Diagnostic pathways to wild-type transthyretin amyloid cardiomyopathy: a multicentre network study.

AIM: Epidemiology of wild-type transthyretin cardiac amyloidosis (ATTRwt-CA) remains poorly defined. A better characterization of pathways leading to ATTRwt-CA diagnosis is of key importance, and potentially informative of disease course and prognosis. The aim of this study was to describe the characteristics of contemporary pathways leading to ATTRwt-CA diagnosis, and their potential association with survival. METHODS AND RESULTS: This was a retrospective study of patients diagnosed with ATTRwt-CA at 17 Italian referral centres for CA. Patients were categorized into different 'pathways' according to the medical reason that triggered the diagnosis of ATTRwt-CA (hypertrophic cardiomyopathy [HCM] pathway, heart failure [HF] pathway, incidental imaging or incidental clinical pathway). Prognosis was investigated with all-cause mortality as endpoint. Overall, 1281 ATTRwt-CA patients were included in the study. The diagnostic pathway leading to ATTRwt-CA diagnosis was HCM in 7% of patients, HF in 51%, incidental imaging in 23%, incidental clinical in 19%. Patients in the HF pathway, as compared to the others, were older and had a greater prevalence of New York Heart Association (NYHA) class III-IV and chronic kidney disease. Survival was significantly worse in the HF versus other pathways, but similar among the three others. In multivariate model, older age at diagnosis, NYHA class III-IV and some comorbidities but not the HF pathway were independently associated with worse survival. CONCLUSIONS: Half of contemporary ATTRwt-CA diagnoses occur in a HF setting. These patients had worse clinical profile and outcome than those diagnosed either due to suspected HCM or incidentally, although prognosis remained primarily related to age, NYHA functional class and comorbidities rather than the diagnostic pathway itself.

Left ventricular assist device in cardiac amyloidosis: friend or foe?

The prevalence of cardiac amyloidosis has progressively increased over the last years, being recognized as a significant cause of heart failure. In fact, the management of advanced heart failure is a cornerstone treatment of amyloid cardiomyopathy due to the frequent delay in its diagnosis. Left ventricular assist devices (LVADs) have been gaining importance in the scenario of end-stage heart failure, representing an alternative to heart transplant. However, only few studies have investigated the role of LVAD in restrictive cardiomyopathies such as cardiac amyloidosis, since there are several problems to consider. In fact, both anatomical factors and the restrictive physiology of this condition make LVAD implant a relevant challenge in this subset of patients. Furthermore, due to the systemic involvement of amyloidosis, several factors have to be considered after LVAD implant, such as an increased risk of bleeding and right ventricular failure. This review attempts to summarize the current evidence of LVAD in cardiac amyloidosis, especially focusing on the challenges that this cardiomyopathy imposes both to the implant and to its management thereafter.

The progression rate of aortic stenosis: key to tailoring the management and potential target for treatment.

Aortic stenosis is the most frequent valvular disease to require intervention in the western world and has always been featured as a progressive disease. The rate of progression can be assessed by carefully performed Doppler echocardiography and can vary greatly between individuals with a profound impact on prognosis. Unfortunately, the determinants of disease progression had been insufficiently studied and remain challenging to define, particularly in the outpatient setting. Multiple factors have been proposed and tested, but at present, there are no proven therapies to slow the course of the stenotic process. Heart valve clinics may be particularly important to define the progression rate and tailor follow-up and management at an individual level. This review enlightens knowledge and gaps regarding the progression-rate of aortic valve stenosis, from the historical perspective to the molecular one.

Identification of cardiac organ damage in arterial hypertension: insights by echocardiography for a comprehensive assessment.

: Arterial hypertension, a widespread disease, whose prevalence increases with age, represents a major risk factor for cardiovascular events, causing damage in several organs, including the heart. In this context, echocardiography has a clear and pivotal role, being able to assess cardiac morphology and detect haemodynamic changes induced by this disease. 2018 European Society of Cardiology/European Society of Hypertension guidelines on AH identified main echo parameters such as left ventricular mass, relative wall thickness and left atrial volume, for detecting cardiac organ damage. The present review highlights the advantage of additional echocardiographic parameters such as diastolic measurement and both thoracic and abdominal aortic dimensions. An overlook on aortic valve should also be suggested to detect aortic regurgitation and stenosis, both frequent complications in hypertensive patients. In this kind of comprehensive assessment, the combination of standard and advanced echocardiography (speckle tracking echocardiography and, with a lesser extent, three-dimensional echocardiography) could be considered to improve the diagnostic accuracy, stratify prognosis and address management in arterial hypertension.

The role of the left atrial function in the surgical management of aortic and mitral valve disease.

The right management of both mitral and aortic disease can be challenging, especially in asymptomatic patients. The current guidelines recommend valve repair or replacement when symptoms arise or when there is an evident left ventricular dysfunction. However, deciding the optimal surgical timing can be very difficult, since the line between the absence of symptoms and being minimally symptomatic, especially in the elderly, is blurred. Another relevant issue regards the second surgical criterion: operating on a patient with a reduced left ventricular ejection fraction or with a dilated left ventricle might jeopardize the possibility of a fully reverse remodeling of the heart after surgery. In this scenario, the left atrium might play an important role. In particular, left atrial deformation might be a very useful tool to detect early ultrastructural alterations, and help or support guiding a patient-tailored treatment at an early stage, optimizing the outcome in the long term.

Prognosis and Risk Stratification of Patients With Advanced Heart Failure (from PROBE).

In recent years, many prognostic scores have been developed for advanced chronic heart failure (CHF), but none of them is comprised of first- and second level echocardiographic indexes. The aim was to create a new prognostic echocardiographic score for patients with advanced CHF. Patients with advanced CHF were analyzed by standard, 3D, and speckle tracking echocardiography and followed prospectively for 2 ± 0.7 years recording major adverse cardiac events (MACE): cardiovascular death, hospitalization for HF, emergency heart transplantation, and left ventricular assist device or intra-aortic balloon pump implantation. A total of 110 patients were enrolled. The best predictors of MACE were selected on the basis of area under the curve by receiver operating characteristic analysis >0.70: left atrial volume index (no MACE vs MACE groups, 51.3 ± 20 ml/m2 vs 67 ± 20 ml/m2, p = 0.0003), right ventricular sphericity index (0.53 ± 0.09 vs 0.61 ± 0.10, p = 0.0002), right ventricular fractional area change (41 ± 9% vs 33 ± 9.5, p <0.0001), free-wall right ventricular longitudinal strain (-20 ± 4.5% vs -16 ± 6%, p = 0.0013). A prognostic score formula was calculated as: PROBE score = 1(if left atrial volume index >65 ml/m2) + 1(if right ventricular sphericity index >0.53) + 0.5(if right ventricular fractional area change <36.5%) + 1(if free-wall right ventricular longitudinal strain >-14%). It presented an area under the curve by receiver operating characteristic analysis of 0.90 and classified patients at low (PROBE ≤1), intermediate (PROBE = 1 to 2), or high (PROBE >2) risk of MACE. The Kaplan-Meier analysis revealed a strong correlation between the event-free survival rate and the 3 groups. In conclusion, the PROBE score, with first- and second level echocardiographic parameters, demonstrated a good predictive value for MACE. It represents a useful tool for a noninvasive, individualized, and accurate evaluation and stratification of prognosis in patients with advanced CHF.

The analysis of left atrial function predicts the severity of functional impairment in chronic heart failure: The FLASH multicenter study.

BACKGROUND: Heart failure (HF) patients present with a variety of symptoms at different stages of the disease, but the underlying pathophysiology still is unclear. Left atrial (LA) function might be tightly related to changes in patients' symptoms, more than morphological and anatomic heart features, measurable by ultrasound imaging technique. This study sought to investigate the correlation between LA function, assessed by Speckle Tracking Echocardiography (STE) and Quality of Life (QoL), assessed by the Minnesota Living with Heart Failure Questionnaire (MLHFQ), in patients with chronic HF. METHODS: Clinically stable HF outpatients (n = 369) were enrolled from 7 different international centres and underwent echocardiographic studies. Patients >75 years old and with atrial fibrillation were excluded. LA strain during reservoir phase (LASr) by STE was measured in all subjects by averaging the 6 atrial segments. LA size was assessed using biplane volume and 4-chamber area acquisition. RESULTS: LASr strongly correlated with both MLHFQ total score (r = -0.87; p < 0.0001). Less significant correlations between MLHFQ and either LA volume or left ventricular global longitudinal strain (LV-GLS) were found (r = 0.28; p = 0.05 and r = 0.30; p = 0.01, respectively). No significant correlation was found between MLHFQ score, LVEF (r = -0.15; p = ns), E/E' ratio (r = 0.19; p = ns), and E/A ratio (r = 0.20; p = ns). Among all echocardiographic parameters analyzed, LASr presented the highest diagnostic accuracy (AUC = 0.74) in predicting a poor QoL (>45), when compared with LV-GLS (AUC = 0.61), LA volume (AUC = 0.54) and E/e' ratio (AUC = 0.51). CONCLUSIONS: In patients with HF, irrespective of etiology, LA function strongly correlates with patients' QoL.

More than 10 years of speckle tracking echocardiography: Still a novel technique or a definite tool for clinical practice?

Speckle tracking echocardiography (STE) cannot be considered a recent technique anymore. Its application has gained growing importance over the last decade in several clinical settings, and the deformation analysis has fully entered in diagnostic algorithms and guidelines of various pathologies. STE allows to track the displacement of "speckles" in two-dimensional (2D) echocardiographic images in an angle-independent way and to assess their movement (strain) during the cardiac cycle. Its high feasibility, reproducibility, and accuracy have been widely demonstrated. In this review, we describe how STE has been applied to different aspects of the daily clinical practice, including ischemic heart diseases, heart valve disease, heart failure, and prognostication, highlighting the possible added value that strain parameters have shown over the years.

Arterial hypertension and atrial fibrillation: standard and advanced echocardiography from diagnosis to prognostication.

: Structural changes in left and right cardiac chambers that occur in arterial hypertension (AH) may lead to an increased risk of atrial fibrillation. Considering that AH is currently the most common cardiovascular disease in the general population, it represents a major risk factor for atrial fibrillation development. This review explores the complex relationship between atrial fibrillation and AH, starting from its pathophysiological basis. It focuses on the role of echocardiography in the management of hypertensive and atrial fibrillation patients, with emphasis on what should be evaluated about left ventricular remodeling, diastolic and systolic function, left atrial (LA) size and function and right ventricular deformation in patients with AH.