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BackgroundCardiac involvement is frequent in systemic light chain amyloidosis (AL). Left-sided valvular thickening (LVT) have been described in AL reflecting heavy infiltration of the valvular endocardium by amyloid proteins. However, the exact prevalence at diagnosis and the prognostic significance of LVT in AL patients have never been investigated.Aimsto study the prevalence and the impact on long-term survival LVT in AL patients.Methods and resultsBetween 1998 and 2013, 150 AL patients were included at diagnosis (mean age was 68±11 ans, 59% were male). A comprehensive transthoracic echocardiography was performed at baseline. The presence of LVT was assessed visually and was found in 42% of patients. Compared to patients without LVT, those with LVT have more frequently advanced NYHA functional class. They also had significantly higher left ventricular (LV) wall thickening (p=0.01), LV mass (p=0.02), and mitral E/e’ (p=0.0009) and larger left atrial size (p

IntroductionThe non-invasive assessment of left ventricular (LV) filling and LA pressure in patients with aortic stenosis (AS) remains challenging. The aim of our study was to identify the echocardiographic parameters that may predict elevated pulmonary capillary wedge pressures (PCWP) measured by cardiac catheterization in patients with severe AS without other valve disease.Methods and resultsFrom January 2010 to December 2012, we included 85 consecutive patients with severe AS scheduled for clinically indicated cardiac catheterization study. Comprehensive transthoracic echocardiography (TTE) was performed in all patients within 24 hours of the hemodynamic study. Mean age was 75±9 years, 65% of them were male, 65%, 22% and 54% had respectively a history of hypertension, diabetes, and dyslipidemia. NYHA functional class was≥III in 63% of patients. By TTE, mean LV ejection fraction, max left atrial (LA) volume indexed, were respectively 60±9%, and 38±16mL/m 2. Mean mitral septal E/e’ ratio was 18.6±9. Cardiac catheterization found 60% cases of coronary artery disease and the mean PCWP was 13.5±7mmHg. As compared to patients with low PCWP (29ml/m 2 predicted a PCWP>13mmhg with a sensitivity of 77% and a specificity of 62% (area under the curve=0.73). Similarly, mitral annular septal E/e’>12 predicted PCP>13mmHg with a sensitivity of 90% and a specificity of 60% (area under the curve=0.73).ConclusionIn severe AS patients, maximal LA indexed volume>29/ml/m2 and E/e’ ratio>12, derived from TTE, appear as good markers of elevated PCWP. Further studies are needed to investigate their prognostic values.

BackgroundPrimary systemic amyloidosis (AL) is a severe plasma cell disorder characterized by amyloid fibrils extracellular deposition in different organs. Myocardial involvement is frequent and has major impact on prognosis. Echocardiography (TTE) is the most common test performed when cardiac involvement is suspected. We hypothesized that a simple measurement of left atrium enlargement (LA) by TTE may provide an important risk marker for this disease.Methods and resultsBetween 1997 and 2010, 109 patients were diagnosed with systemic AL and had first TTE within 21 days. Patients were mainly treated with conventional chemotherapy (M-Dex) with new agents for refractory or relapsing patients We retrospectively collected demographic baseline characteristics along with biological and echo data of these patients. Mean age was 63±11 years; 58% were male; 24% had hypertension. Mean left ventricular ejection fraction and mean LV wall thickness were respectively 62±13% and 13±3mm. Mean follow up time was 2.42±2 years. None had significant valvular heart disease. LA enlargement was defined by M mode as > 40mm in male and > 36mm in female. Patients with enlarged LA were more often male, slightly older (p=0.05) and with slightly more hypertension (p=0.07) but had significantly lower ejection fraction and more hypertrophied LV walls (All P