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Improving outcomes in AL amyloidosis

ESC 2023 - Highlights article 2

Read time: 10 mins
Last updated: 7th Sep 2023
Published:7th Sep 2023

AL amyloidosis at ESC 2023

Watch Professor Stefano Perlini (University of Pavia, Italy) review treatment advances from ESC Congress 2023 for amyloid light-chain (AL) amyloidosis with cardiac dysfunction.

Risk stratification in cardiac amyloidosis

By Eleanor McDermid

Several studies presented at ESC Congress 2023 reported on tools to help predict poor outcomes in people with cardiac amyloidosis.

Poor outcomes with cardiac amyloidosis

People with AL amyloidosis have poorer outcomes after hospitalisation than those with amyloid transthyretin (ATTR) amyloidosis, suggests a study from researchers at Mount Sinai Health System in New York, USA.

Their study drew on data from the US Nationwide Inpatient Sample, comprising 12,430 hospitalisations of people with cardiac amyloidosis. Just 3.7% of these involved people with AL amyloidosis. However, 9.8% of these people died during their hospital stay, which was a significantly higher in-hospital mortality rate than the 6.6% seen for those with ATTR amyloidosis. The difference between the two groups persisted after accounting for baseline variables.

People with AL amyloidosis were also significantly more likely to be hospitalised for heart failure (26.8 vs 21.7%) and to require cardiopulmonary resuscitation (2.6 vs 1.1%). Conversely, they were less likely to have major bleeding (0.2 vs 11.9%), thromboembolism (0.2 vs 1.1%) or a stroke (0.7 vs 17.1%).

The same team, again using the US Nationwide Inpatient Sample, also looked at factors associated with risk of sudden cardiac arrest. This outcome occurred in 1.9% of 103,620 people who were hospitalised with cardiac amyloidosis.

Presenter Kruti Gandhi noted that traditional cardiovascular risk factors, such as obesity and smoking, were not associated with the risk of sudden cardiac arrest in this population.

Traditional cardiovascular risk factors do not predict sudden cardiac arrest in a cardiac amyloidosis population

Instead, people younger than 40 years had a 2.3-fold higher risk of sudden cardiac arrest than younger individuals, and the risk was increased 1.4-fold in people of African American race, in whom the incidence was 30.2%, compared with 23.4% in people with other ethnic backgrounds.

In addition, indicators of atrioventricular nodal dysfunction, distal conduction disease and tachyarrhythmias were significantly associated with sudden cardiac arrest risk – the largest risk increase, of 33.7-fold, was for ventricular fibrillation. Finally, the presence of heart failure was also associated with sudden cardiac arrest risk, with increases of 1.4- and 1.3-fold for preserved and reduced ejection fraction, respectively.

Gandhi suggested these factors could assist with risk stratification of people with cardiac amyloidosis.

Professor Perlini explains why AL amyloidosis management is now viewed as more than “empirical treatment”.

Extracellular volume

Another marker for risk stratification proposed at this year’s ESC congress was extracellular volume (ECV).

This emerged from a prospective registry study by Franz Duca (Medical University of Vienna, Austria) and team. Their findings show that, on average, ECV significantly increased in 103 people with cardiac amyloidosis between baseline and 12-month cardiac magnetic resonance examinations.

Moreover, increase in ECV was significantly associated with the risk of heart failure or all-cause mortality in the cohort overall and in people with AL amyloidosis and with ATTR amyloidosis considered separately. And when dividing people by whether they had increasing ECV versus stable or decreasing ECV, the time to an outcome event was significantly less in the former than the latter group.

Monitoring extracellular volume may help predict heart failure and mortality in people with AL amyloidosis

Transoesophageal echocardiography

Finally, a small study highlighted three transoesophageal echocardiography and cardiac magnetic resonance imaging parameters that could be prognostic in people with AL amyloidosis.

Among 39 such people, global longitudinal strain of 11.5% or greater was associated with an increased risk of mortality within the following year, reported Angel Hernandez Martos (University Hospital of Salamanca, Spain). In addition, T1 mapping higher than 1042 ms and ECV greater than 49% were also significantly associated with this outcome.

The researchers suggested that these markers may warrant inclusion in risk stratification models.

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