Use of Aminophylline to Reverse Acute Kidney Injury in Pediatric Critical Care Patients.

OBJECTIVE: Acute kidney injury (AKI) is a complication encountered in 18% to 51% of pediatric critical care patients admitted for treatment of other primary diagnoses and is an independent risk factor for increased morbidity and mortality. Aminophylline has shown promise as a medication to treat AKI, but published studies have shown conflicting results. Our study seeks to assess the reversal of AKI following the administration of aminophylline in critically ill pediatric patients. METHODS: We performed a single-institution retrospective chart review of pediatric inpatients who were diagnosed with AKI and subsequently treated with non-continuous dose aminophylline between January 2016 and December 2018. Data were collected beginning 2 days prior to the initial dose of aminophylline through completion of the 5-day aminophylline course. RESULTS: Nineteen therapies among 17 patients were included in analysis. Twelve of the therapies resulted in resolution of AKI during the study period. We observed urine output increase of 19% (p = 0.0063) on the day following initiation of aminophylline therapy in the subset of patients whose AKI resolved. Trends toward decreased serum creatinine and lower inotropic support were also noted. CONCLUSIONS: Based on these findings, aminophylline could be considered a potentially effective medication for use as rescue therapy in critically ill children with AKI. Limitations include small study population and retrospective nature. Further research in this area with a larger study population and a randomized control trial would allow for better characterization of the efficacy of aminophylline in reversal of AKI.

Echocardiographic versus Angiographic Measurement of the Patent Ductus Arteriosus in Extremely Low Birth Weight Infants and the Utility of Echo Guidance for Transcatheter Closure.

BACKGROUND: Transthoracic echocardiography (TTE) is increasingly utilized for guiding transcatheter closure of patent ductus arteriosus (PDA) in extremely low birth weight (ELBW) infants. The objectives of this study were to compare PDA size measurements by TTE with angiographic measurements and to describe TTE techniques used in guiding transcatheter PDA closure (TCPC) in ELBW infants. METHODS: One hundred twenty-five consecutive ELBW infants (gestational age < 27 weeks, birth weight < 1 kg) who underwent TCPC before 8 weeks of age under TTE guidance were included. Patent ductus arteriosus sizes were measured from the procedural TTE and angiograms retrospectively by blinded observers. The TTE PDA diameters at the aortic (ED1) and pulmonary end (ED2) were compared with the corresponding angiographic diameters (CD1 and CD2). The TTE PDA lengths, obtained by two techniques (EL1, a straight line between ED1 and ED2; and EL2, a curvilinear line along the PDA), were compared with the PDA length by angiography (CL). Transthoracic echocardiography was used to guide accurate device positioning within the PDA. RESULTS: The procedure weight was 600-1,460 g. The TTE and angiographic PDA diameters were comparable (mean ED1 vs CD1 = 4.5 ± 0.68 vs 4.4 ± 0.85 mm, P = .26; and mean ED2 vs CD2 = 3.1 ± 0.72 vs 3.2 ± 0.94 mm, P = .14). The angiographic length was underestimated by EL1 by 2.6 ± 1.6 mm (P < .0001), while EL2 estimated it better (mean EL2 vs CL = 11.0 ± 1.83 vs 10.8 ± 2.15 mm; P = .40). Transcatheter PDA closure was successful in 100% of the cases using TTE guidance. There were no intraprocedural complications. CONCLUSIONS: Transthoracic echocardiography guidance during TCPC in ELBW infants eliminates the need for aortograms via femoral arterial access, preventing the complications associated with it. Transthoracic echocardiography PDA measurements are comparable to angiographic measurements, thereby assisting in appropriate device size selection.

Experience of a single institution with femoral vein homograft as right ventricle to pulmonary artery conduit in stage 1 Norwood operation.

OBJECTIVE: Femoral vein homograft can be used be used as valved right ventricle to pulmonary artery conduit in the Norwood operation. We describe the results of this approach, including pulmonary artery growth and ventricular function. METHODS: A retrospective chart review of 24 consecutive neonates with hypoplastic left heart syndrome or complex single ventricle undergoing this approach between June 2012 and December 2017 was performed. Conduit valve competency and ventricular function were estimated using transthoracic echocardiogram, and pulmonary artery growth was measured using Nakata's index. Changes in ventricular function pre-Glenn and at latest follow-up were assessed by ordinal logistic regression with a general linear model to account for the correlation within the same patient over time. RESULTS: Median age at surgery was 4 days, and mean weight was 3 kg. There was no interstage mortality. A total of 21 patients have undergone Glenn operation, and 9 patients have completed the Fontan operation. None of the conduits developed thrombosis. Sixty-three percent of conduits remained competent in the first month, and 33% remained competent after 3 months of operation. Catheter interventions on conduits were necessary in 14 patients. Median Nakata index at pre-Glenn catheterization was 228 mm2/m2 (interquartile range, 107-341 mm2/m2). Right ventricular function was preserved in 83% of patients at a median follow-up of 34 (interquartile range, 10-46) months. CONCLUSIONS: Femoral vein homograft as a right ventricle to pulmonary artery conduit in the Norwood operation is safe and associated with good pulmonary artery growth and preserved ventricular function as assessed by subjective echocardiography. Catheter intervention of the conduit may be necessary.

Perioperative serum albumin and its influence on clinical outcomes in neonates and infants undergoing cardiac surgery with cardiopulmonary bypass: a multi-centre retrospective study.

Hypoalbuminemia is associated with morbidity and mortality in critically ill children. In this multi-centre retrospective study, we aimed to determine normative values of serum albumin in neonates and infants with congenital heart disease, evaluate perioperative changes in albumin levels, and determine if low serum albumin influences post-operative outcomes. Consecutive eligible neonates and infants who underwent cardiac surgery with cardiopulmonary bypass at one of three medical centres, January 2012-August 2013, were included. Data on serum albumin levels from five data points (pre-operative, 0-24, 24-48, 48-72, 72 hours post-operative) were collected. Median pre-operative serum albumin level was 2.5 g/dl (IQR, 2.1-2.8) in neonates versus 4 g/dl (IQR, 3.5-4.4) in infants. Hypoalbuminemia was defined as <25th percentile of these values. A total of 203 patients (126 neonates, 77 infants) were included in the study. Post-operative hypoalbuminemia developed in 12% of neonates and 20% of infants; 97% occurred in the first 48 hours. In multivariable analysis, perioperative hypoalbuminemia was not independently associated with any post-operative morbidity. However, when analysed as a continuous variable, lower serum albumin levels were associated with increased post-operative morbidity. Pre-operative low serum albumin level was independently associated with increased odds of post-operative hypoalbuminemia (OR, 3.67; 95% CI, 1.01-13.29) and prolonged length of hospital stay (RR, 1.40; 95% CI, 1.08-1.82). Lower 0-24-hour post-operative serum albumin level was independently associated with an increased duration of mechanical ventilation (RR, 1.35; 95% CI, 1.12-1.64). Future studies should further assess hypoalbuminemia in this population, with emphasis on evaluating clinically meaningful cut-offs and possibly the use of serum albumin levels in perioperative risk stratification models.

A Case of Bilateral Spontaneous Chylothorax with Respiratory Syncytial Virus Bronchiolitis.

A case of bilateral spontaneous chylothorax with respiratory syncytial virus (RSV) bronchiolitis has never been reported. We report the case of a 7-month-old boy born at 33 weeks gestation with a history of Down syndrome, atrial septal defect, pulmonary hypertension, and chronic lung disease, hospitalized due to RSV bronchiolitis who developed bilateral spontaneous chylothorax with exacerbation of pulmonary hypertension (PH). The patient died after 9 weeks of mechanical ventilation and treatment for PH. The autopsy showed acute infectious signs, a chronic interstitial lung disease with pulmonary hypertensive changes and subpleural cysts with no evidence of congenital lymphangiectasia. The cause of chylothorax in this child could be multifactorial. However, worsening pulmonary hypertension with RSV infection might have partially contributed to the development of chylothorax through elevated superior venous cava pressure. Thoracentesis should be considered for patients with Down syndrome and PH associated with congenital heart disease who develop persistent pleural effusion during RSV bronchiolitis to rule out chylothorax.

Femoral vein homograft as Sano shunt results in improved pulmonary artery growth after Norwood operation.

OBJECTIVE: To evaluate differences in interstage growth of pulmonary arteries between use of polytetrafluoroethylene and femoral vein homograft as Sano shunt during stage-I Norwood palliation. METHODS: A retrospective review of all patients who survived to the second stage following Norwood-Sano operation at two institutions was performed. Either polytetrafluoroethylene or the valved segment of femoral vein homograft was used for construction of the Sano shunt. The size of pulmonary arteries was compared at pre-Glenn catheterisation. RESULTS: A total of 48 neonates with the diagnosis of hypoplastic left heart syndrome or its variants comprised the study population. Femoral vein homograft of 5-6 mm diameter was used in 14 and polytetrafluoroethylene graft of 5 mm was used in 34 patients. The two groups were comparable in terms of preoperative demographics and age at time of pre-Glenn catheterisation (3.9±0.7 versus 3.4±0.8 months, p=0.06). Patients who received femoral vein homograft demonstrated a significantly higher pre-Glenn Nakata index [264 (130-460) versus 165 (108-234) mm2/m2, p=0.004]. The individual branch pulmonary arteries were significantly larger in the femoral vein group (right, 7.8±3.6 versus 5.0±1.2, p=0.014; left, 7.2±2.1 versus 5.6±1.9, p=0.02). There were no differences in cardiac index, Qp:Qs, ventricular end-diastolic pressure or systemic oxygen saturations. CONCLUSIONS: Utilisation of a valved segment of femoral vein homograft as right ventricle to pulmonary artery conduit during Norwood-Sano operation confers better interstage growth of the pulmonary arteries. Further studies are needed to evaluate the impact of femoral vein homograft on single ventricle function.

Femoral Vein Homograft as Right Ventricle to Pulmonary Artery Conduit in Stage 1 Norwood Operation.

BACKGROUND: The polytetrafluoroethylene tube used as right ventricle to pulmonary artery conduit in the stage 1 Norwood operation is associated with risks of suboptimal branch pulmonary artery growth, thrombosis, free insufficiency, and long-term right ventricular dysfunction. Our experience with use of valved femoral vein homograft as right ventricle to pulmonary artery conduit is described. METHODS: Between June 2012 and December 2015, 15 neonates with hypoplastic left heart syndrome or complex single ventricle underwent stage 1 Norwood operation with valved segment of femoral vein homograft as right ventricle to pulmonary artery conduit. The median age at surgery was 3 days and the mean weight was 3 kg. The size of the femoral vein homograft was 5 mm in 8 patients and 6 mm in 7 patients. RESULTS: There was no hospital or interstage mortality. Fourteen patients underwent Glenn operation, and 6 have undergone Fontan operation to date. The median Nakata index at pre-Glenn catheterization was 262 mm2/m2 (interquartile range: 121 to 422 mm2/m2). No patient had thrombosis of conduit. Most femoral vein conduits remained competent in the first month after stage 1 Norwood operation, although most became incompetent by 3 months. Catheter intervention on the conduit was necessary in 7 patients. Right ventricular function was preserved in most patients at follow-up. CONCLUSIONS: The use of femoral vein homograft as right ventricle to pulmonary artery conduit in the Norwood operation is safe and associated with good pulmonary artery growth and preserved ventricular function. Balloon dilation of the conduit may be necessary during the interstage period.

Severe Pulmonary Vascular Obstructive Disease After Neonatal Arterial Switch Operation for Simple Transposition of the Great Arteries.

Neonatal arterial switch operation for simple dextro-transposition of the great arteries (d-TGA) has almost eliminated the occurrence of pulmonary vascular obstructive disease compared to patients who underwent Mustard or Senning procedure at an older age. We report a case of a neonate with d-TGA and intact ventricular septum who underwent arterial switch operation and yet developed severe pulmonary vascular obstructive disease within two months.

Partial Anomalous Pulmonary Venous Connection to the Azygous Vein: Unusual Pathology Requiring Unusual Repair.

Connection of the right pulmonary veins to the azygous is an extremely rare variant of partial anomalous pulmonary venous connection. We describe one such case in the setting of an intact atrial septum. Surgical correction in such a situation can be challenging. We describe a successful surgical approach for this unusual variant.

Common pulmonary vein atresia: report of three cases and review of the literature.

Common pulmonary vein atresia is a rare and usually fatal congenital anomaly, in which the pulmonary veins come together to form a confluence that does not connect to the left atrium. We report our experience with three cases of common pulmonary vein atresia and review the literature on this anomaly. The diagnosis of common pulmonary vein atresia must be entertained in any newborn that presents with cyanosis, refractory acidosis, and decreased systemic perfusion within the first 48 hours of life. Echocardiography is a useful screening tool, but cardiac catheterisation is the preferred diagnostic tool. Common pulmonary vein atresia can be fatal without surgical intervention, but survival after surgery continues to be poor.

Systemic and pulmonary artery aneurysms in incomplete Kawasaki disease.

Kawasaki disease (KD) is a systemic vasculitis that can cause coronary artery aneurysms (CAA) in up to 25% if left untreated. Rarely, aneurysms of systemic arteries of all sizes have been reported to occur. The incidence of systemic artery aneurysms (SAA) with typical KD can be as high as 2.2%. Incomplete KD with SAA is not well described. We report a case of a 12-year-old boy with incomplete KD, giant CAA, diffuse SAA, and pulmonary artery aneurysms (PAA). The patient presented with fever, malaise, abdominal pain, maculopapular, rash and cervical lymphadenopathy. Echocardiogram showed multiple giant CAA. By angiography, diffuse ectasia and aneurysms of most medium-sized arteries throughout the body were noted including the lobar pulmonary artery branches. Incomplete KD was suspected. The patient was treated with intravenous gammaglobulin, methylprednisolone, and high-dose aspirin. Due to the systemic vasculitis, he also received cyclophosphamide. The patient's clinical symptoms improved. Anticoagulation with warfarin was maintained. The patient remains asymptomatic three years later but with progressive CAA and stable SAA. The PAA caused no symptoms and resolved after the acute phase. Incomplete KD can manifest with CAA and SAA. This is the first report of PAA associated with KD. Surveillance for PAA in KD must be considered. .

Bumetanide continuous infusions in critically ill pediatric patients.

OBJECTIVE: Limited data exist for the use of bumetanide continuous infusions in children. The purpose of this study was to evaluate the use of bumetanide continuous infusions in critically ill pediatric patients. DESIGN: This study was an institutional review board approved, single-center, retrospective chart review of 95 patients. Dosing practices were described by rate (µg/kg/hr), duration (days), and previous diuretic use. Efficacy, determined by ability to achieve negative fluid balance and time to reach negative fluid balance, was assessed at 12, 24, and 48 hours. Safety was evaluated based on prevalence of adverse drug reactions. Adverse drug reactions were predefined as serum potassium concentration less than 3 mEq/L, serum chloride concentration less than 90 mEq/L, serum carbon dioxide concentration greater than 35 mEq/L, and serum creatinine increased greater than 1.5 times baseline and above patient-specific normal range. SETTING: Le Bonheur Children's Hospital, Memphis, TN. PATIENTS: Critically ill patients who are 18 years old or younger and received bumetanide continuous infusions. A total of 95 patients were included. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: The mean dose of bumetanide was 5.7 ± 2.2 µg/kg/hr (1-10 µg/kg/hr) with a median duration of 3.3 days (0.3-18.5). The total percentage of patients achieving negative fluid balance by 48 hours was 76% with 54% of patients reaching negative fluid balance within 12 hours. CONCLUSIONS: This study showed that a bumetanide dose of 5.7 µg/kg/hr was effective in achieving negative fluid balance in the majority of critically ill pediatric patients. Additionally, bumetanide appears to be a safe loop diuretic for use as a continuous infusion at the doses described in critically ill pediatric patients.

Management of massive diffuse alveolar hemorrhage in a child with systemic lupus erythematosus.

Diffuse alveolar hemorrhage (DAH) from systemic lupus erythematosus (SLE) is a rare but potentially life-threatening condition. We report the case of a 14-year-old female with SLE who developed hypoxia and shock secondary to severe alveolar hemorrhage. She was successfully managed by placement on extracorporeal membrane oxygenation (ECMO) followed by emergent pulmonary lobectomy and medical treatment including high-dose methylprednisolone, cyclophosphamide, intravenous immunoglobulin, and plasmapheresis.