Normal levels of lipoproteins including lipoprotein(a) after liver-heart transplantation in a patient with homozygous familial hypercholesterolaemia.

A 33-year-old female with severe diffuse coronary artery disease and left ventricular outflow tract obstruction secondary to homozygous familial hypercholesterolaemia underwent liver and heart transplantation. During a post-operative follow-up period of 13 months, apart from one episode of cardiac rejection at 3 weeks, she has remained well and without evidence of accelerated coronary disease on angiography. By 3 months after transplantation serum total cholesterol, low density lipoprotein cholesterol, apolipoprotein B and lipoprotein(a) levels had decreased and apolipoprotein A-I and high density lipoprotein cholesterol levels had increased, all to within the normal range. These changes were accompanied by marked regression of xanthomata and were maintained at 13 months. Liver transplantation provides an effective means of correcting serum lipids in patients with homozygous familial hypercholesterolaemia and is an important adjunct to cardiac transplantation.