Heart failure & cardiomyopathies
Rare Forms of Cardiac Amyloidosis: Diagnostic Clues and Phenotype in Apo AI and AIV Amyloidosis
Authors' conclusion
Dysphonia, multisystem involvement, or right-sided cardiac disease should raise suspicion of AApoAI-CA. AApoAIV-CA presents most commonly with heart failure and always displays classical CA imaging features, mimicking common forms of CA. Both AApoAI and AApoAIV are associated with a good prognosis and a lower risk of mortality than matched patients with AL-amyloidosis.
Study context
- Population
- Patients with AApoAI or AApoAIV assessed at one centre, 2000–2021
- Exposure
- AApoAI and AApoAIV cardiac amyloidosis vs matched amyloidosis (AL) and transthyretin CA
- AApoAIn = 45
- AApoAIVn = 21
- Outcome
- Cardiac phenotype by multimodality imaging
Methodological detailssingle-centre, matched comparison cohorts, multimodality imaging
- Single-centre
- Matched comparison cohorts
- Multimodality imaging
Main finding
As reported in the abstract
Cardiac phenotype
- AApoAI: cardiac involvement
- 13 (29%)
- AApoAI: renal involvement
- 32 (71%)
- AApoAI: splenic involvement
- 28 (62%)
- AApoAI: hepatic involvement
- 27 (60%)
- AApoAI: laryngeal involvement
- 7 (16%)
- AApoAI-CA presenting with HF
- n=8, 62%
- AApoAI-CA presenting with dysphonia
- n=7, 54%
- Arg173Pro variant: cardiac and laryngeal involvement
- n=7, 100%
- RV free wall thickness, AApoAI-CA vs AL-CA vs transthyretin CA
- 8.6±1.9 versus 6.3±1.3 mm versus 7.7±1.2 mm, P=0.004
- Tricuspid stenosis, AApoAI-CA vs AL-CA vs transthyretin CA
- 4 [31%] versus 0 [0%] versus 0 [0%], P=0.012
- Tricuspid regurgitation, AApoAI-CA vs AL-CA vs transthyretin CA
- 6 [46%] versus 1 [8%] versus 2 [15%], P=0.048
- Cardiac involvement, AApoAIV vs AApoAI
- 15 [71%] versus 13 [29%], P=0.001
- AApoAIV-CA presenting with HF
- n=12, 80%
- Median eGFR, AApoAIV-CA vs AL-CA vs transthyretin CA
- 36 mL/[min·1.73 m²] versus 65 mL/[min·1.73 m²] versus 63 mL/[min·1.73 m²], P<0.001
- Apical-sparing strain, AApoAIV-CA vs AApoAI-CA
- 15 [100%] versus 7 [54%], P=0.003
- Cardiac uptake on bone scintigraphy, AApoAIV-CA vs AApoAI-CA (all grade 1)
- 14% versus 82%, P<0.001
Observational data: associations, not effects.
Further findings
| Outcome | AApoAI | AApoAIV | HR (95% CI) | P value | |
|---|---|---|---|---|---|
| Survival and mortality vs matched ALALvs AApoAIMortality · matched patients with AL-amyloidosis | median survival >172 months | median survival >30 months | 4.54 (2.02–10.14) | P < 0.001 | |
| Survival and mortality vs matched ALALvs AApoAIVMortality · matched patients with AL-amyloidosis | 3.07 (1.27–7.44) | P = 0.013 | |||
| 0.050.21520Hazard ratio (log scale) |
Original publication
Rare Forms of Cardiac Amyloidosis: Diagnostic Clues and Phenotype in Apo AI and AIV Amyloidosis. Circ Cardiovasc Imaging. Published 11 July 2023. doi:10.
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