targets were achieved (0.9%/day; p = 0.044)., Conclusions: Incidence of CVEs decreased with institutional experience. The risk of CVE is highest in the immediate postoperative period before therapeutic anticoagulation is achieved. Further studies are warranted in pediatric patients supported with the Berlin EXCOR VAD to confirm our findings in a larger cohort., (Copyright © 2013 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Major congenital heart disease (CHD) is seen in 10% to 15% of patients with congenital diaphragmatic hernia (CDH). Some neonates with CDH and major CHD are treated by surgery and some with extracorporeal membrane oxygenation (ECMO). Because of presumed poor survival, there is significant heterogeneity in management approaches for patients with CDH and associated CHD, and there is no published outcome data of patients who were placed on ECMO support., Methods: To examine outcomes of children with CDH with CHD supported with ECMO, Extracorporeal Life Support Organization (ELSO) registry was queried from 1998 to May 2010. There were a total of 3,342 deployments for CDH and 316 (9.5%) neonates with associated CHD. The median values are as follows: age at presentation, 1 (0 to 52) days; gestational age, 38 (29 to 42) weeks; birth weight, 3 (1.35 to 4.7) kg; admission to time to ECMO, 16 (0 to 1,220) hours; duration of ECMO, 194 (3 to 823) hours; time off of ECMO to extubation, 366 (0 to 7,934) hours; and time off of ECMO to death was 114 (0 to 7,272) hours., Results: Initially, ECMO support was venoarterial in 91% (283 of 316), 5 were converted from venovenous to venoarterial ECMO. Overall survival to hospital discharge for all patients with CDH and CHD was 47% (148 of 316). Survival to hospital discharge for hypoplastic left heart syndrome and single-ventricle physiology was 55% (33 of 60), 48% (43 of 89) for ventricular septal defect, and 40% (24 of 60) for coarctation of the aorta patients., Conclusions: Patients with CDH and CHD supported with ECMO have had better than predicted short-term outcomes. In this cohort, overall survival of patient's with CDH with or without CHD was similar. Patients with single-ventricle physiology had similar short-term outcome to those with 2 ventricle physiology., (Copyright © 2013 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

A 10-year-old boy who was receiving support from a ventricular assist device (VAD) experienced heparin-induced thrombocytopenia that was successfully treated with high-dose argatroban infusion to attain therapeutic activated partial thromboplastin time in spite of high serum argatroban levels. This case also highlights bolus argatroban dosing for VAD change in the setting of persistent ventricular fibrillation., (Copyright © 2013 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Some neonates with tetralogy of Fallot (TOF) have rapid progression of right ventricular outflow tract obstruction, requiring early repair irrespective of Doppler gradient as measured in the neonatal period. The aim of this study was to test the hypothesis that infundibular morphology in neonates with TOF is associated with the occurrence of hypercyanotic spells and need for neonatal surgery., Methods: Fifty patients with TOF undergoing surgical repair from 2003 to 2009 were studied. Neonatal echocardiograms were retrospectively analyzed to measure conal septal angle (the angle between the conal septum and the horizontal plane passing through the center of the aortic valve in the parasternal short-axis view, with a larger angle denoting more anterocephalad deviation of conal septum), conal septal thickness and length, the degree of aortic dextroposition, and sizes and Z scores of the pulmonary annulus and the main and branch pulmonary arteries. Outcomes included the occurrence of hypercyanotic spells and the need for neonatal surgery., Results: The median age at first echocardiogram was 2 days (range, 0-12 days). The median age at surgery was 94 days (range, 5-282 days); hypercyanotic spells occurred in 17 patients (34%), and nine (18%) underwent neonatal repair. The presence of a wider conal septal angle was significantly associated with the occurrence of hypercyanotic spells (59 ± 21° vs 48 ± 13°, P = .023) and the need for neonatal surgery (67 ± 13° vs 48 ± 16°, P = .004). The positive and negative predictive values of hypercyanotic spells for conal septal angles ≥60° were 64% and 78%, respectively. Importantly, Doppler right ventricular outflow tract gradient at initial echocardiography, degree of aortic dextroposition, and pulmonary or aortic valve size were not associated with these outcomes., Conclusions: A wider conal septal angle is associated with the occurrence of hypercyanotic spells and the need for neonatal surgery., (Copyright © 2013 American Society of Echocardiography. Published by Mosby, Inc. All rights reserved.)

Objective: Infants are potentially more susceptible to cell death mediated via glutamate excitotoxicity attributed to cardiopulmonary bypass. We hypothesized that ketamine, via N-methyl D-aspartate receptor blockade and anti-inflammatory effects, would reduce central nervous system injury during cardiopulmonary bypass., Methods: We randomized 24 infants, without chromosomal abnormalities, to receive ketamine (2 mg/kg, n = 13) or placebo (saline, n = 11) before cardiopulmonary bypass for repair of ventricular septal defects. Plasma markers of inflammation and central nervous system injury were compared at the end of surgery, and 6, 24, and 48 hrs after surgery. Magnetic resonance imaging and spectroscopy before cardiopulmonary bypass and at the time of hospital discharge were performed in a subset of cases and controls (n = 5 in each group). Cerebral hemodynamics were monitored postoperatively using near-infrared spectroscopy, and neurodevelopmental outcomes were assessed using Bayley Scales of Infant Development-II before and 2-3 wks after surgery., Results: Statistically significant differences were noted in preoperative inspired oxygen levels, intraoperative cooling and postoperative temperature, respiratory rate, platelet count, and bicarbonate levels. The peak concentration of C-reactive protein was lower in cases compared to controls at 24 hrs (p = .048) and 48 hrs (p = .001). No significant differences were noted in the expression of various cytokines, chemokines, S100, and neuron-specific enolase between the cases and controls. Magnetic resonance imaging with spectroscopy studies showed that ketamine administration led to a significant decrease in choline and glutamate plus glutamine/creatine in frontal white matter. No statistically significant differences occurred between pre- and postoperative Bayley Scales of Infant Development-II scores., Conclusions: We did not find any evidence for neuroprotection or neurotoxicity in our pilot study. A large, adequately powered randomized control trial is needed to discern the central nervous system effect of ketamine on the developing brain. brain., Trial Registration: The trial is registered at www.ClinicalTrials.gov, NCT00556361.

Introduction: Neonates frequently require access to their central blood vessels. However, limited data exist relating to the size and the anatomical relation of the femoral and neck vessels for neonates of different postmenstrual ages., Hypothesis: 1) The size of central blood vessels increases with postmenstrual age of the neonate. 2). External rotation with abduction at the hip will decrease the degree of overlap between the femoral artery and vein. 3) The degree of overlap decreases with increasing postmenstrual age., Design: Prospective descriptive cohort study., Measurements and Main Results: Femoral and neck vessel sizes were assessed using ultrasound for three postmenstrual age groups: group A (26 ± 1 wks), group B (32 ± 1 wks), and group C (38 ± 1 wks). The degrees of overlap (major, >50% overlap; minor, ≤50% overlap; no overlap) between the femoral vessels were assessed at the level of the inguinal ligament and 1 cm below the inguinal ligament in a straight hip and in external rotation with abduction of the hip positions. A total of 52 nonconsecutive subjects (group A, seven; group B, 21; group C, 24) were studied. The mean blood vessel dimensions increased with increasing postmenstrual age. Correlation of blood vessel size to growth measurements was better in group A + group B compared to group C. Overlap between the femoral vein and the femoral artery across the neonatal age groups at the level of the inguinal ligament ranged from 57% to 79% and from 43% to 98% at 1 cm below the inguinal ligament. The degree of overlap did not decrease with positioning of the lower extremity in external rotation with abduction of the hip. In the neck blood vessels, the majority of observations showed either minor or major overlap of neck blood vessels in all three groups (group A, 79%; group B, 86%; group C, 90%)., Conclusions: Central blood vessel size increases with increasing postmenstrual age. Correlation of blood vessel size to anthropometric measurements was better in the premature neonates compared to term neonates. A high degree of overlap exists within the femoral and cervical blood vessels. In the femoral vessels, the degree of overlap did not decrease with external rotation with abduction of the hip at any postmenstrual age.

The objective of this study was to investigate the effect of timely peritoneal dialysis (PD) catheter in children with abdominal compartment syndrome (ACS) while supported on extracorporeal membrane oxygenation (ECMO). We present a case series of four patients who developed significant intraperitoneal fluid accumulation and ACS at the general pediatric and cardiac intensive care units in a tertiary children's hospital. The hospital's ECMO database was queried for patients supported on ECMO who required PD catheter placement. These patients were assessed for clinical characteristics and outcomes. Four patients were identified with capillary leak syndrome associated with a primary diagnosis: cardiac transplant rejection in one, septic shock and acute respiratory distress syndrome in two, and neonatal hydrops fetalis in one patient. In each of these patients, a PD catheter was placed for severe abdominal distension and proven/suspected ACS. There was dramatic improvement in venous return after drainage of peritoneal fluid. Two patients were subsequently able to be separated successfully from ECMO support. One patient died of acute neurologic complication and the other because of severe gastrointestinal bleeding. After ruling out common causes for decreased venous return, ACS should be suspected as one of the important causes, especially in patients with massive capillary leak and increasing abdominal distension, among patients supported on ECMO. Timely placement of a PD catheter in patients who develop abdominal distension and ACS can substantially improve venous return and thus help maintain adequate tissue perfusion by improving ECMO flows.

Objectives: To describe the experience with extracorporeal membrane oxygenation support for intractable primary arrhythmias in newborns and infants., Design: Retrospective study., Setting: A tertiary care pediatric hospital., Patients: Patients younger than 1 yr supported with extracorporeal membrane oxygenation for primary cardiac arrhythmias were identified from the institutional extracorporeal membrane oxygenation registry., Interventions: Extracorporeal membrane oxygenation support., Measurements and Main Results: Clinical characteristics and outcomes were investigated for patients with primary cardiac arrhythmia supported with extracorporeal membrane oxygenation. Outcomes investigated were time from initiation of extracorporeal membrane oxygenation support to arrhythmia control, duration of extracorporeal membrane oxygenation support, and results of interventions performed while supported with extracorporeal membrane oxygenation. We summarized the independent categorical and continuous variables using frequencies, percentages, and medians and ranges, respectively. Extracorporeal membrane oxygenation support was used in nine patients for rescue therapy for primary tachyarrhythmia and bradycardia. The primary arrhythmias were: focal atrial tachycardia (n = 2); reentrant supraventricular tachycardia (n = 3); junctional ectopic tachycardia (n = 2); and congenital complete atrioventricular block (n = 2) patients. Seven patients presented with severe hemodynamic compromise, with six patients requiring extracorporeal cardiopulmonary resuscitation. All patients required extracorporeal membrane oxygenation within 24 hrs of initial presentation. Balloon atrial septostomy was performed in three patients and ablation was performed in two patients. Sinus rhythm was achieved in all reentrant supraventricular tachycardia and rate control was established in both patients with focal atrial tachycardia and in one patient with junctional ectopic tachycardia while using extracorporeal membrane oxygenation support. All patients survived to hospital discharge, and median follow-up for the cohort was 5 yrs. There was one late death; all survivors had good overall and neurologic outcomes., Conclusions: The requirement of extracorporeal membrane oxygenation support in newborns and infants with intractable arrhythmia is rare. Extracorporeal membrane oxygenation support does potentially carry morbidity; however, to prevent arrhythmia-related mortality, extracorporeal membrane oxygenation support and/or extracorporeal cardiopulmonary resuscitation should be considered in the management of hemodynamically unstable primary arrhythmias as an emergent lifesaving procedure.

Objective: To investigate if the low dose (1 µg) ACTH stimulation test appropriately assesses adrenal responsiveness in neonates undergoing open-heart surgery requiring cardio-pulmonary bypass., Design: In this retrospective study, adrenal axis response was assessed on the first post-operative day with the low-dose (1 µg) ACTH stimulation test. Age, gender, weight, RACHS category, inotrope score, and baseline and post-stimulation cortisol levels were collected. The association between basal serum cortisol levels and degree of response to the ACTH stimulation test was also investigated., Setting: Tertiary care referral center., Patients: Twenty-one neonates who underwent neonatal cardiac surgery on cardiopulmonary bypass and underwent an ACTH stimulation test. Interventions.  Hydrocortisone 50 mg/m(2) bolus in four divided doses daily., Outcome Measures: Response to the low dose (1 µg) ACTH stimulation was assessed., Results: All neonates with hemodynamic instability in the immediate post-operative period had low basal serum cortisol levels. The basal mean serum cortisol level for the 21 patients who underwent the low dose ACTH stimulation test was 7.3 µg/dL (median 2.2, range 0.7-42). The mean serum cortisol level increased after the ACTH stimulation test in the 21 patients to 39.6 µg/dL (median 38, range 79-17). The mean inotrope score in the first 24 hours after surgery was 24 (median 17.5, range 7-76.5) and decreased to 17 (median 14, range 5-52.3) 24-48 hours after surgery. At 48 hours post-surgery the mean arterial pressure in the groups with a serum cortisol increase after ACTH stimulation (<30 µg/dL vs. >50 µg/dL) was significantly different (P value 0.026)., Conclusions: The low dose (1 µg) ACTH stimulation test is a valid test to assess adrenal responsiveness among neonates after open heart surgery requiring CPB. Traditionally used basal serum cortisol level cutoff of <20 µg/dL used to define relative adrenal insufficiency may not be applicable in neonates undergoing open heart surgery on CPB thus indicating the need for re-defining adrenal insufficiency in this patient population.

Objectives/hypothesis: Propranolol has recently been introduced as a novel pharmacologic treatment for infantile hemangiomas. Systematic examination of this treatment in a tertiary care setting has not been described. This study explores the impact of propranolol on both proliferative and involuting hemangiomas at a tertiary vascular anomalies center., Study Design: Retrospective single institution review., Materials and Methods: We reviewed children treated with propranolol for problematic hemangiomas followed by a blinded prospective analysis of serial photographs taken during the course of their therapy. Parental questionnaires were obtained to evaluate perceived therapeutic response and complications to oral propranolol., Results: Thirty-two children with complete photo documentation were treated with oral propranolol for infantile hemangiomas between September 2008 and June 2009. Twenty-seven patients began therapy during the proliferative phase of their lesions (mean age, 4.9 months), whereas five patients began during the involutional phase (mean age, 19.4 months). Ninety-seven percent of patients displayed improvement in the quality of their hemangiomas during propranolol therapy. Patients were determined to be excellent responders (n = 16, 50%), partial responders (n = 15, 47%), or nonresponders (n = 1, 3%). Partial and nonresponders received adjuvant therapy (75%, laser therapy; 31%, steroid injections). Ten patients experienced minor but reportable side effects to propranolol, including somnolence (27.2%), gastroesophageal reflux (9.1%), respiratory syncytial virus exacerbation (4.5%), and rash (4.5%)., Conclusions: Propranolol may revolutionize the treatment of problematic hemangiomas that cause imminent functional or cosmetic sequelae. At therapeutic doses, propranolol is safe and effective in the majority of patients. Adjunctive therapies may still be required. Minor side effects, expected from beta-blocker therapy, are common but easily managed.

Objective: Early postoperative hyperlactatemia is seen in some children after surgical repair of secundum atrial septal defect despite apparently normal cardiac output. The objective of the study was to investigate the intraoperative risk factors for hyperlactatemia in patients undergoing atrial septal defect repair., Methods and Results: A retrospective review of 68 consecutive patients who underwent isolated atrial septal defect repair at Arkansas Children's Hospital between January 2001 and March 2006 was performed. Perioperative factors in the high lactate group (lactate >3 mmol/L, n = 26) were compared with those in the low lactate group (n = 42). Early hyperlactatemia was seen in 38% of the cohort. The high lactate group showed significantly lower weight-indexed cardiopulmonary bypass flow rate (101 + or - 6.5 mL/kg(-1)/min(-1) vs 131 + or - 6.0 mL/kg(-1)/min(-1), P = .0013), oxygen delivery during cardiopulmonary bypass (mean 12.7 + or - 0. 7 mL/kg(-1)/min(-1) vs 17.0 + or - 1 mL/kg(-1)/min(-1), P = .0009), and higher postoperative glucose (191 + or - 8.6 mg/dL vs 151 + or - 5.4 mg/dL, P = .003) compared with the LL group. Multivariate logistic regression analysis showed that weight-indexed cardiopulmonary bypass flow rate (P = .007) and average mean arterial blood pressure during cardiopulmonary bypass (P = .009) were independent risk factors for postoperative hyperlactatemia. Cardiopulmonary bypass flow rate less than 100 mL/kg(-1)/min(-1) was associated with an odds ratio of 7.67 (95% confidence interval, 1.28-45.86; P = .026) for postoperative hyperlactatemia., Conclusion: Lower weight-indexed cardiopulmonary bypass flow rate is an independent risk factor for early postoperative hyperlactatemia in children after atrial septal defect repair., (Copyright 2010 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

Objective: Lymphopenia is a predictor of adverse clinical outcomes in adults with various systemic diseases. We hypothesized that preoperative absolute lymphopenia (absolute lymphocyte count of less than 3000 cells/microL) is associated with adverse postoperative outcomes in children with congenital heart disease undergoing corrective or palliative surgery on cardiopulmonary bypass during the first 2 years of life., Methods: A retrospective single center cohort study was performed. Categorical variables were analyzed with the chi(2) test. Preoperative variables were analyzed with logistic and linear regression analysis to determine whether they were associated with adverse outcomes., Results: Analysis was performed on 280 patients, of whom 124 were female and 156 were male. Seventy-one patients were neonates (< or =30 days) at the time of the operation. Ninety patients had an absolute lymphocyte count of less than 3000 cells//microL before the operation. Regression models showed that RACHS-1 categories 5 and 6, age, and preoperative lymphopenia were significantly associated with postoperative mortality (P < .0006). Within RACHS-1 groups, lymphopenia remained a significant predictor of mortality for patients in RACHS categories 3 and 4. Lymphopenia and age were associated with longer length of stay and length of mechanical ventilation within RACHS categories 1 to 4 (P < .05). Preoperative lymphopenia was the only predictor of use of postoperative nitric oxide (P < .05)., Conclusions: Preoperative lymphopenia is a predictor of adverse postoperative outcomes in children with congenital heart disease who undergo a corrective or palliative procedure with cardiopulmonary bypass during the first 2 years of life.

Infantile hemangiomas arising in the trachea are rare. These lesions pose a management dilemma as several treatment options can provide safe management. Propranolol, a nonselective beta-blocker, has recently been introduced as a novel modality for the treatment of proliferating hemangiomas. This report illustrates the successful management of tracheal hemangiomas using oral propranolol in a young patient with otherwise treatment-resistant airway lesions. Despite various endoscopic therapeutic attempts, the patient remained stridulous with airway disease that persisted into the involution phase of the average hemangioma cycle. Within 6 weeks of beginning oral propranolol (2 mg/kg/day), her airway compromise was eliminated and she had complete resolution of endoscopically visible disease. No side effects from propranolol occurred. We propose that oral propranolol should be considered for use in airway hemangiomas.

Aim: To describe our experience using extracorporeal cardiopulmonary resuscitation (ECPR) in resuscitating children with refractory cardiac arrest in the intensive care unit (ICU) and to describe hospital survival and neurologic outcomes after ECPR., Methods: A retrospective chart review of a consecutive case series of patients requiring ECPR from 2001 to 2006 at Arkansas Children's Hospital. Data from medical records was abstracted and reviewed. Primary study outcomes were survival to hospital discharge and neurological outcome at hospital discharge., Results: During the 6-year study period, ECPR was deployed 34 times in 32 patients. 24 deployments (73%) resulted in survival to hospital discharge. Twenty-eight deployments (82%) were for underlying cardiac disease, 3 for neonatal non-cardiac (NICU) patients and 3 for paediatric non-cardiac (PICU) patients. On multivariate logistic regression analysis, only serum ALT (p-value=0.043; OR, 1.6; 95% confidence interval, 1.014-2.527) was significantly associated with risk of death prior to hospital discharge. Blood lactate at 24h post-ECPR showed a trend towards significance (p-value=0.059; OR, 1.27; 95% confidence interval, 0.991-1.627). The Hosmer-Lemeshow tests (p-value=0.178) suggested a good fit for the model. Neurological evaluation of the survivors revealed that there was no change in PCPC scores from a baseline of 1-2 in 18/24 (75%) survivors., Conclusions: ECPR can be used successfully to resuscitate children following refractory cardiac arrest in the ICU, and grossly intact neurologic outcomes can be achieved in a majority of cases.

Background: For small children requiring mechanical circulatory support as a bridge to transplantation (BTT), extracorporeal membrane oxygenation (ECMO) has been the only option until the recent introduction of the Berlin Heart EXCOR ventricular assist device (Berlin Heart AG, Berlin, Germany). We reviewed our recent experience with these two technologies with particular focus on early outcomes., Methods: Data for 55 consecutive children undergoing BTT between 2001 and 2008 were abstracted from an institutional database. The analysis excluded 13 patients because EXCOR was not used for acute postcardiotomy BTT. Patients were divided into ECMO (n = 21) and EXCOR groups (n = 21). Specific end points included survival to transplant, overall survival, and bridge to recovery. Incidences of adverse events and the duration of support were determined., Results: Groups were similar in weight, age, and etiologies of heart failure. Likewise, the incidences of stroke and multisystem organ failure were similar. Survival to transplant, recovery, or continued support was 57% in ECMO and 86% in EXCOR (p = 0.040). EXCOR patients had overall significantly better survival (p = 0.049). Two ECMO patients and 1 EXOR patient were bridged to recovery. The mean duration of support was 15 +/- 12 days in the ECMO group and 42 +/- 43 days in the EXCOR group (p < 0.001)., Conclusions: In children requiring BTT, EXCOR provided substantially longer support times than ECMO, without significant increase in the rates of stroke or multisystem organ failure. Survival to transplant and long-term survival was higher with EXCOR.

Objective: To investigate whether a mucolytic agent, recombinant human deoxyribonuclease (rhDNase), improves atelectasis in children with cardiac illness requiring mechanical ventilation., Design: A retrospective cohort study on consecutive patients receiving short-term (< or =14 days) rhDNase therapy for atelectasis in the cardiac intensive care unit from January 2005 through February 2007 was carried out. Data relating to patient characteristics, gas exchange, ventilatory parameters, and chest radiographs were collected and analyzed. The effectiveness of rhDNase therapy in the presence of neutrophils and/or bacteria in the pre-rhDNase therapy tracheal aspirates was also investigated., Results: rhDNase was effective in significantly improving established atelectasis without any major changes in gas exchange and ventilatory parameters. Therapeutic effect of rhDNase is most effective in ameliorating atelectasis in the lungs within 10 doses. rhDNase was more effective in improving chest radiographic atelectasis score in patients who had > moderate amounts of polymophonuclear neutrophils (P value = 0.0008), or bacteria (P value = 0.007) or both (P value = 0.004) present in their pre-rhDNase therapy trachea aspirate. No adverse effects were seen with rhDNase administration in the study cohort., Conclusions: rhDNase can be safely and effectively used to improve atelectasis in mechanically ventilated children with cardiac disease especially in the presence of bacteria and/or moderate amounts of polymophonuclear neutrophils in the pre-rhDNase therapy tracheal aspirate.

Objectives: The clinical outcome of prenatally diagnosed congenital heart defects (CHD) continues to be affected significantly by associated extracardiac and chromosomal abnormalities. We sought to: determine the frequency and type of major extracardiac abnormalities (with impact on quality of life) and chromosomal abnormalities associated with fetal CHD; and compare the extracardiac abnormalities detected prenatally to the postnatal and autopsy findings in affected fetuses, to find the incidence of extracardiac abnormalities missed on prenatal ultrasound., Methods: We reviewed the computerized database of the Division of Cardiology of the Hospital for Sick Children in Toronto to identify all cases of major CHD detected prenatally from 1990 to 2002. Medical records, fetal echocardiograms and ultrasound, cytogenetic and autopsy reports were reviewed. The types of CHD detected were grouped into categories and the frequencies of major extracardiac and chromosomal abnormalities in these categories were noted. Prenatal ultrasound findings were compared with those at autopsy or postnatal examination., Results: Of 491 fetuses with major structural CHD, complete data were obtained for 382. Of these, there were 141 (36.9%) with major extracardiac abnormalities at autopsy or postnatal exam, of which 46 had chromosomal abnormalities and 95 did not. In the absence of chromosomal abnormalities, the organ systems most affected were urogenital (12.2%) and gastrointestinal (11.6%). CHDs with the highest incidence of extracardiac abnormalities (>25%) included: heterotaxy, single left ventricle and tricuspid atresia, hypoplastic left heart syndrome and tetralogy of Fallot. Ninety-four of 334 (28.1%) fetuses tested had chromosomal abnormalities. The most common chromosomal abnormalities were trisomies 21 (43.6%), 18 (19.1%) and 13 (9.6%), monosomy X (7.4%) and 22q11.2 deletion (7.4%). Of 289 extracardiac abnormalities from the complete series, 134 (46.4%) were not identified prenatally. Of the missed extracardiac abnormalities, 65 were considered not detectable at prenatal ultrasound, so 23.9% (69/289) of detectable extracardiac abnormalities were missed prenatally., Conclusions: Major extracardiac and chromosomal abnormalities are common in fetuses with major fetal CHD. Many important associated extracardiac abnormalities may be missed prenatally, which should be taken into consideration in the prenatal counseling for fetal CHD.

A neonate was diagnosed with hypoplastic left heart syndrome, heterotaxy syndrome, and interrupted inferior vena cava soon after birth. At 6 days old she underwent the Norwood procedure using Sano modification. Postoperatively she had persistent direct hyperbilirubinemia and was diagnosed with biliary atresia for which she underwent a Kasai procedure at 29 days old. At 10 months she underwent the Kawashima procedure. She is now 20 months old and has been thriving without any jaundice. This case report illustrates that even in the presence of major multiple congenital anomalies, staged reconstruction for hypoplastic left heart syndrome can be successfully performed.

Lawnmowers are one of the most frequent causes of mutilating injuries to children. The majority of accidents are caused by negligence of the operator. Most injuries of this type are caused by direct contact with the spinning blade and, less frequently, by projectiles propelled by the blade. Such projectiles usually produce bruises, but can penetrate skin and soft tissues. This report presents a 6-year-old child who suffered a small, outwardly insignificant puncture wound of the chest from a lawnmower-propelled projectile who presented with fever and chest pain the following day. The diagnostic work up and treatment of this deceptively life-threatening wound are discussed.

We report the aneurysmal dilation of the ascending aorta and the main pulmonary artery in 2 children with Kabuki syndrome. In 1 patient, there was progressive aneurysmal dilation of the ascending aorta necessitating aortoplasty. Histologic examination of the resected aorta revealed disrupted and fragmented elastic fibers in the medial layer, along with mucinous degeneration of the aortic wall. This is the first recognition and report of these findings as part of the Kabuki syndrome.

We present the case of a 6-month-old previously healthy girl who presented with high fever, labored breathing, and an enlarged cardiac silhouette on her chest radiograph. Comprehensive evaluation discovered a ductus arteriosus aneurysm and pericardial effusion with methicillin-resistant Staphylococcus aureus bacteremia. Despite pericardiocentesis and appropriate intravenous antibiotics, there was rapid enlargement of the aneurysm and accumulation of echogenic material within the ductus arteriosus aneurysm. Infected aneurysm rupture was identified during emergency surgery. This infant also had vocal cord paresis, a likely complication of the surgery. The clinical course, diagnosis, and treatment of this patient are discussed. Infection of a ductus arteriosus or an infected ductal arteriosus aneurysm is a rare and potentially fatal clinical entity. In the era of increasing community-acquired methicillin-resistant S aureus infections, this is a diagnosis that requires a high index of suspicion.

Objectives: We sought to determine trends, and outcomes, for a cohort of patients with pulmonary atresia with intact ventricular septum born between 1965 and 1998., Background: Pulmonary atresia with intact ventricular septum is a complex lesion that remains a therapeutic challenge, particularly regarding the suitability for biventricular repair., Methods: We identified 210 consecutive patients, and reviewed their medical records, initial angiograms, and echocardiograms, along with the relevant surgical and pathology reports., Results: The mean initial Z-score for the diameter of the tricuspid valve was -0.99 +/- 1.95, with Ebstein's malformation in 8%. A right ventricular dependent coronary arterial circulation was found in 23%. The proportion of patients who received treatment increased over time, although placement of an arterial shunt was the predominant initial procedure throughout the experience. At the last follow-up, 107 patients had not reached the planned final stage of their repair, and 79% of these had died. Of the 103 reaching the final stage of planned repair, 58 had undergone attempted biventricular repair, with 34% dying; 14 had undergone attempted one and a half ventricular repair, with 7% dying, and 31 had undergone attempted functionally univentricular repair, with 10% dying. Overall, survival was 57% at the age of 1 year, 48% at 5 years, and 43% at 10 years. Survival improved over time, with survival of 75% at 1 year, and 67% at 5 years, for patients born between 1992 and 1998. An earlier date of birth, the presence of Ebstein's malformation, and prematurity were all significant independent factors associated with decreased survival. A greater severity of coronary arterial abnormalities was significantly associated with a greater likelihood of left ventricular dysfunction during follow-up., Conclusions: The outcomes for patients born with pulmonary atresia with intact ventricular septum have improved over time, albeit that careful initial management, and better selection, is still indicated for those planned to undergo biventricular repair.

Objective: This study was undertaken to compare the clinical and hemodynamic results following aortic valve replacement with a pulmonary valve autograft (Ross procedure) or an allograft valve in children., Methods: The records of 107 pediatric aortic valve replacements from 1994 through 2001 were reviewed, including 78 autografts and 25 allografts. Four mechanical aortic valve replacements performed during this period were excluded from analysis., Results: There were 3 perioperative deaths and 1 late death. Reoperations were required in 5 autograft recipients (with autograft preservation in 4) and in 3 allograft recipients (all requiring valve re-replacement). Seven-year survival (96% in both groups) and reoperation-free survival (88% in the autograft group; 73% in the allograft group, P =.5) were not significantly different. Serial echocardiographic studies showed that in the autograft group, left ventricular outflow tract maximal velocity (2.0-1.8 m/s, P =.02) and left ventricular thickness (10.1-8.4 mm, P <.0001) fell significantly. In the allograft group, maximal velocity (2.3-3.0 m/s, P =.03) increased significantly and left ventricular thickness (9.5-9.0 mm, P =.2) showed minimal change. Analysis according to preoperative physiology (aortic stenosis versus insufficiency), congenital cardiac anatomy, number or type of previous operations, age of patient, and use of balloon valvotomy did not predict outcomes., Conclusions: Aortic valve replacement with either the autograft or allograft provides excellent clinical results in children during an intermediate duration of observation. The Ross procedure achieves a superior hemodynamic result, which may be clinically important with longer follow-up.

Objective: This study was designed to determine whether either of 2 alternative methods of extracardiac Fontan reconstruction provides superior results., Methods: We reviewed 58 consecutive Fontan procedures performed between 1995 and 2001 with a pedicled pericardial tunnel (group P, n = 21) or an extracardiac conduit of polytetrafluoroethylene or allograft aorta (group C, n = 37). Operations were performed with cardiopulmonary bypass at 32 degrees C; an aortic crossclamp was applied in only 6 patients. All group P patients and 33 (89%) group C patients received fenestrations., Results: The groups were similar in terms of age, weight, anatomy, and preoperative hemodynamics. There were 3 hospital deaths (5%; 70% confidence limit, 2%-30%), all in group C. Median durations of mechanical ventilation (group P, 1 day; group C, 1 day), intensive care unit stay (group P, 3 days; group C, 3 days), chest tube drainage (group P, 8 days; group C, 7 days), and hospitalization (group P, 10 days; group C, 9 days) were not significantly different. There were no late deaths. All patients received warfarin sodium, and there were no late strokes. Before the Fontan procedure, 1 patient in group P and 3 patients in group C required pacemaker implants. Of the 51 surviving patients in sinus rhythm before the Fontan procedure, only 1 patient in group C subsequently required a pacemaker., Conclusions: Extracardiac Fontan procedures with either a pericardial baffle or conduit are associated with low operative mortality and low risks of arrhythmia and late thromboembolic complication.

Objectives: The purpose of this study was to describe the clinical characteristics and outcome and to elucidate the pathogenesis of ductus arteriosus aneurysm (DAA)., Background: Ductus arteriosus aneurysm is a rare lesion that can be associated with severe complications including thromboembolism, rupture and death., Method: We reviewed the clinical records, diagnostic imaging studies and available histology of 24 cases of DAA, diagnosed postnatally (PD) in 15 and antenatally (AD) in 9 encountered in five institutions., Results: Of PD cases, 13 presented at <2 months, and all AD cases were detected incidentally after 33 weeks of gestation during a late trimester fetal ultrasound study. Of the 24, only 4 had DAA-related symptoms and 6 had associated syndromes: Marfan, Smith-Lemli-Opitz, trisomies 21 and 13 and one possible Ehlers-Danlos. Three had complications related to the DAA: thrombus extension into the pulmonary artery, spontaneous rupture, and asymptomatic cerebral infarction. Six underwent uncomplicated DAA resection for ductal patency, DAA size or extension of thrombus. In the four examined, there was histologic evidence of reduced intimal cushions in two and abnormal elastin expression in two. Five of the 24 died, with only one death due to DAA. Of 19 survivors, all but one remain clinically asymptomatic at a median follow-up of 35 months; however, two have developed other cardiac lesions that suggest Marfan syndrome. A review of 200 consecutive third trimester fetal ultrasounds suggests an incidence of DAA of 1.5%., Conclusions: Ductus arteriosus aneurysm likely develops in the third trimester perhaps due to abnormal intimal cushion formation or elastin expression. Although it can be associated with syndromes and severe complications, many affected infants have a benign course. Given the potential for development of other cardiac lesions associated with connective tissue disease, follow-up is warranted.

Background: There has been a trend toward advocating earlier repair of tetralogy of Fallot and avoiding palliative procedures. The impact of this trend on perioperative outcomes has not been adequately documented., Methods: Data from consecutive patients undergoing repair of tetralogy of Fallot at less than 18 months of age from May 1987 to September 1994 were reviewed. Independent factors associated with duration of stay in the intensive care unit were sought., Results: Repair was performed in 89 infants at a median age of 13 months (range, 15 days to 18 months). A systemic-pulmonary artery shunt was present in 24% of patients. Mean duration of cardiopulmonary bypass was 119+/-37 minutes; 63% of patients received a transannular patch. There were six deaths (7%), all occurring less than 48 hours after repair. The median duration of stay in the intensive care unit was 5 days (range, 1 day to 8 months). Significant independent factors associated with increasing length of intensive care unit stay included younger age at repair, previous shunt, malformation syndrome, increased total dose and number of inotropic agents used, and respiratory complications. Hemodynamic variables serially recorded in the first 48 hours after repair were independently associated with death or prolonged (>7 days) duration of stay., Conclusions: Although outcomes after repair of tetralogy of Fallot in infants are good, both younger age at repair and previous palliative procedures were associated with longer duration of stay in the intensive care unit.