First European consensus for diagnosis, management, and treatment of transthyretin familial amyloid polyneuropathy

Purpose of reviewEarly and accurate diagnosis of transthyretin familial amyloid polyneuropathy (TTR-FAP) represents one of the major challenges faced by physicians when caring for patients with idiopathic progressive neuropathy. There is little consensus in diagnostic and management approaches across Europe.Recent findingsThe low prevalence of TTR-FAP across Europe and the high variation in both genotype and phenotypic expression of the disease means that recognizing symptoms can be difficult outside of a specialized diagnostic environment. The resulting delay in diagnosis and the possibility of misdiagnosis can misguide clinical decision-making and negatively impact subsequent treatment approaches and outcomes.SummaryThis review summarizes the findings from two meetings of the European Network for TTR-FAP (ATTReuNET). This is an emerging group comprising representatives from 10 European countries with expertise in the diagnosis and management of TTR-FAP, including nine National Reference Centres. The current review presents management strategies and a consensus on the gold standard for diagnosis of TTR-FAP as well as a structured approach to ongoing multidisciplinary care for the patient. Greater communication, not just between members of an individual patient's treatment team, but also between regional and national centres of expertise, is the key to the effective management of TTR-FAP.</p

Robotically-Assisted Coronary Artery Bypass Grafting

Objectives. Robotic surgery enables to perform coronary surgery totally endoscopically. This report describes our experience using the da Vinci system for coronary artery bypass surgery. Methods. Patients requiring single-or-double vessel revascularization were eligible. The procedure was performed without cardiopulmonary bypass on a beating heart. Results. From April 2004 to May 2008, fifty-six patients were enrolled in the study. Twenty-four patients underwent robotic harvesting of the mammary conduit followed by minimal invasive direct coronary artery bypass (MIDCAB), and twenty-three patients had a totally endoscopic coronary artery bypass (TECAB) grafting. Nine patients (16%) were converted to open techniques. The mean total operating time for TECAB was 372 ± 104 minutes and for MIDCAB was 220 ± 69 minutes. Followup was complete for all patients up to one year. There was one hospital death following MIDCAB and two deaths at follow up. Forty-eight patients had an angiogram or CT scan revealing occlusion or anastomotic stenoses (>50%) in 6 patients. Overall permeability was 92%. Conclusions. Robotic surgery can be performed with promising results

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Objectives. Robotic surgery enables to perform coronary surgery totally endoscopically. This report describes our experience using the da Vinci system for coronary artery bypass surgery. Methods. Patients requiring single-or-double vessel revascularization were eligible. The procedure was performed without cardiopulmonary bypass on a beating heart. Results. From April 2004 to May 2008, fifty-six patients were enrolled in the study. Twenty-four patients underwent robotic harvesting of the mammary conduit followed by minimal invasive direct coronary artery bypass (MIDCAB), and twenty-three patients had a totally endoscopic coronary artery bypass (TECAB) grafting. Nine patients (16%) were converted to open techniques. The mean total operating time for TECAB was 372 ± 104 minutes and for MIDCAB was 220 ± 69 minutes. Followup was complete for all patients up to one year. There was one hospital death following MIDCAB and two deaths at follow up. Forty-eight patients had an angiogram or CT scan revealing occlusion or anastomotic stenoses (&gt;50%) in 6 patients. Overall permeability was 92%. Conclusions. Robotic surgery can be performed with promising results

Critical Care Echocardiography Guidelines: Strength in International Cooperation Regarding Terminology and Competency Standards

International audienc

Detecting impaired myocardial relaxation in sepsis with a novel tissue Doppler parameter (septal e′/s′)

Abstract Background Left ventricular diastolic dysfunction is associated with mortality outcomes in severe sepsis and septic shock. There are ongoing issues with diagnosing diastolic dysfunction in this cohort, partly owing to the poor applicability of traditional parameters in the hyperdynamic circulation. In this feasibility study, we sought to assess the utility of a novel parameter (septal e′/s′) to identify diastolic dysfunction in patients with severe sepsis and septic shock who had normal systolic function against the 2016 American Society Echocardiography and European Association of Cardiovascular Imaging (ASE/EACI) guidelines on diastolic dysfunction. Methods In this prospective observational pilot study, patients identified as having severe sepsis and septic shock underwent transthoracic echocardiography on day 1 and day 3 of their intensive care unit admission. In patients with normal systolic function, septal e′/s′ was calculated using the peak modal velocity of the s′ compared with the e′ from the septal annulus tissue Doppler imaging and compared with their diastolic grade according to the 2016 ASE/EACI guidelines on diastolic dysfunction. Results On day 1 of admission, 44 of 62 patients with severe sepsis and septic shock had normal systolic function. There was a strong association of those with diastolic dysfunction having a reduced septal e′/s′ compared with patients with normal diastolic function (AUC 0.91). A similar relationship was seen with patients who had indeterminate diastolic dysfunction. On day 3, 37 patients had normal systolic function. Again, there was a strong association of those with diastolic dysfunction and a reduced septal e′/s′ (AUC 0.95). Conclusions A reduction in septal e′/s′ may indicate diastolic dysfunction in patients with severe sepsis and septic shock who have normal systolic function. As opposed to limited traditional measures of diastolic dysfunction, it is applicable in those with hyperdynamic systolic function