Comparison of computed tomography angiography versus cardiac catheterization for preoperative evaluation of major aortopulmonary collateral arteries in pulmonary atresia with ventricular septal defect.

INTRODUCTION: Pulmonary atresia with the ventricular septal defect is a rare congenital heart defect with high anatomic variability. The most important management question relates to the sources of pulmonary blood flow. The ability to differentiate between ductal dependence and major aortopulmonary collateral arteries is critical to achieving good outcomes and avoiding life-threatening hypoxia in the postneonatal period. Having accurate information about pulmonary arteries, major aortopulmonary collateral arteries, and sources of blood supply to each pulmonary segment is crucial for choosing the optimal surgical strategy. The purpose of this study is to compare computed tomography angiography (CTA) with cardiac catheterization for anatomic delineation of surgically relevant anatomy in pulmonary atresia with ventricular septal defect with major aortopulmonary collateral arteries. MATERIALS AND METHODS: Retrospective review of all children with pulmonary atresia with ventricular septal defect with major aortopulmonary collateral arteries cared for at a large tertiary children's hospital who underwent cardiac catheterization with angiography and CTA close to each other without interval therapy. All studies were performed between 2007 and 2011. RESULTS: There were 9 patients who met the inclusion criteria. Pulmonary artery anatomy (confluent vs. nonconfluent) was correctly identified in 9 patients by CTA and 8 patients by catheterization. There were no significant differences between CTA and catheterization in the identification of major aortopulmonary collateral arteries (mean = 3.4 collaterals/study via catheterization; mean = 3.1 collaterals/study via CTA; P = 0.67). CTA was superior to catheterization in the delineation of segmental pulmonary blood flow (P = 0.006). CONCLUSION: CTA and catheterization are equivalent in their ability to delineate pulmonary artery anatomy and major aortopulmonary collateral arteries.

Importance of Relationship between Ductus and Isthmus in Fetal Diagnosis of Coarctation of Aorta.

BACKGROUND: The prenatal diagnosis of coarctation of aorta (CoA) can prove problematic, with relatively high false-positive and false-negative rates. This significantly impacts both prenatal counseling and postnatal management. We sought to evaluate a variety of prenatal echo indices to determine which would best predict neonatal CoA. METHODS: Fetal echocardiograms of those with prenatal diagnosis of COA were analyzed for the following: diameter of cardiac valves, ascending aorta, distal transverse arch, aortic isthmus, and ductus; right (RV) and left ventricular (LV) length and end-diastolic area and isthmus-ductal angle (IDA). Ratios of RV: LV area, aortic: pulmonary valve diameter, mitral: tricuspid valve ratio (MV:TV ratio), and isthmus: ductal diameter (IDD) were calculated. These measures were compared between those with CoA after birth (CoA group) and those without (no CoA group). RESULTS: Of the 62 subjects, 27 were in CoA and 35 in no CoA group. CoA group had a significantly smaller mitral valve, MV:TV ratio, IDD, and IDA compared to no CoA group. The ROC curves for each of these significant measures showed that mitral valve, IDD, and IDA had an AUC of 0.72, 0.80, and 0.83, respectively. Multiple variable model using at least two of these measures had 85% sensitivity and 60% specificity. CONCLUSIONS: A smaller mitral valve, MV:TV ratio, IDD, and IDA are associated with development of neonatal coarctation. In cases with suspected prenatal diagnosis of CoA, careful evaluation of the relation between the isthmus and the ductus arteriosus using IDD and IDA may enhance the diagnostic accuracy of fetal echocardiograms.

The Miniaturized Multiplane Micro-Transesophageal Echocardiographic Probe: A Comparative Evaluation of Its Accuracy and Image Quality.

BACKGROUND: The initial experience with the miniaturized multiplane micro-transesophageal echocardiographic probe (MTEE) reported high-quality diagnostic imaging in small infants. The aim of this study was to compare the diagnostic accuracy and image quality of the intraoperative MTEE with the pediatric multiplane transesophageal echocardiographic probe (PTEE). METHODS: Infants weighing <5 kg who underwent intraoperative transesophageal echocardiography were identified. Studies using the MTEE were matched 1:1 with those using the PTEE by cardiac diagnosis. The postoperative transesophageal echocardiograms, obtained using either probe, were reviewed for the presence of 11 cardiac abnormalities. Postoperative transesophageal echocardiograms were compared with predischarge transthoracic echocardiograms to assess accuracy. Using receiver operating characteristic curves, the areas under the curve for the MTEE and PTEE were compared. Two pediatric cardiologists scored six image quality metrics on equal numbers of studies obtained with the MTEE and the PTEE. Composite scores from both reviewers were used to compare image quality. RESULTS: The study included 110 transesophageal echocardiograms per probe type. The mean weight for the MTEE was lower than for the PTEE (3.15 ± 0.58 vs 3.70 ± 0.52 kg, P < .001). There was no significant difference in the diagnostic accuracy of the MTEE and PTEE using receiver operating characteristic curves. The numbers of residual anatomic lesions missed by the MTEE and PTEE were similar (19 vs 22, respectively). The composite image quality score was worse for the MTEE compared with the PTEE (81% vs 92%, respectively, P < .0001). CONCLUSIONS: Although the image quality of the MTEE is inferior compared with the PTEE, its diagnostic accuracy in infants weighing <5 kg is comparable.

Perioperative nutritional support and malnutrition in infants and children with congenital heart disease.

OBJECTIVE: To assess the effect of nutritional status and cardiovascular risk on hospital outcomes after congenital heart surgery in infants and children. DESIGN: Retrospective study. SETTING: Cardiac intensive care unit in a tertiary-care children's hospital. PATIENTS: One hundred twenty-one patients <24 months of age admitted to the cardiovascular intensive care unit (CVICU) for >48 hours following cardiac surgery. METHODS: Demographics, Risk Adjustment for Congenital Heart Surgery-1 (RACHS-1), Paediatric Index of Mortality 2, and Pediatric Risk of Mortality III scores were obtained on admission. CVICU nutritional intake was calculated for 7 days. Energy and protein needs were estimated using recommended guidelines. Risk Adjustment for Congenital Heart Surgery-1 was categorized as (1-3) or (4-6). Malnutrition was categorized by Waterlow criteria and correlated with mortality risk, days of mechanical ventilation, and hospital and CVICU length of stay. RESULTS: Ninety-one patients who underwent cardiac surgery were categorized as RACHS-1 (1-3) and RACHS-1 scores of (4-6) (n = 30). Patients with RACHS-1 (4-6) had higher mortality risk by Pediatric Risk of Mortality III (4.9% vs. 2.6%, P < .01), longer CVICU (10.4 days vs. 4.8 days) and hospital stays (28 days vs.14 days), and more days of mechanical ventilation (4 days vs. 2 days) (all P < .005) than RACHS-1 (1-3). The prevalences of acute protein-energy malnutrition and chronic protein-energy malnutrition were 51.2% and 40.5%. The median hospital stay for mild, moderate, and severe chronic protein-energy malnutrition was 31, 10, and 22.5 days, respectively, vs. normal, 15 days (Kruskal-Wallis, P < .005). The average energy and protein requirements met on day 7 were 68 ± 27(SD)% and 68 ± 40%, respectively. CONCLUSION: Although nearly half of the patients were malnourished at surgery, only two-thirds of their recommended caloric and protein requirements were provided by week 1. To improve hospital outcomes, care should be taken to optimize the nutritional condition of infants and children prior to and following surgical correction of congenital heart disease to improve hospital outcomes.

Pediatric off-label use of recombinant factor VIIa.

OBJECTIVE: Our goal was to report our institutional experience with recombinant factor VIIa for the treatment and/or prevention of bleeding in nonhemophiliac children. METHODS: This was a retrospective case series in a tertiary pediatric referral hospital. RESULTS: During 1999-2006, 135 patients received recombinant factor VIIa for off-label use. The median number of doses was 2; the median dose was 88 mug/kg. The most common diagnoses among patients receiving recombinant factor VIIa were disseminated intravascular coagulation/sepsis (28), surgical bleeding (19), procedural prophylaxis (16), and trauma (15). The median volume of blood products administered 24 hours before recombinant factor VIIa treatment was 29.7 vs 11.7 mL/kg 24 hours after treatment. Only 1 high-risk patient had significant bleeding after receiving prophylactic recombinant factor VIIa before an invasive procedure. Nonsurvivors had significantly increased incidence of multiple organ dysfunction syndrome (75%) compared with survivors (23%). The largest group of patients (n = 28) received recombinant factor VIIa for bleeding and/or coagulopathy because of disseminated intravascular coagulation; the mortality in this group was 26 (93%) of 28. Eleven patients received multiple doses of recombinant factor VIIa to treat bleeding complications after hematopoietic stem cell transplant, without improvement in blood use. Mortality in medical patients was 58% vs 16% in surgical patients. Three patients had significant thrombotic adverse events after receiving recombinant factor VIIa, resulting in 2 deaths and 1 leg amputation. CONCLUSIONS: Off-label use of recombinant factor VIIa significantly decreases blood-product administration; surgical patients had control of life-threatening bleeding with low associated mortality. Prophylactic recombinant factor VIIa may be effective in preventing bleeding if given before invasive procedures in children at high risk. Prolonged use of recombinant factor VIIa for bleeding complications after hematopoietic stem cell transplant is not effective in preventing packed red blood cell transfusion. Presence of disseminated intravascular coagulation and mulitorgan dysfunction syndrome may help predict futility of recombinant factor VIIa treatment. Off-label use of recombinant factor VIIa is associated with thromboembolic events in children.

Infectious disease survey of lesser prairie chickens in north Texas.

Lesser prairie chicken (Tympanuchus pallidicinctus) abundance, like that of most grassland birds, has declined rangewide for decades. Although habitat loss and degradation are likely ultimate causes for this decline, infectious agents, particularly microparasites, could be proximate contributors. No surveys of pathogenic bacteria or viruses have been published for this species. We surveyed 24 free-living lesser prairie chickens from Hemphill County, Texas (USA), for evidence of exposure to Salmonella typhimurium, S. pullorum, Mycoplasma gallisepticum, M. synoviae, Chlamydophila psittaci, and the avian influenza, Newcastle disease, infectious bronchitis, and reticuloendotheliosis viruses. Two of 18, and eight of 17 samples were seropositive for the Massachusetts and Arkansas serotypes of infectious bronchitis virus, respectively. Five of the eight positive individuals were juveniles, two of which were seropositive for both serotypes. All other serologic and genetic tests were negative. Because the ecological significance of these results is unknown, the pathogenesis, transmission, and/or population-level influences of infectious bronchitis and related avian coronaviruses for lesser prairie chickens deserves further study.