{
  "abstract": "Introduction Familial hypercholesterolaemia (FH) affects 1:300 individuals and confers significantly increased lifetime cardiovascular risk. Cardiac CT (CCT) effectively detects subclinical coronary artery disease (CAD), enabling timely lipid therapy intensification. However, optimal downstream investigation and management of asymptomatic patients with high atherosclerotic burden remains unclear, with strategies ranging from conservative symptom-guided treatment to early invasive assessment, with some observational data suggesting functional test driven revascularisation, although evidence remains limited.Aim To evaluate downstream investigation strategies and their influence on revascularisation and clinical outcomes in FH patients with subclinical high-risk CAD.Methods We retrospectively screened 2441 patients referred from a specialist lipid clinic for CCT (Coronary calcium scoring and/or CT coronary angiography) between 2012 - 2024, and identified 50 patients with definite and possible FH (32/64% genetically confirmed) and high-risk CCT features: calcium score >400 AU, left main disease, proximal LAD disease, or three-vessel disease ( table 1). Patients were categorised by initial management strategy: no further testing (n=13), functional testing (n=34), or direct angiography (n=3). Data on downstream testing, revascularisation, MI, and cardiac death were collected from electronic records.Results Outcomes Overall, 17/50 (34%) proceeded to invasive angiography during the follow up period and 9/50 (18%) underwent revascularisation (5 PCI, 4 CABG). There were no non-fatal MIs and 1 cardiac death following an MI (2%), 1 death from an intracranial bleed in a patient taking Aspirin, and 4 other deaths from non-cardiac or undocumented causes. Revascularisation rates based on testing strategy and symptoms are illustrated below.Conclusions In asymptomatic FH patients with severe CAD on CCT, symptom development (angina) was the primary driver of revascularisation (PPV 83.3%), not functional test results.MPS had no predictive value for revascularisation (PPV 0%) despite positive results in 5 patients. Stress echocardiography performed better but numbers were small.Calcium score showed no correlation with revascularisation (p=0.92).A watchful waiting approach with symptom-guided intervention appears safe in most patients, with low event rates over median 8.5-year follow-up. However, individualised care is important to identify those that may benefit from early revascularisation.These findings support individualised management of asymptomatic severe CAD in FH, with clinical symptoms rather than routine functional testing guiding intervention in the majority however, larger prospective studies are needed to define the utility of functional testing.Abstract 343 Figure 1Abstract 343 Figure 2References Pérez de Isla L, et al. Coronary computed tomographic angiography findings and their therapeutic implications in asymptomatic patients with familial hypercholesterolemia. Lessons from the SAFEHEART study. J Clin Lipidol. 2018 Jul-Aug;12(4):948–957. doi: 10.1016/j.jacl.2018.04.003. Epub 2018 Apr 17. PMID: 29753733.Shipman KE, et al. The use of CT coronary calcium score in asymptomatic patients with familial hypercholesterolaemia. J Diabetes 2016;15:62–64.",
  "authors": [
    {
      "affiliations": [
        "Russells Hall Hospital, Dudley, United Kingdom"
      ],
      "name": "Raja Khan"
    },
    {
      "affiliations": [
        "Russells Hall Hospital, Dudley, United Kingdom"
      ],
      "name": "Sawan Waidyanatha"
    }
  ],
  "title": "343 Asymptomatic high risk coronary artery disease in familial hypercholesterolaemia: downstream management strategies and outcomes after cardiac CT",
  "uid": "21aa0ebd-9401-50df-8d4b-1f78411ad03f"
}