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9 Prevalence of transthyretin cardiac amyloidosis in patients with high degree AV block
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  1. Douglas Cannie1,
  2. Kush Patel1,
  3. Leon Menezes1,
  4. Imogen Heenan2,
  5. Athanasios Bakalakos1,
  6. Perry Elliott1,
  7. Alexandros Protonotarios1,
  8. Petros Syrris2
  1. 1University College London & Barts Heart Centre
  2. 2University College London

Abstract

Background Transthyretin amyloidosis cardiomyopathy (ATTR-CM) is an infiltrative cardiac disorder caused by deposition of wild type or mutated transthyretin and which is amenable to therapy with TTR stabilisers. As ATTR-CM is associated with conduction disease, we sought to determine its prevalence in patients with idiopathic high-degree atrioventricular (AV) block requiring permanent pacemaker (PPM) implantation.

Methods Consecutive patients aged 70-85 years undergoing PPM implantation for idiopathic high-degree AV block between November 2019 and November 2021 were offered a 3,3-diphosphono-1,2-propanodicarboxylic acid (DPD) scan. Demographics, comorbidities, electrocardiographic and imaging data from the time of device implantation were retrospectively collected.

Results Thirty nine patients (79.5% male, mean (SD) age at device implantation 76.2 (2.9) years) had a DPD scan (Table 1). Three (7.7%, all male) had a result consistent with ATTR-CM (Perugini grade 2 or 3). Mean (SD) maximum wall thickness of those with a positive DPD scan was 19.0 mm (3.6 mm) versus 11.4 mm (2.7 mm) in those with a negative scan (p value = 0.06). All patients diagnosed with ATTR-CM had spinal canal stenosis and 2 had carpal tunnel syndrome.

Conclusions ATTR-CM should be considered in older patients requiring permanent pacing for high-degree AV block, particularly in the presence of left ventricular hypertrophy, carpal tunnel syndrome or spinal canal stenosis.

Abstract 9 Table 1

Demographic, clinical and echocardiographic features of 39 patients undergoing device implantation for high-degree AV block, stratified by outcome of DPD scan. Abbreviations: DPD, 3,3-diphosphono-1,2-propanodicarboxylic acid; IQR, interquartile range; LV, left ventricular; PPM, permanent pacemaker; SD, standard deviation

Abstract 9 Figure 1

Investigations for an 85 year old male requiring ICD implantation for 2:1 AV block and ventricular arrhythmia. Perugini grade 3 uptake on DPD Scan (top left) consistent with a diagnosis cardiomyopathy. Electrocardiogram showing low voltage QRS Complexes in limb leads and first-degree heart block (top right). Traces from holter monitoring showing 2:1 fixed AV block and non-sustained ventricular tachycardia (bottom left). Echocardiography showing concentric left ventricular hypertrophy and near complete cavity oblitration in systole(bottom right)

Conflict of Interest None

  • Transthyretin amyloidosis
  • Pacemaker
  • Conduction disease

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