Aortic Valve Replacement in Familial Hypercholesterolemia: Not an Ordinary Procedure

Authors

  • Mirko Muretti Department of Cardiac Surgery, Santissima Annunziata Hospital, Sassari
  • Francesco Massi Department of Cardiac Surgery, Santissima Annunziata Hospital, Sassari
  • Enrico Coradduzza Department of Cardiac Surgery, Santissima Annunziata Hospital, Sassari
  • Michele Portoghese Department of Cardiac Surgery, Santissima Annunziata Hospital, Sassari

DOI:

https://doi.org/10.1532/hsf.1240

Abstract

Familial hypercholesterolemia is an inherited disorder with incidences of approximately 1:500 and 1:1,000,000 in heterozygous and homozygous form respectively. Affected patients usually show early coronary artery disease and severe aortic root calcification, despite optimization of therapy. We report a case of a 64-year-old woman affected by heterozygous familial hypercholesterolemia which presented dyspnea and anginal symptoms due to a severely calcified aortic root causing valve stenosis and narrowed sinotubular junction. Aortic valve replacement and aortic root enlargement were performed using the Manougian procedure. Even for experiences surgeons, this surgery could prove challenging for this group of patients due to aggressive degenerative tissue calcification of the aortic root, which often presents an extremely calcified aortic valve with a small annulus associated to a narrowed sinotubular junction.

Published

2015-04-28

How to Cite

Muretti, M., Massi, F., Coradduzza, E., & Portoghese, M. (2015). Aortic Valve Replacement in Familial Hypercholesterolemia: Not an Ordinary Procedure. The Heart Surgery Forum, 18(2), E056-E058. https://doi.org/10.1532/hsf.1240

Issue

Section

Article