title: Echocardiographic dimensions and function in adults with primary growth hormone resistance (Laron syndrome).
abstract: Patients with primary growth hormone (GH) resistance-Laron Syndrome (LS)-have no GH signal transmission, and thus, no generation of circulating insulin-like growth factor-I (IGF-I), and should serve as a unique model to explore the controversies concerning the longterm effect of GH/IGF-I deficiency on cardiac dimension and function. We assessed 8 patients with LS (4 men, 4 women) with a mean (+/- SD) age of 38+/-7 years (range 22 to 45), and 8 aged-matched controls (4 men, 4 women) with a mean age of 38+/-9 years (range 18 to 47) by echocardiography at rest, following exercise, and during dobutamine administration. Left ventricular (LV) septum, posterior wall, and end-diastolic diameter were significantly reduced in untreated patients with LS compared with the control group (p<0.05 for all). Systolic Doppler-derived parameters, including LV stroke volume, stroke index, cardiac output, and cardiac index, were significantly lower (p<0.05 for all) than in the control subjects, whereas LV diastolic Doppler parameters, including mitral valve waves E, A, E/A ratio, and E deceleration time, were similar in both groups. LV ejection fraction at rest as well as the stress-induced increment of the LV ejection fraction were similar in both groups. Our results show that untreated patients with long-term IGF-I deficiency have reduced cardiac dimensions and output but normal LV ejection fraction at rest and LV contractile reserve following stress.
authors.affiliation: The Heart Institute, Sheba Medical Center, Tel-Hashomer, Israel.
authors.forename: M S
authors.initials: MS
authors.lastname: Feinberg
authors.forename: M
authors.initials: M
authors.lastname: Scheinowitz
authors.forename: Z
authors.initials: Z
authors.lastname: Laron
mesh_terms: D000328:Adult; D002316:Cardiotonic Agents; D004280:Dobutamine; D005080:Exercise Test; D005260:Female; D006330:Heart Defects, Congenital / Q000000981:diagnostic imaging* / Q000503:physiopathology; D019382:Human Growth Hormone / Q000172:deficiency / Q000378:metabolism*; D006801:Humans; D008297:Male; D008875:Middle Aged; D013577:Syndrome; D014463:Ultrasonography; D016277:Ventricular Function, Left
chemical_list: D002316:Cardiotonic Agents; D019382:Human Growth Hormone; D004280:Dobutamine