Development and Initial Validation of a Frailty Score for Pediatric Patients with Congenital and Acquired Heart Disease.

Frailty is a multi-dimensional clinical syndrome that is associated with increased morbidity and mortality and decreased quality of life. Children/adolescents with heart disease (HD) perform significantly worse for each frailty domain compared to non-HD peers. Our study aimed to create a composite frailty score (CFS) that can be applied to children/adolescents with HD and evaluate associations between the CFS and outcomes. Children and adolescents (n = 30) with HD (73% single ventricle, 20% heart failure, 7% pulmonary hypertension) were recruited from 2016 to 2017 (baseline). Five frailty domains were assessed at baseline using measures validated for pediatrics: (1) Slowness: 6-min walk test; (2) Weakness: handgrip strength; (3) Fatigue: PedsQL Multi-dimensional Fatigue Scale; (4) Body composition: triceps skinfold thickness; and (5) Physical activity questionnaire. Frailty points per domain (range = 0-5) were assigned based on z-scores or raw questionnaire scores and summed to produce a CFS (0 = least frail; 25 = most frail). Nonparametric bootstrapping was used to identify correlations between CFS and cross-sectional change in outcomes over 2.2 ± 0.2 years. The mean CFS was 12.5 ± 3.5. In cross-sectional analyses of baseline data, correlations (|r|≥ 0.30) were observed between CFS and NYHA class, the number of ancillary specialists, total prescribed medications, heart failure medications/day, exercise test derived chronotropic index and percent predicted VO2peak, and between child and parent proxy PEDsQL. At follow-up, CFS was correlated with an increase in the number of heart failure medications (r = 0.31). CFS was associated with cross-sectional outcomes in youth with heart disease. Longitudinal analyses were limited by small sample sizes due to loss to follow-up.

Cardiac troponin and outcome in decompensated heart failure with preserved ejection fraction.

BACKGROUND: Cardiac troponin (cTn) has been established as an effective prognostic marker in acute heart failure (HF) with predominantly reduced ejection fraction. However, it's prognostic value in heart failure with preserved ejection fraction (HFpEF) is unclear. The aim of this study is to describe the prognostic role of troponin I in patients hospitalized for HFpEF decompensation. METHODS: We included 363 consecutive patients admitted for HFpEF decompensation that was not associated with acute coronary syndrome (ACS). Patients with troponin level elevation (troponin I level ≥0.04 ng/mL) were compared to patients with normal troponin level. The primary outcome was short-, intermediate-, and long-term all-cause mortality. The secondary outcomes were differences in B-type natriuretic peptide level (BNP), length of stay, and readmission rates between the two groups. RESULTS: Nearly half of the patients in the Cohort had troponin level elevation. Troponin level elevation was significantly associated with higher 30-day (4.8% vs. 0.6%, P=0.014), 1-year (12.2% vs. 4.6%, P=0.009), and 2-year mortality (13.8% vs. 5.1%, P=0.005) when compared to a normal troponin level. Troponin level elevation was an independent predictor of mortality after adjusting for clinical and laboratory risk factors seen in HFpEF decompensation. Additionally, BNP level >287 pg/mL, age, and history of atrial fibrillation were identified as statistically significant predictors of mortality. CONCLUSIONS: Troponin level elevation, in hospitalized patients with HFpEF decompensation, was associated with higher short-, intermediate-, and long-term mortality.

Posterior reversible encephalopathy syndrome associated with left horizontal gaze palsy.

Posterior reversible encephalopathy syndrome (PRES) is characterized by rapid onset of symptoms including headache, seizures, altered consciousness, and visual disturbance, as well as radiologic findings of focal reversible vasogenic edema. Multiple visual disturbances have been described in PRES, such as hemianopia, visual neglect, auras, visual hallucinations, and cortical blindness. However, horizontal gaze palsy has not been previously reported. We report a 72-year-old female who presented with blurred vision, severe headache, lethargy, and later developed seizures. She was found to have left horizontal gaze palsy with intact vestibulo-ocular reflex. Brain magnetic resonance imaging (MRI) showed severe edema throughout the subcortical white matter, and signal in the posterior parietal and occipital lobes. She was diagnosed with PRES associated with supranuclear gaze palsy.

Euglycemic Diabetic Ketoacidosis, a Misleading Presentation of Diabetic Ketoacidosis.

CONTEXT: Diabetic ketoacidosis (DKA) is one of the most serious complications of diabetes. It is characterized by a triad of increased total body ketone concentration, metabolic acidosis, and uncontrolled hyperglycemia. Hyperglycemia is a key diagnostic criterion of DKA; however, in some rare cases, normal glucose levels can be present. CASE REPORTS: We describe two patients with type 1 diabetes mellitus (DM1); one who presented with a Bartholin's gland abscess and the other with acute pancreatitis. Both patients had maintained adequate hydration and continued to take their insulin without sufficient carbohydrate intake in the previous days prior to presentation. Despite their normal serum glucose levels upon presentation, they were found to have ketonemia and acidosis consistent with DKA. If only the serum glucose level was taken into consideration, while ignoring the rest of their biochemical profiles and failing to obtain ketone levels, the diagnoses would have been missed. CONCLUSION: Euglycemic DKA is usually seen in otherwise healthy patients with type 1 diabetes mellitus who have decreased carbohydrate intake in the presence of adequate hydration and a degree of insulin intake. Recognition of this entity by the emergency provider is crucial when patients with DM1 present with a picture of DKA, regardless of their blood sugar.