Paediatric Restrictive Cardiomyopathy - Diagnosis and Challenges: A report of two cases.

Restrictive cardiomyopathy is one of the rarest forms of cardiomyopathies in paediatric patients characterised by impaired myocardial relaxation or compliance with restricted ventricular filling, leading to a reduced diastolic volume with a preserved systolic function. We report 2 cases-a 5-year-old boy who presented with abdominal distension and palpitation with family history of similar complaints but no definite genetic diagnosis as yet and a 5-year-old girl who presented with chronic cough and shortness of breath. Both cases were diagnosed in a tertiary care hospital in Muscat, Oman, in 2019 and are managed supportively with regular outpatient follow-up. This is the first series of reported cases of paediatric restrictive cardiomyopathy from Oman.

Standard and New Echocardio Techniques, Such as Global Longitudinal Strain, to Monitor the Impact of Diets on Cardiovascular Diseases and Heart Function.

"The Seven Countries Study", published in 1984, was the first study to find a correlation between diet and mortality related to cardiovascular diseases (CVDs). Since then, many investigations have addressed the relationship between type of diet, or specific nutrients, and CVDs. Based on these findings, some traditional dietary models, such as the Mediterranean or Nordic diet, are recommended to prevent CVDs. Meanwhile, new diets have been proposed for optimal nutrition therapy, for example, the Dietary Approaches to Stop Hypertension (DASH) and the Mediterranean-DASH Intervention Diet for Neurodegenerative Delay (MIND). The main outcomes evaluated after implementing these dietary models are as follows: CVD-related death; the development of specific CVDs, such as myocardial infarction and hypertension; or biochemical parameters related to CVDs, i.e., non-HDL cholesterol, C-reactive protein (CPR) and homocysteine. However, the early impact of diet on heart functionality is less evaluated. Recently, the echographic measurement of left ventricle (LV) deformation by global longitudinal strain (GLS) has been introduced as a novel marker of clinical and subclinical cardiac dysfunction. This technology allows a subclinical evaluation of heart functionality since, differently from the traditional evaluation of left ventricle ejection fraction (LVEF), it is capable of detecting early myocardial dysfunction. In this review, we analyzed the available studies that correlate dietetic regimens to cardiovascular diseases, focusing on the relevance of LV strain to detect subclinical myocardial alteration related to diet. Evidence is presented that DASH and MIND can have a positive impact on heart functionality and that myocardial strain is useful for early detection of diet-related changes in cardiac function.

CMR provides comparable measurements of diastolic function as echocardiography.

Clinical application of cardiac magnetic resonance (CMR) is expanding but CMR assessment of LV diastolic function is still being validated. The purpose of this study was to validate assessments of left ventricular (LV) diastolic dysfunction (DD) using CMR by comparing with transthoracic echocardiography (TTE) performed on the same day. Patients with suspected or diagnosed cardiomyopathy (n = 63) and healthy volunteers (n = 24) were prospectively recruited and included in the study. CMR diastolic parameters were measured on cine images and velocity-encoded phase contrast cine images and compared with corresponding parameters measured on TTE. A contextual correlation feature tracking method was developed to calculate the mitral annular velocity curve. LV DD was classified by CMR and TTE following 2016 guidelines. Overall DD classification was 78.1% concordant between CMR and TTE (p < 0.0001). The trans-mitral inflow parameters correlated well between the two modalities (E, r = 0.78; A, r = 0.90; E/A, r = 0.82; all p < 0.0001) while the remaining diastolic parameters showed moderate correlation (e', r = 0.64; E/e', r = 0.54; left atrial volume index (LAVi), r = 0.61; all p < 0.0001). Classification of LV diastolic function by CMR showed good concordance with standardized grades established for TTE. CMR-based LV diastolic function may be integrated in routine clinical practice.Name of the registry: Technical Development of Cardiovascular Magnetic Resonance Imaging. Trial registration number: NCT00027170. Date of registration: November 26, 2001. URL of trial registry record: https://clinicaltrials.gov/ct2/show/NCT00027170.

Multimodal preoperative imaging for transcatheter mitral valve replacement in the domestic sheep model.

Preclinical in vivo evaluation is an essential step in the progression of new cardiac devices into patient use, with studies predominantly performed in the domestic sheep model. A growing area of interest in cardiac device development is transcatheter mitral valve replacement (TMVR). Clinically, multimodal imaging, or computed tomography (CT) and echocardiography (echo) are used extensively to preoperatively determine mitral valve morphology prior to an intervention, but there is no description on how these modalities can be implemented to support preclinical studies. The purpose of this study is to apply clinically relevant CT and echo acquisition and assessment techniques to a large group of naive research sheep in order to analyze and report modality-related effects on mitral valve dimensional reference intervals in the sheep model. To this end, fifty-five adult domestic sheep underwent preoperative CT and echo exams and resultant images were analyzed using a landmark-based multiplanar measurement protocol and compiled into a master dataset for statistical analysis. We found moderate agreement between CT and echo-derived measurements of the mitral valve in sheep and propose the first clinically-relevant dimensional indices for the sheep's naive mitral valve which can be used to guide future studies evaluating novel TMVR devices. This study is the first of its kind in proposing a reproducible method for detailed examination of the mitral valve in the sheep model using clinically-relevant multimodal imaging. As in patients, CT and echo can reveal accurate native mitral valve dimensions in the sheep prior to preclinical TMVR studies.

Evaluation of Inter-atrial Septal Defect Diameter and Left Ventricular Systolic Function in Children with Ostium Secundum Atrial Septal Defect in Enugu, Nigeria.

BACKGROUND: Children with ostium secundum atrial septal defect (OS ASD) usually present with varying degrees of ventricular dysfunction. OBJECTIVES: This study aimed to evaluate the left ventricular systolic function of children with OS ASD compared with control. METHODS: This was a cross-sectional study involving 113 children with OS ASD and 113 age- and sex- matched controls. Their age ranged from 1 month to 16 years. There were 49 males and 64 females in each group. Left ventricular systolic function of children with isolated OS ASD were compared with that of controls using echocardiography. Subjects and controls who fulfilled the inclusion criteria were consecutively recruited in the study. The data elicited from the study were analyzed with the IBM SPSS version 20 (IBM Corp, Chicago). RESULTS: The mean left ventricular mass (LV mass) of the control, 93.9±113.6 gm was significantly higher than that of subjects with OS ASD, 39.4±74.3 gm, (Mann Whitney U= 4.266, p< 0.001). Similarly, the mean left ventricular internal diameter in diastole (LVIDd) of the control,25.8±11.7 mm was significantly higher than that of participants with OS ASD, 19.9±7.1 mm (Mann Whitney U=4.522, p<0.001). The mean interventricular septal diameter in systole (IVSs) of the control, 7.2±3.5 mm was higher than that of participants with OS ASD, 5.0±1.9 mm and the mean difference was found to be statistically significant, (Student t=5.738, p<0.001). The mean left ventricular internal diameter in systole (LVIDs) of the control, 16.2±7.6 mm was significantly higher than that of participants with OS ASD, 12.9±5.8 mm, (Student t= 3.660, p< 0.001). There was no significant correlation between the size of interatrial septal defect of children with OS ASD and left ventricular systolic function and age :(Corr. Coef. 0.065, p= 0.492) ; EF( Corr. Coef. 0.121,p=0.202), FS (Corr. Coef. 0.139, p=0.143). CONCLUSION: Children with OS ASD had lower left ventricular mass compared with those with control. Females seem to have higher function than males.

CONTEXTE: Les enfants atteints de communication interauriculaire type ostium secundum (CIASOS) présentent généralement des degrés variables de dysfonction ventriculaire. OBJECTIFS: Cette étude visait à évaluer la fonction systolique ventriculaire gauche des enfants atteints de CIASOS par rapport à des témoins. MÉTHODES: Il s'agissait d'une étude transversale portant sur 113 enfants atteints de CIASOS et 113 témoins appariés selon l'âge et le sexe. Leur âge variait de 1 mois à 16 ans. Il y avait 49 garçons et 64 filles dans chaque groupe. La fonction systolique ventriculaire gauche des enfants présentant un CIASOS isolé a été comparée à celle des témoins à l'aide de l'échocardiographie. Les sujets et les témoins qui remplissaient les critères d'inclusion ont été recrutés consécutivement dans l'étude. Les données recueillies ont été analysées avec la version 20 d'IBM SPSS (IBM Corp, Chicago). RÉSULTATS: La masse moyenne du ventricule gauche (VG) du groupe témoin, 93,9±113,6 g, était significativement plus élevée que celle des sujets atteints d'OS ASD, 39,4±74,3 g (Mann-Whitney U = 4,266, p < 0,001). De même, le diamètre interne moyen du ventricule gauche en diastole (LVIDd) du groupe témoin, 25,8±11,7 mm, était significativement plus élevé que celui des participants atteints d'OS ASD, 19,9±7,1 mm (Mann-Whitney U = 4,522, p < 0,001). Le diamètre moyen du septum interventriculaire en systole (IVSs) du groupe témoin, 7,2±3,5 mm, était plus élevé que celui des participants atteints d'OS ASD, 5,0±1,9 mm, et la différence moyenne était statistiquement significative (Student t = 5,738, p < 0,001). Le diamètre interne moyen du ventricule gauche en systole (LVIDs) du groupe témoin, 16,2±7,6 mm, était significativement plus élevé que celui des participants atteints d'OS ASD, 12,9±5,8 mm (Student t = 3,660, p < 0,001). Il n'y avait pas de corrélation significative entre la taille du défaut septal interauriculaire chez les enfants atteints d'OS ASD et la fonction systolique du ventricule gauche et l'âge : (Coef. Corr. 0,065, p = 0,492) ; FE (Coef. Corr. 0,121, p = 0,202), FS (Coef. Corr. 0,139, p = 0,143). CONCLUSION: Les enfants atteints de CIASOS avaient une masse ventriculaire gauche plus faible que ceux du groupe témoin. Les filles semblent avoir une fonction plus élevée que les garçons. MOTS-CLÉS: Fonction ventriculaire gauche; Communication interauriculaire type ostium secundum (CIASOS); Malformation septale interauriculaire; Enfants; Échocardiographie.

Prognostic value of the echocardiographic ratio tricuspid annular plane systolic excursion / pulmonary arterial systolic pressure in acute pulmonary embolism.

INTRODUCTION: The occurrence of death from acute pulmonary embolism (PE) is often linked to right ventricular (RV) failure, arising from an imbalance between RV systolic function and heightened RV afterload. In our study, we posited that an echocardiographic ratio derived from this disparity [RV systolic function assessed by tricuspid annular plane systolic excursion (TAPSE) divided by pulmonary arterial systolic pressure (PASP)] could offer superior predictive value for adverse outcomes compared to individual measurements of TAPSE and PASP alone. METHODS: We conducted a retrospective analysis using data from a University Hospital Centre spanning from 2017 to 2023. All individuals with confirmed PE and a formal transthoracic echocardiogram within 7 days of diagnosis were included. The primary endpoint was a composite outcome of death, hemodynamic deterioration needing introduction of inotropes or thrombolysis within 30 days. Secondary endpoints included 6 months all-cause mortality and onset of right-sided heart failure. RESULTS: Thirty-eight patients were included. Mean age was 58 ±15 years old. A male predominance was noted: 23 male patients (60.5%) and 15 female patients (39.5%). Eight patients met the primary composite endpoint while nine patients met the secondary composite endpoint. In multivariate analysis, the TAPSE/PASP ratio was independently associated with the primary outcome (OR=2.77, 95% CI 1.101-10.23, P=0.042). A TAPSE/PASP ratio <0.3 was independently associated with the secondary outcome (OR=3.07, 95% CI 1.185-10.18, P=0.034). CONCLUSION: This study suggests that a combined echocardiographic ratio of RV function to afterload is effective in predicting adverse outcomes in acute PE.

Relationship between ocular manifestations, laboratory findings, echocardiographic findings, and intravenous immunoglobulin resistance in Kawasaki disease.

BACKGROUND: This study investigates the incidence of ocular involvement in Kawasaki disease (KD) and evaluates the relationship between ocular manifestations, laboratory findings, echocardiographic findings, and intravenous immunoglobulin (IVIG) resistance. METHODS: We conducted a cross-sectional study with 58 KD patients from June 2021 to March 2023. For all patients, a complete ophthalmologic examination and echocardiography were performed in the acute phase before starting the treatment. We analyzed the age, sex, mean of white blood cell (WBC) count, platelet count, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), levels of alanine aminotransferase (ALT) and aspartate aminotransferase (AST), echocardiographic findings and IVIG responses for all patients and compared the group with ocular involvement with the group without involvement. RESULTS: The incidence of bilateral acute conjunctivitis was 70.7%, while that of acute uveitis was 30%. Patients with uveitis had significantly higher rates of Coronary artery dilatation and IVIG resistance, as well as higher mean levels of WBC, platelet, and CRP compared to those without uveitis. (P < 0.05). Additionally, the age of patients with uveitis involvement was lower than those without involvement. No significant relationships existed between ESR, AST, or ALT values and uveitis (P > 0.05). Furthermore, no significant correlations existed between any examined items and acute bilateral conjunctivitis. CONCLUSION: Uveitis in KD is significantly associated with coronary artery dilatation, IVIG resistance, higher WBC count, platelet count, and CRP level.

Speckle-Tracking: Incremental Role in Diastolic Assessment of Pediatric Patients with Chronic Kidney Disease. / Strain do Átrio Esquerdo pela Técnica de Speckle-Tracking: Contribuição para a Avaliação da Função Diastólica em Pacientes Pediátricos com Doença Renal Crônica.

BACKGROUND: Cardiovascular complications are the leading cause of mortality in pediatric patients with chronic kidney disease (CKD). Echocardiographic assessment of diastolic function in CKD has been limited to spectral and tissue Doppler imaging, known to be less reliable techniques in pediatrics. Two-dimensional Speckle tracking echocardiography (2DST) derived left atrial (LA) strain has recently been confirmed as a robust measure of diastolic function. OBJECTIVES: To investigate LA strain role in diastolic assessment of children at different stages of CKD. METHODS: From February 2019 to July 2022, 55 CKD patients without cardiovascular symptoms and 55 controls were evaluated by standard and 2DST echocardiograms. The level of significance was set at 5% (p<0.05). RESULTS: Patients and controls had similar age [9.78 (0.89 - 17.54) vs. 10.72 (1.03 -18,44) years; p = 0.41] and gender (36M:19F vs. 34M:21F; p=0.84). There were 25 non-dialysis patients and 30 dialysis patients. Left ventricular ejection fraction was ≥ 55% in all of them. Comparing CKD and controls, LA reservoir strain was lower (48.22±10.62% vs. 58.52±10.70%) and LA stiffness index was higher [0.14 (0.08-0.48)%-1 vs. 0.11 (0.06-0.23) %-1]; p<0.0001. LV hypertrophy was associated with lower LA reservoir strain (42.05±8.74% vs. 52.99±9.52%), higher LA stiffness [0.23(0.11 - 0.48)%-1 vs. 0.13 (0.08-0.23) %-1 and filling indexes (2.39±0.63 cm/s x %-1 vs. 1.74±0.47 cm/s x %-1; p<0.0001. Uncontrolled hypertension was associated with lower LA reservoir strain (41.9±10.6% vs. 50.6±9.7; p=0.005). CONCLUSIONS: LA strain proved to be a feasible tool in the assessment of pediatric CKD patients and was associated with known cardiovascular risk factors.

FUNDAMENTO: As complicações cardiovasculares são a principal causa de morte em pacientes pediátricos com doença renal crônica (DRC). A avaliação ecocardiográfica da função diastólica na DRC tem se limitado à avaliação espectral por Doppler espectral e por Doppler tecidual, técnicas sabidamente menos confiáveis na pediatria. O strain do átrio esquerdo (AE) pela técnica do speckle tracking bidimensional (2DST) foi recentemente confirmada como uma medida robusta da função diastólica. OBJETIVOS: Investigar o papel do strain do AE na avaliação da função diastólica de crianças em diferentes estágios da DRC. MÉTODOS: De fevereiro de 2019 a julho de 2022, 55 pacientes com DRC sem sintomas cardiovasculares e 55 controles foram avaliados por ecocardiografia convencional e por ecocardiografia com 2DST. O nível de significância adotado foi de 5% (p < 0,05). RESULTADOS: Pacientes e controles tinham idade similares [9,78 (0,89 ­ 17,54) vs. 10,72 (1,03 ­18,44) anos; p = 0,41] e sexo (36M:19F vs. 34M:21F; p = 0,84) similares. Havia 25 pacientes não dialíticos e 30 pacientes dialíticos. A fração de ejeção do ventrículo esquerdo foi ≥ 55% em todos. Em comparação aos controles, os pacientes com DRC apresentaram strain de reservatório mais baixo (48,22±10,62% vs. 58,52±10,70%) e índice de rigidez do AE mais alto [0,14 (0,08­0,48)%-1 vs. 0,11 (0,06­0,23) %-1]; p<0,0001. A hipertrofia ventricular esquerda associou-se com um strain de reservatório mais baixo (42,05±8,74% vs. 52,99±9,52%), e valores mais altos de índice de rigidez [0,23 (0,11 ­ 0,48)%-1 vs. 0,13 (0,08­0,23) %-1 e de índice de enchimento do AE (2,39±0,63 cm/s x %-1 vs. 1,74±0,47 cm/s x %-1; p<0,0001). Hipertensão não controlada associou-se com strain de reservatório do AE mais baixo (41,9±10,6% vs. 50,6±9,7; p=0,005). CONCLUSÃO: O strain do AE mostrou-se uma ferramenta útil na avaliação de pacientes pediátricos com DRC e associado com fatores de risco cardiovasculares conhecidos.

Targeting subclinical ventricular dysfunction in atrial fibrillation: What can myocardial work add?

Atrial fibrillation (AF) could impact on left ventricular function leading to a sublinical myocardial dysfunction, as identified by myocardial work parameters in a population-based cohort of AF patients compared with healthy individuals; factors associated with these parameters are also shown. SBP: systolic blood pressure; LAVI: left atrial volume index.

A novel multi-task machine learning classifier for rare disease patterning using cardiac strain imaging data.

To provide accurate predictions, current machine learning-based solutions require large, manually labeled training datasets. We implement persistent homology (PH), a topological tool for studying the pattern of data, to analyze echocardiography-based strain data and differentiate between rare diseases like constrictive pericarditis (CP) and restrictive cardiomyopathy (RCM). Patient population (retrospectively registered) included those presenting with heart failure due to CP (n = 51), RCM (n = 47), and patients without heart failure symptoms (n = 53). Longitudinal, radial, and circumferential strains/strain rates for left ventricular segments were processed into topological feature vectors using Machine learning PH workflow. In differentiating CP and RCM, the PH workflow model had a ROC AUC of 0.94 (Sensitivity = 92%, Specificity = 81%), compared with the GLS model AUC of 0.69 (Sensitivity = 65%, Specificity = 66%). In differentiating between all three conditions, the PH workflow model had an AUC of 0.83 (Sensitivity = 68%, Specificity = 84%), compared with the GLS model AUC of 0.68 (Sensitivity = 52% and Specificity = 76%). By employing persistent homology to differentiate the "pattern" of cardiac deformations, our machine-learning approach provides reasonable accuracy when evaluating small datasets and aids in understanding and visualizing patterns of cardiac imaging data in clinically challenging disease states.

[Giant coronary aneurysm incidentally detected on transthoracic echocardiography in a patient with previous Bentall procedure]. / Riscontro incidentale di aneurisma coronarico gigante tramite ecocardiogramma transtoracico in esiti di pregresso intervento di Bentall.

Coronary artery aneurysms represent a rare pathology (0.2-4.9% of patients undergoing coronary angiography) that may reach considerable size. The clinical presentation is various, manifesting as acute coronary syndrome or, conversely, remaining silent lifelong. We here report the case of an incidental finding by transthoracic echocardiography of a paracardiac mass of considerable size in a patient with vasculopathy that underwent a Bentall procedure for acute aortic dissection 18 years earlier. On thoracic computed tomography angiography, a 62 mm-sized giant aneurysm located in the proximal right coronary artery was evidenced. The optimal treatment of patients affected by coronary artery aneurysms remains debated; therefore, the therapeutic strategy should be individualized considering the etiology, clinical presentation, anatomical characteristics and concomitant presence of obstructive coronary artery disease.

Incidence, Risk Factors, and Outcomes of Left Atrial Enlargement in Patients Requiring Right Ventricular Pacing: A Retrospective Study of 461 Cases from 2012 to 2020.

BACKGROUND Long-term right ventricular (RV) pacing has been linked to left atrial enlargement (LAE). The incidence and risk factors associated with significant LAE after RV pacing remain unknown. This retrospective study included 461 patients requiring RV pacing at 2 centers between 2012 and 2020 and aimed to evaluate the incidence, risk factors, outcomes, and complications of LAE. MATERIAL AND METHODS A total of 461 patients with normal-sized pre-implant left atrial dimension and dual-chamber pacing pacemaker implantation for complete atrioventricular block were enrolled. Patients were grouped based on a ≥20% increase from their baseline left atrial dimension by echocardiography, indicating significant LAE, and initial characteristics, echocardiographic data, and outcomes were compared. RESULTS During a mean 7.0±4.9 years follow-up period, 96 patients (20.8%) developed significant LAE, whereas 365 patients did not. In multivariate logistic regression analysis, smaller pre-implant left atrial dimension (OR, 0.776; 95% CI, 0.728-0.828; P<0.001), lower post-implant left ventricular ejection fraction (OR, 0.976; 95% CI, 0.957-0.995; P=0.014), post-implant development of moderate to severe mitral regurgitation (OR, 2.357; 95% CI, 1.172-4.740; P=0.016), and RV pacing duration ≥3.3 years (OR, 1.576; 95% CI, 1.039-2.646; P=0.045) were independent predictors of significant LAE after RV-dependent pacing. There was a significant difference in the incident stroke events between patients without and with significant LAE (9.9% vs 17.7%; log-rank P=0.047). CONCLUSIONS Long-term RV pacing was linked to significant LAE in 20.8% of patients with complete atrioventricular block, with those affected experiencing a higher stroke rate during follow-up.

Real-world experience utilizing the nuvision 4D intracardiac echocardiography catheter for left atrial appendage closure.

Transesophageal echocardiography (TEE) has been the preferred imaging modality to help guide left atrial appendage closure. Newer technologies such as the Nuvision 4D Intracardiac echocardiography (ICE) catheter allow for real-time 3D imaging of cardiac anatomy. There are no direct comparison studies for procedural imaging between TEE and 4D ICE. To evaluate the performance and safety of left atrial appendage (LAA) closure procedures with the Watchman FLX and Amulet, guided by the Nuvision 4D ICE Catheter. This retrospective observational analysis was conducted on institutional LAAO National Cardiovascular Data Registry from January 2022 to March 2023. Patients had undergone LAA closure procedures with the Watchman FLX or Amulet device guided by TEE or a 4D ICE Catheter. The primary outcome evaluated was successful LAAO device placement. A total of 121 patients underwent LAAO device placement with 46 (38.0%) patients guided by 4D ICE during LAAO implantation. The 4D ICE group had a shorter procedural time compared with TEE guidance. Post procedural 45-day TEE post implant was also comparable for both groups with no patients in either group having incomplete closure of the left atrial appendage and peri-device leak > 5 mm. No device related complications (device related access, stroke, or pericardial effusion) occurred in either group at follow-up. There was no significant difference in device implant success or post procedural outcomes at 45 days in either the TEE or 4D ICE group. However, there was a noticeable improvement in procedural time with the 4D ICE catheter.

Incremental value of myocardial global longitudinal strain in predicting major adverse cardiac events among patients with hypertrophic cardiomyopathy.

OBJECTIVES: Endocardial global longitudinal strain (endo-GLS) measured with echocardiography (echo) has been demonstrated to be associated with myocardial fibrosis (MF) and is a prognostic predictor in patients with hypertrophic cardiomyopathy (HCM). Late gadolinium enhancement cardiac magnetic resonance (LGE-CMR) imaging showed that MF is primarily located in the myocardial layer of the extremely hypertrophic septal or ventricular wall. We hypothesized that GLS of the myocardial layer (myo-GLS) is more strongly correlated with the extent of LGE (%LGE) and is a more powerful prognostic factor than endo-GLS. METHODS: A total of 177 inpatients (54.0 [IQR: 43.0, 64.0] years, female 37.3%) with HCM were retrospectively included from May 2019 to April 2021. Among them, 162 patients underwent echocardiographic examination and contrast-enhanced CMR within 7 days. Myo-GLS and %LGE were blindly assessed in a core laboratory. All the patients were followed after they were discharged. RESULTS: During a mean follow-up of 33.77 [IQR 30.05, 35.40] months, 14 participants (7.91%) experienced major adverse cardiac events (MACE). The MACE (+) group showed lower absolute endo-GLS and myo-GLS than the MACE (-) group. Myo-GLS was more associated with %LGE (r = -.68, P < .001) than endo-GLS (r = -.64, P < .001). Cox multivariable analysis indicated that absolute myo-GLS was independently associated with MACE (adjusted hazard ratio = .75, P < .05). Myo-GLS was better than endo-GLS at detecting MACE (+) patients (-8.64%, AUC .939 vs. - 16.375%, AUC .898, P < .05). CONCLUSIONS: Myo-GLS is a stronger predictor of MACE than endo-GLS in patients with HCM and is highly correlated with %LGE.

Prognostic value of left atrial strain in aortic stenosis: A systematic review.

PURPOSE: Aortic stenosis (AS) is a common valvular heart disease with high morbidity and mortality. Recently, the association between peak atrial longitudinal strain (PALS) and AS clinical outcomes has been identified. This systematic review evaluates the prognostic value of PALS for adverse events in AS. METHODS: We performed a systematic literature review to identify clinical studies that evaluated Speckle-Tracking Echocardiography (STE)-derived PALS to predict adverse outcomes in patients with AS. We excluded studies that compared echocardiography to computed tomography and studies that focused on diseases other than AS. RESULTS: We included 18 studies reporting on 2660 patients. Patients with symptomatic AS had decreased PALS when compared to patients with asymptomatic AS. Patients with AS who had adverse events had decreased PALS when compared to patients with AS with no events. Each unit increase of PALS was independently associated with decreased risk for the primary endpoint. PALS cut-off values were associated with increased risk for the primary endpoint. CONCLUSION: This systematic review suggests PALS as an independent predictor for cardiovascular events in patients with AS and highlights the importance of evaluating LA mechanics for AS prognosis.

Role of aortic valve replacement in moderate aortic stenosis: a 10-year outcomes study.

OBJECTIVE: Patients with moderate aortic stenosis (AS) exhibit high morbidity and mortality. Limited evidence exists on the role of aortic valve replacement (AVR) in this patient population. To investigate the benefit of AVR in moderate AS on survival and left ventricular function. METHODS: In a retrospective cohort study, patients with moderate AS between 2008 and 2016 were selected from the Cleveland Clinic echocardiography database and followed until 2018. Patients were classified as receiving AVR or managed medically (clinical surveillance). All-cause and cardiovascular mortality were assessed by survival analyses. Temporal haemodynamic and structural changes were assessed with longitudinal analyses using linear mixed effects models. RESULTS: We included 1421 patients (mean age, 75.3±5.4 years and 39.9% women) followed over a median duration of 6 years. Patients in the AVR group had lower risk of all-cause (adjusted HR (aHR)=0.51, 95% CI: 0.34 to 0.77; p=0.001) and cardiovascular mortality (aHR=0.50, 95% CI: 0.31 to 0.80; p=0.004) compared with those in the clinical surveillance group irrespective of sex, receipt of other open-heart surgeries and underlying malignancy. These findings were seen only in those with preserved left ventricular ejection fraction (LVEF) ≥50%. Further, patients in the AVR group had a significant trend towards an increase in LVEF and a decrease in right ventricular systolic pressure compared with those in the clinical surveillance group. CONCLUSIONS: In patients with moderate AS, AVR was associated with favourable clinical outcomes and left ventricular remodelling.

Normative Values of Echocardiographic Chamber Size and Function in Older Healthy Adults: The Multi-Ethnic Study of Atherosclerosis.

BACKGROUND: Echocardiographic (2-dimensional echocardiography) thresholds indicating disease or impaired functional status compared with normal physiological aging in individuals aged ≥65 years are not clearly defined. In the present study, we sought to establish standard values for 2-dimensional echocardiography parameters related to chamber size and function in older adults without cardiopulmonary or cardiometabolic conditions. METHODS: In this cross-sectional study of 3032 individuals who underwent 2-dimensional echocardiography at exam 6 in the MESA (Multi-Ethnic Study of Atherosclerosis), 608 participants fulfilled our inclusion criteria of healthy aging, with normative values defined as the mean ± 1.96 standard deviation and compared across sex and race and ethnicity. Functional status measures included NT-proBNP (N-terminal pro-B-type natriuretic peptide), 6-minute walk distance, and Kansas City Cardiomyopathy Questionnaire. Prognostic performance using MESA cutoffs was compared with established guideline cutoffs using time-to-event analysis. RESULTS: The normative aging cohort (69.5±7.0 years, 46.2% male, 47.5% White) had lower NT-proBNP, higher 6-minute walk distance, and higher (better) Kansas City Cardiomyopathy Questionnaire summary values. Women had significantly smaller chamber sizes and better biventricular systolic function. White participants had the largest chamber dimensions, whereas Chinese participants had the smallest, even after adjustment for body size. Current guidelines identified 81.6% of healthy older adults in MESA as having cardiac abnormalities. CONCLUSIONS: Among a large, diverse group of healthy older adults, we found significant differences in cardiac structure and function by sex and race/ethnicity, which may signal sex-specific cardiac remodeling with advancing age. It is crucial for existing guidelines to consider the observed and clinically significant differences in cardiac structure and function associated with healthy aging. Our study highlights that existing guidelines, which grade abnormalities in echocardiographic cardiac chamber size and function based on younger individuals, may not adequately address the anticipated changes associated with normal aging.

A study protocol to characterise pathophysiological and molecular markers of rheumatic heart disease and degenerative aortic stenosis using multiparametric cardiovascular imaging and multiomics techniques.

INTRODUCTION: Rheumatic heart disease (RHD), degenerative aortic stenosis (AS), and congenital valve diseases are prevalent in sub-Saharan Africa. Many knowledge gaps remain in understanding disease mechanisms, stratifying phenotypes, and prognostication. Therefore, we aimed to characterise patients through clinical profiling, imaging, histology, and molecular biomarkers to improve our understanding of the pathophysiology, diagnosis, and prognosis of RHD and AS. METHODS: In this cross-sectional, case-controlled study, we plan to recruit RHD and AS patients and compare them to matched controls. Living participants will undergo clinical assessment, echocardiography, CMR and blood sampling for circulatory biomarker analyses. Tissue samples will be obtained from patients undergoing valve replacement, while healthy tissues will be obtained from cadavers. Immunohistology, proteomics, metabolomics, and transcriptome analyses will be used to analyse circulatory- and tissue-specific biomarkers. Univariate and multivariate statistical analyses will be used for hypothesis testing and identification of important biomarkers. In summary, this study aims to delineate the pathophysiology of RHD and degenerative AS using multiparametric CMR imaging. In addition to discover novel biomarkers and explore the pathomechanisms associated with RHD and AS through high-throughput profiling of the tissue and blood proteome and metabolome and provide a proof of concept of the suitability of using cadaveric tissues as controls for cardiovascular disease studies.