Amyloidosis and anesthesia

Abstract

Aim:The aim of this article is to provide a view of amyloidosis and discuss implications for the anesthetic management of patients with this condition.Material and Method:Urine samples from patients with plasma cell dyscrasias were obtained from a urine bank that gathers urine samples from patients who gave research use consent for specimens that would otherwise be considered waste.Results:Patients with amyloidosis may present to the anesthesiologist for procedures relating to diagnosis, surgery relating to the underlying condition (e.g., bronchial laser and organ transplant), or for incidental surgery. The condition carries a significant risk of perioperative morbidity and mortality.Conclusion:The term amyloid was coined by Virchow in the mid 19th century, meaning “starch like.” Amyloidosis is a disease complex, in which there is an abnormal deposition of extracellular hyaline material with particular staining characteristics and which contains protein fibrils embedded in a relatively amorphous ground substance. There are numerous clinical manifestations, the onset is insidious, and the diagnosis may not be made in a patient undergoing anesthesia and surgery for an apparently straightforward problem. Unexpected complications such as heart or kidney failure may arise, either before operation or in the postoperative period. Bullous lesions of the skin or oral mucosa and extensive areas of purpura are but two of the ways, in which amyloidosis may first present. The disease spectrum may be inherited or acquired, localized or systemic, and life threatening or an incidental finding.

Pulmonary Fat Embolism and Coronary Amyloidosis

“…However, because of its rarity, its insidious onset, and the vague nature of symptoms, cardiac amyloidosis is hard to recognize and the diagnosis may not be made in a patient undergoing anaesthesia and surgery for an apparently straightforward problem. It is important to point out that, in the presence of cardiac amyloidosis, several unexpected complications (including heart failure) may arise, either before the operation or in the postoperative period, especially if a surgical complication (such as the fat embolism in our patient) occurs [ 20 ]. A variety of diagnostic modalities (e.g., endomyocardial biopsy, echocardiography, electrocardiography (ECG), cardiovascular magnetic resonance imaging, radionuclide imaging) are available, but no single test is sufficient by itself; hence, early and accurate diagnosis remains difficult [ 21 ].…”

Systemic amyloidosis: A challenge to the anaesthesiologists

“…Amyloidosis is a rare disorder, characterised by extracellular deposition of abnormal protein fibrils in organs or tissues which progressively leads to organ dysfunction. [ 1 ] It may be inherited or acquired, localised or systemic, and life-threatening or an incidental finding. Amyloidosis disease is classified according to the type of amyloid protein as AL, AA, ATTR, Aβ2M, or Aβ.…”

“…Cardiac involvement represents the worst prognostic factor. [ 1 ] Heart failure is the most common terminal event for primary and hereditary amyloidosis. There are case reports mentioning cardiac adverse events like intraoperative death,[ 3 ] fatal perioperative myocardial infarction[ 4 ] and postoperative ventricular fibrillation.…”