Normative Echocardiographic Values for Right and Left Ventricular Function in Extremely Premature Neonates.

OBJECTIVE: To identify the measures of biventricular function and surrogates of pulmonary vascular resistance that can be expected in relatively stable extremely premature neonates, and evaluate maturational changes in myocardial performance in this patient population. STUDY DESIGN: This was a prospective observational study. Clinically stable extremely preterm newborns were divided into 3 cohorts based on gestational age: cohort 1 (240/7-256/7 weeks), cohort 2 (260/7-276/7 weeks), and cohort 3 (280/7-296/7 weeks). Serial echocardiograms were obtained on day of life 3-5, 7-10, and just prior to discharge. RESULTS: In total, 46 subjects met the criteria aimed at capturing only the most clinically healthy and stable newborns less than 296/7 weeks of gestational age. Myocardial performance was reliably assessed by echocardiography with high inter-reader correlation. Normative values were identified for right ventricular function, left ventricular function, and surrogates of pulmonary vascular resistance. CONCLUSIONS: Biventricular systolic performance is significantly different in the clinically stable extremely premature neonate when compared with healthy full-term newborns. All participants had evidence of poor biventricular compliance at birth which improved with maturation. Extreme prematurity does not appear to adversely affect myocardial functional maturation at the time of term corrected age and/or discharge.

Application of the Pediatric Appropriate Use Criteria for Chest Pain.

OBJECTIVES: To characterize the subgroup of outpatient pediatric patients presenting with chest pain and to determine the effectiveness of published pediatric appropriate use criteria (PAUC) to detect pathology. STUDY DESIGN: The Pediatric Appropriate Use of Echocardiography study evaluated the use and yield of transthoracic echocardiography (TTE) before and after PAUC release. Data were reviewed on patients ?18 years of age who underwent TTE for chest pain. Indications were classified as appropriate (A), may be appropriate (M), and rarely appropriate (R) based on PAUC ratings, and findings were normal, incidental, or abnormal. RESULTS: Chest pain was the primary indication in 772 of 4562 outpatient TTE studies (17%) (median age 14 years, IQR 10-16) ordered during the study period: 458 of 772 before (59%) and 314 of 772 after (41 %) the release of PAUC with no change in appropriateness. In A indications (n?=?654), 642 (98%) were normal, 5 (1%) had incidental findings, and 7 (1%) were abnormal. A and M detected 100% of all abnormal findings (A: n?=?7; M: n?=?6; R: n?=?0), with an association between ratings and findings (P?<.001). There was no association between R rating and any pathology. CONCLUSIONS: There was no change in ordering patterns with publication of the PAUC. Despite the high rate of TTEs ordered for indications rated A, most studies were normal. Studies that detected pathology were performed for indications rated A or M, but not R. This study supports PAUC as a useful tool in pediatric chest pain evaluation that may subsequently improve the use of TTE.

Assessment of Growth 6 Years after the Norwood Procedure.

At 6 years of age, patients with hypoplastic left heart syndrome had mean age-adjusted z-scores for weight and height below the normative population, and body mass index was similar to the normative population. Males had the greatest increase in z-scores for body mass index. TRIAL REGISTRATION: ClinicalTrials.gov: NCT00115934.

Assessment of Quality of Life in Young Patients with Single Ventricle after the Fontan Operation.

OBJECTIVES: To assess self-reported quality of life (QOL) in a large multicenter cohort of adolescent and young adults surviving Fontan. STUDY DESIGN: Cross-sectional. The Pediatric Quality of Life Inventory (PedsQL) was administered to 408 survivors of Fontan ages 13-25 years enrolled in the Pediatric Heart Network Fontan Follow-up Study. Subjects also completed either the Child Health Questionnaire (age <19 years) or Short Form Health Survey (age ≥ 19 years). PedsQL data were compared with matched controls without a chronic health condition. Correlations between the measures were examined. RESULTS: Mean PedsQL scores for subjects receiving Fontan were significantly lower than those for the control group for physical and psychosocial QOL (P < .001). Overall, 45% of subjects receiving Fontan had scores in the clinically significant impaired range for physical QOL with 30% in the impaired range for psychosocial QOL. For each 1 year increase in age, the physical functioning score decreased by an average of 0.76 points (P = .004) and the emotional functioning score decreased by an average of 0.64 points (P = .03). Among subjects ≥19 years of age, the physical functioning score decreased by an average of 2 points for each year increase in age (P = .02). PedsQL scale scores were significantly correlated with conceptually related Child Health Questionnaire (P < .001) and Short Form Health Survey scores (P < .001). CONCLUSIONS: Survivors of Fontan are at risk for significantly impaired QOL which may decline with advancing age. Routine assessment of QOL is essential to inform interventions to improve health outcomes. The PedsQL allowed QOL assessment from pediatrics to young adulthood. TRIAL REGISTRATION: ClinicalTrials.gov: NCT00132782.

Incidence of cardiac abnormalities in children with human immunodeficiency virus infection: The prospective P2C2 HIV study.

OBJECTIVE: To describe the 5-year cumulative incidence of cardiac dysfunction in human immunodeficiency virus (HIV)-infected children. STUDY DESIGN: We used a prospective cohort design, enrolling children at 10 hospitals. Group I included 205 vertically HIV-infected children enrolled at a median age of 1.9 years. Group II consisted of 600 HIV-exposed children enrolled prenatally or as neonates, of whom 93 were ultimately HIV-infected. The main outcome measures were echocardiographic indexes of left ventricular dysfunction. RESULTS: In group I, the 5-year cumulative incidence of left ventricular fractional shortening