Nucleated red blood cells as a biomarker for mortality in infants and neonates requiring veno-arterial extracorporeal membrane oxygenation for cardiac disease.

INTRODUCTION: Nucleated red blood cells (NRBC) are rare in the peripheral circulation of healthy individuals and their presence have been associated with mortality in adults and very low birth weight newborns, however, its value as a biomarker for mortality in infants requiring veno-arterial (VA) extracorporeal membrane oxygenation (ECMO) has yet to be studied. We sought to determine if NRBC can serve as a biomarker for ECMO mortality and inpatient mortality in infants requiring V-A ECMO. METHODS: A single-center retrospective chart review analyzing infants <1 year of age requiring VA ECMO due to myocardial dysfunction or post-cardiotomy between January 1, 2011 to June 30, 2020. RESULTS: One hundred two patients required VA ECMO. Sixty-five patients required ECMO post-cardiotomy, 19 for perioperative deterioration, and 18 for myocardial dysfunction. Fifty-one patients (50%) died (21 died on ECMO, 30 died post-ECMO decannulation). Multivariable analysis found Age <60 days (OR 13.0, 95% CI 1.9-89.6, p = 0.009), NRBC increase by >50% post-ECMO decannulation (OR 17.1, 95% CI 3.1-95.1, p = 0.001), Single Ventricle (OR 9.0, 95% CI 1.7-47.7, p = 0.01), and lactate at ECMO decannulation (OR 3.0, 95% CI 1.3-7.1, p = 0.011) to be independently associated with inpatient mortality. ROC curves evaluating NRBC pre-ECMO decannulation as a biomarker for mortality on ECMO (AUC 0.80, 95% CI 0.68-0.92, p ⩽ 0.001) and post-ECMO decannulation (AUC 0.75, 95% CI 0.65-0.84, p ⩽ 0.001) show NRBC to be an accurate biomarker for mortality. CONCLUSIONS: Greater than 50% increase in NRBC post-ECMO decannulation is associated with inpatient mortality. NRBC value pre-ECMO decannulation may be a useful biomarker for mortality while on ECMO and post-decannulation.

Nucleated red blood cells as a biomarker for mortality in neonates following cardiac surgery.

INTRODUCTION: Nucleated red blood cells (NRBCs) are immature red cells that under normal conditions are not present in the peripheral circulation. Several studies have suggested an association between elevated NRBC and poor outcome in critically ill adults and neonates. We sought to determine if elevations in NRBC value following cardiac surgery and following clinical events during the hospital stay can be used as a biomarker to monitor for mortality risk in neonates post-cardiac surgery. MATERIALS AND METHODS: We constructed a retrospective study of 264 neonates who underwent cardiac surgery at Children's Hospital, New Orleans between 2011 and 2020. Variables included mortality and NRBC value were recorded following cardiac surgery and following peri-operative clinical events. The study was approved by LSU Health IRB. Sensitivity, specificity, receiver operating characteristic (ROC) curves with area under the curve (AUC) and logistic regression analysis were performed. RESULTS: Thirty-six patients (13.6%) died, of which 32 had an NRBC value ≥10/100 white blood cell (WBC) during hospitalisation. Multi-variable analysis found extracorporeal membrane oxygenation use (OR 10, 95% CI 2.9-33, p=<0.001), NRBC ≥10/100 WBC (OR 16.1, CI 4.1-62.5, p ≤ 0.001) and peak NRBC in the 14-day period post-cardiac surgery (continuous variable, OR 1.05, 95% CI 1.0-1.09, p = 0.03), to be independently associated with mortality. Using a cut-off NRBC value of 10/100 WBC, there was an 88.9% sensitivity and a 90.8% specificity, with ROC curve showing an AUC of 0.9 and 0.914 for peak NRBC value in 14 days post-surgery and entire hospitalisation, respectively. CONCLUSIONS: NRBC ≥10/100 WBC post-cardiac surgery is strongly associated with mortality. Additionally, NRBC trend appears to show promise as an accurate biomarker for mortality.

Pre-Fontan Cardiac Catheterization Data as a Predictor of Prolonged Hospital Stay and Post-Discharge Adverse Outcomes Following the Fontan Procedure: A Single-Center Study.

Despite improved outcomes following modifications to the Fontan technique, significant morbidity and mortality persist. We sought to determine if abnormal pre-Fontan catheterization hemodynamic data will predict postoperative prolonged hospital stay (PHLOS) and adverse post-discharge outcomes. This is a retrospective study of patients who underwent the Fontan procedure at Children's Hospital of New Orleans from 2008 to 2018. PHLOS was defined as ≥ 14 and ≥ 21 days to discharge post Fontan. We defined post-discharge adverse outcomes as thromboembolic phenomena requiring anticoagulation therapy, protein-losing enteropathy, plastic bronchitis, transplantation, persistent chylous effusion requiring fenestration creation, or death. Statistical analysis was performed using student t test, Chi-square test, and multivariable logistic regression analysis using IBM SPSS version 22. Ninety-seven patients underwent extracardiac Fontan. Forty-one patients (42.3%) experienced hospitalization ≥ 14 days, 31 patients (32%) experienced hospitalization ≥ 21 days, and 14 patients (14.4%) experienced adverse post-discharge outcome. Elevated end-diastolic pressure (EDP) ≥ 10 mmHg (p = 0.005, OR 4.2, CI 1.5-11.4) was independently associated with ≥ 14 days of hospitalization, while a CI < 4 L/minute/meters2 combined with one abnormal catheterization variable was associated with PHLOS and post-discharge adverse outcomes (p = 0.03, OR 2.8, CI 1.1-7.3 and p = 0.043, OR 6.42, OR 1.1-38.9, respectively). The absence of fenestration was also associated with post-discharge adverse outcomes (p = 0.007, OR 5.8, CI1.6-20.7). Elevated EDP may be associated with PHLOS, while CI < 4 L/minute/meters2 combined with abnormal catheterization hemodynamics may be associated with PHLOS and adverse post-discharge outcomes, while absence of fenestration may be associated with post-discharge adverse events.

Near-infrared spectroscopy for prediction of extubation success after neonatal cardiac surgery.

INTRODUCTION: Reliable predictors of extubation readiness are needed and may reduce morbidity related to extubation failure. We aimed to examine the relationship between changes in pre-extubation near-infrared spectroscopy measurements from baseline and extubation outcomes after neonatal cardiac surgery. MATERIALS AND METHODS: In this retrospective cross-sectional multi-centre study, a secondary analysis of prospectively collected data from neonates who underwent cardiac surgery at seven tertiary-care children's hospitals in 2015 was performed. Extubation failure was defined as need for re-intubation within 72 hours of the first planned extubation attempt. Near-infrared spectroscopy measurements obtained before surgery and before extubation in patients who failed extubation were compared to those of patients who extubated successfully using t-tests. RESULTS: Near-infrared spectroscopy measurements were available for 159 neonates, including 52 with single ventricle physiology. Median age at surgery was 6 days (range: 1-29 days). A total of 15 patients (9.4 %) failed extubation. Baseline cerebral and renal near-infrared spectroscopy measurements were not statistically different between those who were successfully extubated and those who failed, but pre-extubation cerebral and renal values were significantly higher in neonates who extubated successfully. An increase from baseline to time of extubation values in cerebral oximetry saturation by ≥ 5 % had a positive predictive value for extubation success of 98.6 % (95%CI: 91.1-99.8 %). CONCLUSION: Pre-extubation cerebral near-infrared spectroscopy measurements, when compared to baseline, were significantly associated with extubation outcomes. These findings demonstrate the potential of this tool as a valuable adjunct in assessing extubation readiness after paediatric cardiac surgery and warrant further evaluation in a larger prospective study.

Multicenter Validation of the Vasoactive-Ventilation-Renal Score as a Predictor of Prolonged Mechanical Ventilation After Neonatal Cardiac Surgery.

OBJECTIVES: We sought to validate the Vasoactive-Ventilation-Renal score, a novel disease severity index, as a predictor of outcome in a multicenter cohort of neonates who underwent cardiac surgery. DESIGN: Retrospective chart review. SETTING: Seven tertiary-care referral centers. PATIENTS: Neonates defined as age less than or equal to 30 days at the time of cardiac surgery. INTERVENTIONS: Ventilation index, Vasoactive-Inotrope Score, serum lactate, and Vasoactive-Ventilation-Renal score were recorded for three postoperative time points: ICU admission, 6 hours, and 12 hours. Peak values, defined as the highest of the three measurements, were also noted. Vasoactive-Ventilation-Renal was calculated as follows: ventilation index + Vasoactive-Inotrope Score + Δ creatinine (change in creatinine from baseline × 10). Primary outcome was prolonged duration of mechanical ventilation, defined as greater than 96 hours. Receiver operative characteristic curves were generated, and abilities of variables to correctly classify prolonged duration of mechanical ventilation were compared using area under the curve values. Multivariable logistic regression modeling was also performed. MEASUREMENTS AND MAIN RESULTS: We reviewed 275 neonates. Median age at surgery was 7 days (25th-75th percentile, 5-12 d), 86 (31%) had single ventricle anatomy, and 183 (67%) were classified as Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery Congenital Heart Surgery Mortality Category 4 or 5. Prolonged duration of mechanical ventilation occurred in 89 patients (32%). At each postoperative time point, the area under the curve for prolonged duration of mechanical ventilation was significantly greater for the Vasoactive-Ventilation-Renal score as compared to the ventilation index, Vasoactive-Inotrope Score, and serum lactate, with an area under the curve for peak Vasoactive-Ventilation-Renal score of 0.82 (95% CI, 0.77-0.88). On multivariable analysis, peak Vasoactive-Ventilation-Renal score was independently associated with prolonged duration of mechanical ventilation, odds ratio (per 1 unit increase): 1.08 (95% CI, 1.04-1.12). CONCLUSIONS: In this multicenter cohort of neonates who underwent cardiac surgery, the Vasoactive-Ventilation-Renal score was a reliable predictor of postoperative outcome and outperformed more traditional measures of disease complexity and severity.

Extubation Failure after Neonatal Cardiac Surgery: A Multicenter Analysis.

OBJECTIVES: To describe the epidemiology of extubation failure and identify risk factors for its occurrence in a multicenter population of neonates undergoing surgery for congenital heart disease. STUDY DESIGN: We conducted a prospective observational study of neonates ≤30 days of age who underwent cardiac surgery at 7 centers within the US in 2015. Extubation failure was defined as reintubation within 72 hours of the first planned extubation. Risk factors were identified with the use of multivariable logistic regression analysis and reported as OR with 95% CIs. Multivariable logistic regression analysis was conducted to examine the relationship between extubation failure and worse clinical outcome, defined as hospital length of stay in the upper 25% or operative mortality. RESULTS: We enrolled 283 neonates, of whom 35 (12%) failed their first extubation at a median time of 7.5 hours (range 1-70 hours). In a multivariable model, use of uncuffed endotracheal tubes (OR 4.6; 95% CI 1.8-11.6) and open sternotomy of 4 days or more (OR 4.8; 95% CI 1.3-17.1) were associated independently with extubation failure. Accordingly, extubation failure was determined to be an independent risk factor for worse clinical outcome (OR 5.1; 95% CI 2-13). CONCLUSIONS: In this multicenter cohort of neonates who underwent surgery for congenital heart disease, extubation failure occurred in 12% of cases and was associated independently with worse clinical outcome. Use of uncuffed endotracheal tubes and prolonged open sternotomy were identified as independent and potentially modifiable risk factors for the occurrence of this precarious complication.

Nutritional markers accompanying acquired chylothorax in infants: a systematic review.

CONTEXT: Chylothorax is a well-established acquired complication of thoracic surgery in infants. Current data suggest acquired chylothorax may affect infant growth and nutrition because of a loss of essential nutrients via chylous effusion. OBJECTIVE: The 3 objectives for this study were: (1) identify nutritional markers affected by the development of acquired chylothorax in infants; (2) highlight the variability in methods used to assess nutritional status and growth in this patient population; and (3) highlight nutritional deficits that can serve as treatment targets during postoperative feeding protocols. DATA SOURCES: A systematic literature search was conducted between May 31, 2021, and June 21, 2022, using the PubMed, Embase, CINAHL, and Web of Science databases. Search terms included, but were not limited to, "chylothorax," "infants," and "nutrition." DATA EXTRACTION: Inclusion criteria required studies that measured quantitative markers of nutrition in ≥10 participants aged <1 year with acquired chylothorax. A total of 575 studies were screened and all but 4 were eliminated. Nutritional markers were categorized into 4 different groups: total serum protein level, triglyceride levels, growth velocity, and weight for length. DATA ANALYSIS: The variation in methods, time points, interventional groups, and nutritional markers did not facilitate a meta-analysis. Risk of bias was assessed using the Cochrane Risk of Bias in Nonrandomized Studies assessment tool. CONCLUSION: This review highlights the need for reliable quantitative markers of nutrition that will enable providers to assess the nutritional needs of infants with chylothorax. Future studies must focus on measuring markers of nutrition at regular intervals in larger study populations.

Congenital cardiac defects: a possible association of aminopterin syndrome and in utero methotrexate exposure?

Folate antagonist are chemotherapeutic agents used in many neoplastic, autoimmune, and inflammatory disorders. The first suggestions that folic acid antagonists were teratogenic in humans were based on reports of failed terminations in mothers given aminopterin in the first trimester. Newborns who survived after aminopterin exposure were noted for years to have defects of the neural tube, skull, or limbs. There is now a well-defined syndrome of congenital anomalies associated with the use of aminopterin. The aminopterin syndrome consists of cranial dysostosis, hypertelorism, anomalies of the external ears, micrognathia, limb anomalies, and cleft palate. The use of aminopterin has now fallen out of favor. Methotrexate is a folate antagonist that is now used more frequently. A similar pattern of malformations has been found in fetuses exposed to methotrexate. If used during pregnancy, it can cause congenital malformations or fetal death. A consistent association between methotrexate exposure and cardiac, renal, or gastrointestinal malformations has not been reported. We report two patients who presented with classic features of aminopterin syndrome combined with significant congenital cardiac malformations after first-trimester in utero methotrexate exposure. Both of these patients survived to undergo corrective cardiac surgery.

Occlusive Pulmonary Artery Thrombosis in a Healthy Neonate With No Identifiable Risk Factors.

Pulmonary artery thrombosis is reported in neonates with risk factors for hypercoagulability. No consensus exists regarding standard therapy for this condition. We present a neonate, with no risk factors for thrombosis, who was admitted after birth to the Pediatric Cardiac Intensive Care Unit with an occlusive left pulmonary artery thrombus. (Level of Difficulty: Intermediate.).

Risk Factors for Gastrostomy Tube Placement in Single Ventricle Patients Following The Norwood Procedure.

Neonates with single ventricle heart disease frequently experience poor oral feeding and inconsistent weight gain, often requiring gastrostomy tube (gtube) placement. We sought to identify risk factors for gtube placement in neonates following the Norwood procedure at our institution. We retrospectively reviewed multiple preoperative, operative, and postoperative variables in neonates <30 days with single ventricle heart disease following the Norwood procedure. Study outcomes included duration of mechanical ventilation, hospital length of stay (HLOS), and gtube requirement. Multivariable logistic regression was used to analyze for associated risk factors. Seventy-nine neonates were included in the study, of which 47 underwent gtube placement (59.5%). Multivariable regression analysis found vocal cord dysfunction (P = 0.001, odds ratio 1.1, 95% confidence interval 1.0-1.4) and longer duration of sedative or narcotic infusion (P = 0.01, odds ratio 1.1, 1.03-1.2) to be independently associated with the requirement for gtube among patients who underwent the Norwood procedure. There was a significant difference in HLOS (median 69 vs 33, P = 0.003) between the gtube and the no gtube groups. Univariate analysis comparing the era of surgery was performed and found a significant difference between the groups in terms of the number of gtubes placed (P = 0.02) and duration of sedative or narcotic infusion days (P = 0.038). Both were greater in the era from 2011 to 2015. In a single-institution analysis of neonates following the Norwood procedure, gtube requirement was independently associated with vocal cord dysfunction and longer duration of sedative or narcotic infusions. gtube placement was also associated with longer HLOS.

Inadequate preoperative nutrition might be associated with acute kidney injury and greater illness severity postoperatively.

OBJECTIVE: Nutrition is vital for maintaining optimal cellular and organ function, particularly in neonates who undergo cardiac surgery. Achieving nutritional goals preoperatively can be challenging because of fluid restrictions, suboptimal oral intake, and concerns for inadequate gastrointestinal circulation. We examined preoperative caloric intake and its effects on postoperative course in neonates who underwent cardiac surgery. METHODS: We retrospectively reviewed records of neonates (younger than 30 days) who underwent congenital heart surgery requiring cardiopulmonary bypass from 2008 to 2014 at Arnold Palmer Hospital for Children. Data on multiple nutritional and postoperative variables were collected. Study outcomes included hospital length of stay, duration of mechanical ventilation, and acute kidney injury (AKI). RESULTS: Records of 95 neonates were reviewed. Sixty-six patients (69.5%) with a median age of 5 days did not achieve preoperative caloric goal, whereas 29 patients (30.5%) with a median age of 11 days did. Of those who achieved caloric goal, 6 (20.6%) achieved it via total parental nutrition, 9 (31.1%) with a combination of total parental nutrition and enteral feeds, and 14 (48.3%) via enteral route. There was a significant difference in peak lactate (P = .002), inotropic score (P = .02), and duration of mechanical ventilation (P = .013) between those who did and did not achieve caloric goal. In multivariable analysis we found that failure to achieve caloric goal preoperatively was independently associated with stage 2 or 3 AKI (P = .04; odds ratio, 4.48; 95% confidence interval, 1.02-19.63) and younger age at the time of surgery (P < .001; odds ratio, 0.12; 95% confidence interval, 0.04-0.33). CONCLUSIONS: Failure to achieve preoperative caloric goal might contribute to development of AKI and might be associated with greater severity of illness postoperatively.

Comparing del Nido and Conventional Cardioplegia in Infants and Neonates in Congenital Heart Surgery.

BACKGROUND: The aim of this study was to evaluate outcome measures after the use of del Nido (dN) cardioplegia compared with conventional multidose high-potassium (non-dN) cardioplegia in neonates and infants. METHODS: We retrospectively analyzed data in patients, aged younger than 1 year, undergoing cardiopulmonary bypass (CPB) from January 2012 to August 2015. We changed our cardioplegia protocol from non-dN to dN administered in a single or infrequently dosed strategy in September 2013. The outcomes of the dN group (n = 107) are compared with the non-dN group (n = 118). We analyzed variables for demographic, intraoperative, early postoperative, and discharge variables. RESULTS: The two groups were similar in age, weight, height, CPB, and cross-clamp time; preoperative and postoperative echocardiographic systolic functions; first 24-hour postoperative urine output and inotropic score; length of stay; and mortality rate. The Society of Thoracic Surgeons/European Association for Cardio-Thoracic Surgery Congenital Heart Surgery (STAT) mortality category was significantly higher in the dN group (p = 0.03). The cardioplegia dosing interval was lower for the non-dN group (p < 0.001). The volume and doses of cardioplegia per patient were significantly higher in the non-dN group (p < 0.001). In a subanalysis, when the Norwood patients were excluded from both groups, the overall STAT mortality category difference was no longer significant. The demographic, early postoperative, and discharge variables still showed no significant difference when the two groups were compared. CONCLUSIONS: Similar outcomes can be achieved with less frequent interruption of the operation and lower volume of cardioplegia when using dN cardioplegia solution compared with conventional cardioplegia. The dN cardioplegia with extended ischemic interval can be used as an alternative strategy in the neonatal and infant population during cardiac operations.

Single institution experience with the Ladd's procedure in patients with heterotaxy and stage I palliated single-ventricle.

AIM: To investigate and describe our current institutional management protocol for single-ventricle patients who must undergo a Ladd's procedure. METHODS: We retrospectively reviewed the charts of all patients from January 2005 to March 2014 who were diagnosed with heterotaxy syndrome and an associated intestinal rotation anomaly who carried a cardiac diagnosis of functional single ventricle and were status post stage I palliation. A total of 8 patients with a history of stage I single-ventricle palliation underwent Ladd's procedure during this time period. We reviewed each patients chart to determine if significant intraoperative or post-operative morbidity or mortality occurred. We also described our protocolized management of these patients in the cardiac intensive care unit, which included pre-operative labs, echocardiography, milrinone infusion, as well as protocolized fluid administration and anticoagulation regimines. We also reviewed the literature to determine the reported morbidity and mortality associated with the Ladd's procedure in this particular cardiac physiology and if other institutions have reported protocolized care of these patients. RESULTS: A total of 8 patients were identified to have heterotaxy with an intestinal rotation anomaly and single-ventricle heart disease that was status post single ventricle palliation. Six of these patients were palliated with a Blaylock-Taussig shunt, one of whom underwent a Norwood procedure. The two other patients were palliated with a stent, which was placed in the ductus arteriosus. These eight patients all underwent elective Ladd's procedure at the time of gastrostomy tube placement. Per our protocol, all patients remained on aspirin prior to surgery and had no period where they were without anticoagulation. All patients remained on milrinone during and after the procedure and received fluid administration upon arrival to the cardiac intensive care unit to account for losses. All 8 patients experienced no intraoperative or post-operative complications. All patients survived to discharge. One patient presented to the emergency room two months after discharge in cardiac arrest and died due to bowel obstruction and perforation. CONCLUSION: Protocolized intensive care management may have contributed to favorable outcomes following Ladd's procedure at our institution.

Culture Negative Stent Infection in an Infant with Hypoplastic Left Heart and Persistent Fever.

We present an infant with hypoplastic left heart with persistent fever despite two courses of antibiotics and repeatedly negative blood cultures. He eventually underwent surgical extraction of two stents. The stent cultures became positive; he was treated with 4 weeks of antibiotics and the fever resolved.

Are We Overdiagnosing Acute Kidney Injury in Pediatric Patients Following Cardiac Surgery?

BACKGROUND: The use of two diagnostic criteria in the current literature has led to some degree of ambiguity in the precise diagnosis of acute kidney injury in pediatric patients undergoing surgery for congenital heart disease. This study aims to determine which criteria is the most accurate diagnostic indicator of acute kidney injury and determine whether the incidence is being overestimated based on the current criteria. METHODS: This retrospective study consisted of 389 patients with congenital heart disease from birth to 18 years, who underwent cardiac surgery. The statistical tests conducted were the student t test and chi-square test. Outcomes measured included hospital length of stay, duration of mechanical ventilation, and mortality. RESULTS: The incidence rate of acute kidney injury diagnosed by the pediatric Risk, Injury, Failure, Loss, and End-Stage Renal Disease (RIFLE) criterion was 56% compared to 24.4% for the Acute Kidney Injury Network criterion. The pediatric RIFLE criterion consists of the following subsets: risk, injury, failure, loss, and end-stage renal disease. Patients classified in the "risk" subset of the pediatric RIFLE criterion who failed to meet Acute Kidney Injury Network criterion were compared to patients without acute kidney injury. Comparison of intensive care unit outcomes between these groups lacked statistical significance for all variables except the duration of mechanical ventilation postoperatively. CONCLUSION: Although recent research in this field identified the pediatric RIFLE criterion as the most sensitive indicator of acute kidney injury, the results of this study suggest the pediatric RIFLE criterion overestimates acute kidney injury incidence and that the Acute Kidney Injury Network criterion is the more accurate diagnostic indicator.

Acute Kidney Injury and Fluid Overload in Neonates Following Surgery for Congenital Heart Disease.

BACKGROUND: Acute kidney injury (AKI) and fluid overload have been shown to increase morbidity and mortality. The reported incidence of AKI in pediatric patients following surgery for congenital heart disease is between 15% and 59%. Limited data exist looking at risk factors and outcomes of AKI or fluid overload in neonates undergoing surgery for congenital heart disease. METHODS: Neonates aged 6 to 29 days who underwent surgery for congenital heart disease and who were without preoperative kidney disease were included in the study. The AKI was determined utilizing the Acute Kidney Injury Network criteria. RESULTS: Ninety-five neonates were included in the study. The incidence of neonatal AKI was 45% (n = 43), of which 86% had stage 1 AKI. Risk factors for AKI included cardiopulmonary bypass time, selective cerebral perfusion, preoperative aminoglycoside use, small kidneys by renal ultrasound, and risk adjustment for congenital heart surgery category. There were eight mortalities (five from stage 1 AKI group, three from stage 2, and zero from stage 3). Fluid overload and AKI both increased hospital length of stay and postoperative ventilator days. CONCLUSION: To avoid increased risk of morbidity and possibly mortality, every attempt should be made to identify and intervene on those risk factors, which may be modifiable or identifiable preoperatively, such as small kidneys by renal ultrasound.

Computed tomography angiography successfully used to diagnose postoperative systemic-pulmonary artery shunt narrowing.

In recent years, there has been a marked reduction in surgical mortality for many complex forms of congenital heart disease. Treatment or palliative strategies vary but may include systemic-pulmonary central or Blalock-Taussig shunt. These shunts can be complicated by overcirculation, infection, thrombosis, and thromboembolism. Many diagnostic modalities are available to aide in diagnosis of postoperative shunt complications including echocardiography and cardiac catheterization but these may be invasive, inconclusive, or difficult to obtain adequate images. Computed tomography angiography (CTA) has many attributes that make it potentially useful in the evaluation of congenital heart disease and postoperative shunt complications. We report one patient where CTA guided the post-operative algorithm and appropriately identified a shunt narrowing despite repeated echocardiograms showing a patent shunt. These findings along with clinical suspicion appropriately guided us toward cardiac catheterization. To our knowledge, this is the first paper where CTA appropriately suspected a shunt narrowing in the absence of echocardiographic confirmation.

Multifocal lymphangioendotheliomatosis with thrombocytopenia: a rare cause of gastrointestinal bleeding in the newborn period.

Severe gastrointestinal bleeding in the newborn period is a serious but uncommon phenomenon that has a broad differential diagnosis. In the following case report we describe a rare phenomenon in which a newborn presents with severe hematemesis, hematochezia, and thrombocytopenia that are resistant to repeated platelet and packed red blood cell transfusions. Previous cases have been reported, but none of the patients described presented within the first 8 days of life. The early age of presentation and refractory nature of this disease entity to multiple therapies make it a diagnostic and therapeutic dilemma for all physicians involved in the care of newborns.