Video-Assisted Minimally Invasive Endoscopic Aortic Valve Replacement in a Patient With Alkaptonuria.

ABSTRACT:

Background

Alkaptonuria is a rare metabolic disorder causing homogentisic acid accumulation, leading to ochronotic pigmentation and associated cardiac complications, including aortic valve disease. Although its exact role in aortic stenosis is unclear, pigment deposition may accelerate degeneration.

Case summary

A 75-year-old patient with alkaptonuria and ochronotic aortic valve stenosis successfully underwent a video assisted, minimally invasive endoscopic aortic valve replacement (AVR). The procedure used the novel automated suturing device RAM 5.0 (LSI SOLUTIONS®) and Cor-Knot device (LSI SOLUTIONS®).

Discussion

Although uncommon, the prevalence of ochronotic aortic valve disease associated with alkaptonuria increases with age, often requiring AVR. The impact of ho

SUBMITTER: Origel Romero CA 

PROVIDER: S-EPMC12441352 | biostudies-literature | 2025 Jul

REPOSITORIES: biostudies-literature