title: Usefulness of insulin like growth factor 1 as a marker of heart failure in children and young adults after the Fontan palliation procedure.
abstract: Growth hormone and its mediator, insulinlike growth factor 1 (IGF-1), are key determinants of growth in children and young adults. As patients with Fontan physiology often experience diminished longitudinal growth, we sought to describe IGF-1 levels in this population and to identify factors associated with IGF-1 deficiency. Forty-one Fontan subjects ≥5 years were evaluated in this cross-sectional study. Age- and gender-specific height Z scores were generated using national data. Laboratory testing included IGF-1 and brain natriuretic peptide (BNP) levels. IGF-1 levels were converted to age-, gender-, and Tanner stage-specific Z scores. BNP levels were log transformed to achieve a normal distribution (log-BNP). Medical records were reviewed for pertinent clinical variables. Predictors of IGF-1 Z score were assessed through the Student t test and Pearson's correlation. Median age was 11.1 years (range 5.1 to 33.5 years), and time from Fontan was 8.2 years (1.1 to 26.7). Mean height Z score was -0.2 ± 0.9 with a mean IGF-1 Z score of -0.1 ± 1.3. There was no association between IGF-1 Z score and height Z score. Longer interval since Fontan (R = -0.32, p = 0.04), higher log-BNP (R = -0.40; p = 0.01), and lower indexed systemic flow on cardiac magnetic resonance (R = 0.55, p = 0.02) were associated with lower IGF-1 Z scores. In conclusion, in this cohort with Fontan physiology, higher BNP and lower systemic flow were associated with lower IGF-1 Z score. Longitudinal studies are needed to determine if these relations represent a mechanistic explanation for diminished growth in children with this physiology and with other forms of congenital heart disease.
authors.affiliation: Division of Cardiology, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania. Electronic address: Catherine.avitabile@tenethealth.com.
authors.forename: Catherine M
authors.initials: CM
authors.lastname: Avitabile
authors.affiliation: Department of Pediatrics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania; Division of Nephrology, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania; Department of Biostatistics and Epidemiology, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania.
authors.forename: Mary B
authors.initials: MB
authors.lastname: Leonard
authors.affiliation: Mid-Hudson Medical Group, Poughkeepsie, New York.
authors.forename: Jill L
authors.initials: JL
authors.lastname: Brodsky
authors.affiliation: Division of Cardiology, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania; Department of Pediatrics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania.
authors.forename: Kevin K
authors.initials: KK
authors.lastname: Whitehead
authors.affiliation: Division of Cardiology, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania; Department of Pediatrics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania.
authors.forename: Chitra
authors.initials: C
authors.lastname: Ravishankar
authors.affiliation: Division of Cardiology, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania; Department of Pediatrics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania.
authors.forename: Meryl S
authors.initials: MS
authors.lastname: Cohen
authors.affiliation: Department of Pediatrics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania; Division of Cardiothoracic Surgery, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.
authors.forename: J William
authors.initials: JW
authors.lastname: Gaynor
authors.affiliation: Division of Cardiology, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania; Department of Pediatrics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania.
authors.forename: Jack
authors.initials: J
authors.lastname: Rychik
authors.affiliation: Division of Cardiology, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania; Department of Pediatrics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania.
authors.forename: David J
authors.initials: DJ
authors.lastname: Goldberg
mesh_terms: D000293:Adolescent; D000328:Adult; D015415:Biomarkers / Q000097:blood; D001827:Body Height; D001835:Body Weight; D002648:Child; D002675:Child, Preschool; D003430:Cross-Sectional Studies; D005260:Female; D018729:Fontan Procedure; D006330:Heart Defects, Congenital / Q000097:blood / Q000150:complications / Q000601:surgery*; D006333:Heart Failure / Q000097:blood* / Q000175:diagnosis* / Q000209:etiology; D006801:Humans; D007334:Insulin-Like Growth Factor I / Q000378:metabolism*; D008297:Male; D045283:Natriuretic Agents / Q000097:blood*; D020097:Natriuretic Peptide, Brain / Q000097:blood*; D010166:Palliative Care / Q000379:methods*; D011237:Predictive Value of Tests; D011379:Prognosis; D015203:Reproducibility of Results; D012307:Risk Factors; D012680:Sensitivity and Specificity; D012720:Severity of Illness Index; D013997:Time Factors; D055815:Young Adult
chemical_list: D015415:Biomarkers; D045283:Natriuretic Agents; D020097:Natriuretic Peptide, Brain; D007334:Insulin-Like Growth Factor I