Conditional cardiovascular response to growth hormone therapy in adult patients with Prader-Willi syndrome.
BACKGROUND
In Prader-Willi syndrome (PWS), an altered GH secretion has been related to reduced cardiac mass and systolic function when compared with controls.


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


METHODS
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.
Marzullo
Paolo
P
Department of General Medicine, Ospedale S. Giuseppe Instituto di Ricovero e Cura a Carattere Scientifico Istituto Auxologico Italiano, Casella Postale 1, I-28921 Verbania, and Department of Medical Sciences, University of Padua, Italy. marzullop@yahoo.com
Marcassa
Claudio
C
Campini
Riccardo
R
Eleuteri
Ermanno
E
Minocci
Alessandro
A
Sartorio
Alessandro
A
Vettor
Roberto
R
Liuzzi
Antonio
A
Grugni
Graziano
G
D000328:Adult; D001786:Blood Glucose / Q000378:metabolism; D015992:Body Mass Index; D049628:Body Size; D002319:Cardiovascular System / Q000187:drug effects / Q000503:physiopathology*; D004452:Echocardiography; D019382:Human Growth Hormone / Q000627:therapeutic use*; D006801:Humans; D007328:Insulin / Q000097:blood; D011218:Prader-Willi Syndrome / Q000188:drug therapy*
D001786:Blood Glucose; D007328:Insulin; D019382:Human Growth Hormone