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Anesthesia for aortic valve replacement in a patient with acute intermittent porphyria

1991; Elsevier BV; Volume: 5; Issue: 3 Linguagem: Inglês

10.1016/1053-0770(91)90285-2

1532-8422

Javier H. Campos, David Stein, Marci K. Michel, John R. Moyers,

Methemoglobinemia and Tumor Lysis Syndrome

P ORPHYRIA IS a genetic and inherited group of inborn metabolic disorders of uncertain origin. Biochemically, there is an enzymatic deficiency of heme biosynthesis, which results in the accumulation and excretion of excessive amounts of porphyrins and porphyrin precursors.’ Activity of 6 aminolevulinic acid (GALA) synthetase is increased in some of the porphyria-type diseases, which is responsible for the inappropriate hypersynthesis of porphyrin precursors.* The classification for the four genetically transmitted porphyrias includes: (1) acute intermittent porphyria (AIP); (2) hereditary coproporphyria; (3) variegate porphyria; and (4) plumboporphyria. In vivo or in vitro studies of this rare disorder are difficulti,‘; thus, clinicians are dependent on case reports to develop appropriate anesthetic management.‘.’ Acute attacks are precipitated by certain drugs, the majority of which are lipid soluble and have the property of inducing cytochrome P-450 and &ALA synthetase. Barbiturates are well known to precipitate an attack of porphyria.’ Multiple symptoms may occur during an acute attack and include severe abdominal pain, stupor, progressive paralysis, autonomic neuropathy, and ascending polyneuropathy. Sympathetic overactivity, hypertensive crisis, and cardiac dysrhythmias have also been reported.Y These symptoms are more common in the young adult, and there is a preference for females. Clinical manifestations are rare prior to puberty. Hormonal changes, for example menstruation and pregnancy, can exacerbate a crisis of porphyria. Two previous reports of patients with variegate porphyria undergoing anesthesia for coronary artery bypass surgery have been published.“‘,” The present case report is of a patient with AIP and aortic valvular disease. The patient had the type of porphyria more commonly found in the Northern hemisphere and received some of the more recently developed anesthetic agents.