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[Valve Replacement for Severe Aortic Stenosis in a Patient with Tangier Disease]

Atsushi Tamura1, Takamichi Yoshizaki, Sho Kusadokoro

  • 1Department of Cardiovascular Surgery, Kawaguchi Municipal Medical Center, Kawaguchi, Japan.

Kyobu Geka. the Japanese Journal of Thoracic Surgery
|August 10, 2017
PubMed

Insights

This case study details severe aortic valve stenosis in a Tangier disease patient, a rare occurrence. Surgical aortic valve replacement was successfully performed, highlighting a novel treatment approach for this genetic lipid disorder.

Area of Science:

  • Cardiovascular Medicine
  • Genetics
  • Lipid Metabolism

Background:

  • Tangier disease is a rare genetic disorder characterized by defects in high-density lipoprotein (HDL) metabolism.
  • Patients typically present with low HDL cholesterol and cholesterol ester accumulation in tissues.
  • Aortic valve stenosis is a significant cardiovascular condition, often requiring surgical intervention.

Observation:

  • A 64-year-old female diagnosed with Tangier disease presented with dyspnea.
  • Echocardiography revealed severe aortic valve stenosis with a maximum gradient of 60.5 mmHg.
  • Chest CT demonstrated severe calcification of the ascending aorta and aortic root.

Findings:

  • The patient had pathologically low serum high-density lipoprotein (HDL) levels (4.3 mg/dl) and total cholesterol of 124 mg/dl.
  • Severe aortic valve stenosis was confirmed, necessitating surgical intervention.
  • Aortic valve replacement using a bioprosthetic valve was successfully performed.

Implications:

  • This case highlights the rare association between Tangier disease and severe aortic valve stenosis.
  • Surgical treatment for aortic valve stenosis in Tangier disease patients is exceptionally uncommon.
  • Understanding this link may improve cardiovascular risk assessment and management in Tangier disease patients.

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