Postoperative optimization of cochlear implantation for single sided deafness and asymmetric hearing loss: a systematic review.

OBJECTIVE: Identify and evaluate the effectiveness of methods for improving postoperative cochlear implant (CI) hearing performance in subjects with single-sided deafness (SSD) and asymmetric hearing loss (AHL). DATA SOURCES: Embase, PubMed, Scopus. REVIEW METHODS: Systematic review and narrative synthesis. English language studies of adult CI recipients with SSD and AHL reporting a postoperative intervention and comparative audiometric data pertaining to speech in noise, speech in quiet and sound localization were included. RESULTS: 32 studies met criteria for full text review and 6 (n = 81) met final inclusion criteria. Interventions were categorized as: formal auditory training, programming techniques, or hardware optimization. Formal auditory training (n = 10) found no objective improvement in hearing outcomes. Experimental CI maps did not improve audiologic outcomes (n = 9). Programed CI signal delays to improve synchronization demonstrated improved sound localization (n = 12). Hardware optimization, including multidirectional (n = 29) and remote (n = 11) microphones, improved sound localization and speech in noise, respectively. CONCLUSION: Few studies meeting inclusion criteria and small sample sizes highlight the need for further study. Formal auditory training did not appear to improve hearing outcomes. Programming techniques, such as CI signal delay, and hardware optimization, such as multidirectional and remote microphones, show promise to improve outcomes for SSD and AHL CI users.

Life-Threatening Hemoptysis in a Pediatric Referral Center.

OBJECTIVES: Hemoptysis is uncommon in children, even among the critically ill, with a paucity of epidemiological data to inform clinical decision-making. We describe hemoptysis-associated ICU admissions, including those who were critically ill at hemoptysis onset or who became critically ill as a result of hemoptysis, and identify predictors of mortality. DESIGN: Retrospective cohort study. Demographics, hemoptysis location, and management were collected. Pediatric Logistic Organ Dysfunction-2 score within 24 hours of hemoptysis described illness severity. Primary outcome was inhospital mortality. SETTING: Quaternary pediatric referral center between July 1, 2010, and June 30, 2017. PATIENTS: Medical/surgical (PICU), cardiac ICU, and term neonatal ICU admissions with hemoptysis during or within 24 hours of ICU admission. INTERVENTIONS: No intervention. MEASUREMENTS AND MAIN RESULTS: There were 326 hemoptysis-associated ICU admissions in 300 patients. Most common diagnoses were cardiac (46%), infection (15%), bronchiectasis (10%), and neoplasm (7%). Demographics, interventions, and outcomes differed by diagnostic category. Overall, 79 patients (26%) died inhospital and 109 (36%) had died during follow-up (survivor mean 2.8 ± 1.9 yr). Neoplasm, bronchiectasis, renal dysfunction, inhospital hemoptysis onset, and higher Pediatric Logistic Organ Dysfunction-2 score were independent risk factors for inhospital mortality (p < 0.02). Pharmacotherapy (32%), blood products (29%), computerized tomography angiography (26%), bronchoscopy (44%), and cardiac catheterization (36%) were common. Targeted surgical interventions were rare. Of survivors, 15% were discharged with new respiratory support. Of the deaths, 93 (85%) occurred within 12 months of admission. For patients surviving 12 months, 5-year survival was 87% (95% CI, 78-92) and mortality risk remained only for those with neoplasm (log-rank p = 0.001). CONCLUSIONS: We observed high inhospital mortality from hemoptysis-associated ICU admissions. Mortality was independently associated with hemoptysis onset location, underlying diagnosis, and severity of critical illness at event. Additional mortality was observed in the 12-month posthospital discharge. Future directions include further characterization of this vulnerable population and management recommendations for life-threatening pediatric hemoptysis incorporating underlying disease pathophysiology.

Mortality and Factors Associated With Hemorrhage During Pediatric Extracorporeal Membrane Oxygenation.

OBJECTIVES: To quantify and identify factors associated with bleeding events during pediatric extracorporeal membrane oxygenation. DESIGN: Retrospective cohort study with primary outcome of bleeding days on extracorporeal membrane oxygenation. SETTING: Single tertiary care children's hospital. SUBJECTS: One-hundred twenty-two children supported with extracorporeal membrane oxygenation for greater than 12 hours during January 2015 through December 2016. INTERVENTIONS: Bleeding days were identified if mediastinal or cannula site exploration, activated factor VII administration, gastrointestinal, pulmonary, or intracranial hemorrhages occurred. Logistic regression was used to assess factors associated with bleeding days. MEASUREMENTS AND MAIN RESULTS: Study population was identified from institutional extracorporeal membrane oxygenation database. Clinical, laboratory, and survival data were obtained from medical records. Only data from patients' first extracorporeal membrane oxygenation run were used. One-hundred twenty-two patients with median age of 17 weeks (interquartile range, 1-148 wk) were analyzed. Congenital heart disease (n = 56, 46%) was the most common diagnosis. Bleeding days comprised 179 (16%) of the 1,121 observed extracorporeal membrane oxygenation-patient-days. By extracorporeal membrane oxygenation day 4, 50% of users had experienced a bleeding day. Central rather than peripheral cannulation (odds ratio, 2.58; 95% CI, 1.47-4.52; p < 0.001), older age (odds ratio, 1.31 per increased week; 95% CI, 1.14-1.52; p < 0.001), higher lactate (odds ratio, 1.08 per 1 mmol/L increase; 95% CI, 1.05-1.12; p < 0.001), and lower platelets (odds ratio, 0.87 per 25,000 cell/µL increase; 95% CI, 0.77-0.99; p = 0.005) were associated with bleeding days. Patients who experienced more frequent bleeding (> 75th percentile) had fewer ventilator-free and hospital-free days in the 60 days after cannulation (0 vs 31; p = 0.002 and 0 vs 0; p = 0.008) and higher in-hospital mortality (68 vs 34%; p < 0.001). CONCLUSIONS: Central cannulation, older age, low platelets, and high lactate are associated with bleeding days during pediatric extracorporeal membrane oxygenation. Patients who bleed more frequently during extracorporeal membrane oxygenation have higher in-hospital mortality, longer technological dependence, and reduced hospital-free days.

RBC Exposure in Pediatric Extracorporeal Membrane Oxygenation: Epidemiology and Factors Associated With Large Blood Transfusion Volume.

OBJECTIVES: To quantify and identify factors associated with large RBC exposure in children supported with extracorporeal membrane oxygenation. DESIGN: Retrospective cohort study. SETTING: Single tertiary care children's hospital. PATIENTS: One-hundred twenty-two children supported with extracorporeal membrane oxygenation for greater than 12 hours during January 1, 2015, to December 31, 2016. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Clinical, laboratory, and survival data were obtained from medical records. Only data from patients' first extracorporeal membrane oxygenation run were used. The primary outcome was RBC volume exposure during extracorporeal membrane oxygenation (mL/kg/d). Patients with RBC exposure volume greater than 75th percentile were categorized as "high RBC use" patients. A "bleeding day" was identified if mediastinum or cannula sites were explored and/or Factor VIIa administration, gastrointestinal, pulmonary, or intracranial hemorrhages occurred. Median age was 0.3 years (interquartile range, 0-3 yr). Congenital heart disease (n = 56; 46%) was the most common diagnosis. Median RBC volume transfused during extracorporeal membrane oxygenation was 39 mL/kg/d (interquartile range, 21-66 mL/kg/d). High RBC use patients were more likely be supported by venoarterial extracorporeal membrane oxygenation (100 vs 76%; p = 0.006), have congenital heart disease (68 vs 39%; p = 0.02), and experience bleeding (33 vs 11% d; p < 0.001). High RBC use patients showed a trend toward higher in-hospital mortality (58 vs 37%; p = 0.07). In the multivariable analysis, younger age (-9% per year; 95% CI, -10% to -7%; p < 0.001), more blood draws per day (+8%; 95% CI, 6-11%; p < 0.001), and higher proportion of bleeding days (+22% per 10% increase; 95% CI, 16-29%; p < 0.001) were associated with larger RBC exposure (model R = 0.66). CONCLUSIONS: Bleeding during extracorporeal membrane oxygenation, frequent laboratory draws, and younger age were associated with increased RBC exposure during extracorporeal membrane oxygenation. Higher transfusion volume was associated with increased mortality.

The impact of right ventricular pressure and function on survival in patients with pulmonary vein stenosis.

Pulmonary vein stenosis (PVS) is associated with pulmonary hypertension (PH), but there is little information regarding the impact of PH on right ventricular (RV) systolic function and survival. We conducted a retrospective cohort study of our patients to explore this and other aspects of pulmonary hemodynamics with PVS. RV function was assessed using qualitative two-dimensional echocardiography. The ratio of systolic pulmonary artery (PA) and aortic pressures (PA:Ao) at cardiac catheterization reflected pulmonary hemodynamics. Reactivity testing employed inhaled nitric oxide + 100% fiO2, or 100% fiO2 only; "reactivity" was a ≥ 20% decrease in PA:Ao. There were 105 PVS patients, although not all had data at every time point. (1) The mean PA:Ao at first cardiac catheterization (n = 77) was 0.79 ± 0.36; at last catheterization (n = 54), PA:Ao = 0.69 ± 0.30; 90% had systolic PAP > one-half systemic. Survival was shorter with PA:Ao > 0.5. (2) Differences in survival relative to RV dysfunction on the first echocardiogram were not significant, although they were using the last echocardiogram. (3) The magnitude of RV dysfunction was positively correlated with PA:Ao. (4) Balloon dilation of PV acutely decreased PA:Ao (-0.13 ± 0.37, P = 0.03 [n = 40 patients]). 5. Of 20 patients tested, 13 were acutely reactive to vasodilators. PH is a major feature of PVS. Reduced RV function and PA:Ao appear to be predictors of survival. Given the importance of PH in this disease, clinical studies of PVS treatments should include measures of PAP and RV function as important variables of interest.