Treatment of obstructive aortic atheroma in homozygotic familial hypercholesterolemia

The object of this report is to describe the surgical treatment of a rare clinical form of homozygotic familial hypercholesterolaemia (HFH) associating valvular and supravalvular stenosis with coronary ostial stenosis. Three patients, two male and one female, aged 15, 23 and 41 respectively, suffering from HFH diagnosed in early childhood, presented with obstacles to left ventricular ejection and myocardial ischaemia due to coronary ostial stenosis. Surgery consisted of corrections in a single procedure of all abnormalities by aortic valve replacement, ascending aortic replacement and widening of the coronary artery ostia which were reimplanted on the aortic tube. The postoperative course of all three patients was favourable. Postoperative echocardiography showed the normal position of the valvular prosthesis, normalisation of the left ventricular ejection fraction with no significant residual obstruction. Angioscan of the coronary arteries showed a good result of coronary ostial widening. The authors conclude that HFH is a rare condition and that disease of the ascending aorta is common in this variety with involvement of the aortic valve, the ascending aorta and the coronary ostia. The surgical procedure described by the authors allows correction of all the abnormalities with the hope of a good long-term result.