Ochronosis of Mitral Valve and Coronary Arteries

Gottschalk, Byron H.; Blankenstein, Jonathan; Guo, Lina

doi:10.1016/j.athoracsur.2018.01.074

Cited by 11 publications

(20 citation statements)

References 7 publications

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“…Previous to this case, the feasibility to successfully utilize ochronotic arterial conduits in a bypass setting and follow the patient outcomes has yet to be reported 6 . Our patient's postoperative care and 20‐month follow‐up, including TTE, revealed no deterioration to either the aortic valve or the overall ventricular function suggesting compromise of the arterial grafts.…”

Section: Discussionmentioning

confidence: 80%

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: 80%

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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“…Less commonly affected are the mitral valve, then the pulmonary and tricuspid valve. Some have even noted coronary artery, sternal and aortic wall involvement 8,9 . Although pigmentation has been noted within the walls of the aorta with evidence of impaired aortic distensibility in patients with alkaptonuria, 10 there have been no cases of aortic aneurysm or aortic dissection due to ochronosis reported in the literature.…”

Section: Discussionmentioning

confidence: 99%

“…7 Although alkaptonuria-related cardiac manifestations are rare, it has been widely reported in the surgical literature. 1,[4][5][6]8 Aortic ochronosis leading to aortic stenosis requiring surgical replacement is the most frequent. Less commonly affected are the mitral valve, then the pulmonary and tricuspid valve.…”

Section: Discussionmentioning

confidence: 99%

A black heart: Aortic valve ochronosis secondary to alkaptonuria causing aortic stenosis

Edwards

2021

J Card Surg

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Alkaptonuria is a rare autosomal recessive genetic disorder where an accumulation of homogentisic acid in the tissues leads to ochronosis-a pathological dark pigmentation. It can affect various tissues and the weight bearing joints of the body, leading to degenerative arthropathy. On the rare occasion, it causes cardiac manifestations. We describe a case of aortic valve stenosis due to ochronosis secondary to alkaptonuria requiring aortic valve replacement.

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“…There are several other reports of ITA use in patients with cardiac ochronosis requiring coronary bypass. 5,6 Good results of aortic valve replacement are generally expected with the standard surgical approach, 7 but the problem of which valve should be implanted remains. In this case, a bioprosthetic valve was used due to the patient's age.…”

Section: Discussionmentioning

confidence: 99%

“…There are several other reports of ITA use in patients with cardiac ochronosis requiring coronary bypass. 5,6…”

Section: Discussionmentioning

confidence: 99%

No ochronosis was seen at internal thoracic artery with alkaptonuria

Watanabe

Harada

Mikami

et al. 2018

Asian Cardiovasc Thorac Ann

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Alkaptonuria is a rare disorder of amino acid metabolism that causes premature large joint spine arthropathy and cardiac disease. We describe a case of aortic stenosis and coronary artery disease associated with ochronosis in a 76-year-old man who underwent aortic valve replacement and coronary artery bypass grafting. Although the ochronosis was extensive, no ochronosis was seen in the internal thoracic artery which used for coronary artery bypass grafting. Ochronosis with alkaptonuria is considered to accelerate the atherosclerotic process, so the internal thoracic artery may ensure a better long-term outcome. Long-term follow-up may allow better understanding of this rare condition.

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Ochronosis of Mitral Valve and Coronary Arteries

Cited by 11 publications

References 7 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

A black heart: Aortic valve ochronosis secondary to alkaptonuria causing aortic stenosis

No ochronosis was seen at internal thoracic artery with alkaptonuria

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