Cardiovascular ochronosis.

Cardiovasc Pathol

Baylor Scott & White Heart and Vascular Institute, Baylor University Medical Center, Baylor Scott & White Health, Dallas, Texas, USA; Department of Pathology, Baylor University Medical Center, Baylor Scott & White Health, Dallas, Texas, USA; Department of Internal Medicine (Division of Cardiology), Baylor University Medical Center, Baylor Scott & White Health, Dallas, Texas, USA. Electronic address:

Published: September 2020

In this review, we summarize previously reported case reports (n=66) in which the presence of ochronotic pigment was found in one or more cardiovascular structures either at necropsy or after operative excision of a cardiac valve or portions of arteries or both. As illustration, we describe black pigment in operatively excised aortic valves and aorta in 2 patients, both probably examples of secondary ochronosis. Ochronosis appears to have fascinated a number of prominent historical figures in medicine, and this review also summarizes their important contributions to this topic.

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.carpath.2020.107219DOI Listing

Publication Analysis

Top Keywords

cardiovascular ochronosis
4
ochronosis review
4
review summarize
4
summarize reported
4
reported case
4
case reports
4
reports n=66
4
n=66 presence
4
presence ochronotic
4
ochronotic pigment
4

Similar Publications

Alkaptonuria is characterized by the accumulation of homogentisic acid which causes dark coloration of urine upon standing, ochronosis, and arthritis. A 4-year old child was referred to our pediatric nephrologist with hyperoxaluria and a history of unexplained pink-to-brown discolouration of his diapers associated with a brown-staining of clothes and skin since he was six months old. He had no other symptoms and his past medical history only included minor child illnesses.

View Article and Find Full Text PDF
Article Synopsis
  • Cardiac ochronosis is a rare condition linked to alkaptonuria, characterized by black pigmentation in cardiac structures, discovered in a 60-year-old woman during valve replacement surgery for severe aortic stenosis.
  • Intraoperatively, the presence of a pigmented aortic valve led to the diagnosis of alkaptonuria, prompting a switch from a bioprosthetic to a mechanical valve for better long-term outcomes.
  • Post-surgery, the patient was discharged without complications, with plans for regular follow-up to monitor her valve function and manage her condition's progression.
View Article and Find Full Text PDF
Article Synopsis
  • * The condition can cause serious heart problems, such as valve stenosis and regurgitation, impacting about 10% of individuals and potentially leading to heart failure.
  • * This report details the effective anesthesia management of a 74-year-old man with cardiac issues from alkaptonuria who successfully underwent surgery for an aortic valve replacement and coronary artery bypass grafting.
View Article and Find Full Text PDF

Ease of sutureless aortic valve replacement in a patient with unexpected ochronosis: a case report.

J Cardiothorac Surg

June 2024

Heart Valve Diseases Research Center, Rajaie Cardiovascular Medical and Research Center, School of Medicine, Iran University of Medical Sciences, Tehran, Iran.

Article Synopsis
  • Alkaptonuria is a rare genetic disorder that leads to the buildup of homogentisic acid in the body, causing various health issues like joint pain, skin and eye pigmentation, and potential heart problems.
  • In a recent surgical case, a patient with alkaptonuria was found to have cardiac ochronosis, which affected the approach to aortic valve replacement surgery.
  • Surgeons should be aware of cardiac ochronosis in patients with aortic stenosis and consider underlying alkaptonuria symptoms when planning treatment.
View Article and Find Full Text PDF

Surgical management of calcific valvular and coronary disease in a patient with alkaptonuria: a case report.

Eur Heart J Case Rep

February 2024

Division of Cardiac Surgery, Bluhm Cardiovascular Institute, Northwestern Medicine, 676 North Saint Clair St, Arkes Family Pavilion, Suite 730, Chicago, IL 60611, USA.

Article Synopsis
  • - Alkaptonuria is a rare metabolic disorder that leads to the accumulation of homogentisic acid, causing symptoms like dark skin discoloration (ochronosis) and issues such as arthritis and cardiac diseases.
  • - A case study describes a 62-year-old man with alkaptonuria who underwent cardiac surgery for multiple valve replacements and bypass grafting, during which significant tissue discoloration and calcification were observed.
  • - The discussion emphasizes the link between alkaptonuria and heart disease, noting challenges in monitoring cerebral oxygenation during surgery and the importance of careful pre-operative planning for successful treatment.
View Article and Find Full Text PDF

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!