Cardiovascular ochronosis

Ather, Nuvaira; Roberts, William C.

doi:10.1016/j.carpath.2020.107219

Cited by 8 publications

(11 citation statements)

References 58 publications

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“…Ather et al reviewed 66 case reports of ochronotic cardiovascular disease. 4 Until now, there has been only one other case report with a presentation similar to the this case: a patient with severe aortic stenosis and evidence of ochronosis of the aortic valve, coronary arteries, and the internal thoracic artery. 5 Another case series reported that 83% and 100% of patients over 60 years of age had aortic and intracardiac calcification, respectively, without correlation to standard cardiac risk factors.…”

Section: Discussionmentioning

confidence: 59%

“…Ather et al reviewed 66 case reports of ochronotic cardiovascular disease 4 . Until now, there has been only one other case report with a presentation similar to the this case: a patient with severe aortic stenosis and evidence of ochronosis of the aortic valve, coronary arteries, and the internal thoracic artery 5 .…”

Section: Discussionmentioning

confidence: 69%

“…3 Most cases of ochronotic heart disease have reported involvement of the aortic valve, but manifestations can be extensive and affect the myocardium, coronary vessels, and internal thoracic arteries as well. 4…”

Section: Introductionmentioning

confidence: 99%

“…While the involvement of the connective tissue and genitourinary system have been well documented, and are often regarded as the most common manifestations of alkaptonuria, the cardiovascular system has been shown to be severely affected in many patients with alkaptonuria as well 3 . Most cases of ochronotic heart disease have reported involvement of the aortic valve, but manifestations can be extensive and affect the myocardium, coronary vessels, and internal thoracic arteries as well 4 …”

Section: Introductionmentioning

confidence: 99%

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Ochronotic heart disease leading to severe aortic valve and coronary artery stenosis

Velez

Gaughan

Thomas

et al. 2021

Journal of Cardiac Surgery

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Cardiac ochronosis is a rare disease, estimated to affect 1 in 250,000 persons. While there is extensive evidence of the musculoskeletal alterations of the disease, cardiac involvement has not been widely studied and most information we currently have derives from case reports and case series. We report the case of a 64‐year old patient with a known history of alkaptonuria who presented with dyspnea and weight loss. On evaluation, he was found to have severe aortic stenosis, coronary artery disease, and interventricular septal hypertrophy. Surgery revealed extensive ochronotic pigment deposition affecting the cardiac septum, both internal thoracic arteries, the native coronary arteries, and the aortic valve. Ochronotic heart disease is an often disregarded presentation of alkaptonuria. More information is needed on the course of the disease, as well as long‐term outcomes after valve replacement surgery and/or coronary artery bypass grafting in patients with alkaptonuria.

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Section: Discussionmentioning

confidence: 59%

Section: Discussionmentioning

confidence: 69%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Ochronotic heart disease leading to severe aortic valve and coronary artery stenosis

Velez

Gaughan

Thomas

et al. 2021

Journal of Cardiac Surgery

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“…While involvement on the aortic valve or in the aortic vessel is observed in almost all of these cases, only mitral valve involvement has been reported very rarely. [ 7 ] Alkaptonuria causes calcific deposits in the aortic valve that typically result in stenosis. There are four aortic insufficiency cases with other cardiovascular pathologies reported in the literature.…”

Section: Discussionmentioning

confidence: 99%

Incidental diagnosis of ochronosis by aortic valve replacement

Çoban¹,

Uçak²,

Güldür³

et al. 2022

Turk Gogus Kalp Dama

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Alkaptonuria is a rare inherited metabolic disease caused by homogentisic acid oxidase enzyme deficiency. Homogentisic acid formed during phenylalanine and tyrosine metabolism cannot be further metabolized and accumulates due to this enzyme deficiency. Some of the homogentisic acid that cannot be removed by metabolism is excreted with urine, some of it causes this accumulation known as ochronosis, which is characterized by dark pigmented color change in tissues. The classic clinical triad of the disease is darkening of the urine color, degenerative arthritis in the joints and dark colored pigmentation in the connective tissue. Herein, we present a case of ochronosis detected incidentally during aortic valve replacement with the diagnosis of aortic insufficiency.

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Minocycline

2020

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Cardiovascular ochronosis

Cited by 8 publications

References 58 publications

Ochronotic heart disease leading to severe aortic valve and coronary artery stenosis

Ochronotic heart disease leading to severe aortic valve and coronary artery stenosis

Incidental diagnosis of ochronosis by aortic valve replacement

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