Implantable Cardioverter Defibrillators in Patients with Cardiac Amyloidosis

Lin, Grace; Dispenzieri, Angela; Kyle, Robert A.; Grogan, Martha; Brady, Peter A.

doi:10.1111/jce.12123

Cited by 166 publications

(115 citation statements)

References 25 publications

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“…Another report suggested a benefit of an implantable cardioverter defibrillator (ICD) in a subset of patients with a history of sustained and/or nonsustained ventricular tachycardia [143], but lacked a comparator arm. Our experience suggests that despite appropriate shock delivery, lack of a survival benefit for ICD implantation indicates a difficulty in appropriate patient selection [144]. Identification of indications for ICD installation await collaborative trials.…”

Section: Treatmentmentioning

confidence: 99%

Immunoglobulin Light-Chain Amyloidosis: From Basics to New Developments in Diagnosis, Prognosis and Therapy

et al. 2016

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Immunoglobulin amyloid light-chain (AL) amyloidosis is the most common form of systemic amyloidosis, where the culprit amyloidogenic protein is immunoglobulin light chains produced by marrow clonal plasma cells. AL amyloidosis is an infrequent disease, and since presentation is variable and often nonspecific, diagnosis is often delayed. This results in cumulative organ damage and has a negative prognostic effect. AL amyloidosis can also be challenging on the diagnostic level, especially when demonstration of Congo red-positive tissue is not readily obtained. Since as many as 31 known amyloidogenic proteins have been identified to date, determination of the amyloid type is required. While several typing methods are available, mass spectrometry has become the gold standard for amyloid typing. Upon confirming the diagnosis of amyloidosis, a pursuit for organ involvement is essential, with a focus on heart involvement, even in the absence of suggestive symptoms for involvement, as this has both prognostic and treatment implications. Details regarding initial treatment options, including stem cell transplantation, are provided in this review. AL amyloidosis management requires a multidisciplinary approach with careful patient monitoring, as organ impairment has a major effect on morbidity and treatment tolerability until a response to treatment is achieved and recovery emerges.

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Section: Treatmentmentioning

confidence: 99%

Immunoglobulin Light-Chain Amyloidosis: From Basics to New Developments in Diagnosis, Prognosis and Therapy

et al. 2016

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“…[179][180][181][182] Although appropriate implantable cardioverter defibrillator device therapy has been observed in patients with AL cardiac amyloid, studies to date have not demonstrated a survival advantage. 183 A study of implanted cardiac rhythm recorders found that sudden death in AL amyloidosis is commonly due to pulseless electrical activity, often preceded by bradycardia. 184 Given the absence of randomized studies, the role of implantable cardioverter defibrillator therapy for primary prevention in cardiac AL amyloidosis remains unclear.…”

Section: Grade Of Recommendation Cmentioning

confidence: 99%

Treatment of Immunoglobulin Light Chain Amyloidosis

Dispenzieri

Buadi

Kumar

et al. 2015

Mayo Clinic Proceedings

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104

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Immunoglobulin light chain amyloidosis (AL amyloidosis) has an incidence of approximately 1 case per 100,000 person-years in Western countries. The rarity of the condition not only poses a challenge for making a prompt diagnosis but also makes evidenced decision making about treatment even more challenging. Physicians caring for patients with AL amyloidosis have been borrowing and customizing the therapies used for patients with multiple myeloma with varying degrees of success. One of the biggest failings in the science of the treatment of AL amyloidosis is the paucity of prospective trials, especially phase 3 trials. Herein, we present an extensive review of the literature with an aim of making recommendations in the context of the best evidence and expert opinion.

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“…3 Studies have not shown any survival benefit from implantable defibrillators, 4,5 and no studies have yet examined the benefit of biventricular pacing.…”

Section: Lessonmentioning

confidence: 99%