Yonsei Med J.  1993 Dec;34(4):352-355. 10.3349/ymj.1993.34.4.352.

Cardiovascular findings of Hutchinson-Gilford syndrome: a Doppler and two-dimensional echocardiographic study

• Ha JW
• Shim WH
• Chung NS

• KMID: 1758499
• DOI: http://doi.org/10.3349/ymj.1993.34.4.352

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

Keyword

Hutchison-Gilford syndrome; Doppler and two-dimensional echocardiography

MeSH Terms

Adult
Cardiovascular System/*ultrasonography
Case Report
Coronary Angiography
*Echocardiography
*Echocardiography, Doppler
Heart Catheterization
Human
Male
Progeria/diagnosis/*ultrasonography