Left ventricular assessment in patients with systemic light chain amyloidosis: a 3-dimensional speckle tracking transthoracic echocardiographic study.

Cardiac involvement in systemic light chain (AL) amyloidosis carries a poor prognosis mainly through involvement of the left ventricular (LV) myocardium. Despite its limitations, two-dimensional transthoracic echocardiography (2D-TTE) remains the main tool used for the assessment of LV systolic function in AL patients. We hypothesize that 3D-TTE coupled with speckle tracking imaging allows earlier detection of LV systolic dysfunction than 2D-TTE in AL amyloidosis. We prospectively studied 71 subjects including 58 patients with confirmed AL amyloidosis (mean age 66 ± 10 years, 60% male) and 21 healthy control (mean age 64 ± 7 years, 48% male) from 2011 to 2014 at the University Hospital of Limoges. The AL patients were divided into three groups according to Mayo Clinic (MC) staging and all subjects underwent 2D-TTE and 3D-TTE at the same setting. Using 2D-TTE, there was no significant difference in LV ejection fraction (EF) between the groups [LVEF = 63 ± 7% (control), 59 ± 6% (MC stage I), 60 ± 8% (MC stage II) and 57 ± 14% (MC stage III) (p = 0.24)]. In contrast, 3D-TTE demonstrated significantly worse LV systolic function in stage II and III patients using 3D-LVEF [MC II and III 45 ± 8% and 39 ± 12% vs. control 53 ± 8% (p < 0.0001)], global longitudinal strain (GLS) [MC II and III - 11 ± 4% and - 8 ± 3% vs. control - 15 ± 3% (p < 0.0001)] and global radial strain (GRS) [MC II and III 14 ± 9% and 10 ± 8% vs. control 25 ± 10% (p < 0.0001)]. Furthermore, MC III patients had significantly worse global circumferential strain and area tracking [- 17 ± 6% and - 25 ± 8% vs. - 24 ± 7% and - 36 ± 7% for control (p < 0.0001)]. Additionally, MC I had significantly better 3D GLS, GRS and global strain (- 15 ± 3%, 25 ± 10% and 28 ± 12%) than MC II (- 11 ± 4%, 14 ± 9% and 16 ± 10%) and MC III patients (- 8 ± 3%, 10 ± 8% and 12 ± 8%), respectively. Despite an apparently preserved LVEF by 2D-TTE, AL patients in MC stage II and III demonstrate evidence of LV systolic dysfunction by 3D imaging using LVEF and strain analysis. Worse LV involvement by AL amyloidosis was associated with more impaired 3D-TTE LV systolic parameters.

[Quantitative assessment of left ventricular regional wall motion in endomyocardial fibrosis]. / Avaliação quantitativa da movimentação parietal regional do ventrículo esquerdo na endomiocardiofibrose.

OBJECTIVE: To analyze left ventricular (LV) regional wall motion in patients with endomyocardial fibrosis (EMF). METHODS: The study comprised 88 patients, 59 of the female sex, with a mean age of 39+/-13 years (range, 9 to 65) and with echocardiographic and angiographic evidence of left ventricular EMF. The intensity of fibrous tissue buildup on contrast cineventriculography was classified as mild, moderate, or severe. The overall left ventricular ejection fraction (LVEF) was determined by using the area-length method on ventriculography. The motion was measured in 100 equidistant chords perpendicular to the centerline drawn in the middle of the final diastolic and systolic contours and normalized to cardiac size. Five left ventricular segments were analyzed: A--apical; AL--anterolateral; AB--anterobasal; IA--inferoapical; IB--inferobasal. Abnormality was expressed in units of standard deviation of the mean motion in a normal population of reference, comprised of 103 patients with normal LV according to clinical and electrocardiographic data, and angiographic standards. RESULTS: Mean LVEF was 0.47+/-0.12. Fibrous tissue buildup in the left ventricle was mild in 12 patients, moderate in 40, and severe in 36. The regions with the poorest ventricular wall motion were A (-1.4+/-1.6 standard deviation/chords) and IA (-1.6+/-1.8 standard deviation/chords) compared with that in AB (-0.3+/-1.9 standard deviation/chords), AL (-0.5+/-1.8 standard deviation/chords) and IB (-0.9+/-1.3 standard deviation/chords). No relation was observed between the intensity of fibrous tissue buildup and regional ventricular wall motion. CONCLUSION: A change in LV regional wall motion exists in EMF, and it is independent of the intensity of fibrous tissue buildup qualitatively assessed. Nonuniform involvement of the LV should be considered when planning surgery for this disease.

Avaliação quantitativa da movimentação parietal regional do ventrículo esquerdo na endomiocardiofibrose / Quantitative assessment of left ventricular regional wall motion in endomyocardial fibrosis

OBJETIVO: Analisar a movimentação parietal regional do ventrículo esquerdo (VE) em pacientes com endomiocardiofibrose (EMF). MÉTODOS: Estudados 88 pacientes, 59 do sexo feminino, com idade média de 39±13 anos (variação de 9 a 65) com evidência ecocardiográfica e angiográfica de EMF do VE. A intensidade da deposição de tecido fibroso na cineventriculografia contrastada foi classificada como discreta, moderada ou importante. A fração de ejeção global do ventrículo esquerdo (FEVE) foi determinada pelo método área-comprimento por meio da ventriculografia. O movimento foi medido em 100 cordas eqüidistantes e perpendiculares à linha média desenhada no meio dos contornos diastólico e sistólico finais e normalizadas para o tamanho cardíaco. Analisaram-se cinco segmentos do VE: A - apical; AL - ântero-lateral; AB - ântero-basal; IA - ínfero-apical; IB - ínfero-basal. A anormalidade foi expressa em unidades de desvio padrão do movimento médio em uma população de referência normal, composta por 103 pacientes com VE normal, conforme dados de clínica, eletrocardiograma e padrões angiográficos. RESULTADOS: A FEVE média foi de 0,47±0,12. O envolvimento de tecido fibroso do VE foi discreto em 12 pacientes, moderado em 40 e importante em 36. As regiões com pior movimentação parietal foram A (-1,4±1,6 desvio-padrão/cordas) e IA (-1,6±1,8 desvio-padrão/cordas) comparadas com AB (-0,3±1,9 desvio-padrão /cordas), AL (-0,5±1,8 desvio-padrão/cordas) e IB (-0,9±1,3 desvio-padrão/cordas). Não se observou relação entre a intensidade de envolvimento do tecido fibroso e a manutenção parietal regional. CONCLUSAO : Existe alteração da movimentação parietal regional na EMF e é independente da intensidade de deposição de tecido fibroso avaliada qualitativamente. O envolvimento não uniforme do VE deve ser levado em conta no planejamento cirúrgico dessa doença.