Left atrial function in patients with light chain amyloidosis: A transthoracic 3D speckle tracking imaging study.

BACKGROUND: Systemic light chain amyloidosis (AL) is characterized by the extracellular deposition of amyloid fibrils. Transthoracic echocardiography is the modality of choice to assess cardiac function in patients with AL. Whereas left ventricular (LV) function has been well studied in this patient population, data regarding the value of left atrial (LA) function in AL patients are lacking. In this study, we aim to examine the impact of LA volumes and function on survival in AL patients as assessed by real-time 3D echocardiography. METHODS: A total of 77 patients (67±10 years, 60% men) with confirmed AL and 39 healthy controls were included. All standard 2D echocardiographic and 3D-LA parameters were obtained. RESULTS: Fourteen patients (18%) were in Mayo Clinic (MC) stage I, 30 (39%) in stage II, and 33 (43%) in stage III at initial evaluation. There was no significant difference among the MC stages groups in terms of age, gender, or cardiovascular risk factors. As compared to patients in MC II and MC I, those in MC III had significantly larger indexed 3D-LA volumes (MCIII: 46±15mL/m2, MC II: 38±12mL/m2, and MC I: 23±9mL/m2, p<0.0001), lower 3D-LA total emptying fraction (3D-tLAEF) (21±13% vs. 31±15% vs. 43±7%, respectively, p<0.0001), and worse 3D peak atrial longitudinal strain (3D-PALS) (11±9% vs. 18±13% vs. 20±7%, respectively, p=0.007). Two-year survival was significantly lower in patients with 3D-tLAEF <+34% (p=0.003) and in those with 3D-PALS <+14% (p=0.034). Both parameters provided incremental prognostic value over maximal LA volume in multivariate analysis. CONCLUSION: Functional LA parameters are progressively altered in AL patients according to the MC stage. A decrease in 3D-PALS is associated with worse outcome, independently of LA volume.

Impact of Pulmonary Hypertension on Outcome in Patients with Severe Aortic Stenosis and Preserved Left Ventricular Ejection Fraction.

AIMS: The prognostic impact of elevated pulmonary arterial pressure (PAP) remains controversial in aortic stenosis (AS) and few studies focused on patients with preserved left ventricular ejection fraction (LVEF). We aimed to investigate the impact of pulmonary hypertension (PH), invasively derived, on survival in severe AS with preserved LVEF. METHODS AND RESULTS: Between 2000 and 2010, 749 patients (74 ± 8 years, 57% males) with preserved LVEF and severe AS without other valvular heart disease underwent cardiac catheterization. PH was defined as mean PAP > 25 mmHg. The mean follow-up was 4.6 ± 3.0 years. Overall, 32% (n = 241) of patients had PH. Surgical aortic valve replacement (SAVR) was performed in 91% of patients with 4.5% of 30-day mortality rate, significantly higher in patients with PH than without PH (7.5 vs. 3.5%, p = 0.014). In logistic regression, PH was an independent predictor of increased 30-day mortality (odds-ratio = 2.2, p = 0.04). Overall long-term survival was significantly reduced in patients with PH as compared to those without (10-year: 52 ± 5 vs. 68 ± 3%, p < 0.0001). Likewise, focusing on patients with SAVR showed significant reduced survival in those with PH (10-year: 57 ± 5 vs. 72 ± 3%, p < 0.0001). In multivariate analysis, after adjustment for relevant cofactors, PH was an independent predictor of mortality (hazard ratio = 1.5, p = 0.009). Using quartiles of mean PAP, only patients with most elevated values (Q4: mean PAP: 27-67mmHg) had significantly reduced survival, as compared to other quartiles (all p < 0.0001). CONCLUSION: In patients with severe AS and preserved LVEF, PH is an independent predictor of 30-day and long-term mortality patients. Nevertheless, only severely elevated PAP seems associated with reduced survival.

Prevalence and prognostic impact of left-sided valve thickening in systemic light-chain amyloidosis.

BACKGROUND: Left heart valve thickening (LVT) was described in patients with light-chain amyloidosis (AL). This phenomenon reflects likely infiltration of the valve by amyloid proteins. However, the prevalence of LVT and its prognostic value have not been investigated in patients with AL. METHODS AND RESULTS: Comprehensive transthoracic echocardiography was performed at baseline in 150 patients [median age 68 (33-87) years; 59% male] with confirmed AL. The presence of abnormal mitral and/or aortic valve thickening (>3 mm) was assessed in all included patients. Overall, 42% had LVT at the time of diagnosis. Compared to patients without LVT, those with LVT were older and had a more advanced NYHA functional class (63% in patients with NYHA III-IV vs. 33% in NYHA I-II, p < 0.001). They also had higher left ventricular (LV) wall thickness and mass, larger left atrium, higher mitral annulus E/E' ratio and systolic pulmonary artery pressures, and lower LV ejection fraction (all p < 0.05). Patients with more advanced Mayo Clinic stage had a higher incidence of LVT: 58% in stage III vs. 45% in stage II and 5% in stage I (p < 0.001). During a median follow-up of 2 years, 79 deaths occurred. The presence of LVT was significantly associated with reduced 5-year survival (32 ± 7 vs. 64 ± 6%). In multivariate analysis, after adjusting for age, gender, NYHA functional class, and LV ejection fraction, LVT remained significantly associated with higher all-cause mortality (hazard ratio 1.90, 95% CI 1.10-3.34, p = 0.02). CONCLUSION: Left heart valve thickening is common in patients with AL and is associated with worse functional class, LV systolic and diastolic function, and more advanced stage of the disease. In addition, LVT appears to be a powerful marker of all-cause mortality.