Diagnostic delay in wild type transthyretin cardiac amyloidosis – A clinical challenge

Ladefoged, Bertil; Dybro, Anne M.; Povlsen, Jonas Agerlund; Vase, Henrik; Clemmensen, Tor Skibsted; Poulsen, Steen Hvitfeldt

doi:10.1016/j.ijcard.2019.12.063

Cited by 38 publications

(79 citation statements)

References 22 publications

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“…Lane reports a median life expectancy of 57 months. In the Danish study by Ladefoged et al 10 , 42% of patients were in stage 1, 40% in stage 2, and 18% in stage 3. Using the UK NAC-specific survival data and the Danish distribution of patients according to the NAC distribution, the average weighted median life expectancy is 52 months.…”

Section: Time Horizonmentioning

confidence: 90%

“…Estimates of resource use associated with diagnosis are based on current Danish treatment guidelines and clinical practice with treatment and monitoring of patients with ATTRwt. Frequencies of cardiac implants and hospitals admissions prior to diagnosis are estimated from a Danish study by Ladefoged et al 10 . Ladefoged et al (2020) includes 50 contemporary ATTRwt patients diagnosed between 2017 and 2019 and present the only available Danish data on ATTRwt.…”

Section: Data Sourcesmentioning

confidence: 99%

“…Three studies report the median diagnostic delay measured as the time from the first cardiological examination to the date of the diagnosis by endomyocardial biopsy or DPD scintigraphy. Ladefoged et al 10 reports median delays of 13 (2-47) months, Bishop et al 11 reports median delays of 34 (15-72) months, and Lane et al 9 reports median delays of 39 (8-78) months in diagnosis. We use the Danish estimate by Ladefoged et al (2020) with a median diagnostic delay of 13 months in our base case.…”

Section: Time Horizonmentioning

confidence: 99%

“…For patients with ATTRwt, a pacemaker is a standard treatment for symptom management of particular arrhythmias. According to Ladefoged et al 10 , 30% of Danish ATTRwt patients have received a pacemaker 10 . Another recent study from Spain found that up to 44% of patients with ATTRwt carry a pacemaker .…”

Section: Cardiac Implantsmentioning

confidence: 99%

“…The epidemiology of ATTRwt is poorly characterized, however, new data suggest that the disease is substantially under-and misdiagnosed [5][6][7][8] . Studies have reported delays in diagnosis from 13 to 78 months [9][10][11] . Before the correct diagnosis is established, patients with ATTRwt experience multiple hospitalizations due to misdiagnosis and mistreatments, which has been reported to be as high as six hospital admissions annually [9][10][11] .…”

Section: Introductionmentioning

confidence: 99%

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Diagnostic and lifetime hospital costs of patients suffering from wild-type transthyretin amyloid cardiomyopathy in Denmark

Pilgaard¹,

Pedersen²,

Poulsen

2020

Journal of Medical Economics

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Aims: Wild-type transthyretin amyloid cardiomyopathy (ATTRwt) is a fast progressing and fatal disease associated with substantial delays in diagnosis. Between the first symptoms and diagnosis, patients are frequently hospitalized, primarily with cardiac symptoms. After diagnosis, patients continue to experience frequent hospital admissions. The objective of this study was to estimate the Danish diagnostic and lifetime hospital costs associated with the treatment of patients with ATTRwt both before and after they are diagnosed. Materials and Methods: We developed a cost model for Danish hospital costs associated with ATTRwt, including the costs of diagnosis, cardiac implants, and hospital admissions covering inpatient hospitalization and outpatient hospital care (ambulatory and emergency services). The number of diagnoses, cardiac implants, inpatient hospitalization and outpatient hospital care were estimated based on published data. Estimates of the unit costs were based on publicly available Danish reference costs. We calculated the total hospital costs covering the median lifespan of patients from onset of symptoms, which is 13 months prior to diagnosis, to 52 months after diagnosis which is the median survival time after diagnosis. Results: The average cost of diagnosing ATTRwt was USD 3,424 per patient; the average costs of cardiac implants were USD 1,851 per patient. Hospital admissions costs totaled USD 3,345 pre-diagnosis and USD 59,449 post-diagnosis per patient, on average. The total diagnostic and lifetime (65 months) hospital costs associated with ATTRwt were USD 68,069. Conclusions: Caring for patients with ATTRwt places a significant economic burden on the healthcare system. The study emphasizes the cost saving potential for medical interventions in this patient population.

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Section: Time Horizonmentioning

confidence: 90%

Section: Data Sourcesmentioning

confidence: 99%

Section: Time Horizonmentioning

confidence: 99%

Section: Cardiac Implantsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Diagnostic and lifetime hospital costs of patients suffering from wild-type transthyretin amyloid cardiomyopathy in Denmark

Pilgaard¹,

Pedersen²,

Poulsen

2020

Journal of Medical Economics

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Cardiac amyloidosis: A survey of current awareness, diagnostic modalities, treatment practices, and clinical challenges among cardiologists in selected Middle Eastern countries

Mohty

Nasr

Ragy

et al. 2023

Clinical Cardiology

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Background: Cardiac amyloidosis (CA) is a chronic progressive disease caused by the deposition of amyloid fibrils in cardiac tissues. Diagnosis and management of CA are complicated and have developed over the years. Hypothesis: Middle Eastern countries have significant knowledge disparities in diagnosing, managing, and treating different subtypes of CA. Methods: An online survey was sent to cardiologists in four countries (Saudi Arabia, Lebanon, Egypt, and Iraq) interested in heart failure and practicing for more than a year. The survey questioned the characteristics of the participants and their institutions. It addressed their knowledge and practices in CA specifically diagnostic modalities, treatment options, and interest in education and knowledge exchange.Results: A total of 85 physicians participated in the survey. There was a variation in the participating cardiologists' knowledge, experience level, and readiness of their institutes to manage patients with ATTR-CM. Most participants believed that a high rate of ATTR-CM misdiagnosis existed. Participants' knowledge of the diagnostic modalities and "red flags" raising suspicion about ATTR-CM varied. Another challenge was the availability of essential diagnostic modalities among various cardiology centers. A knowledge gap was also observed regarding updates in ATTR-CM management. However, there was a high endorsement of the need for more education, physician networking, and knowledge exchange.Conclusions: This survey highlighted the need for increasing awareness levels among cardiologists in the four selected Middle Eastern countries. Cardiologists are most likely to benefit from additional training and knowledge exchange on the latest management advances of this disease. Thus, measures must be taken to focus on the

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History of extracardiac/cardiac events in cardiac amyloidosis: prevalence and time from initial onset to diagnosis

et al. 2021

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Aims Cardiac amyloidosis (CA) has a poor prognosis which is aggravated by diagnostic delay. Amyloidosis extracardiac and cardiac events (AECE and ACE) may help improve CA diagnosis and typing. The aim of this study was to compare AECE and ACE between different CA types and assess their relationship with survival. Methods and results This retrospective cohort study conducted in France from June 2008 to May 2019, at the Henry Mondor Hospital. This cohort included 983 patients with CA. Mean age at inclusion was 73.1 ± 11.4 years, 726 (75.1%) were male and the mean body mass index was 24.5 ± 4.1 kg/m2. Among them, 321 had immunoglobulin light chain (AL) amyloidosis, 434 had wild‐type transthyretin (ATTRwt), and 212 had hereditary transthyretin (ATTRv). The first AECE and/or ACE occurred at a mean age of 63 ± 11 years for AL and ATTRv, and 70 ± 12 years for ATTRwt (P < 0.01). The median (Q1–Q3) delay between declaration of the first events and diagnosis varied from 11.1 (5.9; 34.8) months for AL to 92.2 (39.0; 174.7) months for ATTRwt (P < 0.01). The nature of the onset of AECE or ACE varied based on amyloidosis type, heart failure symptoms for AL (26%) and integumentary symptoms for ATTRv with cardiologic or mixed phenotype (39%) and ATTRwt (42%). In AL and ATTRwt, a short delay between the onset of the first AECE or ACE and diagnosis was associated with reduced survival rate (log‐rank test P‐value <0.01). Conclusions This study highlights the impact of amyloidosis type and evolution on diagnostic delay and on prognosis. Physicians must be aware and vigilant in front of extracardiac and cardiac events to considerably improve early diagnosis of amyloidosis.

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Diagnostic delay in wild type transthyretin cardiac amyloidosis – A clinical challenge

Cited by 38 publications

References 22 publications

Diagnostic and lifetime hospital costs of patients suffering from wild-type transthyretin amyloid cardiomyopathy in Denmark

Diagnostic and lifetime hospital costs of patients suffering from wild-type transthyretin amyloid cardiomyopathy in Denmark

Cardiac amyloidosis: A survey of current awareness, diagnostic modalities, treatment practices, and clinical challenges among cardiologists in selected Middle Eastern countries

History of extracardiac/cardiac events in cardiac amyloidosis: prevalence and time from initial onset to diagnosis

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