Integration of imaging and circulating biomarkers in heart failure: a consensus document by the Biomarkers and Imaging Study Groups of the Heart Failure Association of the European Society of Cardiology.

Circulating biomarkers and imaging techniques provide independent and complementary information to guide management of heart failure (HF). This consensus document by the Heart Failure Association (HFA) of the European Society of Cardiology (ESC) presents current evidence-based indications relevant to integration of imaging techniques and biomarkers in HF. The document first focuses on application of circulating biomarkers together with imaging findings, in the broad domains of screening, diagnosis, risk stratification, guidance of treatment and monitoring, and then discusses specific challenging settings. In each section we crystallize clinically relevant recommendations and identify directions for future research. The target readership of this document includes cardiologists, internal medicine specialists and other clinicians dealing with HF patients.

Role of cardiovascular imaging in cancer patients receiving cardiotoxic therapies: a position statement on behalf of the Heart Failure Association (HFA), the European Association of Cardiovascular Imaging (EACVI) and the Cardio-Oncology Council of the European Society of Cardiology (ESC).

Cardiovascular (CV) imaging is an important tool in baseline risk assessment and detection of CV disease in oncology patients receiving cardiotoxic cancer therapies. This position statement examines the role of echocardiography, cardiac magnetic resonance, nuclear cardiac imaging and computed tomography in the management of cancer patients. The Imaging and Cardio-Oncology Study Groups of the Heart Failure Association (HFA) of the European Society of Cardiology (ESC) in collaboration with the European Association of Cardiovascular Imaging (EACVI) and the Cardio-Oncology Council of the ESC have evaluated the current evidence for the value of modern CV imaging in the cardio-oncology field. The most relevant echocardiographic parameters, including global longitudinal strain and three-dimensional ejection fraction, are proposed. The protocol for baseline pre-treatment evaluation and specific surveillance algorithms or pathways for anthracycline chemotherapy, HER2-targeted therapies such as trastuzumab, vascular endothelial growth factor tyrosine kinase inhibitors, BCr-Abl tyrosine kinase inhibitors, proteasome inhibitors and immune checkpoint inhibitors are presented. The indications for CV imaging after completion of oncology treatment are considered. The typical consequences of radiation therapy and the possibility of their identification in the long term are also summarized. Special populations are discussed including female survivors planning pregnancy, patients with carcinoid disease, patients with cardiac tumours and patients with right heart failure. Future directions and ongoing CV imaging research in cardio-oncology are discussed.

Identification of novel splice mutation in SMAD3 in two Cypriot families with nonsyndromic thoracic aortic aneurysm. Two case reports.

BACKGROUND: Thoracic aortic aneurysm and dissection (TAA/D) represents a potentially lethal disease group characterized by an increased risk of dissection or rupture. Only a small percentage (approximately 30%) of individuals with nonsyndromic familial TAA/D have a pathogenic variant in one of the genes that have been found to be associated with the disease. METHODS: A targeted sequencing panel and direct sequencing approach were used to identify causative mutations in the index patients and other family members. RESULTS: In this study we report two apparently unrelated Cypriot families with nonsyndromic familial TAA/D. The proband A is a female patient diagnosed with TAA/D and intracranial aneurysm and opted for an elective intervention. The proband B is a male patient who was diagnosed with TAA/D and underwent cardiac surgery. Sequencing analysis identified a novel splice site variant (c.871+1G>A) in SMAD3 which is shown to be associated with the disease. Analysis of mRNA from the patient's tissue confirmed aberrant splicing and exon 6 skipping. CONCLUSION: Our findings expand the mutation spectrum of variants that have been shown to be associated with nonsyndromic familial TAA/D. This study demonstrates the importance of a comprehensive clinical and genetic evaluation aiming at early diagnosis and intervention.

Innovative imaging methods in heart failure: a shifting paradigm in cardiac assessment. Position statement on behalf of the Heart Failure Association of the European Society of Cardiology.

Myriad advances in all fields of cardiac imaging have stimulated and reflected new understanding of cardiac performance, myocardial damage and the mechanisms of heart failure. In this paper, the Heart Failure Association assesses the potential usefulness of innovative imaging modalities in enabling more precise diagnostic and prognostic evaluation, as well as in guiding treatment strategies. Many new methods have gradually penetrated clinical practice and are on their way to becoming a part of routine evaluation. This paper focuses on myocardial deformation and three-dimensional ultrasound imaging; stress tests for the evaluation of contractile and filling function; the progress of magnetic resonance techniques; molecular imaging and other sound innovations. The Heart Failure Association aims to highlight the ways in which paradigms have shifted in several areas of cardiac assessment. These include reassessing of the simplified concept of ejection fraction and implementation of the new parameters of cardiac performance applicable to all heart failure phenotypes; switching from two-dimensional to more accurate and reproducible three-dimensional ultrasound volumetric evaluation; greater tissue characterization via recently developed magnetic resonance modalities; moving from assessing cardiac function and congestion at rest to assessing it during stress; from invasive to novel non-invasive hybrid techniques depicting coronary anatomy and myocardial perfusion; as well as from morphometry to the imaging of pathophysiologic processes such as inflammation and apoptosis. This position paper examines the specific benefits of imaging innovations for practitioners dealing with heart failure aetiology, risk stratification and monitoring, and, in addition, for scientists involved in the development of future research.

The Greek version of the 9-item European Heart Failure Self-care Behaviour Scale: a multidimensional or a uni-dimensional scale?

OBJECTIVES: To evaluate the dimensionality of the Greek version of the European Heart Failure Self-care Behaviour Scale (Gr9-EHFScBS) in a Greek-Cypriot population. BACKGROUND: EHFScBS is a valid and reliable scale which is widely used for assessing heart failure (HF) patients' self-care behaviors. METHODS: EHFScBS was translated into Greek and was administered to 128 Greek-Cypriot HF patients. The internal consistency, construct validity and discriminant validity of the scale were assessed. RESULTS: Confirmatory factor analysis failed to capture the proposed theoretical structure. Further exploratory factor analysis provided a three-factor solution accounting for 53.35% of the variance, though the scale is better used as a whole. Cronbach's alpha was moderate 0.66, but deletion of any item decreased the alpha coefficient. Discriminant validity was supported by the poor correlation between EHFScBS and Minnesota Living with Heart Failure Questionnaire scores. CONCLUSION: Even though results do not conform to the multidimensionality of the scale, assessment of the tool provided acceptable validity and reliability measures to support its usage among Greek speaking populations.

Evaluation of the psychometric properties of the Greek version of the Minnesota Living With Heart Failure questionnaire.

PURPOSE: One of the major challenges for health care professionals in heart failure (HF) management is to maintain and/or improve HF patient health-related quality of life. The Minnesota Living With Heart Failure Questionnaire (MLHFQ) is one of the most comprehensive and widely used tools for measuring health-related quality of life among patients with HF. The aim of the study was to assess the psychometric properties of the Greek version of the tool. METHODS: The MLHFQ was administered among 128 Greek-Cypriot HF patients to assess the internal consistency, content validity, and contrast validity of its Greek version. Exploratory factor analysis was undertaken to establish its construct validity. RESULTS: The factor analysis in this study provided support for a 3-factor solution explaining 64.15% of the variance (physical, emotional, and social subscales). The internal consistency for the Greek version of the MLHFQ total scale (0.95) and subscales (0.80-0.94) were found to be high. The contrast validity of the Greek version of the MLHFQ was explored through cumulative MLHFQ scores and comparisons that were able to distinguish among all different levels of HF severity, as defined by the New York Heart Association functional class grouping. CONCLUSION: This study provides support for the reliability and validity of the Greek version of the MLHFQ.

Dobutamine stress echocardiography for the evaluation of cardiac reserve late after Fontan operation.

INTRODUCTION: This study aimed to assess the potential role and safety of dobutamine stress echocardiography (DSE) in the evaluation of cardiac reserve in asymptomatic patients several years after a Fontan operation. METHODS: We studied 10 asymptomatic patients, 28 +/- 5 years old, 14 +/- 6 years after their Fontan operation. All patients and 10 healthy, matched controls underwent two-dimensional and Doppler echocardiography at baseline and throughout dobutamine infusion (given in 3-minute increments of 5, 10, 20, 30, and 40 microg/kg/min). Multivariate analysis for repeated measurements was used to detect differences between patients and controls. RESULTS: There were no adverse events during dobutamine infusion. Heart rate increased appropriately in both patients and controls. Patients reached peak stroke volume at infusion rates of 20 microg/kg/min, whereas controls peaked at 10 microg/kg/min. Mean stroke volume, cardiac output and cardiac index were significantly different between the two groups only up to infusion rates of 10 microg/kg/min. The velocity time integral of the left ventricular outflow tract flow was significantly lower in patients than controls throughout the study. CONCLUSION: DSE is a safe method of stress testing for the assessment of adult Fontan patients and provides insights into the pathophysiology of their cardiac performance. Cardiac reserve in these patients is impaired compared to healthy controls.

Hepatocellular carcinoma with right atrial extension in a young patient with congenitally unguarded tricuspid orifice.

We report a case of a young female patient with congenitally unguarded tricuspid orifice and underdeveloped pulmonary valve leaflets, who had developed severe right-sided congestive heart failure and cardiac cirrhosis early in life. She was admitted to our hospital with deterioration of dyspnea and abdominal tenderness. Hepatocellular carcinoma was diagnosed on the basis of computed tomography findings, high plasma alpha-fetoprotein levels and evidence of chronic hepatitis B viral infection. Transthoracic echocardiogram revealed a mass into the right atrium, considered to be an extension of the tumor.

Acute thoracic aortic thrombosis after intra-aortic balloon pumping.

We present two transesophageal echocardiographic images of a patient with acute myocardial infarction, demonstrating a large thrombus attached to the thoracic aortic wall, considered to be a complication of intra-aortic balloon pumping. The patient had received the device because of hemodynamic instability due to an infarct-related ventricular septal defect. Clinical manifestations which led to the diagnosis of thromboembolism were abdominal pain and deterioration of renal function, without signs of limb ischemia.