Acute Methemoglobinemia Associated with Ochronotic Valvular Heart Disease: Report of a Case

Abstract

We describe the first reported case of acute methemoglobinemia associated with ochronotic valvular heart disease. A 79-year-old man with ochronotic valvular heart disease experienced decreased urinary output starting 9 days after an operation. Thereafter, the patient's methemoglobin concentration acutely increased, indicating systemic cyanosis, while the arterial partial oxygen pressure (PaO₂) was maintained at around 200 mmHg. In patients with ochronotic valvular heart disease, acute methemoglobinemia may occur, as in cases of renal failure or oliguresis.

Cardiovascular surgery - heart valve surgery - heart disease

References

• 1 Phornphutkul C, Introne W J, Perry M B et al. Natural history of alkaptonuria.  N Engl J Med. 2002;  347 2111-2121
• 2 Introne W J, Phornphutkul C, Bernardini I, McLaughlin K, Fitzpatrick D, Gahl W A. Exacerbation of the ochronosis of alkaptonuria due to renal insufficiency and improvement after renal transplantation.  Mol Genet Metab. 2002;  77 136-142
• 3 Martin Jr J P, Batkoff B. Homogentisic acid autoxidation and oxygen radical generation: implications for the etiology of alkaptonuric arthritis.  Free Radic Biol Med. 1987;  3 241-250
• 4 Ffolkes L V, Brull D, Krywawych S, Hayward M, Hughes S E. Aortic stenosis in cardiovascular ochronosis.  J Clin Pathol. 2007;  60 92-93
• 5 Prchal J T, Gregg X T. Red cell enzymes.  Hematology Am Soc Hematol Educ Program. 2005;  19-23
• 6 Berlin G, Brodin B, Hilden J O, Martensson J. Acute dapsone intoxication: a case treated with continuous infusion of methylene blue, forced diuresis and plasma exchange.  J Toxicol Clin Toxicol. 1984–1985;  22 537-548