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

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.

journal_name

Yonsei Med J

journal_title

Yonsei medical journal

authors

Ha JW,Shim WH,Chung NS

doi

10.3349/ymj.1993.34.4.352

subject

Has Abstract

pub_date

1993-12-01 00:00:00

pages

352-5

issue

4

eissn

0513-5796

issn

1976-2437

journal_volume

34

pub_type

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