Amyloid cardiomyopathy in a large integrated health care system

Czobor, Peter; Hung, Yun‐Yi; Baer, David; McGlothlin, Dana; Weisshaar, Dana; Zaroff, Jonathan G.

doi:10.1016/j.ahj.2019.06.008

Cited by 10 publications

(7 citation statements)

References 32 publications

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“…Patients with the cardiac phenotype already-exhibited symptoms of heart failure (68% had NYHA stage II-IV) and a clinically meaningful increase in left ventricular wall thickness at diagnosis [18]. A similar lesson can be learnt from a cohort study of 198 patients with ATTR-CM in the US, in which elevated concentrations of B-type natriuretic peptide were significantly associated with mortality, emphasizing the need for careful cardiac management of these patients to prevent premature deterioration of their condition [19].…”

Section: Discussionmentioning

confidence: 90%

Diagnosis of Amyloidosis: A Survey of Current Awareness and Clinical Challenges Among Cardiologists in Switzerland

Mircsof

2020

Cardiol Ther

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Introduction Transthyretin amyloidosis (ATTR) is a progressive disease in which amyloid fibril deposition disrupts tissue structure and organ function. Many patients with ATTR present with cardiac involvement; recent studies indicate that ATTR prevalence is higher than expected in patients with certain heart conditions. Although long delays to diagnosis are common, recent treatment advances have made timely diagnosis critical to ensure appropriate patient management. Despite clinical guideline updates, it remains unclear how these are being implemented in routine patient care. Methods We performed a survey of practicing cardiologists in Switzerland to assess their knowledge of ATTR and current clinical practice. Results Overall, 72 cardiologists completed the questionnaire in a face-to-face interview (n = 16) or online (n = 56). Key findings highlighted wide variation in cardiologists’ knowledge about the diagnostic tests required for a differential diagnosis of ATTR. In particular, many cardiologists lacked familiarity with radiolabeled bone scintigraphy, an important non-invasive test that is part of the diagnostic algorithm for ATTR. Another challenge in diagnosing amyloidosis appears to be general awareness of ATTR among cardiologists. Conclusions Survey results highlight that cardiologists in Switzerland would benefit from information on the latest advances in ATTR to support them in screening heart failure patients and ensure that patients benefit from treatment advances.

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Section: Discussionmentioning

confidence: 90%

Diagnosis of Amyloidosis: A Survey of Current Awareness and Clinical Challenges Among Cardiologists in Switzerland

Mircsof

2020

Cardiol Ther

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“…A formal bias assessment of identified studies is provided in online supplementary Table . The individual studies and the extracted aggregate data are summarized in Table 2 2,3,10,11,13,26,27,29,30,52,54,65,71,78–158 . An exponential growth in the annual publication of studies on clinical outcomes of ATTR was observed over the recent years ( Figure ).…”

Section: Resultsmentioning

confidence: 99%

Prevalence and clinical outcomes of transthyretin amyloidosis: a systematic review and meta‐analysis

Antonopoulos

Panagiotopoulos

Kouroutzoglou

et al. 2022

European J of Heart Fail

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“…Czobor et al 44 identified all patients with cardiac amyloidosis using International Classification of Disease codes at Kaiser Permanente hospital systems in San Francisco, United States, and found that among all ATTR-CM patients, Black race was associated with higher mortality.…”

Section: Resultsmentioning

confidence: 99%

“…Among those diagnosed with vATTR-CM phenotype in the United States and United Kingdom, 66% to 79% carried the p.Val142Ile variant compared with 0% to 22% reported from the rest of the world (Table 2). [42][43][44][45][46][47][48][49][50][51] Among studies of clinically diagnosed vATTR polyneuropathy, the p.Val142Ile variant frequency was expectedly low ranging from 0% to 1.7% (Table IV in the Data Supplement). [52][53][54][55][56][57][58][59][60][61]…”

Section: Prevalence Of Pval142ile Variant Among Those With Confirmed ...mentioning

confidence: 99%

Prevalence and Outcomes of p.Val142Ile TTR Amyloidosis Cardiomyopathy: A Systematic Review

Chandrashekar

Alhuneafat

Mannello

et al. 2021

Circ: Genomic and Precision Medicine

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Background: The p.Val142Ile variant, predominantly found among people of African descent, is the most common cause of variant transthyretin amyloidosis and carriers predominantly develop a cardiomyopathy (variant transthyretin amyloidosis cardiomyopathy) phenotype. Yet, there are conflicting data on the prevalence and outcomes of p.Val142Ile variant carriers. Methods: We performed a systematic review of the prevalence and outcomes of p.Val142Ile variant transthyretin amyloidosis cardiomyopathy among subjects of African descent. We found 62 relevant articles after searching the MEDLINE databases from 1980 to 2020 that reported data for ≈150 000 subjects. Results: The reported worldwide prevalence of the p.Val142Ile variant is 0.3% to 1.6% in the general population. Among people of African descent, the reported prevalence from all studies ranges from 1.1% to 9.8%, but for studies with >1000 subjects, it is 3% to 3.5%. The prevalence of the p.Val142Ile variant in a region is dependent on the reported percentage of subjects who are of African descent in that region. p.Val142Ile variant transthyretin amyloidosis cardiomyopathy typically presents in the seventh to eighth decade of life and the majority of cases reported were male, with 25% to 38% diagnosed with atrial fibrillation. It was associated with a longitudinally worse quality of life and a lower adjusted survival compared with other types of transthyretin amyloidosis cardiomyopathy. Conclusion: The p.Val142Ile variant is the most common variant of the transthyretin gene with most carriers being of African descent. The true penetrance is unknown but the p.Val142Ile variant is associated with increased rates of incident heart failure and portends a lower overall survival. Increased awareness could lead to earlier diagnosis and improved heart failure outcomes among those of African descent, which is of increasing importance given the advent of novel therapeutics for this disease.

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Amyloid cardiomyopathy in a large integrated health care system

Cited by 10 publications

References 32 publications

Diagnosis of Amyloidosis: A Survey of Current Awareness and Clinical Challenges Among Cardiologists in Switzerland

Diagnosis of Amyloidosis: A Survey of Current Awareness and Clinical Challenges Among Cardiologists in Switzerland

Prevalence and clinical outcomes of transthyretin amyloidosis: a systematic review and meta‐analysis

Prevalence and Outcomes of p.Val142Ile TTR Amyloidosis Cardiomyopathy: A Systematic Review

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