Surgical management of HLP and DLP can be categorized from different perspectives, particularly in terms of the organs involved. Liver transplantation, sometimes in conjunction with heart transplantation, is truly experimental treatment. Portocaval shunts were equally experimental, but have since been abandoned. Let us therefore primarily focus on contemporary “metabolic surgery” interventions of the bowel (and, in a broader sense, the stomach as well).
Truly experimental treatment
While the aforementioned surgical procedure may not be considered overly routine, it is nevertheless encountered in clinical practice for management of HLP/DLP. Other methods, however, exist only in the framework of experimental institutions.
In children with homozygous FH, individual cases of liver transplantation have been performed with the intention of introducing functional LDL receptors. The results should be considered with caution and cannot be clearly evaluated at this time. This method remains reserved for international super-specialized centers in cases where other treatments have failed. In cases of severe coronary involvement and the development of heart failure, FH homozygotes may be able to consider combined transplantation of the heart and liver.
Portocaval shunts have already been abandoned due to their negative side effects (and for other indications, as well).
Gene therapy has been verified in FH, but has yet to reach its full potential.
That said, gene therapy in patients with lipoprotein lipase deficiencies (i.e. Type I HLP) has already become experimental – several dozen patients have already started commercial treatment.
Further experimental treatments (delipidation of plasma, apo
A-IMilano, and others) are still exorbitantly expensive, remain far from routine clinical practice, and do not focus on the primary treatment goals of LDL-C or FH (to which this publication is dedicated), but instead focus on HDL-C.