Trusted Resources: Education
Scientific literature and patient education texts
Newer Therapies for Amyloid Cardiomyopathy
source: Current Heart Failure Reports
year: 2016
authors: Chakraborty R, Muchtar E, Gertz MA
summary/abstract:The heart and the kidneys are the most commonly involved organs in systemic amyloidosis. Cardiac involvement is associated with an increased morbidity, treatment intolerance, and poorer overall survival. The most common types of amyloidosis that are associated with cardiac involvement include light chain (AL) amyloidosis and transthyretin (TTR) amyloidosis (both mutant and wild type).
The traditional first-line treatment for AL amyloidosis includes alkylator-based chemotherapy or high-dose melphalan followed by autologous stem cell transplantation (ASCT). Novel agents, including proteasome inhibitors, immunomodulators, and monoclonal antibodies, have shown promising activity in both frontline and relapsed settings.
Orthotopic heart transplantation (OHT) followed by ASCT has led to superior outcomes compared to OHT alone. Orthotopic liver transplantation (OLT) is the first-line treatment for TTR amyloidosis. However, progression of cardiac amyloidosis after OLT is often noted due to deposition of wild TTR. Combined OLT and OHT also has a role in treatment and leads to superior outcomes in carefully selected candidates. Pharmacologic agents, including diflunisal, tafamidis, small interfering ribonucleic acid, and doxycycline, have shown promising activity in stabilizing TTR from misfolding into fibrils and are being actively investigated.
Best supportive care and management of heart failure symptoms with diuretics are a mainstay of treatment in all cardiac amyloidosis subtypes. Robust data on the benefit of angiotensin-converting enzyme inhibitors, angiotensin receptor blockers, or beta blockers in amyloid cardiomyopathy is lacking.
organization: Mayo Clinic,USADOI: 10.1007/s11897-016-0300-1
read more full text
Related Content
-
How Can I Avoid Fluid Balance Problems?Many patients with AL amyloidosis should...
-
The Challenges in Chemotherapy and Stem Cell Transplantation for Light-Chain AmyloidosisLight-chain (AL) amyloidosis is a system...
-
FDA Approves Subcutaneous Daratumumab Plus VCd for Newly Diagnosed Light-Chain AmyloidosisThe FDA has approved daratumumab and h...
-
Dara-CyBorD Treatment – ASG Webinar 1/13https://www.youtube.com/watch?v=4gUXPbOk...
-
FDA OKs Inotersen (Tegsedi) for Hereditary ATTR With PolyneuropathyThe US Food and Drug Administration (FDA...
-
Chafic Karam, MDDr. Chafic Karam is a Neurologist at the...
-
Steroids, IMiDs, and Alkylators in Amyoidosis: Dr. Larry Anderson – ASG Webinar 2/13https://www.youtube.com/watch?v=NrEjyVeJ...
To improve your experience on this site, we use cookies. This includes cookies essential for the basic functioning of our website, cookies for analytics purposes, and cookies enabling us to personalize site content. By clicking on 'Accept' or any content on this site, you agree that cookies can be placed. You may adjust your browser's cookie settings to suit your preferences.
More information
The cookie settings on this website are set to "allow cookies" to give you the best browsing experience possible. If you continue to use this website without changing your cookie settings or you click "Accept" below then you are consenting to this.
To improve your experience on this site, we use cookies. This includes cookies essential for the basic functioning of our website, cookies for analytics purposes, and cookies enabling us to personalize site content. By clicking on 'Accept' or any content on this site, you agree that cookies can be placed. You may adjust your browser's cookie settings to suit your preferences.
More information
The cookie settings on this website are set to "allow cookies" to give you the best browsing experience possible. If you continue to use this website without changing your cookie settings or you click "Accept" below then you are consenting to this.