Association between Left Atrial Deformation and Brain Involvement in Patients with Anderson-Fabry Disease at Diagnosis.

BACKGROUND: Anderson-Fabry disease (AFD) can induce both central nervous system white matter lesions (WMLs) and cardiac abnormalities including left atrial (LA) dysfunction. We sought to evaluate the possible interrelations of LA structure and function impairment with the presence of WMLs in AFD patients. Methods 22 AFD patients and 22 controls, matched for age and sex, underwent an echo-Doppler exam including quantification of peak atrial longitudinal strain (PALS). AFD patients underwent also a 3-T brain magnetic resonance imaging with a visual quantification of WMLs by Fazekas' score (FS) on 3D FLAIR images. Results AFD patients had significantly higher left ventricular (LV) mass index (LVMi) and relative wall thickness, and lower PALS compared to controls. Among AFD patients, 9 showed a FS = 0, and 13 a FS > 1. AFD patients with FS ≥ 1 showed lower PALS (29.4 ± 6.7 vs. 37.2 ± 3.9%, p = 0.003) than those with FS = 0, without difference in LA volume index and LVMi. In AFD patients, FS was inversely related to PALS (r = -0.49, p < 0.0001), even after adjusting for LVMi (r = -0.43, p < 0.05). Conclusions In the absence of significant alterations in LA size, AFD patients had lower PALS compared to controls. The inverse association between PALS and presence of WMLs indicates a possible parallel early involvement of heart and brain.

Practical Impact of New Diastolic Recommendations on Noninvasive Estimation of Left Ventricular Diastolic Function and Filling Pressures.

BACKGROUND: In 2016, an update of the 2009 recommendations for the evaluation of left ventricular (LV) diastolic function (DF) was released by the American Society of Echocardiography and the European Association of Cardiovascular Imaging. The aims of this study were to assess the concordance between the 2016 and 2009 recommendations and to test the impact of the consideration of "myocardial disease" recommended in the 2016 update on the evaluation of diastolic dysfunction (DD) and LV filling pressures in patients with normal and reduced LV ejection fractions referred to a general echocardiography laboratory. METHODS: A total of 1,508 outpatients referred to an echocardiography laboratory during a predefined 5-month period were prospectively enrolled. All patients underwent targeted clinical history and Doppler echocardiographic examination. DD and LV filling pressures were assessed according to 2009 and 2016 recommendations. Concordance was calculated using the κ coefficient and overall proportion of agreement. RESULTS: Overall proportion of agreement between the two recommendations was 64.7% (κ = 0.43). Comparing the 2009 and 2016 recommendations, 47.5% and 36.1% patients, respectively, had DD (P < .0001), and 22.7% and 12.6% had elevated LV filling pressures (P < .0001). This difference remained significant in the setting of patients with normal LV ejection fractions (21.6% vs 10.7%, P < .0001). In the application of the 2016 recommendations, whether or not the presence of "myocardial disease" was considered, the prevalence of indeterminate diastolic function was, respectively, 7.3% versus 13.7%, while patients in whom the DD grade could not be determined were 8.1% versus 14.4% (P < .0001 for all). CONCLUSIONS: Considering the presence of myocardial disease when applying the 2016 recommendations resulted in a lower prevalence of inconclusive diagnosis.

77 Routine outpatients visits during SARS-CoV2 global pandemic.

Aims: The inability to carry office visits was collateral damage caused by the Coronavirus (COVID-19) pandemic. Tele-health is a relatively new, and yet fundamental amid the current crisis, resource to bridge the gap between phisicians and patients. Methods and results: We report our experience with telemedicine and describe the major events occured in our patients. 121 consecutive adult patients with arterial hypertension (F/M: 56/65; mean age: 66.8 years) were enrolled. 33 patients (27%) had also diabetes, 94 (78%) were also affected from dyslipidemia and 11 (9%) had CAD. They all referred to our ambulatory of hypertension, in most of case for several years. Given the impossibility to continue routine outpatient visits during lockdown, they were all phone called by three residents in order to detect their state of health or any events they could have experienced over this period. They were all asked about their own blood pressure values, the occurrence of new symptoms and of new-onset both cardiovascular and non cardiovascular events. We also followed a self-made preset form. 31 of them (26%) experienced cardiovascular symptoms/events during this period: 11 had hypertensive peaks, in one case associated with nausea and vomiting while 2 of them had hypotensive episodes; 10 had typical angina and/or dyspnoea while 4 had atypical angina; 6 had palpitations; 1 of them developed new onset atrial fibrillation resolved with pharmacologic cardioversion during hospitalization; 1 had syncope; 1 patient reported new onset peripheral oedema; 2 patients died during lockdown for non cardiovascular causes. 17 of them also developed non cardiovascular symptoms, 7 of whom were severe anxiety and/or panic attacks. Almost all patients had important lifestyle changes, in 15 cases (12.3%) associated with weight increase. Conclusion: The impossibility to access to routine outpatient visits during lockdown due to global pandemic of SARS-CoV2, has brought out the risk of underestimating consequences of chronic disease, in absence of appropriate Follow-up. Nevertheless, the two deaths we report were not related to cardiovascular disease. The risk is that both the missing of cardiovascular control visit and the extension of the waiting list, could provoke serious complications in patients suffering from chronic cardiovascular disease.

Strain-oriented strategy for guiding cardioprotection initiation of breast cancer patients experiencing cardiac dysfunction.

AIMS: This study assessed the impact of the strain-guided therapeutic approach on cancer therapy-related cardiac dysfunction (CTRCD) and rate of cancer therapy (CT) interruption in breast cancer. METHODS AND RESULTS: We enrolled 116 consecutive female patients with HER2-positive breast cancer undergoing a standard protocol by EC (epirubicine + cyclophosphamide) followed by paclitaxel + trastuzumab (TRZ). Coronary artery, valvular and congenital heart disease, heart failure, primary cardiomyopathies, permanent or persistent atrial fibrillation, and inadequate echo-imaging were exclusion criteria. Patients underwent an echo-Doppler exam with determination of ejection fraction (EF) and global longitudinal strain (GLS) at baseline and every 3 months during CT. All patients developing subclinical (GLS drop >15%) or overt CTRCD (EF reduction <50%) initiated cardiac treatment (ramipril+ carvedilol). In the 99.1% (115/116) of patients successfully completing CT, GLS and EF were significantly reduced and E/e' ratio increased at therapy completion. Combined subclinical and overt CTRCD was diagnosed in 27 patients (23.3%), 8 at the end of EC and 19 during TRZ courses. Of these, 4 (3.4%) developed subsequent overt CTRCD and interrupted CT. By cardiac treatment, complete EF recovery was observed in two of these patients and partial recovery in one. These patients with EF recovery re-started and successfully completed CT. The remaining patient, not showing EF increase, permanently stopped CT. The other 23 patients with subclinical CTRCD continued and completed CT. CONCLUSION: These findings highlight the usefulness of 'strain oriented' approach in reducing the rate of overt CTRCD and CT interruption by a timely cardioprotective treatment initiation.

Sex-specific echocardiographic reference values: the women's point of view.

: Clinical presentation, diagnosis and outcomes of cardiac diseases are influenced by the activity of sex steroid hormones. These hormonal differences explain the later development of heart diseases in women in comparison with men and the different clinical picture, management and prognosis. Echocardiography is a noninvasive and easily available technique for the analysis of cardiac structure and function. The aim of the present review is to underline the most important echocardiographic differences between sexes. Several echocardiographic studies have found differences in healthy populations between women and men. Sex-specific difference of some of these parameters, such as left ventricular (LV) linear dimensions and left atrial volume, can be explained on the grounds of smaller body size of women, but other parameters (LV volumes, stroke volume and ejection fraction, right ventricular size and systolic function) are specifically lower in women, even after adjusting for body size and age. Sex-specific differences of standard Doppler and Tissue Doppler diastolic indices remain controversial, but it is likely for aging to affect LV diastolic function more in women than in men. Global longitudinal strain appears to be higher in women during the childbearing age - a finding that also highlights a possible hormonal influence in women. All these findings have practical implications, and sex-specific reference values are necessary for the majority of echocardiographic parameters in order to distinguish normalcy from disease. Careful attention on specific cut-off points in women could avoid misinterpretation, inappropriate management and delayed treatment of cardiac diseases such as valvular disease and heart failure.