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There are two forms of transthyretin (TTR) amyloidosis: non-hereditary and hereditary. The non-hereditary form (ATTRwt) is caused by native or wild-type TTR and was previously referred to as senile systemic amyloidosis. The hereditary form (ATTRm) is caused by variant TTR which results from a genetic mutation of TTR. The predominant effect of ATTRwt amyloidosis is on the heart, with patients having a greater left ventricular wall thickness at presentation than the devastating form which is light chain (AL) amyloidosis. ATTRm amyloidosis is broadly split into two categories: a type that predominantly affects the nervous system (often called familial amyloid polyneuropathy (FAP)) and one with a predilection for the heart (often called familial amyloid cardiomyopathy (FAC)). Approximately half of all TTR mutations known to express a clinical phenotype cause a cardiomyopathy. Since the introduction of orthotopic liver transplantation for ATTRm amyloidosis in 1991, several additional therapies have been developed. These therapies aim to provide a reduction or elimination of TTR from the plasma (through genetic approaches), stabilisation of the TTR molecule (to prevent deposition) and dissolution of the amyloid matrix. We describe the latest developments in these approaches to management, many of which are also applicable to wild-type amyloidosis. [ABSTRACT FROM AUTHOR]

Heart involvement by amyloid deposition remains the most challenging of all organ sytems that may become involved, in what is generally a systemic disease. The correct diagnosis of amyloid type is critical to selection of the appropriate and wide range of therapies. The treatment of amyloid heart disease comprises two strategies: conventional management of a restrictive cardiomyopathy, and varied therapies aimed at the underlying amyloidogenic process. In light chain (AL) amyloidosis, many of the most efficacious therapies involve chemotherapeutic agents with their own inherent toxicities to the heart and bone marrow. In the case of the hereditary amyloidosis, major surgery in the form of liver transplantation is usually required. Moreover, consideration should be given to screening of family members for a potentially hereditary disease. Several types of amyloidosis may require one or more, of heart, liver and/or kidney transplantation, sometimes in addition to high-dose chemotherapy. The objective is to provide a schematic overview of available therapies in the management of AL, hereditary, senile systemic, isolated atrial and secondary forms of amyloidosis. [ABSTRACT FROM AUTHOR]

The article provides an overview of amyloidosis and discusses implications for the anesthetic management of patients with the condition. The key findings on amyloidosis suggest that the condition frequently masquerades as other diseases hence early diagnosis is essential to allow all treatment options. Key concerns for the anesthetist include unpredictable perioperatve mortality, heart failure, arrythmias, airway difficulties, bleeding and altered response to pharmacological measures.

Describes the features and prognosis of exertional syncope in light-chain associated cardiac amyloidosis (AL). Clinical presentations of cardiac AL; Common presenting symptoms of the disease; Contribution of the failure of the atria to prime the ventricles to their decreased filling in late diastole.

Presents a study characterizing the geometry and function of the right ventricle (RV) and its significance in patients with primary cardiac amyloidosis. Shorter median survival exhibited by patients with dilated RV; Ventricular area ratio as an independent predictor of survival; Association of RV dilation in amyloid heart disease patients with poor prognosis.