Trisomy 21 and Congenital Heart Disease: Impact on Health and Functional Outcomes From Birth Through Adolescence: A Scientific Statement From the American Heart Association.

Due to improvements in recognition and management of their multisystem disease, the long-term survival of infants, children, and adolescents with trisomy 21 and congenital heart disease now matches children with congenital heart disease and no genetic condition in many scenarios. Although this improved survival is a triumph, individuals with trisomy 21 and congenital heart disease have unique and complex care needs in the domains of physical, developmental, and psychosocial health, which affect functional status and quality of life. Pulmonary hypertension and single ventricle heart disease are 2 known cardiovascular conditions that reduce life expectancy in individuals with trisomy 21. Multisystem involvement with respiratory, endocrine, gastrointestinal, hematological, neurological, and sensory systems can interact with cardiovascular health concerns to amplify adverse effects. Neurodevelopmental, psychological, and functional challenges can also affect quality of life. A highly coordinated interdisciplinary care team model, or medical home, can help address these complex and interactive conditions from infancy through the transition to adult care settings. The purpose of this Scientific Statement is to identify ongoing cardiovascular and multisystem, developmental, and psychosocial health concerns for children with trisomy 21 and congenital heart disease from birth through adolescence and to provide a framework for monitoring and management to optimize quality of life and functional status.

Nurses' perceptions and use of near infrared spectroscopy in paediatric cardiac intensive care.

BACKGROUND: Near-infrared spectroscopy (NIRS) is a non-invasive technology that estimates regional oxygen saturation. Literature demonstrates that NIRS can provide valuable data for clinical staff. However, little research has addressed the nursing care and management of NIRS in the critical care environment. AIMS: To assess nurses' perception around the use of NIRS and current NIRS practice within paediatric cardiac intensive care unit (PCICUs). STUDY DESIGN: A 53-item cross-sectional electronic survey was developed to assess indications for NIRS, critical value thresholds and interventions, barriers to use, policies and procedures, and nursing perceptions. Descriptive statistics were used to summarize and aggregate data. RESULTS: Among the 28 responding sites (63.6% response rate), usage of NIRS was variable and patient-dependent. Most nurses reported using NIRS in patients with unstable physiology such as post-operative single ventricle (n = 25, 89.3%) and concern for shock (n = 21, 75.0%). Critically low cerebral values varied among respondents from less than 40 (n = 3, 10.7%) to less than 60 (n = 4, 14.3%), with lower critical values permitted for single ventricle physiology: less than 40 (n = 8, 28.6%) to less than 50 (n = 6, 21.4%). Reported barriers to using NIRS included skin breakdown (n = 9, 32.1%), lack of consistency in decision-making among physicians (n = 13, 46.4%), and not using NIRS data when developing a plan of care (n = 11, 39.3%). Most (n = 24, 85.7%) nurses reported that NIRS provided valuable information and was perceived to be beneficial for patients. CONCLUSIONS: NIRS monitoring is a common technology in the care of complex congenital heart disease patients. Most nurses valued this technology, but inconsistencies and practicalities around its use in guiding patient management were found to be problematic. RELEVANCE TO CLINICAL PRACTICE: NIRS is commonly used in the PCICU and although nurses perceived NIRS to be useful for their practice, the variability in the interpretation of values and inconsistent protocols and decision-making by physicians was challenging.

Standardized Feeding Approach Mitigates Weight Loss in Infants with Congenital Heart Disease.

OBJECTIVE: To evaluate the effect of a standardized feeding approach using a clinical nutrition pathway on weight-for-age Z score (WAZ) over hospital length of stay (HLOS) for infants with congenital heart disease (CHD). STUDY DESIGN: A 10-year retrospective cohort study examined eligible infants who underwent neonatal cardiac surgery between July 2009 and December 2018 (n = 987). Eligibility criteria included infants born at least 37 weeks of gestation and a minimum birth weight of 2 kg who underwent cardiac surgery for CHD within the first 30 days of life. Using the best linear unbiased predictions from a linear mixed effects model, WAZ change over HLOS was estimated before and after January 2013, when the standardized feeding approach was initiated. The best linear unbiased predictions model included adjustment for patient characteristics including sex, race, HLOS, and class of cardiac defect. RESULTS: The change in WAZ over HLOS was significantly higher from 2013 to 2018 than from 2009 to 2012 (ß = 0.16; SE = 0.02; P < .001), after controlling for sex, race, HLOS, and CHD category, indicating that infants experienced a decreased WAZ loss over HLOS after the standardized feeding approach was initiated. Additionally, differences were found in WAZ loss over HLOS between infants with single ventricle CHD (ß = 0.26; SE = 0.04; P < .001) and 2 ventricle CHD (ß = 0.04; SE = 0.02; P = .04). CONCLUSIONS: These data suggest that an organized, focused approach for nutrition therapy using a standardized pathway improves weight change outcomes before hospital discharge for infants with single and 2 ventricle CHD who require neonatal cardiac surgery.