A complicated Glenn procedure: risk factors and association with adverse long-term neurodevelopmental and functional outcomes.

OBJECTIVES: To determine potentially modifiable risk factors for a complicated Glenn procedure (cGP) and whether a cGP predicted adverse neurodevelopmental and functional outcomes. A cGP was defined as post-operative death, heart transplant, extracorporeal life support, Glenn takedown, or prolonged ventilation. METHODS: All 169 patients having a Glenn procedure from 2012 to 2017 were included. Neurodevelopmental assessments were performed at age 2 years in consenting survivors (n = 156/159 survivors). The Bayley Scales of Infant and Toddler Development-3rd Edition (Bayley-III) and the Adaptive Behavior Assessment System-2nd Edition (ABAS-II) were administered. Adaptive functional outcomes were determined by the General Adaptive Composite (GAC) score from the ABAS-II. Predictors of outcomes were determined using univariate and multiple variable linear or Cox regressions. RESULTS: Of patients who had a Glenn procedure, 10/169 (6%) died by 2 years of age and 27/169 (16%) had a cGP. Variables statistically significantly associated with a cGP were the inotrope score on post-operative day 1 (HR 1.04, 95%CI 1.01, 1.06; p = 0.010) and use of inhaled nitric oxide post-operatively (HR 7.31, 95%CI 3.19, 16.76; p < 0.001). A cGP was independently statistically significantly associated with adverse Bayley-III Cognitive (ES -10.60, 95%CI -17.09, -4.11; p = 0.002) and Language (ES -11.43, 95%CI -19.25, -3.60; p = 0.004) scores and adverse GAC score (ES -14.89, 95%CI -22.86, -6.92; p < 0.001). CONCLUSIONS: Higher inotrope score and inhaled nitric oxide used post-operatively were associated with a cGP. A cGP was independently associated with adverse 2-year neurodevelopmental and functional outcomes. Whether early recognition and intervention for risk of a cGP can prevent adverse outcomes warrants study.

Near-fatal pediatric methemoglobinemia secondary to intentional sodium nitrite ingestion.

Methemoglobinemia is the result of inappropriate oxidation of hemoglobin iron groups, leading to a failure of oxygen transport and delivery, resulting in a clinical state of refractory hypoxia. Methemoglobin levels above 70% are often considered fatal. Acquired methemoglobinemia can be caused by a variety of substances, including sodium nitrite, a commercially available food preservative and color fixative. This report describes a patient presenting with a methemoglobin level of 83% secondary to intentional sodium nitrite ingestion. The methemoglobin level recorded is amongst some of the highest found in surviving patients.

Setting up a Rescue Extracorporeal Life Support Program.

Extracorporeal life support (ECLS) is a high-risk, lifesaving medical treatment that is typically limited to centers that can support a comprehensive ECLS program. Rescue programs can bridge the gap in care between ECLS centers and other tertiary pediatric centers without cardiac surgical and comprehensive ECLS support. We describe how our pediatric center without cardiac surgery successfully partnered with an established ECLS center to develop a Rescue ECLS Cannulation Program. This formalized program provides cannulation and stabilization by a specialized team at the presenting hospital before being transported to a partner hospital. This article outlines how we established our unique Rescue ECLS Cannulation program. We outline the planning, development, and implementation of the program and describe the unique aspects contributing to successful implementation including longitudinal training, staged program evolution, and a bundled approach to care. We also describe the patients who we have cannulated since its inception. Rescue ECLS Cannulation Programs provide access to consistent, high-quality, and lifesaving care to critically ill patients at sites without the resources to support a full ECLS program.