This Article Full Text Full Text (PDF) All Versions of this Article: 92/4/1364    most recent Author Manuscript (PDF) Submit a related Letter to the Editor Purchase Article View Shopping Cart Alert me when this article is cited Alert me when eLetters are posted Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Request Copyright Permission Citing Articles Citing Articles via Google Scholar Google Scholar Articles by Marzullo, P. Articles by Grugni, G. Search for Related Content PubMed PubMed Citation Articles by Marzullo, P. Articles by Grugni, G. Pubmed/NCBI databases OMIM Compound via MeSH Substance via MeSH Genetics Home Reference Medline Plus Health Information Nutrition Prader-Willi Syndrome Related Collections Cardiovascular Endocrinology Obesity Neuroendocrinology and Pituitary

Conditional Cardiovascular Response to Growth Hormone Therapy in Adult Patients with Prader-Willi Syndrome

Departments of General Medicine (P.M., A.M., A.L.) and Auxology (A.S., G.G.), Ospedale S. Giuseppe, Instituto di Ricovero e Cura a Carattere Scientifico Istituto Auxologico Italiano, I-28921 Verbania, Italy; Departments of Nuclear Medicine (C.M., R.C.) and Cardiology (C.M., E.E.), Fondazione S. Maugeri, I-27100 Veruno, Italy; and Department of Medical Sciences (R.V.), University of Padua, I-35122 Padua, Italy

Address all correspondence and requests for reprints to: Paolo Marzullo, M.D., Ph.D., General Medicine, Ospedale S. Giuseppe Instituto di Ricovero e Cura a Carattere Scientifico Istituto Auxologico Italiano, Casella Postale 1, I-28921 Verbania, Italy. E-mail: marzullop{at}yahoo.com.

Context: In Prader-Willi syndrome (PWS), an altered GH secretion has been related to reduced cardiac mass and systolic function when compared with controls.

Objectives: The objective of the study was to evaluate the cardiovascular response to GH therapy in adult PWS patients.

Study Participants: Thirteen obese PWS adults (seven males and six females, aged 26.9 ± 1.2 yr, body mass index 46.3 ± 1.6 kg/m²) participated in the study.

Methods: Determination of IGF-I, metabolic parameters, echocardiography, and cardioscintigraphy with dobutamine stimulation was made during 12 months GH therapy, with results analyzed by repeated-measures ANOVA.

Results: GH therapy increased IGF-I (P < 0.0001); decreased C-reactive protein levels (P < 0.05); and improved lean mass (P < 0.001), fat mass (P < 0.05), and visceral fat (P < 0.001). Echocardiography showed that 6- and 12-month GH therapy increased left ventricle mass in 76 and in 61% of patients, respectively (P < 0.05), did not change diastolic function, and slightly decreased the left ventricle ejection fraction (LVEF) (P = 0.054). Cardioscintigraphy documented stable values of LVEF throughout the study, whereas right ventricle ejection fraction decreased significantly (P < 0.05) being normally responsive to dobutamine infusion. A positive association between IGF-I z-scores and LVEF occurred at the 6- and 12-month follow-up (P < 0.05).

Conclusions: In PWS, GH therapy increased cardiac mass devoid of diastolic consequences. The observation of a slight deterioration of right heart function as well as the association between IGF-I and left ventricular function during GH therapy suggest the need for appropriate cardiac and hormonal monitoring in the therapeutic strategy for Prader-Willi syndrome.