Introduction
Werner syndrome (adult progeria) is a rare disease, caused by LMNA mutations, located on chromosome 8 (8p12). Only three cases of coronary artery surgery in classical adult-onset Hutchinson-Gilford Progeria Syndrome (Werner Syndrome) have been reported in the English literature. Here, we report on a 42-years-old male who presented with severe disabling angina and left main coronary artery disease who underwent coronary bypass surgery.
Case
The patient had the typical stigmata of Werner syndrome with senile face, prematurely aged skin, small stature (1.59m, 39kg) and was being followed with the diagnosis of Werner syndrome since young adulthood. He had a younger brother with similar clinical features and diagnosis who had a history of a coronary stent procedure. He had diabetes mellitus and was on insulin for 4 years. He presented with class IV angina pectoris which made him bed ridden. He also had un-healing ulcers on both feet. His resting ECG showed significant ST segment depression on anterior chest leads. A coronary angiogram revealed severe left main stenosis encompassing proximal left anterior descending artery (LAD) and a mid right coronary stenosis. Left ventricular function was mildly depressed with an ejection fraction of 45%. The patient underwent on pump coronary bypass surgery with left internal thoracic artery to LAD, the right internal thoracic artery to the circumflex artery (free graft) and a saphenous vein graft to the right coronary. He had an uneventful postoperative course and was discharged on postoperative day 7. On his follow-up he was angina free and mobilizing freely.
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