Longitudinal systolic strain, cardiac function improvement, and survival following treatment of light-chain (AL) cardiac amyloidosis.

Aims: To determine whether echocardiographic longitudinal systolic strain (LS) parameters identify short-term improvement following chemotherapy for light-chain (AL) cardiac amyloidosis (CA). Among patients with CA, standard echocardiographic measures are commonly unchanged at 1 year following successful chemotherapy, despite observed reductions in cardiac biomarkers.

Methods And Results: We retrospectively identified 61 patients with AL-CA treated with high-dose melphalan or bortezomib-based regimens.

Operability assessment in CTEPH: Lessons from the CHEST-1 study.

Pulmonary endarterectomy is the gold standard treatment for chronic thromboembolic pulmonary hypertension and is potentially curative, although some patients are unsuitable for pulmonary endarterectomy and require alternative management. Lack of standardized assessment of pulmonary endarterectomy eligibility risks suboptimal treatment in some patients. We discuss the implications for future clinical trials and practice of a unique operability assessment in patients who have chronic thromboembolic pulmonary hypertension and were initially screened for inclusion in the CHEST-1 (Chronic Thromboembolic Pulmonary Hypertension Soluble Guanylate Cyclase Stimulator Trial-1) study.

Predictors of long-term outcomes in patients treated with riociguat for chronic thromboembolic pulmonary hypertension: data from the CHEST-2 open-label, randomised, long-term extension trial.

Background: Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare, debilitating, and life-threatening disease. We investigated associations between markers of disease severity and long-term outcomes in patients with inoperable CTEPH or persistent or recurrent pulmonary hypertension after pulmonary endarterectomy (PEA) who were receiving the soluble guanylate cyclase stimulator riociguat. We also present safety and efficacy from the final data cutoff of CHEST-2, where most patients had received riociguat for at least 2 years.

Relationship between electrocardiographic findings and Cardiac Magnetic Resonance phenotypes in patients with Hypertrophic Cardiomyopathy.

Background: Q waves and negative T waves are common electrocardiographic (ECG) abnormalities in patients with Hypertrophic Cardiomyopathy (HCM). Several studies correlated ECG findings with presence and extent of fibrosis and hypertrophy; however, their significance remains incompletely clarified. Our study aimed to explain the mechanism behind Q and negative T waves by comparing their positions on a 12-lead ECG with phenotypes observed at Late Gadolinium Enhancement (LGE) Cardiac Magnetic Resonance (CMR).

Changes in surface electrocardiogram in patients with chronic thromboembolic pulmonary hypertension undergoing pulmonary endarterectomy. Correlations with hemodynamic and echocardiographic improvements after surgery.

Background: The aim of the present study was to evaluate the changes of electrocardiographic (ECG) markers of right ventricular (RV) hypertrophy/overload in patients with chronic thromboembolic pulmonary hypertension (CTEPH) undergoing pulmonary endarterectomy (PEA).

Methods And Results: We evaluated 99 CTEPH patients who underwent PEA. P wave amplitude in DII, R wave amplitude in V1 and the number of patients with negative T wave in V1-V3 decreased significantly at 1month after surgery with no further change at 1year, in parallel with the rapid improvement in right heart hemodynamics.

Diagnostic advances and opportunities in chronic thromboembolic pulmonary hypertension.

Chronic thromboembolic pulmonary hypertension (CTEPH) is characterised by the presence of thromboembolic material in the pulmonary circulation, and patients have a poor prognosis without treatment. Patients present with nonspecific symptoms, such as breathlessness and syncope, which means that other more common conditions are sometimes suspected before CTEPH, leading to delayed diagnosis and treatment. This is problematic because CTEPH is potentially curable with surgical pulmonary endarterectomy (PEA); indeed, CTEPH should always be considered in any patient with unexplained pulmonary hypertension (PH).

Use of responder threshold criteria to evaluate the response to treatment in the phase III CHEST-1 study.

Background: In the Chronic Thromboembolic Pulmonary Hypertension Soluble Guanylate Cyclase - Stimulator Trial 1 (CHEST-1) study, riociguat improved 6-minute walking distance (6MWD) vs placebo in patients with inoperable chronic thromboembolic pulmonary hypertension or persistent/recurrent pulmonary hypertension after pulmonary endarterectomy. In this study, the proportion of patients who achieved responder thresholds that correlate with improved outcome in patients with pulmonary arterial hypertension was determined at baseline and at the end of CHEST-1.

Methods: Patients received placebo or riociguat individually adjusted up to 2.

Riociguat for the treatment of chronic thromboembolic pulmonary hypertension: a long-term extension study (CHEST-2).

Riociguat is a soluble guanylate cyclase stimulator approved for the treatment of inoperable and persistent/recurrent chronic thromboembolic pulmonary hypertension (CTEPH). In the 16-week CHEST-1 study, riociguat showed a favourable benefit-risk profile and improved several clinically relevant end-points in patients with CTEPH. The CHEST-2 open-label extension evaluated the long-term safety and efficacy of riociguat.

Pulmonary endarterectomy for distal chronic thromboembolic pulmonary hypertension.

Objectives: Chronic thromboembolic pulmonary hypertension can be cured by pulmonary endarterectomy. Operability assessment remains a major concern, because there are no well-defined criteria to discriminate proximal from distal obstructions, and surgical candidacy depends mostly on the surgeon's experience. The intraoperative classification of chronic thromboembolic pulmonary hypertension describes 4 types of lesions, based on anatomy and location.

Heart transplantation in patients with eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome).

Background: Heart involvement is the leading cause of death of patients with eosinophilic granulomatosis with polyangiitis (EGPA; formerly Churg-Strauss syndrome) and is more frequent in anti-neutrophil cytoplasm antibody (ANCA)-negative patients. Post-transplant outcome has only been reported once.

Methods: We conducted a retrospective international multicenter study.

Leukocytoclastic vasculitis as a complication of recombinant granulocyte colony-stimulating factor therapy in a heart transplant patient.

Recombinant granulocyte colony-stimulating factor (rG-CSF) is a myeloid growth factor that is widely used in haematology to recover neutropenia secondary to myelosuppressive chemotherapy. Leukocytoclastic vasculitis is an acknowledged side effect of the above therapy. Its pathogenesis involves many mechanisms that collectively induce an increase in neutrophil function and a subsequent release of cytokines.

Grover's Disease after Heart Transplantation: A Case Report.

Grover's disease is a transient acantholytic dermatosis of unknown cause, manifesting clinically as a papular skin eruption that is usually located on the anterior chest and abdomen. Histologically characterized by an acantholytic pattern, it has been associated with numerous disorders, including hematologic malignancies, chronic renal failure, and HIV infection, as well as with chemotherapy and bone marrow and/or kidney transplant. Evaluation of followup and treatment is often complicated by spontaneous remission and the occasionally fluctuant course of the disease.

Aortic valve replacement performed twice through ministernotomy 15 years after lung transplantation.

After transplantation, steroids and calcineurin inhibitors together with end-stage renal failure may lead to associated cardiovascular diseases, particularly in long-term survivors. We present a case of aortic valve replacement 15 years after lung transplantation, followed by reoperative valve replacement for late infective endocarditis. Lung compliance and gas exchange were excellent during recovery.

Assessment of synovitis to predict bone erosions in rheumatoid arthritis.

Although rheumatoid arthritis (RA) is traditionally considered as the prototype of destructive arthritis, the course of the disease varies considerably, with some patients experiencing more rapid progression of joint damage and disability than others. Given the increasing availability of treatment targets and options, timely recognition of individual's outcomes could allow therapeutic allocation according to personalized benefit-risk profiles. Research efforts are thus increasingly focused at discovering predictive markers that could identify patients with aggressive, rapidly progressive disease and poor prognosis.

Evolving surgical techniques for pulmonary endarterectomy according to the changing features of chronic thromboembolic pulmonary hypertension patients during 17-year single-center experience.

Objective: An increasing number of elderly patients are referred for pulmonary endarterectomy. The distinction between operable and inoperable lesions has been challenged over time. Hence, we developed alternative cardiopulmonary bypass management and cerebral protection strategies to obtain satisfactory surgical results according to the changing patient features.

Serum levels of CXCL13 are associated with ultrasonographic synovitis and predict power Doppler persistence in early rheumatoid arthritis treated with non-biological disease-modifying anti-rheumatic drugs.

Introduction: Biological markers specifically reflecting pathological processes may add value in the assessment of inter-individual variations in the course of rheumatoid arthritis (RA). The current study was undertaken to investigate whether baseline serum levels of the chemokine CXCL13 might predict clinical and ultrasonographic (US) outcomes in patients with recent-onset RA.

Methods: The study included 161 early RA patients (disease duration < 12 months) treated according to a disease activity score (DAS) driven step-up protocol aiming at DAS < 2.

Is mitral E/E' ratio a reliable predictor of left ventricular diastolic pressures in patients without heart failure?

Unlabelled: Aim Conflicting evidence exists as to whether the mitral E/E' ratio can be a reliable predictor of the left ventricular end-diastolic pressure (LVEDP). Our aim was to assess the value of the mitral E/E' ratio for the estimation of left ventricular diastolic pressures (LVDP) in patients without heart failure (HF).

Methods And Results: Echo-Doppler examination and left heart catheterization were carried out in 100 consecutive patients to assess the correlation between echo-Doppler parameters and the LVDP.

Synovial tissue heterogeneity and peripheral blood biomarkers.

Rheumatoid arthritis is characterized by multiple pathobiological processes and heterogeneous clinical phenotypes. Not surprisingly, the inflamed synovium harbors an equally complex pathology. This includes variability in infiltrating and resident cell populations, spatial arrangements, and cell-cell interactions, as well as gene expression profiles.

Clinical and immunological evaluation of 12-month azithromycin therapy in chronic lung allograft rejection.

Background: Bronchiolitis obliterans syndrome (BOS) is the leading cause of morbidity/mortality in lung-transplant recipients (LTRs). Recent studies demonstrated that azithromycin (AZI) can improve graft function in BOS. We here investigated whether a 12-month course of AZI could more efficiently impact the course of BOS if administered early in BOS development.

Subclinical remodelling of draining lymph node structure in early and established rheumatoid arthritis assessed by power Doppler ultrasonography.

Objective: To investigate the suitability of power Doppler ultrasonography (PD-US) for the assessment of lymph node (LN) status in RA, evaluating the existence of structural and dynamic modifications in well-characterized stages of the disease.

Methods: Ten patients with active disease and five patients in clinical remission underwent complete clinical and PD-US examination of hands, wrists, axillary and cervical LNs on the same day. Synovitis and PD were graded 0-3.

Clinical characteristics and outcome of left ventricular ballooning syndrome in a European population.

We assessed the clinical characteristics and determinants of the prognosis of patients with left ventricular ballooning syndrome (LVBS) in an European population. A total of 128 patients with LVBS (98% women, age 67 ± 11 years) were prospectively followed up for a median of 13 months. A trigger event was identifiable in 58% of the patients.

Dobutamine stress echocardiography for assessing the role of dynamic intraventricular obstruction in left ventricular ballooning syndrome.

Background: Dynamic intraventricular obstruction has been observed in patients with left ventricular ballooning syndrome (LVBS) and has been hypothesized as a possible mechanism of the syndrome. The aim of this study was to assess the prevalence and significance of dynamic intraventricular obstruction in patients with LVBS.

Methods And Results: Dobutamine stress echocardiography was carried out in 22 patients with LVBS (82% apical), all women, aged 68 +/- 9 years.

Effect of dietary glycemic index on food intake, adiposity, and fasting plasma ghrelin levels in animals.

Background: An increase in lipid storage as a consequence of feeding animals with high-glycemic index (GI) diets has been observed by many authors. Ghrelin is one of the most important orexigenic hormones, and curiously, its fasting plasma levels are decreased in human obesity.

Aim: As ghrelin secretion is affected by insulin concentration, we hypothesized that carbohydrates with different glycemic responses might influence fasting plasma ghrelin levels.