Author + information
- Candida Cristina Quarta,
- Imran Uraizee,
- Rodney H. Falk,
- Jenna Kruger,
- Simone Longhi,
- Claudio Rapezzi and
- Scott D. Solomon
Light chain (AL)-related cardiac amyloidosis (CA) has a far worse prognosis than either wild type (ATTRwt) or mutant (ATTRm) transthyretin amyloidosis. This has been attributed, in part, to light chain cardiotoxicity. We analyzed LV function with speckle tracking echocardiography in CA and compared the three types of CA to determine whether differences can be explained simply by greater infiltration (assessed by wall thickness) or lower EF.
We analyzed 172 patients with CA. Standard echocardiographic parameters were measured as well as longitudinal (LS) and circumferential strain (CS) by speckle tracking.
In the overall population, LV EF was preserved (55±11%) but global (−13±4%), basal (−8±5%) and mid LV (−9±4%) LS were reduced. ATTRwt patients were older and had higher LV wall thickness and lower LV EF. Global, basal and mid LV LS was significantly less in AL and ATTRwt than in ATTRm even after adjustment by age and wall thickness, although all were abnormal compared with reference values. CS was preserved in all the three forms.
Despite preserved CS and LV EF, patients with AL and ATTRwt have significantly worse LS than those with ATTRm. Decreased LS in ATTRwt may represent a lower LV ejection fraction or a longer time-course of infiltration. However, this explanation cannot account for the impaired LS in AL. Decreased LS in AL gives further credence to a cardiotoxic effect of circulating light chains as a cause of LV dysfunction in AL amyloidosis.
|AL (n=80)||ATTRm (n=37)||ATTRwt (n=55)||p|
|Male gender, n (%)||53 (66)||28 (76)||48 (87)||0.021|
|NYHA class III-IV, n (%)||23 (29)||13 (35)||16 (29)||0.8|
|LV wall thickness, cm||1.5±0.2||1.5±0.2||1.7±0.2||<0.001|
|LV ejection fraction, %||56±11||56±13||51±11||0.03|
|E wave, cm/sec||0.84 [0.69–0.94]||0.79 [0.65–0.98]||0.76 [0.65–0.93]||0.37|
|A wave, cm/sec||0.56 [0.33–0.8]||0.66 [0.47–0.78]||0.38 [0.29–0.55]||0.004|
|E/A ratio||1.63 [1.02–2.54]||1.3 [0.9–1.8]||2 [1.24–2.84]||0.06|
|Septal S' wave, cm/sec||5 [4–6]||6 [4–7]||4 [3–5]||<0.001|
|Septal E' wave, cm/sec||5 [4–6]||5 [4–7]||4 [3–5]||0.14|
|Global LV longitudinal strain, %||−12 ± 4||−15±5||−12±4||0.006*|
|Basal LV longitudinal strain, %||−9±5||−12±6||−7±4||0.009*|
|Mid LV longitudinal strain, %||−9±4||−11±5||−8±4||0.01*|
|Apical LV longitudinal strain, %||−20±7||−22±6||−19±7||0.11*|
|Circumferential strain, %||−24±7||−26±7||−25±7||0.34*|
↵* Adjusted by age and LV wall thickness
Poster Sessions, Expo North
Saturday, March 09, 2013, 3:45 p.m.-4:30 p.m.
Session Title: What Is Unfolding in Cardiac Amyloidosis Research?
Abstract Category: 23. Pericardial/Myocardial Disease
Presentation Number: 1163-144
- 2013 American College of Cardiology Foundation