Non-cardiac surgery in patients with valvular heart disease.

In patients with significant valvular heart disease (VHD) undergoing non-cardiac surgery (NCS), perioperative adverse cardiac events are a relevant issue. Although postoperative outcomes can be adversely affected by valve-related haemodynamic instability, recommended perioperative risk scores prioritise the risk of the surgical procedure and the presence of cardiovascular risk factors, neglecting the presence or extent of VHD. Perioperative management and anaesthetic approach should focus on the underlying type and severity of VHD, the compensatory mechanisms deployed by left ventricle and right ventricle and the type and risk of NCS. Due to the lack of randomised trials investigating different therapeutic approaches of valvular intervention prior to NCS, recommendations mainly rely on consensus opinion and inference based on large observational registries. As a general rule, valvular intervention is recommended prior to NCS in symptomatic patients or in those who meet standard criteria for cardiac intervention. In the absence of such conditions, it is reasonable to perform NCS with tailored anaesthetic management and close invasive perioperative haemodynamic monitoring. However, patient-specific management strategies should be discussed with the heart team preoperatively. Symptomatic patients with severe VHD or those undergoing high-risk NCS should ideally be treated at a high-volume medical centre that is equipped to manage haemodynamically complex patients during the perioperative period.

Cardiac Imaging in Anderson-Fabry Disease: Past, Present and Future.

Anderson-Fabrydisease is an X-linked lysosomal storage disorder caused by a deficiency in the lysosomal enzyme α-galactosidase A. This results in pathological accumulation of glycosphingolipids in several tissues and multi-organ progressive dysfunction. The typical clinical phenotype of Anderson-Fabry cardiomyopathy is progressive hypertrophic cardiomyopathy associated with rhythm and conduction disturbances. Cardiac imaging plays a key role in the evaluation and management of Anderson-Fabry disease patients. The present review highlights the value and perspectives of standard and advanced cardiovascular imaging in Anderson-Fabry disease.

Cor pulmonale: the role of traditional and advanced echocardiography in the acute and chronic settings.

Cor pulmonale is the condition in which the right ventricle undergoes morphological and/or functional changes due to diseases that affect the lungs, the pulmonary circulation, or the breathing process. Depending on the speed of onset of the pathological condition and subsequent effects on the right ventricle, it is possible to distinguish the acute cor pulmonale from the chronic type of disease. Echocardiography plays a central role in the diagnostic and therapeutic work-up of these patients, because of its non-invasive nature and wide accessibility, providing its greatest usefulness in the acute setting. It also represents a valuable tool for tracking right ventricular function in patients with cor pulmonale, assessing its stability, deterioration, or improvement during follow-up. In fact, not only it provides parameters with prognostic value, but also it can be used to assess the efficacy of treatment. This review attempts to provide the current standards of an echocardiographic evaluation in both acute and chronic cor pulmonale, focusing also on the findings present in the most common pathologies causing this condition.

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.

Atrial Fibrillation, Cancer and Echocardiography.

Nonvalvular atrial fibrillation (AF) is a relatively frequent arrhythmia in cancer patients; it is possibly due to direct effect of cancer or consequence of cancer therapies. AF creates important problems for both therapeutic management and prognosis in cancer patients. The anticoagulation of cancer patients presenting AF is a main issue because of the difficult balance between thromboembolic and bleeding risks, both elevated in this clinical setting. A comprehensive echo Doppler examination is mandatory to identify the eventual sources of emboli in left atrial (LA) cavity, mainly the transesophageal echocardiography (TEE), but also to predict the subsequent development of heart failure. This evaluation is particularly important to graduate anticoagulation and to prevent and manage symptoms/signs of heart failure. The performance of a TEE precardioversion is highly encouraged to detect possible thrombi in LA appendage. A careful assessment of LA size (LA volume index) and function (LA emptying fraction and/or LA strain) should always be planned to predict the possible recurrence of AF paroxysmal episodes. This is in fact a key action, not only from the cardiologic point of view but also for the oncologic perspectives in individual situations. Patients with larger left atrium and more impaired LA function should be addressed toward a less aggressive cancer treatment, with drugs which are not associated or are poorly related with the risk of AF development. A correct and comprehensive echocardiographic assessment could even induce the oncologist to change the cancer management balancing the oncologic and the cardiac risk.

Impaired Right and Left Ventricular Longitudinal Function in Patients with Fibrotic Interstitial Lung Diseases.

Background: Left ventricular (LV) and right ventricular (RV) dysfunction is recognized in idiopathic pulmonary fibrosis (IPF). Little is known about cardiac involvement in non-idiopathic pulmonary fibrosis (no-IPF). This issue can be explored by advanced echocardiography. Methods: Thirty-three clinically stable and therapy-naive fibrotic IPF and 28 no-IPF patients, and 30 healthy controls were enrolled. Exclusion criteria were autoimmune systemic diseases, coronary disease, heart failure, primary cardiomyopathies, chronic obstructive lung diseases, pulmonary embolism, primary pulmonary hypertension. Lung damage was evaluated by diffusion capacity for carbon monoxide (DLCOsb). All participants underwent an echo-Doppler exam including 2D global longitudinal strain (GLS) of both ventricles and 3D echocardiographic RV ejection fraction (RVEF). Results: We observed LV diastolic dysfunction in IPF and no-IPF, and LV GLS but not LV EF reduction only in IPF. RV diastolic and RV GLS abnormalities were observed in IPF versus both controls and no-IPF. RV EF did not differ significantly between IPF and no-IPF. DLCOsb and RV GLS were associated in the pooled pulmonary fibrosis population and in the IPF subgroup (ß = 0.708, p < 0.001), independently of confounders including pulmonary arterial systolic pressure. Conclusion: Our data highlight the unique diagnostic capabilities of GLS in distinguishing early cardiac damage of IPF from no-IPF patients.

Prominent basal and middle strain longitudinal involvement in newly-diagnosed and never treated hypertensive patients without clear-cut hypertrophy.

BACKGROUND: Left ventricular (LV) global longitudinal strain (GLS) can detect an early dysfunction in arterial hypertension. We investigated regional LV patterns of longitudinal strain (LS) and base-to-apex behaviour in newly diagnosed, never-treated hypertensive patients (HTN) without LV hypertrophy. METHODS: 180 HTN and 115 healthy controls underwent standard echocardiography, including regional LS and GLS assessment (in absolute values). The average LS of six basal (BLS), six middle (MLS), and six apical (ALS) segments and relative regional strain ratio = [ALS/(BLS + MLS)] were also computed. RESULTS: The two groups were comparable for sex, age and heart rate. Body mass index (BMI), systolic, diastolic and mean blood pressure (BP) (all p < 0.0001) were higher in HTN. Despite LV ejection fraction (EF) was comparable, GLS, BLS and MLS resulted lower in HTN (all p < 0.0001), without difference in ALS. Relative regional strain ratio resulted higher in HTN (p < 0.001). Dividing HTN group according to lower normal values derived from the controls, BLS was able to identify a higher rate of LV dysfunction than GLS. By a multiple linear regression analysis performed in the pooled population after adjusting for age, sex, BMI, end-systolic stress, relative wall thickness and LV mass index, the association between BLS and mean BP remained significant (ß coefficient = -0.42, p < 0.0001), despite the significant impact of male sex. In a similar model, MLS and mean BP resulted also independently associated (ß = -0.21, p < 0.002). CONCLUSIONS: Despite normal LV EF, LS dysfunction is detectable in HTN, mainly involving basal and middle segments, resulting in higher relative regional strain ratio.

Basic and advanced echocardiography in advanced heart failure: an overview.

Advanced chronic heart failure (ACHF) is the last phase in the evolution of heart failure and is characterized by high hospitalization and mortality rates and is refractory to medical therapy, therefore requiring more aggressive therapies, such as mechanical circulatory support or heart transplantation. Over the last years, the incidence of ACHF was continuously growing, together with the increase in population survival rates. Therefore, the early recognition of the transition to ACHF is of crucial importance in HF patients, which also helps in prognostication of such patients, since advanced therapeutic options are limited to selected patients and they also have some important risk implications. Echocardiography is the gold standard tool for the evaluation of patients with HF; moreover, the recent technological advances provided new structural and functional indices of the four cardiac chambers that showed to be comparable to advanced imaging or invasive hemodynamic parameters. This allows us to operate an accurate study of ACHF with first- and second-level echocardiographic techniques, which are now being integrated in daily clinical practice. The present review presents an overview of the currently available tools for the echocardiographic examination of patients with ACHF, with its advantages and limitations, based on the latest supporting evidences.

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.

Interrelation between midwall mechanics and longitudinal strain in newly diagnosed and never-treated hypertensive patients without clinically defined hypertrophy.

BACKGROUND: In hypertensive patients, an impairment of midwall myocardial mechanics was described in presence of left ventricular (LV) concentric geometry. Under these circumstances, also LV longitudinal dysfunction was found. PURPOSE: Our aim was to evaluate longitudinal and circumferential systolic function and correlations between these two functional components in newly diagnosed hypertensive patients without clinically defined LV hypertrophy (LVH). One hundred and thirty-eight newly diagnosed, never-treated hypertensive patients without LVH and a control group of 105 healthy normotensive individuals underwent two-dimensional and speckle tracking echocardiography. Global longitudinal strain (GLS) was derived (in absolute value) and midwall fractional shortening (MFS) computed. In addition, the hypertensive population was divided into two groups according to GLS: normal GLS (≥20%, n = 94) and reduced GLS (<20%, n = 44). RESULTS: Hypertensive patients had lower MFS (P < 0.001) and GLS (P < 0.0001) than healthy controls. By dividing hypertensive patients according to GLS thresholds of normalcy, MFS was lower in patients with GLS less than 20% (P < 0.0001) while no significant difference was found in LV geometry, ejection fraction and diastolic parameters in comparison with patients with GLS at least 20%. In the pooled hypertensive population, GLS resulted positively related to MFS (r = 0.33, P < 0.0001). By a multiple linear regression analysis, after adjusting for female sex, age, BMI, circumferential end-systolic stress, average e', ejection fraction and relative wall thickness, MFS remained independently associated with GLS (ß = 0.222, P < 0.005). CONCLUSION: In newly diagnosed and never-treated hypertensive patients without LVH, an early LV systolic dysfunction is testified by the reduction of both MFS and GLS. These two parameters resulted independently associated after adjusting for several confounders.

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.

[Stress echocardiography of the righ heart-pulmonary circulation unit: rationale and possibilities of application in clinical practice]. / L'eco-stress dell'unità cuore destro-circolo polmonare: razionale e possibilità applicative nella pratica clinica.

The right heart has been traditionally considered a passive conduit between the venous system and the lungs. Today, we know that a close hemodynamic interdependence exists between right ventricle, pulmonary circulation, and left ventricle, and that the right ventricle plays a key role in the ability to adapt to many super-physiological and pathological conditions. Dynamic exercise is a considerable stress for the "right heart-pulmonary circulation unit", which responds, in healthy subjects, with an increase of right ventricular contractility and cardiac output, a decrease of pulmonary vascular resistance, and a non-significant rise in pulmonary pressures. In patients affected by cardiac and/or pulmonary diseases (e.g. ischemic heart disease, heart failure, severe valvular stenosis or regurgitation, systemic sclerosis, chronic obstructive pulmonary disease, etc.) right ventricular contractile reserve may be impaired, and pulmonary artery systolic pressure may abnormally increase during exercise. From this perspective, stress echocardiography is a primary tool to evaluate right ventricle and pulmonary circulation during exercise, and can be useful in setting diagnosis, prognosis, and therapeutic timing of many cardiopulmonary diseases.

Cardiopulmonary exercise testing and echocardiographic exam: an useful interaction.

Cardiopulmonary exercise test (CPET) is a functional assessment that helps to detect disorders affecting the system involved in oxygen transport and utilization through the analysis of the gas exchange during exercise. The clinical application of CPET is various, it including training prescription, evaluation of treatment efficacy and outcome prediction in a broad spectrum of conditions. Furthermore, in patients with shortness of breath it provides pivotal information to bring out an accurate differential diagnosis between physical deconditioning, cardiopulmonary disease and muscular diseases. Modern software allows the breath-by-breath analysis of the volume of oxygen intake (VO2), volume of carbon dioxide output (VCO2) and expired air (VE). Through this analysis, CPET provides a series of additional parameters (peak VO2, ventilatory threshold, VE/VCO2 slope, end-tidal carbon dioxide exhaled) that characterize different patterns, helping in diagnosis process. Limitations to the routine use of CPET are mainly represented from the lack of measurement standardization and limited data from randomized multicentric studies. The integration of CPET with exercise stress echocardiography has been recently introduced in the clinical practice by integrating the diagnostic power offered by both the tools. This combined approach has been demonstrated to be valuable for diagnosing several cardiac diseases, including heart failure with preserved or reduced ejection fraction, cardiomyopathies, pulmonary arterial hypertension, valvular heart disease and coronary artery disease. Future investigations are needed to further promote this intriguing combination in the clinical and research setting.

Global longitudinal strain is a hallmark of cardiac damage in mitral regurgitation: the Italian arm of the European Registry of mitral regurgitation (EuMiClip).

BACKGROUND: The search for reliable cardiac functional parameters is crucial in patients with mitral regurgitation (MR). In the Italian arm of the European Registry of MR, we compared the ability of global longitudinal strain (GLS) and left ventricular (LV) ejection fraction (LVEF) to detect cardiac damage in MR. METHODS: Five hundred four consecutive patients with MR underwent a complete echo-Doppler exam. A total of 431, 53 and 20 patients had degenerative, secondary and mixed MR, respectively. The main echocardiographic parameters, including LV and left atrial (LA) size measurements, pulmonary artery systolic pressure (PASP) and GLS were compared between patients with mild MR (n = 392) vs. moderate to severe MR (n = 112). RESULTS: LVEF and GLS were related one another in the pooled population, and separately in patients with mild and moderate/severe MR (all p < 0.0001). However, a certain number of patients were above the upper or below the lower limits of the 95% confidence interval (CI) of the normal relation in the pooled population and in patients with mild MR. Only 2 patients were below the 95% CI in moderate to severe MR. After adjusting for confounders by separate multivariate models, LVEF and GLS were independently associated with LV and left atrial size in the pooled population and in mild and moderate/severe MR. GLS, but not LVEF, was also independently associated with PASP in patients with mild and moderate to severe MR. CONCLUSIONS: Both LVEF and GLS are independently associated with LV and LA size, but only GLS is related to pulmonary arterial pressure. GLS is a powerful hallmark of cardiac damage in MR.

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.

Impact of left ventricular mass/end-diastolic volume ratio by three-dimensional echocardiography on two-dimensional global longitudinal strain and diastolic function in native hypertensive patients.

BACKGROUND: In hypertensive patients, high left ventricular (LV) mass/end-diastolic volume ratio (LVM/EDV) is related to LV dysfunction and myocardial fibrosis. PURPOSE: We examined the ability of 3D-echo-derived LVM/EDV ratio in identifying early systolic and diastolic dysfunction in relation with LV concentric geometry in native hypertensive patients. METHODS: One-hundred and forty-four newly diagnosed, never treated hypertensive patients underwent 2D-echo, including computation of 2D-derived global longitudinal strain (GLS), and 3D-echo. The study population was divided into two groups: elevated 3D-LVM/EDV (≥1.23 in women and ≥1.22 in men), corresponding to LV concentric geometry (n = 50), and normal ratio (<1.23 in women and <1.22 in men) corresponding to LV normal or eccentric geometry (n = 94). RESULTS: The two groups were comparable for sex, heart rate, BMI, and blood pressure (BP). Patients with elevated 3D-LVM/EDV ratio were older and had lower GLS (P < 0.001) than patients with normal LVM/EDV ratio. Transmitral E/A ratio (P < 0.0001) and e' velocity (P < 0.0001) were lower, and E/e' ratio (P < 0.0001) higher in patients with elevated LVM/EDV ratio. In the pooled population, LVM/EDV ratio was positively correlated to E/e' (r = 0.39, P < 0.0001) and negatively to GLS (r = -0.29, P < 0.001). By separate multilinear regression analyses, after adjusting for sex, age, heart rate, mean BP and BMI, LVM/EDV ratio - but not 2D-relative wall thickness - was independently associated with E/e' (ß = 0.304, P = 0.003) and GLS (ß = -0.501, P < 0.0001). CONCLUSION: Three-dimensional echocardiographic assessment of LV concentric geometry allows identifying an early diastolic and longitudinal systolic dysfunction in native hypertensive patients. In particular, 3D-LVM/EDV ratio is independently associated with both E/e' ratio and GLS.

Layer-specific longitudinal strain in Anderson-Fabry disease at diagnosis: A speckle tracking echocardiography analysis.

BACKGROUND: Speckle tracking advancements make now available the analysis of layer-specific myocardial deformation. This study investigated multilayer longitudinal strain in Anderson-Fabry disease (AFD) patients at diagnosis. METHODS: In a case-control study, 33 newly diagnosed, untreated AFD patients and 33 healthy age- and sex-matched healthy controls underwent a complete echocardiogram, including assessment of left ventricular (LV) transmural global longitudinal strain (GLS), subendocardial longitudinal strain (LSsubendo), subepicardial longitudinal strain (LSsubepi), and strain gradient (LSsubendo-LSsubpepi). RESULTS: Anderson-Fabry disease patients had similar blood pressure, heart rate, and ejection fraction but higher body mass index in comparison with controls. LV mass index, maximal, and relative wall thickness were significantly greater in AFD patients. LSsubendo was significantly higher than LSsubepi in both groups, but GLS (P < 0.0001), LSsubendo (P = 0.003), and particularly LSsubepi (21.4 ± 1.7 vs 18.8 ± 1.4%, P < 0.0001) were lower in AFD patients than in controls. Accordingly, LS gradient was higher in AFD patients (P = 0.003). Three patients symptomatic for dyspnoea presented a combination of LV hypertrophy and reduced LSsubepi. After adjusting for confounders by multivariate analyses, LV mass index or maximal wall thickness were independently and inversely associated with transmural GLS and LSsubepi, but not with LSsubendo in the AFD group. At receiver operating curve curves, LSsubepi best discriminated AFD and normals. CONCLUSIONS: In newly diagnosed, untreated AFD patients, layer-specific strain imaging highlights an impairment of LV longitudinal deformation, mainly involving subepicardial strain and causing increase in longitudinal strain myocardial gradient. These findings could be useful for identifying the mechanisms underlying early LV dysfunction in AFD patients.

Right ventricular function after cardiac surgery: the diagnostic and prognostic role of echocardiography.

Cardiac surgical techniques and circulatory supports have strongly evolved in the last years. Right ventricular (RV) function during the post-operatory period is still subject of study, although its relevant prognostic impact has been variably described in different papers. RV post-surgical dysfunction's underlying mechanisms are still not clear and include a different hypothesis. Echocardiography, with both first and second level parameters, offers the possibility to accurately analyze the right ventricle and optimize these patients' management. This paper describes the pathophysiology of the right ventricle, the most used echo indexes of RV function, whether they alter after surgery, the different supposed mechanisms of RV dysfunction and its role in the prognosis of patients undergoing cardiac surgery.

Prominent longitudinal strain reduction of left ventricular basal segments in treatment-naïve Anderson-Fabry disease patients.

AIMS: Little is known about regional longitudinal strain (LS) distribution in early stages of Anderson-Fabry disease (AFD) cardiomyopathy. We investigated regional left ventricular (LV) patterns of LS strain and base-to-apex behaviour of LS in treatment-naïve AFD patients. METHODS AND RESULTS: Twenty-three consecutive AFD patients at diagnosis and 23 healthy controls without cardiovascular risk factors and matched for age and sex to the patients, underwent a comprehensive evaluation of target organs. An echo-Doppler exam, including determination of regional and global LS strain (GLS) was obtained. The average LS of 6 basal (BLS), 6 middle (MLS), and 5 apical (ALS) segments and relative regional strain ratio [ALS/(BLS + MLS)] were also calculated. Ejection fraction and diastolic indices did not differ between the two groups. LV mass index was greater in AFD (P < 0.01). GLS (P = 0.006), BLS (P < 0.0001), and MLS (P = 0.003), but not ALS, were lower in AFD patients and relative regional strain ratio was higher in AFD (P < 0.01) than in controls. These analyses were confirmed separately in the two genders and even after excluding patients with wall hypertrophy. By subdividing AFD patients according to lysoGB3 levels, 9 patients with lysoGB3 ≥ 1.8 ng/L had lower ALS compared to 11 patients with lysoGB3 < 1.8 ng/L (P < 0.01). CONCLUSION: In naïve AFD patients, we observed an early reduction of LV LS, involving mainly LV basal myocardial segments. Nevertheless, the association found between the higher lysoGB3 levels and the lower apical cap LS demonstrates that apical segments LS, despite still normal, is not spared at diagnosis.