Cardiac Transplantation and Mechanical Circulatory Support in Amyloidosis

Witteles, Ronald

doi:10.1016/j.jaccao.2021.05.007

Cited by 11 publications

(9 citation statements)

References 24 publications

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“…Hence, it is imperative to diagnose ATTR-CM at earlier stages with available non-invasive testing and FDA approved treatments. Heart transplantation can be considered in select patients with Stage D HF[ 12 ]. The current allocation system provides priority as Status 4 to these stage D ATTR-CM patients due to a lack of durable mechanical support options[ 10 ].…”

Section: Discussionmentioning

confidence: 99%

Early and aggressive presentation of wild-type transthyretin amyloid cardiomyopathy: A case report

Boda¹,

Farhoud²,

Dalia³

et al. 2022

World J Cardiol

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BACKGROUND Wild-type transthyretin amyloidosis (ATTRwt) is the most common form of transthyretin amyloid cardiomyopathy, occurring mostly over age of 60 years (mean age of 80 years). Mean survival without treatment is 3.6 years, making early detection imperative. We report an unusual case of a 58-year-old patient with ATTRwt cardiomyopathy requiring heart transplantation. CASE SUMMARY A 58-year-old male presented with progressive fatigue, shortness of breath, weight gain, leg swelling, orthopnoea, and paroxysmal nocturnal dyspnoea for several months. Approximately ten months before this clinical presentation, the patient had first received a diagnosis of heart failure with reduced ejection fraction (EF) of 15% to 20%. The patient was started on appropriate guideline-directed medical therapy with only mild improvement in his EF. Upon further investigation, echocardiogram, technetium pyrophosphate scan (Tc PYP), and cardiac magnetic resonance imaging (cMRI) suggested a diagnosis of amyloidosis, and ATTRwt was subsequently confirmed with native heart tissue biopsy, congo red staining, liquid chromatography-tandem mass spectrometry, and genetic testing. The patient was successfully treated with heart transplantation and is doing well post-transplant. CONCLUSION Wild-type ATTR amyloidosis should be kept on differentials in all patients (even less than 60 years old) with non-ischemic cardiomyopathy, especially in the setting of increased ventricular wall thickness and other classic echocardiogram, cMRI, and Tc PYP findings. Early diagnosis and management can be consequential in improving patient outcomes.

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Section: Discussionmentioning

confidence: 99%

Early and aggressive presentation of wild-type transthyretin amyloid cardiomyopathy: A case report

Boda¹,

Farhoud²,

Dalia³

et al. 2022

World J Cardiol

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“…However, it is reasonable to consider silencer therapy if patients have lifestyle-limiting neuropathy in the postheart transplant phase [64 ▪▪ ]. Historically, liver transplant was considered a first-line therapeutic modality in patients with hATTR to replace the source of mutant TTR production [62,64 ▪▪ ]. However, studies have shown that patients who underwent liver transplant continued to have amyloid deposition of the wild-type TTR subtype [62,66–68].…”

Section: Advanced Heart Failure Therapiesmentioning

confidence: 99%

“…Small left ventricular size presents technical challenges for inflow cannula placement and advanced stages of the disease are less responsive to mechanical support [62]. However, there may be a subset of patients with larger left ventricular cavities who may benefit, particularly when used as a bridge to transplant strategy [63,64 ▪▪ ]. Furthermore, RV failure is often progressive in this disease process, further underscoring the limitations of left sided ventricular support [62,63].…”

Section: Advanced Heart Failure Therapiesmentioning

confidence: 99%

“…In contrast, cardiac transplant is a viable option for carefully selected patients with both AL and ATTR advanced cardiomyopathy and the current allocation system provides priority as status 4 to these patients [27 ▪▪ ,64 ▪▪ ,65]. The transplant selection process in AL amyloidosis is more nuanced, as it is important to carefully screen for the extent of multiorgan involvement and demonstrate hematologic response to chemotherapy prior to pursuing organ transplantation.…”

Section: Advanced Heart Failure Therapiesmentioning

confidence: 99%

“…There is no data to support, the optimal therapeutic strategy posttransplant in these patients. However, it is reasonable to consider silencer therapy if patients have lifestyle-limiting neuropathy in the postheart transplant phase [64 ▪▪ ]. Historically, liver transplant was considered a first-line therapeutic modality in patients with hATTR to replace the source of mutant TTR production [62,64 ▪▪ ].…”

Section: Advanced Heart Failure Therapiesmentioning

confidence: 99%

See 2 more Smart Citations

Cardiac amyloidosis: an update on diagnosis, current therapy, and future directions

Khedraki

2022

Current Opinion in Cardiology

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Purpose of reviewThis review aims to serve as a practical guide for differentiating the two most common forms of cardiac amyloidosis, as well as reviewing the approach to diagnosis and management, particularly as it pertains to transthyretin cardiac amyloidosis. Recent findingsEmerging literature continues to unravel new understandings and challenges in the field of cardiac amyloidosis. Although cardiac amyloidosis has historically been thought of as a 'zebra diagnosis', current evidence has shown that this is a common cause of heart failure. Furthermore, it has become increasingly apparent that earlier diagnosis leads to improved outcomes and quality of life for patients. SummaryBy leveraging an understanding of the pathophysiology leading to amyloid fibril formation, new drug therapeutics are under investigation as promising candidates for the treatment of amyloid cardiomyopathy.

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