{
  "abstract": "Introduction Transthyretin cardiomyopathy (ATTR-CM) is a treatable cause of heart failure, yet up to 47% of patients are initially misdiagnosed with 65% seeing ≥2 cardiologists before diagnosis. 1 Diagnostic delays result in a median of 17 hospital attendances prior to diagnosis.2 Timely diagnosis facilitates early access to disease modifying drugs resulting in improved outcomes and reduced cost. Echocardiography plays a central role in the nonbiopsy diagnostic algorithm for ATTR-CM.Aim To evaluate the local cardiac amyloidosis diagnostic pathway to understand how patients with echocardiographic features suggestive of amyloidosis progress through subsequent investigations in real-world practice, and to assess adherence to guideline-recommended investigations at University Hospitals Dorset.Methods All transthoracic echocardiograms performed between January 2024 and November 2020 with the term ‘amyloid’ in the report were reviewed against the 2023 ESC Guidelines for the management of cardiomyopathies. Demographic data, echocardiographic features, and subsequent investigations along the amyloidosis diagnostic pathway were analysed. For patients who did not undergo guideline-recommended follow-up investigations, electronic health records were reviewed to identify documented clinical reasons. Where no justification was evident, cases were flagged for further diagnostic work-up including clinic review.Results Of 97 patients with echocardiographic reports referencing amyloid, 44% were female, mean age 80 (39-97) years), 14% (n=14) had a pre-existing diagnosis of cardiac amyloidosis (11 ATTR-CM, 3 AL) (see figure 1). Thirty-three patients underwent further investigation following the echocardiogram, including bone scintigraphy (n=8), cardiac MRI (n=20), or both (n=5). Among those investigated, 42% (n=14) were diagnosed with amyloidosis, of whom 10 patients were referred to the National Amyloidosis Centre (NAC). Mean time from echocardiographic report to NAC referral was 116 (9-263) days. Three patients were on treatment with tafamidis, 6 were awaiting appointment and 1 was diagnosed with AL amyloid.Fifty patients did not undergo further investigation. In 34 cases this was due to frailty, death, alternative diagnoses, or patient choice. However, 16 patients had no documented clinical reason for omission of follow-up; these patients are currently undergoing investigations as a result of this evaluation.Conclusions This evaluation demonstrated both successful identification of previously unrecognised amyloidosis and a substantial gap between echocardiographic suspicion and downstream diagnostics. Although 12% (n=10) of undiagnosed patients with echocardiographic features of amyloidosis underwent appropriate follow-up investigations enabling referral to the NAC, 19% (n=16) did not progress along the recommended diagnostic pathway. These findings highlight persistent diagnostic delays that limit timely access to disease modifying therapy. Further analyses are underway to assess the impact of pathway deviation on healthcare utilisation and clinical outcomes.References Lousada I, et al. Amyloidosis research consortium cardiac amyloidosis survey: results from patients with AL and ATTR amyloidosis and their caregivers. Journal of Cardiac Failure 2019;20(8):S69.Lane T, et al. Natural history, quality of life, and outcome in cardiac transthyretin amyloidosis. Circulation 2019;140(1):16–26.Abstract 183 Figure 1Sankey diagram of diagnostic pathway of patients with echocardiographic feature of amyloidosis. ATTR-CM = Transthyretin cardiomyopathy, DPD = 99mTc-DPD bone scintigraphy, cMRI = cardiac Magnetic Resonance Imaging, NAC = National Amyloidosis Centre",
  "authors": [
    {
      "affiliations": [
        "University Hospitals Dorset, Bournemouth, United Kingdom"
      ],
      "name": "Samantha Montandon"
    },
    {
      "affiliations": [
        "University Hospitals Dorset, Bournemouth, United Kingdom"
      ],
      "name": "Lucy Carter"
    },
    {
      "affiliations": [
        "University Hospitals Dorset, Bournemouth, United Kingdom"
      ],
      "name": "Ana-Maria Bologan"
    },
    {
      "affiliations": [
        "University Hospitals Dorset, Bournemouth, United Kingdom"
      ],
      "name": "Kayleigh Romagnoli"
    },
    {
      "affiliations": [
        "University Hospitals Dorset, Bournemouth, United Kingdom"
      ],
      "name": "Ryan Cooper"
    },
    {
      "affiliations": [
        "University Hospitals Dorset, Bournemouth, United Kingdom"
      ],
      "name": "Rachel Hall"
    },
    {
      "affiliations": [
        "University Hospitals Dorset, Bournemouth, United Kingdom"
      ],
      "name": "Russell Bull"
    },
    {
      "affiliations": [
        "University Hospitals Dorset, Bournemouth, United Kingdom"
      ],
      "name": "Chris Steadman"
    },
    {
      "affiliations": [
        "University Hospitals Dorset, Bournemouth, United Kingdom"
      ],
      "name": "Raj Chahal"
    },
    {
      "affiliations": [
        "University Hospitals Dorset, Bournemouth, United Kingdom"
      ],
      "name": "Chris Critoph"
    },
    {
      "affiliations": [
        "University Hospitals Dorset, Bournemouth, United Kingdom"
      ],
      "name": "Ki Yap"
    },
    {
      "affiliations": [
        "University Hospitals Dorset, Bournemouth, United Kingdom"
      ],
      "name": "Kat Dixon"
    },
    {
      "affiliations": [
        "University Hospitals Dorset, Bournemouth, United Kingdom"
      ],
      "name": "Sarah Birkhoelzer"
    }
  ],
  "title": "183 Assessing cardiac amyloidosis pathways in left ventricular hypertrophy: a service evaluation from the university hospitals Dorset, UK",
  "uid": "615d51d1-8577-519b-9fb0-3480b220106b"
}