In this document, we propose a universal definition of heart failure (HF) as a clinical syndrome with symptoms and/or signs caused by a structural and/or functional cardiac abnormality and ...corroborated by elevated natriuretic peptide levels and/or objective evidence of pulmonary or systemic congestion. We also propose revised stages of HF as: At risk for HF (Stage A), Pre‐HF (Stage B), Symptomatic HF (Stage C) and Advanced HF (Stage D). Finally, we propose a new and revised classification of HF according to left ventricular ejection fraction (LVEF). This includes HF with reduced ejection fraction (HFrEF): symptomatic HF with LVEF ≤40%; HF with mildly reduced ejection fraction (HFmrEF): symptomatic HF with LVEF 41–49%; HF with preserved ejection fraction (HFpEF): symptomatic HF with LVEF ≥50%; and HF with improved ejection fraction (HFimpEF): symptomatic HF with a baseline LVEF ≤40%, a ≥10 point increase from baseline LVEF, and a second measurement of LVEF > 40%.
Congestion is one of the most prominent characteristics of patients presented with decompensated heart failure and it implies unfavorable prognosis for the heart failure patient. Neurohumoral and ...immuno-inflammatory activation secondary to cardiac dysfunction constitute the pivotal mechanisms driving the heart failure syndrome that results in progressive fluid accumulation. In addition, fluid redistribution between different vascular compartments in human body guided from sympathetic activity constitutes another mechanism for heart failure decompensation. Ultrasound applied in the form of echocardiography provides invaluable data for the assessment of intracardiac filling pressures. The type of renal venous flow can provide the degree of renal congestion and probably insight into the pathophysiology of the decompensation of heart failure. Assessment of lung congestion in the patient with heart failure can be accomplished by lung ultrasonography. Additionally, clinical studies on the role of ultrasound in the management and prognosis of the congested patient are reviewed. Special heart failure population supported with left ventricular assist devices and extracorporeal membrane oxygenation support constitute an area where ultrasound guidance of fluid management has gained important role.
Myocardial iron overload in patients with thalassemia major (TM) is one of the most important complications. The purpose of the study was to identify advanced echocardiography parameters for early ...identification of myocardial dysfunction during follow-up of patients with TM.
Forty TM patients who were 41 ± 5 years old were included in the study and divided into two groups according to cardiac magnetic resonance T2* results (Group 1: Τ2* > 25 ms, Group 2: Τ2* ≤ 25 ms). Liver T2* parameters were also measured. Conventional and deformational echocardiographic parameters were measured at baseline and approximately 2 years later.
Thirty-two patients had Τ2* = 34 ± 4 ms (Group 1), and 8 had Τ2* = 17 ± 9 ms (Group 2). Blood consumption was 185 ± 60 and 199 ± 37 ml/kg/yr (p = 0.64), and liver T2* was 4 ± 5 and 17 ± 21 ms (p = 0.01) in Groups 1 and 2, respectively. At baseline, Group 1 had better left ventricular global longitudinal strain (GLS) (- 22 ± 3 vs. - 18 ± 5, p = 0.01) and similar left ventricular ejection fraction (LVEF) (62 ± 5% vs. 58 ± 10%, p = 0.086) than Group 2. At the 28 ± 11-month follow-up, LVEF, GLS, and T2* values in Group 1 (63 ± 3%, - 21 ± 3%, 34 ± 4 ms) and Group 2 (56 ± 11%, - 17 ± 4%, 17 ± 9 ms) did not change significantly compared to their corresponding baseline values. In 8 patients from Group 1, a worsening (> 15%) in LS (p = 0.001) was detected during follow-up, with a marginal reduction in LVEF.
GLS seems to be an efficient echocardiographic parameter for detecting hemochromatosis-related cardiac dysfunction earlier than LVEF. It also seems to be affected by other factors (free radical oxygen, immunogenetic mechanisms or viral infections) in a minority of patients, underscoring the multifactorial etiology of cardiomyopathy.
•Arrhythmogenic right ventricular cardiomyopathy is a rare but significant cause of advanced heart failure with biventricular compromise.•Biventricular mechanical circulatory support is often ...necessary in low organ donation environments but with suboptimal outcomes.•Heart transplant is the advanced heart failure therapy of choice in this population.
Arrhythmogenic right ventricular cardiomyopathy (ARVC) may be complicated by heart failure. Management of advanced heart failure in this context is challenging.
We reviewed our center's experience with advanced heart failure therapies in patients with ARVC. Three rapidly deteriorating patients with ARVC with biventricular heart failure were found. Their management and outcomes are presented. Data on ventricular fibrosis were available in 2 of them and are also included.
The first patient underwent initially successful paracorporeal pulsatile biventricular assist device (BiVAD) implantation. However, a large ischemic stroke occurred 2 weeks later, and the patient died after 2 months. The second patient underwent urgent BiVAD implantation after extracorporeal membrane oxygenation support because of cardiogenic shock, but his course was complicated by multiorgan failure due to systemic infection and the patient died. The last patient, being at Interagency Registry for Mechanically Assisted Circulatory Support 3-4 profile, underwent heart transplant with uneventful recovery. Extensive fibrosis was present in both ventricles of 2 patients undergoing pathology examination.
Patients with ARVC and advanced biventricular heart failure are characterized by extensive ventricular fibrosis and considerable risk, but data on their management are limited. Biventricular circulatory support is associated with suboptimal outcomes, and prioritization for heart transplant seems preferable.
The accepted use of left ventricular assist device (LVAD) technology as a good alternative for the treatment of patients with advanced heart failure together with the improved survival of patients on ...the device and the scarcity of donor hearts has significantly increased the population of LVAD supported patients. Device‐related, and patient–device interaction complications impose a significant burden on the medical system exceeding the capacity of LVAD implanting centres. The probability of an LVAD supported patient presenting with medical emergency to a local ambulance team, emergency department medical team and internal or surgical wards in a non‐LVAD implanting centre is increasing. The purpose of this paper is to supply the immediate tools needed by the non‐LVAD specialized physician — ambulance clinicians, emergency ward physicians, general cardiologists, and internists — to comply with the medical needs of this fast‐growing population of LVAD supported patients. The different issues discussed will follow the patient's pathway from the ambulance to the emergency department, and from the emergency department to the internal or surgical wards and eventually back to the general practitioner.
Abstract Accessibility to the available traditional forms of cardiac rehabilitation programmes in heart failure patients is not adequate and adherence to the programmes remains unsatisfactory. The ...home-based telerehabilitation model has been proposed as a promising new option to improve this situation. This paper's aims are to discuss the tools available for telemonitoring, and describing their characteristics, applicability, and effectiveness in providing optimal long term management for heart failure patients who are unable to attend traditional cardiac rehabilitation programmes. The critical issues of psychological support and adherence to the telerehabilitation programmes are outlined. The advantages and limitations of this long term management modality are presented and compared with alternatives. Finally, the importance of further research, multicentre studies of telerehabilitation for heart failure patients and the technological development needs are outlined, in particular interactive remotely controlled intelligent telemedicine systems with increased inter-device compatibility.
Primary cardiac lymphomas (PCL) represent extremely rare cardiac tumors which are accompanied by poor prognosis, unless they are timely diagnosed and treated.
Herein we present a 28-year-old, ...immunocompetent man who presented to our hospital due to progressively worsening symptoms and signs of superior vena cava syndrome. Multi-modality imaging demonstrated a large intracardiac tumor, which was proven, by biopsy, to be a PCL. The patient received targeted chemotherapy which led to total remission of his disease, with no relapse over a 15-month follow-up period.
Although PCLs are rare, they should always be kept in mind in the differential diagnosis of cardiac tumors. Timely diagnosis of PCLs and appropriate chemotherapy, alone or in combination with radiotherapy, seems to provide the best results.
Restrictive cardiomyopathy is a rare cardiac disease, for which several genes including TNNT2, MYPN, FLNC and TNNI3 have been associated with its familial form.
Here we describe a female proband with ...a severely manifested restrictive phenotype leading to heart transplantation at the age of 41, who was found homozygous for the novel TNNI3 mutation: NM_000363.4:c.586G > C, p.(Asp196His). Her parents were third-degree cousins originating from a small village and although they were found heterozygous for the same variant they displayed no symptoms of the disease. Her older sister who was also found heterozygous was asymptomatic. Her twin sister and her brother who were homozygous for the same variant displayed a restrictive and a hypertrophic phenotype, respectively. Their children are all carriers of the mutation and remain asymptomatic until the age of 21.
These observations point to a recessive mode of inheritance reported for the first time for this combination of gene/disease.
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