Avoiding Furosemide Ototoxicity Associated With Single-Ventricle Repair in Young Infants.

OBJECTIVE: To reduce bilateral delayed-onset progressive sensory permanent hearing loss using a systems-wide quality improvement project with adherence to best practice for the administration of furosemide. DESIGN: Prospective cohort study with regular audiologic follow-up assessment of survivors both before and after a 2007-2008 quality improvement practice change. SETTING: The referral center in Western Canada for complex cardiac surgery, with comprehensive multidisciplinary follow-up by the Complex Pediatric Therapies Follow-up Program. PATIENTS: All consecutive patients having single-ventricle palliative cardiac surgery at age 6 weeks old or younger. INTERVENTIONS: A 2007-2008 quality improvement practice change consisted of a Parenteral Drug Monograph revision indicating slow IV administration of furosemide, an educational program, and an evaluation. MEASUREMENTS AND MAIN RESULTS: The outcome measure was the prevalence of permanent hearing loss by 4 years old. Firth multiple logistic regression compared pre (1996-2008) to post (2008-2012) practice change occurrence of permanent hearing loss, adjusting for confounding variables, including all hospital days, extracorporeal membrane oxygenation, cardiopulmonary bypass time, age at first surgery, dialysis, and sepsis. From 1996 to 2012, 259 infants had single-ventricle palliative surgery at age 6 weeks old or younger, with 173 (64%) surviving to age 4 years. Of survivors, 106 (61%) were male, age at surgery was 11.6 days (9.0 d), and total hospitalization days by age 4 years were 64 (42); 18 (10%) had cardiopulmonary resuscitation and 38 (22%) had sepsis at any time. All 173 (100%) had 4-year follow-up. Pre- to postpractice change permanent hearing loss dropped from 17/100 (17%) to 0/73 (0%) of survivors. On Firth multiple logistic regression, the only variable statistically associated with permanent hearing loss was the pre- to postpractice change time period (odds ratio, 0.03; 95% CI, 0-0.35; p = 0.001). CONCLUSIONS: A practice change to ensure slow IV administration of furosemide eliminated permanent hearing loss. Centers caring for critically ill infants, particularly those with single-ventricle anatomy or hypoxia, should review their drug administration guidelines and adhere to best practice for administration of IV furosemide.

Prevalence of Childhood Permanent Hearing Loss after Early Complex Cardiac Surgery.

OBJECTIVES: To estimate the prevalence of childhood permanent hearing loss (PHL) after early cardiac surgery. STUDY DESIGN: This prospective observational (1996-2015) study after complex cardiac surgery with cardiopulmonary bypass at ≤6 weeks of life reports audiology follow-up by registered pediatric-experienced audiologists at 6-8 months postsurgery, age 2 years, and as required throughout and thereafter to complete diagnoses. PHL at any frequency (500-4000 Hz) is defined as responses of >25-decibel hearing level in either ear. PHL was evaluated by type (conductive or sensorineural), pattern (flat or sloping), and severity (mild to profound). RESULTS: Survival rate was 83.4% (706 of 841 children) with a 97.9% follow-up rate (691 children); 41 children had PHL, 5.9% (95% CI 4.3%, 8.0%). By cardiac defect, prevalence was biventricular, 4.0% (95%CI 2.5%, 6.1%); single ventricle, 10.8% (95%CI 6.8%, 16.1%). Eighty-seven (12.6%) of 691 had syndromes/genetic abnormalities with known association with PHL; of these, 17 (41.5%) had PHL. Of 41 children, 4 had permanent conductive, moderate to severe loss (1 bilateral); 37 had moderate to profound sensorineural loss (29 bilateral with 20 sloping and 9 flat), 6 with cochlear implant done or recommended. CONCLUSIONS: Infants surviving complex cardiac surgery are at high risk for PHL. Over 40% with PHL have known syndromes/genetic abnormalities, but others do not have easily identifiable risk indicators. Early cardiac surgery should be considered a risk indicator for PHL.

Bilateral sensory permanent hearing loss after palliative hypoplastic left heart syndrome operation.

BACKGROUND: Bilateral sensory permanent hearing loss (PHL) has been reported after neonatal respiratory failure but has rarely been noted in survivors after cardiac operations. We report the prevalence and severity of PHL after Norwood right ventricular-pulmonary artery shunt for hypoplastic left heart syndrome (HLHS), document progressive loss, and explore markers of acute illness and ototoxic medications for PHL. METHODS: This interprovincial longitudinal outcome study after neonatal complex cardiac operations at Stollery Children's Hospital, Edmonton, Alberta, Canada, 2002 to 2007, completed repeated diagnostic audiologic assessments for all survivors by registered pediatric-experienced audiologists. Demographic, surgical, and perisurgical variables, including ototoxic medications, were collected. The association of potentially predictive variables with PHL and its severity were determined by univariate analysis and multiple logistic and linear regression analysis. RESULTS: At an age older than 3.5 years, progressive PHL was present in 12 of 42 survivors (28.6%, 95% confidence interval, 16.2% to 44.8%; mortality, 20.9%). Overall lowest partial pressure of arterial oxygen (odds ratio, 1.315; 95% confidence interval, 1.051 to 1.506), and cumulative dose of furosemide given as bolus (odds ratio, 1.062; 95% confidence interval, 1.018 to 1.109) combined to predict PHL and gave 39% of the variance of PHL severity. Antibiotics and neuromuscular blockers were not associated with PHL. CONCLUSIONS: Monitoring outcomes of neonates after HLHS surgery revealed unexpected PHL associated with hypoxia and bolus administration of furosemide. As survival improves, close follow-up is necessary to identify outcomes and seek modifiable predictive variables. Changes in the mode of furosemide administration may prevent this complication.