Short- and medium-term outcomes for patent ductus arteriosus stenting in neonates ≤2.5 kg with duct-dependent pulmonary circulation.

BACKGROUND: Morbidity with surgical systemic-to-pulmonary artery shunting (SPS) in infants ≤2.5 kg has remained high. Patent ductus arteriosus (PDA) stenting may be a valid alternative. The objective of this study is to evaluate outcomes following PDA stenting in patients ≤2.5 kg from four large tertiary centers. METHODS: Retrospective review of all neonates ≤2.5 kg with duct-dependent pulmonary circulation who underwent PDA stenting. Procedural details, pulmonary arterial growth, reinterventions, surgery type, and outcomes were assessed. RESULTS: PDA stents were implanted in 37 of 38 patients attempted (18 female) at a median procedural weight of 2.2 kg (interquartile range [IQR], 2-2.4 kg). Seven patients (18%) had a genetic abnormality and 16 (42%) had associated comorbidities. The median intensive care unit stay was 4 days (IQR, 2-6.75 days), and the median hospital stay was 20 days (IQR, 16-57.25). One patient required a rescue shunt procedure, with three others requiring early SPS (<30 days postprocedure). Twenty patients (54%) required reintervention with either balloon angioplasty, restenting, or both. At 6-month follow-up, right pulmonary artery growth (median z-score -1.16 to 0.01, p = 0.05) was greater than the left pulmonary artery (median z-score -0.9 to -0.64, p = 0.35). Serious adverse effects (SAEs) were seen in 18% (N = 7) of our cohort. One patient developed an SAE during planned reintervention There were no intraprocedural deaths, with one early procedure-related mortality, and three interstage mortalities not directly related to PDA stenting. CONCLUSIONS: PDA stenting in infants ≤2.5 kg is feasible and effective, promoting pulmonary artery growth. Reintervention rates are relatively high, though many are planned to allow for optimal growth before a definitive operation.

Aortopulmonary Window With Pulmonary Atresia and Interrupted Aortic Arch: A Very Rare Triad.

Aortopulmonary window (APW) is a rare lesion, accounting for 0.2% to 0.6% of all congenital heart diseases. We report a rare case of an infant with APW, interrupted aortic arch, and pulmonary atresia with intact interventricular septum and right ventricle-dependent coronary circulation. This report describes the anatomy of this lesion set, the complex surgical palliation that was required, and the management of postoperative complications.

Evaluation of Fontan liver disease: Correlation of transjugular liver biopsy with magnetic resonance and hemodynamics.

INTRODUCTION: Liver fibrosis and cirrhosis are late complications in Fontan palliation. Liver biopsy is the gold standard. The goal of this study is to correlate transjugular liver biopsy (TJLB) in the setting of Fontan palliation with noninvasive testing and hemodynamics. METHODS: Between August 2014 and July 2017, 49 Fontan patients underwent TJLB. All the patients had hemodynamic evaluation, 28 patients had MRE (magnetic resonance elastography) and 40 patients had cardiopulmonary exercise test. Histologic liver fibrosis was quantitated using traditional histologic scoring systems and a modified Ishak congestive hepatic fibrosis score. RESULTS: Median age 17.8 years, median time since Fontan 15.2 years. Primary diagnosis and Fontan type were variables, but predominantly LV morphology (30/49), lateral tunnel Fontan (29/49), originally fenestrated (37/49), and 11/49 had a pacemaker. Histologic fibrosis correlated with MRE (R = 0.62, P ≤ .001). Histologic fibrosis and MRE correlated with Fontan pressure (R = 0.38, P = .008 & R = 0.59, P ≤ .001). Morphology of the single ventricle did not correlate with liver fibrosis. The presence of a fenestration resulted in a higher cardiac index (P = .026) but did not resulted in lower liver fibrosis (P = .64). CONCLUSION: Noninvasive tests, such as MRE, may be suitable for longitudinal follow-up in patients with single ventricle physiology. Our data suggest that there is reasonable correlation of MRE liver stiffness with biopsy scoring systems and Fontan pressures. We demonstrated the feasibility of TJLB in the setting of Fontan palliation and demonstrated its correlation with noninvasive measures particularly MRE. We recommend selective use of TJLB when MRE score is >5 KPa or when there are other clinical signs of cirrhosis.

Pulmonary artery interventions after the arterial switch operation: Unique and significant risks.

BACKGROUND: In the modern era, results of the arterial switch operation (ASO) for transposition of the great arteries are excellent. However, because of the LeCompte maneuver, there may be a propensity for development of pulmonary artery stenosis. We encountered atypical complications of pulmonary artery stenting in patients after the ASO, including aorto-pulmonary fistula and coronary compression. METHODS: We performed a 10-year retrospective review of catheterizations performed in patients after ASO in our institution with a focus on adverse events. RESULTS: Diagnostic and interventional catheterizations were performed in 47 patients. In 29 patients, 37 interventional procedures performed, which included pulmonary artery angioplasty and/or stenting. In this group, there were five major adverse events (14%), including three aorto-pulmonary fistulae and one coronary artery compression among patients having stent implantation or stent redilation. In addition, there were 6/37 (16%) intended stent procedures, which were aborted because there appeared to be high-risk of significant adverse events. CONCLUSIONS: This review suggests that percutaneous intervention on pulmonary artery stenosis after ASO has high-risk and should be undertaken advisedly. Prior thorough evaluation of coronary arteries is mandatory as coronary reimplantation sites may be adjacent to sites of pulmonary artery stenosis. Furthermore, if pulmonary artery stent implantation or stent redilation is contemplated, the risk of stent fracture and possible AP fistula should be recognized. Primary use of reinforced covered stents should be considered.

Impact of iron deficiency anemia on the function of the immune system in children.

The importance of iron deficiency as a public health problem is based ultimately on the seriousness of its consequences on health. The most extensively investigated consequences of iron deficiency involve work performance and immune function. The significance of the effects on work performance is generally accepted. In contrast, data on the influence of iron deficiency on immune function are often perceived as being confusing and contradictory.We aimed to evaluate the effect of iron deficiency anemia on humoral, cellular, nonspecific immunity, and also the effect on the cytokines that are the key factors of many immunologic steps.Forty children with iron deficiency anemia and 20 age and sex-matched healthy children were included. All children were subjected to full medical history, thorough clinical examination, complete blood count, iron indices (serum iron, serum total iron-binding capacity, serum ferritin, and transferrin saturation), immunoglobulin assay (IgA, IgG, and IgM), interleukin (IL)-6 serum level, study of T-lymphocyte subsets, and evaluation of phagocytic function of macrophages and oxidative burst activity of neutrophils.Patients had significantly lower IgG levels, IL-6, phagocytic activity, and oxidative burst of neutrophils than controls, although there was no significant difference between patients and controls with regard to other immunoglobulins and CD4/CD8 ratio. There was significantly positive correlation between serum iron and IL-6 serum level.We concluded that humoral, nonspecific immunity (phagocytic activity and oxidative burst), and the IL-6 are influenced in patients with iron deficiency anemia. Study of these abnormalities after correction of iron deficiency is strongly needed.

Chemotherapy-induced neutropenia among pediatric cancer patients in Egypt: Risks and consequences.

Chemotherapy-induced neutropenia (CIN) is the major dose-limiting toxicity of systemic chemotherapy and it is associated with significant morbidity, mortality and treatment cost. The aim of the present study was to identify the risk factors that may predispose pediatric cancer patients who receive myelosuppressive chemotherapy to CIN and associated sequelae. A total of 113 neutropenia episodes were analyzed and the risk factors for CIN were classified as patient-specific, disease-specific and regimen-specific, while the consequences of CIN were divided into infectious and dose-modifying sequelae. The risks and consequences were analyzed to target high-risk patients with appropriate preventive strategies. Among our patients, 28% presented with a single neutropenia attack, while 72% experienced recurrent attacks during their treatment cycles. The mean absolute neutrophil count was 225.5±128.5 ×109/l (range, 10-497 ×109/l), starting 14.2±16.3 days (range, 2-100 days) after the onset of chemotherapy and resolving within 11.2±7.3 days, either with (45.1%) or without (54.9%) granulocyte colony-stimulating factor (G-CSF). No significant association was observed between any patient characteristics or disease stage and the risk for CIN. However, certain malignancies, such as acute lymphocytic leukemia (ALL), neuroblastoma and Burkitt's lymphoma, and certain regimens, such as induction block for ALL and acute myelocytic leukemia, exerted the most potent myelotoxic effect, with severe and prolonged episodes of neutropenia. G-CSF significantly shortened the duration of the episodes and enhanced bone marrow recovery. Febrile neutropenia was the leading complication among our cases (73.5%) and was associated with several documented infections, particularly mucositis (54.9%), respiratory (45.1%), gastrointestinal tract (38.9%) and skin (23.9%) infections. A total of 6% of our patients succumbed to infection-related complications. Neutropenia was responsible for treatment discontinuation (13.3%), dose delay (13.3%) and dose reduction (5.3%) in our patients. The mean cost for each episode in our institution was 9,386.5±6,688.9 Egyptian pounds, which represented a significant burden on health care providers.

NT-proBNP as early marker of subclinical late cardiotoxicity after doxorubicin therapy and mediastinal irradiation in childhood cancer survivors.

BACKGROUND: Childhood cancer survivors treated with anthracyclines and mediastinal irradiation are at risk for late onset cardiotoxicity. AIMS OF THE STUDY: To assess the role of N-terminal pro-brain natriuretic peptide (NT-proBNP) and tissue Doppler imaging (TDI) as early predictors of late onset cardiotoxicity in asymptomatic survivors of childhood cancer treated with doxorubicin with or without mediastinal irradiation. METHODS: A cross-sectional study on 58 asymptomatic survivors of childhood cancer who received doxorubicin in their treatment protocols and 32 asymptomatic Hodgkin's lymphoma survivors who received anthracycline and mediastinal irradiation. Levels of NT-proBNP, TDI, and conventional echocardiography were determined. RESULTS: Thirty percent of survivors had abnormal NT-proBNP levels. It was significantly related to age at diagnosis, duration of follow-up, and cumulative dose of doxorubicin. TDI detected myocardial affection in 20% more than conventional echocardiography. Furthermore, abnormalities in TDI and NT-pro-BNP levels were more common in Hodgkin lymphoma survivors receiving both chemotherapy and radiotherapy. CONCLUSIONS: TDI could detect early cardiac dysfunction even in those with normal conventional echocardiography. Measurement of NT-proBNP represents an interesting strategy for detecting subclinical cardiotoxicity. We recommend prospective and multicenter studies to validate the role of NT-proBNP as an early marker for late onset doxorubicin-induced cardiotoxicity.

Detection of early renal injury in children with solid tumors undergoing chemotherapy by urinary neutrophil gelatinase-associated lipocalin.

Acute kidney injury (AKI) is a complication in children with solid tumors undergoing chemotherapy, as it may prevent the use of therapy protocols and also hinder the supportive and diagnostic procedures. Thus, there is an urgent requirement for early predictive biomarkers of AKI. The most promising novel AKI biomarker is neutrophil gelatinase-associated lipocalin (NGAL). The aim of the present study was to compare the predictability of NGAL as a biomarker of AKI with creatinine as a traditional biomarker in children with solid tumors under chemotherapy. The study was performed on 30 patients with different types of solid tumors (reuroblastoma, Wilms tumor, medulloblastoma, rhabdomyosarcoma and Ewing sarcoma) and 20 control subjects. Urinary NGAL (uNGAL) and serum creatinine samples were taken three times: Baseline before the beginning of the treatment, one week after chemotherapy and at the end of the chemotherapy protocol. AKI is defined as a change in creatinine level by >50% of the baseline. The creatinine level only rises to this level in the third sample, while uNGAL increases significantly in the second and third samples with percentage of change 376.8 and 698.2%, respectively, which is highly significant (P<0.001). When comparing the predictive value of serum creatinine for AKI depending on the receiver operating characteristic curve with that of uNGAL, the area under the curve (AUC) for creatinine was 0.60 with a standard error (SE) of 0.086 and 95% confidence interval (CI) between 0.432 and 0.768, while that of uNGAL was highly predictive with an AUC of 0.847, SE 0.55 and 95% CI between 0.739 and 0.955. Depending only on the creatinine level for detecting the AKI will markedly delay the diagnosis; however, uNGAL is detected earlier, and is easier and more reliable as a marker for AKI in children with solid tumors undergoing chemotherapy.