[Acute on chronic liver failure: more than just cirrhotic decompensation]. / Acute on chronic liver failure : bien plus qu'une simple décompensation cirrhotique.

Acute on Chronic Liver Failure (ACLF) is an unfavorable form of cirrhotic disease progression, distinguished from decompensated cirrhosis by a very high short-term mortality associated with damage to one or more organs. The pathophysiology is based on an intense systemic inflammatory reaction, the triggering factor of which can be identified (infection, toxic agent, etc.) in around two thirds of cases. The analogy with sepsis has enabled us to derive prognostic scores linked to organ damage, and thus to better guide these patients, who most often require close monitoring. Treatment remains limited and relies on support for the affected organs. Given the poor prognosis of these patients, attitude discussions should also be part of early management.

L'Acute on Chronic Liver Failure (ACLF) est une forme d'évolution défavorable de la maladie cirrhotique se distinguant de la cirrhose décompensée par une mortalité à court terme très élevée liée à une atteinte d'un ou plusieurs organes. La physiopathologie repose sur une réaction inflammatoire systémique, dont le facteur déclenchant peut être mis en évidence (infection, toxique, etc.) dans environ deux tiers des cas. L'analogie avec le sepsis a permis d'établir des scores pronostiques liés aux atteintes d'organes et ainsi de mieux orienter ces patients nécessitant le plus souvent une surveillance rapprochée. Le traitement reste limité et repose sur le soutien des organes atteints. Au vu du pronostic sombre de ces patients, des discussions d'attitude devraient également faire partie de la prise en charge précoce.

2D cine vs. 3D self-navigated free-breathing high-resolution whole heart cardiovascular magnetic resonance for aortic root measurements in congenital heart disease.

BACKGROUND: Cardiovascular magnetic resonance (CMR) is considered the method of choice for evaluation of aortic root dilatation in congenital heart disease. Usually, a cross-sectional 2D cine stack is acquired perpendicular to the vessel's axis. However, this method requires a considerable patient collaboration and precise planning of image planes. The present study compares a recently introduced 3D self-navigated free-breathing high-resolution whole heart CMR sequence (3D self nav) allowing a multiplanar retrospective reconstruction of the aortic root as an alternative to the 2D cine technique for determination of aortic root diameters. METHODS: A total of 6 cusp-commissure (CuCo) and cusp-cusp (CuCu) enddiastolic diameters were measured by two observers on 2D cine and 3D self nav cross-sectional planes of the aortic root acquired on a 1.5 T CMR scanner. Asymmetry of the aortic root was evaluated by the ratio of the minimal to the maximum 3D self nav CuCu diameter. CuCu diameters were compared to standard transthoracic echocardiographic (TTE) aortic root diameters. RESULTS: Sixty-five exams in 58 patients (32 ± 15 years) were included. Typically, 2D cine and 3D self nav spatial resolution was 1.1-1.52 × 4.5-7 mm and 0.9-1.153 mm, respectively. 3D self nav yielded larger maximum diameters than 2D cine: CuCo 37.2 ± 6.4 vs. 36.2 ± 7.0 mm (p = 0.006), CuCu 39.7 ± 6.3 vs. 38.5 ± 6.5 mm (p < 0.001). CuCu diameters were significantly larger (2.3-3.9 mm, p < 0.001) than CuCo and TTE diameters on both 2D cine and 3D self nav. Intra- and interobserver variabilities were excellent for both techniques with bias of -0.5 to 1.0 mm. Intra-observer variability of the more experienced observer was better for 3D self nav (F-test p < 0.05). Aortic root asymmetry was more pronounced in patients with bicuspid aortic valve (BAV: 0.73 (interquartile (IQ) 0.69; 0.78) vs. 0.93 (IQ 0.9; 0.96), p < 0.001), which was associated to a larger difference of maximum CuCu to TTE diameters: 5.5 ± 3.3 vs. 3.3 ± 3.8 mm, p = 0.033. CONCLUSION: Both, the 3D self nav and 2D cine CMR techniques allow reliable determination of aortic root diameters. However, we propose to privilege the 3D self nav technique and measurement of CuCu diameters to avoid underestimation of the maximum diameter, particularly in patients with asymmetric aortic roots and/or BAV.

Prognostic value of right ventricular dyssynchrony in adults with repaired tetralogy of Fallot.

OBJECTIVE: Residual sequelae after surgical repair of tetralogy of Fallot (rTOF) affect clinical outcome. We investigated the prognostic impact of right ventricular (RV) dyssynchrony in adults with rTOF years after the surgical repair. METHODS: Patients from the Swiss Adult Congenital HEart disease Registry were included. NT-proBNP levels, echocardiography, exercise testing and MRI data were collected. An offline strain analysis to quantify RV-ventricular and interventricular dyssynchrony was performed. The standard deviation of the time-to-peak shortening (TTP) of six RV segments defined the RV Dyssynchrony Index (RVDI). Maximal difference of TTP between RV and left ventricular segments defined the interventricular shortening delay (IVSD). Predictors of a composite adverse event (arrhythmias, hospitalisation for heart failure and death) were identified by multivariate Cox regression analysis. Their median values were used to create a risk score. RESULTS: Out of 285 included patients (mean age 34±14 years), 33 patients (12%) experienced an adverse event during a mean follow-up of 48±21 months. No correlation was found between RVDI, IVSD and clinical events. NT-proBNP, right atrial area and peak heart rate were independent predictors of outcomes. After 4 years-follow-up, no adverse events occurred in patients at low risk (score=0 points), while an adverse event occurred in 62% of patients at high risk (score=3 points, p<0.001). CONCLUSION: In our cohort of adults with rTOF, surrogates of RV dyssynchrony did not correlate with outcomes. A multimodality approach was effective in predicting the risk for adverse events.

Association of reduced peak left atrial strain with supraventricular arrhythmia in adults with congenital heart disease.

Atrial arrhythmias are an important cause of morbidity and mortality in adults with congenital heart disease (ACHD). In acquired heart disease, the left atrial (LA) strain has been shown to predict supraventricular tachyarrhythmias (SVT). This study aimed to investigate whether reduced LA strain is associated with SVT in ACHD patients. This retrospective, single-center cohort study collected baseline clinical and echocardiographic data of 206 ACHD patients (157 left heart defect, 49 right heart defect). Patients with sinus rhythm at baseline and a 5-year follow-up (median age 29, IQR 22-41 years) were included. Diagnosis of sustained SVT was determined from clinical reports during the follow-up period. New or recurrent sustained SVT occurred in 16 patients (7.8%, median follow-up of 6.2 years). Patients who developed SVT were older, more likely to have diastolic dysfunction, and had larger LA dimensions, left ventricular mass, and a lower peak LA longitudinal strain (PALS). Lower PALS was associated with higher risk of SVT in patients with left and right heart defects. Patients in the lowest quartile for PALS had a 15.9-fold higher hazard ratio of SVT (95% confidence interval, 4.5 to 56.0, p < 0.001) in comparison with the top three quartiles. PALS provides information about the occurrence of SVT in the ACHD population. Including measurement of LA strain in the follow-up of these patients may allow to better identify patients at risk of future atrial arrhythmias.