Multimodality Imaging in Cardiomyopathies.

Cardiomyopathy, which is shortly defined as a disease of the myocardium, has a broad definition that includes many different diagnoses. Recent advances in cardiac imaging techniques, including basic and advanced echocardiography, computed tomography, nuclear medicine, and cardiac magnetic resonance, allow for a more accurate evaluation of volumes and thickness of cardiac chambers, systolic and diastolic function of the ventricules, and tissue structure. Multimodality imaging often provides the first clinical suspicion for specific etiologies, especially when the medical and family history is unclear, by identification of red flags of underlying systemic diseases.

A gray zone: Role of transesophageal echocardiography before atrial tachycardia catheter ablation.

Introduction: There is a lack of studies in the literature directly investigating the relationship between atrial tachycardia (AT) and left atrial (LA)/left atrial appendage (LAA) thrombus, and current guidelines do not provide strong recommendations regarding the use of transesophageal echocardiography (TEE) before AT catheter ablation. This study aims to elucidate the relationship between AT and the presence of LA/LAA thrombus and contribute to the literature on the use of TEE before AT catheter ablation.

Methods: This single-center retrospective observational study screened patients who underwent TEE between February 10, 2019, and February 10, 2023.

Multinational experience with next-generation sequencing: opportunity to identify transthyretin cardiac amyloidosis and Fabry disease.

Background: Sarcomeric hypertrophic cardiomyopathy (HCM) must be differentiated from phenotypically similar conditions because clinical management and prognosis may greatly differ. Patients with unexplained left ventricular hypertrophy require an early, confirmed genetic diagnosis through diagnostic or predictive genetic testing. We tested the feasibility and practicality of the application of a 17-gene next-generation sequencing (NGS) panel to detect the most common genetic causes of HCM and HCM phenocopies, including treatable phenocopies, and report detection rates.

Current barriers and recommendations on the diagnosis of transthyretin amyloid cardiomyopathy: a Delphi study.

Objectives: This study has been conducted to investigate the non-invasive diagnostic journey of patients with a transthyretin amyloid cardiomyopathy (aTTR-CM) in Turkey, identify the challenges and uncertainties encountered on the path to diagnosis from the perspectives of expert physicians, and develop recommendations that can be applied in such cases.

Methods: This study employed a three-round modified Delphi method and included 10 cardiologists and five nuclear medicine specialists. Two hematologists also shared their expert opinions on the survey results related to hematological tests during a final face-to-face discussion.

Naples Prognostic Score and Clinical Outcomes in Pulmonary Arterial Hypertension Patients.

Objectives: Pulmonary arterial hypertension (PAH) is a specific form of pulmonary hypertension characterized by an increased mean pulmonary arterial pressure. Risk stratification is crucial in managing PAH, using various clinical, laboratory, and imaging parameters. The Naples prognostic score (NPS), incorporating nutritional and inflammatory markers, has demonstrated prognostic value in other conditions but not in PAH.

Tc-99m pyrophosphate scintigraphy for cardiac amyloidosis: concordance between planar and SPECT/CT imaging.

The imaging protocol and the optimal cut-off points for quantitative assessment of technetium-99m pyrophosphate (Tc-99m PYP) cardiac amyloidosis scintigraphy remain controversial. The aim of this study was to evaluate the concordance between planar and SPECT images, and to investigate the contribution of SPECT/CT to diagnostic precision. All patients referred to our department for Tc-99m PYP cardiac imaging between April 2019 and April 2022 were included in the study.

The Definition of Sarcomeric and Non-Sarcomeric Gene Mutations in Hypertrophic Cardiomyopathy Patients: A Multicenter Diagnostic Study Across Türkiye.

Background: Hypertrophic cardiomyopathy is a common genetic heart disease and up to 40%-60% of patients have mutations in cardiac sarcomere protein genes. This genetic diagnosis study aimed to detect pathogenic or likely pathogenic sarcomeric and non-sarcomeric gene mutations and to confirm a final molecular diagnosis in patients diagnosed with hypertrophic cardiomyopathy.

Methods: A total of 392 patients with hypertrophic cardiomyopathy were included in this nationwide multicenter study conducted at 23 centers across Türkiye.

The Frequency of Fabry Disease in Patients with Cardiac Hypertrophy of Various Phenotypes Including Prominent Papillary Muscle: The TUCARFAB Study in Turkey.

Background: The present study aimed to identify the frequency of Fabry disease in patients with cardiac hypertrophy of unknown etiology and to evaluate demographic and clinical characteristics, enzyme activity levels, and genetic mutations at the time of diagnosis.

Methods: This national, multicenter, cross-sectional, single-arm, observational registry study was conducted in adult patients with a clinical echocardiographic diagnosis of left ventricular hypertrophy and/or the presence of prominent papillary muscle. In both genders, genetic analysis was performed by DNA Sanger sequence analysis.

Expert opinion on the recognition, diagnosis and management of children and adults with Fabry disease: a multidisciplinary Turkey perspective.

This consensus statement by a panel of Fabry experts aimed to identify areas of consensus on conceptual, clinical and therapeutic aspects of Fabry disease (FD) and to provide guidance to healthcare providers on best practice in the management of pediatric and adult patients with FD. This consensus statement indicated the clinical heterogeneity of FD as well as a large number of pathogenic variants in the GLA gene, emphasizing a need for an individualized approach to patient care. The experts reached consensus on the critical role of a high index of suspicion in symptomatic patients and screening of certain at-risk groups to reveal timely and accurate diagnosis of FD along with an increased awareness of the treating physician about the different kinds of pathogenic variants and their clinical implications.

Prevalence of cardiac involvement in home-based recovered coronavirus disease 2019 (COVID-19) patients: a retrospective observational study.

Objective: Coronavirus disease 2019 (COVID-19) has a risk of cardiac arrhythmia, acute coronary syndrome, heart failure and myocarditis, and the prognosis of COVID-19 has been associated with cardiovascular symptoms. However, there has not been enough information about cardiovascular involvement in patients who had recovered home-based mild symptoms of COVID-19 infection. Therefore, this study evaluates the prevalence of cardiac involvement and associated factors in home-based recovered COVİD-19 patients.

Pulmonary vascular alterations in explanted lung after transplantation.

Widespread pulmonary destruction and fibrosis can be seen in end-stage pulmonary diseases. This situation causes vascular remodeling of the pulmonary circulation and pulmonary hypertension. Lung transplantation is an alternative treatment for end-stage pulmonary diseases.

Selvester score predicts implantable cardioverter defibrillator shocks in patients with non-ischemic cardiomyopathy.

Background: The implantable cardiac defibrillator is the cornerstone of prevention of sudden cardiac death in non-ischemic cardiomyopathy. The Selvester score, which is frequently investigated in ischemic cardiomyopathy, has not been investigated in the field of non-ischemic cardiomyopathy.

Aim: The aim of this study was to evaluate the Selvester score for determining appropriate implantable cardiac defibrillator shocks in non-ischemic cardiomyopathy patients.

[Fabry disease in cardiology: Diagnosis and therapeutic approaches].

Fabry disease is a rare, progressive, X-linked inherited storage disorder due to absent or deficient of lysosomal alfa galactosidase A activity. Deficient activity of alfa-galactosidase A results in progressive accumulation of globotriaosylceramide in a variety of tissues and organs including myocardium, kidney and nerve system. This disorder predominantly affects males; however, female heterozygotes may also be affected with a less severe clinical picture.

Whole blood viscosity in the evaluation of thrombogenic milieu in mitral stenosis.

We aimed to assess the association of whole blood with thromboembolic milieu in significant mitral stenosis patients. We included 122 patients and classified patients into two groups as having thrombogenic milieu, thrombogenic milieu (+), otherwise patients without thrombogenic milieu, thrombogenic milieu (-). Whole blood viscosity (WBV) in both shear rates were higher in thrombogenic milieu (+) group comparing with thrombogenic milieu (-).

A new risk model for the evaluation of the thromboembolic milieu in patients with atrial fibrillation: the PALSE score.

Background: The evaluation of thromboembolic risk is the cornerstone of atrial fibrillation (AF) management. Thromboembolic risk is associated with the presence of left atrial (LA) thrombus and spontaneous echo contrast (SEC), namely the thromboembolic milieu.

Aims: We aimed to assess the predictors of the thromboembolic milieu in terms of LA thrombus and/ or SEC in patients with paroxysmal AF undergoing electrical cardioversion or catheter ablation, and to develop an effective risk model for detecting the thromboembolic milieu.

Usefulness of positive T wave in lead aVR in predicting arrhythmic events and mortality in patients with hypertrophic cardiomyopathy.

Background: Positive T wave in lead aVR (TaVR) has been associated with increased risk of adverse events in patients with various cardiovascular diseases.

Objective: The purpose of this study was to investigate the prevalence and prognostic significance of positive TaVR in patients with hypertrophic cardiomyopathy (HCM).

Methods: This study investigated 421 consecutive patients with HCM (177 women; age 51.

Where is the right ventricle? Accurate diagnosis with cardiovascular multimodality imaging.

Endomyocardial fibrosis (EMF) is a globally unattended disease with significant rates of morbidity and mortality. It has a higher prevalence in tropical and subtropical countries compared to the rest of the world. Endomyocardial fibrosis can affect the atrioventricular valves, along with all four chambers of the heart, but spares the myocardium.

Relationship between CHA2DS2-VASc and CHADS2 scores with pulmonary hypertension in patients with acute pulmonary embolism.

Introduction: Pulmonary hypertension (PH) is the most important prognostic factor after acute pulmonary embolism (PE). Therefore, determination of patients who will develop PH after acute PE is crucial. The aim of the present study was to evaluate the predictive value of the CHADS2 and CHA2DS2-VASc scores for PH in patients with acute PE.

Importance of lead aVR on predicting adverse cardiac events in patients with noncompaction cardiomyopathy.

Background: Noncompaction cardiomyopathy (NCCM) is a relatively rare cardiac abnormality with high rates of mortality and morbidity. T-wave amplitudes during ventricular repolarization in lead aVR (TaVR) have been reported to be associated with the prognosis of various cardiovascular diseases. This study sought to investigate the prevalence and prognostic role of positive TaVR in patients with NCCM.

Cardiac amyloidosis: Recent advances in the diagnosis and therapy.

Cardiac amyloidosis (CA) is a progressive cardiomyopathy in which misfolded endogenous proteins form amyloid fibrils that deposit in the heart as well as kidneys, liver, gastrointestinal tract and soft tissues. The most common forms of CA include immunoglobulin light chain (AL) amyloidosis and transthyretin (TTR) amyloidosis. Although cardiac amyloidosis is thought to be a very rare disease, emerging data suggested that 13% of heart failure patients with preserved ejection fraction and 16-26% of advanced aged patients with severe aortic stenosis may have TTR-CA.

Monocyte to high-density lipoprotein cholesterol ratio predicts adverse cardiac events in patients with hypertrophic cardiomyopathy.

The aim of the study was to investigate the monocyte count to HDL cholesterol ratio (MHR) on the prognosis of patients with hypertrophic cardiomyopathy (HCM). A total of 411 patients with HCM were assessed. The primary end point was cardiovascular death or malignant arrhythmic events.

Prediction of new onset atrial fibrillation in patients with acute pulmonary embolism: The role of sPESI Score.

Objective: Acute pulmonary embolism (APE) is a serious clinical situation and atrial fibrillation (AF) is the most common arrhytmia in clinical practice. The Pulmonary Embolism Severity Index (PESI) is an accepted risk stratification tool used to predict short term mortality in APE. The aim of this study was to evaluate the relationship between the PESI score and new-onset AF in patients with APE.

[Increased myocardial energy expenditure in cardiac syndrome X: More work, more pain].

Objective: The aim of this study was to assess the myocardial energy expenditure (MEE) in patients with cardiac syndrome X (CSX) and to examine its association with exercise electrocardiogram (ECG) parameters.

Methods: A total of 99 patients who underwent coronary angiography and who were diagnosed as having normal coronary arteries were included. The patients were divided into 2 groups based on symptoms and exercise ECG parameters: 56 CSX patients and 43 control patients with a negative stress test.

Flail Tricuspid Leaflet During the Percutaneous Closure of Post-Myocardial Infarction Ventricular Septal Defect.

• A post-MI ventricular septal defect is a complication of ST-elevation MI. • Iatrogenic tricuspid regurgitations have not been reported after percutaneous closure. • Care should be taken with the degree of tricuspid regurgitation to prevent iatrogenic tricuspid injury.