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- Title
Ease of sutureless aortic valve replacement in a patient with unexpected ochronosis: a case report.
- Authors
Hosseini, Saeid; Salari, Soheila; Ansari, Mohammad Javad Alemzadeh; Hesami, Mahshid; Banar, Sepideh; Shojaei, Mohammad Amin
- Abstract
Background: Alkaptonuria is a rare congenital metabolic disorder characterized by homogentisic acid accumulation in body cartilage and connective tissues due to a deficient homogentisic acid dioxygenase enzyme. This disorder manifests in various clinical symptoms, including spondyloarthropathy, ocular and dermal pigmentation, genitourinary tract obstruction by ochronosis stones, and cardiovascular system involvement. Cardiac ochronosis is a rare manifestation of alkaptonuria that may present as aortic stenosis, sometimes accompanied by other cardiovascular complications. Case presentation: We report an unexpected case of ochronosis diagnosed during cardiac surgery. Due to the fragile, thin, and atheromatous nature of the ascending aorta in patients with ochronosis, we opted for a sutureless aortic valve replacement procedure. This approach appears to be more suitable for patients with ochronosis. Conclusions: Although cardiac ochronosis is rare, surgeons should remain vigilant and consider the possibility of this condition when examining patients with aortic valve stenosis, paying close attention to the clinical manifestations of alkaptonuria.
- Subjects
AORTIC valve transplantation; AORTIC stenosis; CARDIOVASCULAR system; CARDIOLOGICAL manifestations of general diseases; CONGENITAL disorders; PULMONARY alveolar proteinosis
- Publication
Journal of Cardiothoracic Surgery, 2024, Vol 19, Issue 1, p1
- ISSN
1749-8090
- Publication type
Case Study
- DOI
10.1186/s13019-024-02834-4