Multidisciplinary coordinated care of hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu disease).

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.

The effect of clinical and haemodynamic variables on post-operative length of stay immediately upon admission after biventricular repair with Yasui operation following an earlier Norwood operation.

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.

Acute Effects of Sodium Bicarbonate in Children with Congenital Heart Disease with Biventricular Circulation in Non-cardiac Arrest Situations.

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), paCO2 (45 ± 26 to 42 ± 7 mmHg), paO2 (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.

Association of Immediate Postoperative Hemodynamic and Laboratory Values in Predicting Norwood Admission Outcomes.

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/m2 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.

Early Infantile Diagnosis of Hereditary Hemorrhagic Telangiectasia Complicated by Child Abuse.

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.

Dimension and overlap of femoral and neck blood vessels in neonates.

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.

Extracorporeal membrane oxygenation support for intractable primary arrhythmias and complete congenital heart block in newborns and infants: short-term and medium-term outcomes.

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.

Ketamine as a neuroprotective and anti-inflammatory agent in children undergoing surgery on cardiopulmonary bypass: a pilot randomized, double-blind, placebo-controlled trial.

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.

Novel KDM6A Kabuki Syndrome Mutation With Hyperinsulinemic Hypoglycemia and Pulmonary Hypertension Requiring ECMO.

Kabuki syndrome (KS) is a multisystem disorder estimated to occur in 1:32 000 newborns. Pathogenic mutations cause the majority but not all cases of KS in either KMT2D or KDM6A. KS can be suspected by phenotypic features, including infantile hypotonia, developmental delay, dysmorphic features, congenital heart defects, and others. Still, many of these features are not readily apparent in a newborn. Although neonatal hypoglycemia has been reported in 8% to 10% of patients with KS, the incidence and severity of hyperinsulinemic hypoglycemia (HH) is not well-studied. We present a full-term female infant with HH who was responsive to low-dose diazoxide. At 3 months of age, she was admitted for septic shock, worsening respiratory status, and severe pulmonary hypertension, requiring extracorporeal membrane oxygenation support. Her neonatal history was notable for hypotonia, dysphagia with aspiration requiring gastrostomy tube placement, and a cardiac defect-hypoplastic aortic arch requiring aortic arch repair. She has characteristic facial features, including prominent eyelashes, long palpebral fissures, and a short nasal columella. Next-generation sequencing for HH revealed a de novo likely pathogenic missense variant in KDM6A gene: c.3479G > T, p.Gly1160Val that was absent from population databases. Genetic testing for causes of HH should include testing of the KS genes KMT2D and KDM6A. Early detection of the underlying genetic defect will help guide management as all reported HH cases associated with KS have been responsive to diazoxide. Affected infants with underlying cardiac conditions may be at higher risk of serious respiratory complications such as pulmonary hypertension.

Patent foramen ovale in children: Unique pediatric challenges and lessons learned from adult literature.

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.

Intrahospital transport of children on extracorporeal membrane oxygenation: indications, process, interventions, and effectiveness.

OBJECTIVE: To evaluate indications, process, interventions, and effectiveness of patients undergoing intrahospital transport. Critically ill patients supported with extracorporeal membrane oxygenation are transported within the hospital to the radiology suite, cardiac catheterization suite, operating room, and from one intensive care unit to another. No studies to date have systematically evaluated intrahospital transport for patients on extracorporeal membrane oxygenation. DESIGN: Retrospective cohort analysis. SETTING: Cardiac intensive care unit in a tertiary care children's hospital. PATIENTS: All patients on extracorporeal membrane oxygenation who required intrahospital transport between January 1996 and March 2007 were included and analyzed. MEASUREMENTS AND MAIN RESULTS: A total of 57 intrahospital transports for cardiac catheterization and head computed tomography scans were analyzed. In 14 (70%) of 20 of patients with cardiac catheterization, a management change occurred as a result of the diagnostic cardiac catheterization. In ten (59%) of 17 patients, bedside echocardiography was of limited value in defining the critical problem. In the interventional group, the majority of transports were for atrial septostomy. In the head computed tomography group, significant pathology was identified, which led to management change. No major complications occurred during these intrahospital transports. CONCLUSIONS: Although transporting patients on extracorporeal membrane oxygenation is labor intensive and requires extensive logistic support, it can be carried out safely in experienced hands and it can result in important therapeutic and diagnostic yields. To our knowledge, this is the first study designed to evaluate safety and efficacy of intrahospital transport for patients receiving extracorporeal membrane oxygenation support.

Pleuroparenchymal fibroelastosis in an 8-year old treated for neuroblastoma.

Pleuroparenchymal fibroelastosis (PPFE), which is primarily diagnosed in adults, is a progressive lung pathology associated with significant morbidity and mortality. PPFE is characterized by pleural and subpleural parenchymal disease causing dyspnea, cough, and recurrent pneumothoraces. PPFE can be precipitated by autoimmune disorders, recurrent respiratory infections, chemotherapy, and transplant. We describe the youngest recorded patient to develop PPFE, whose symptoms began several years after treatment for neuroblastoma. Her symptoms were initially mistaken for worsening asthma, and multiple comorbidities developed during the prolonged time to recognition of PPFE and she progressed to fatal lung disease before potentially curative lung transplantation could occur.

Superior vena cava perforation in a child from a lawnmower projectile.

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.

Risk factors for prolonged mechanical ventilation for children on ventricular assist device support.

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.

Steroid therapy attenuates acute phase reactant response among children on ventricular assist device support.

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.

Comparison of autograft and allograft aortic valve replacement in children.

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.

Comparison of extracardiac Fontan techniques: pedicled pericardial tunnel versus conduit reconstruction.

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.

Dynamic pulmonary computed tomography angiography: a new standard for evaluation of combined airway and vascular abnormalities in infants.

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 supplementation on extracorporeal membrane oxygenation: impact on heparin dose and circuit life.

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.