Cardiovascular findings of Hutchinson-Gilford syndrome: a Doppler and two-dimensional echocardiographic study .
10.3349/ymj.1993.34.4.352
- Author:
Jong Won HA
1
;
Won Heum SHIM
;
Nam Sik CHUNG
Author Information
1. Cardiology Division, Yonsei Cardiovascular Center, Yonsei University College of Medicine, Seoul, Korea.
- Publication Type:Case Reports
- Keywords:
Hutchison-Gilford syndrome;
Doppler and two-dimensional echocardiography
- MeSH:
Adult;
Cardiovascular System/*ultrasonography;
Case Report;
Coronary Angiography;
*Echocardiography;
*Echocardiography, Doppler;
Heart Catheterization;
Human;
Male;
Progeria/diagnosis/*ultrasonography
- From:Yonsei Medical Journal
1993;34(4):352-355
- CountryRepublic of Korea
- Language:English
-
Abstract:
Progeria, also known as Hutchinson-Gilford syndrome, is an extremely rare condition originally described by Hutchinson in 1886. Death results from cardiac complications in the majority of cases and usually occurs at an average age of fourteen years. We recently experienced a patient with progeria who died suddenly after symptomatic improvement with conservative treatment. A Doppler and two-dimensional echocardiographic study revealed an enlarged and hypertrophied left ventricle with reduced global systolic function and senile aortic calcific stenosis (peak systolic pressure gradient: 50 mmHg) with a moderate degree of aortic regurgitation. Doppler findings of restrictive hemodynamic suggest severe left ventricular dysfunction due to multiple influences from the aging process, coronary artery and valvular heart disease
