Prognostic value of left atrial mechanics in cardiac light-chain amyloidosis with preserved ejection fraction: a cohort study.

BACKGROUND: Light-chain amyloidosis is a plasma cell disorder associated with poor outcomes, especially when the heart is involved. The characteristics of left atrial (LA) function and its prognostic implications in cardiac amyloidosis (CA) have not been fully investigated. METHODS: Between April 2014 and June 2019, 93 patients with a diagnosis of CA, normal left ventricular ejection fraction (LVEF) and sinus rhythm were included. Their clinical, baseline echocardiographic and follow-up data were investigated. LA function, including LA strain and strain rate, was assessed using 2D speckle tracking echocardiography in different LA functional phases. RESULTS: Among all patients, 38 (40.9%) died. Multivariate Cox regression analyses showed that LA mechanics regarding LA reservoir and booster pump functions were independent predictors for overall survival. Traditional echocardiographic parameters for LA structure like LA volume index and LA width were not associated with mortality. Moreover, LA strain and strain rate in reservoir and contractile phases improved the discrimination and goodness of fit of the conventional prognostic model, the Mayo criteria 2004 and 2012, in our study population. Decreased LA mechanics were associated with impaired left ventricular (LV) systolic and diastolic function, and LA reservoir and contractile functions were associated with LA structure. CONCLUSIONS: Assessment of LA reservoir and contractile functions via 2D speckle tracking echocardiographic LA mechanical indices provide clinical and prognostic insights into cardiac light-chain amyloidosis patients, especially those with preserved EF and sinus rhythm. Emphasizing the monitoring of LA function may be beneficial for the prognosis prediction of CA.

The quest for diagnostic approaches of cardiac involvement in polymyositis and dermatomyositis.

Polymyositis (PM) and dermatomyositis (DM) are autoimmune diseases characterized by inflammation of skeletal muscle, primarily manifesting as chronic muscle weakness. Extramuscular organs can also be affected. Cardiac involvement is one of the visceral organ damages whose prevalence is underestimated and is a marker of poor prognosis leading to irreversible dysfunction or even death. Although early and accurate recognition of cardiac involvement remains a key barrier to improving survival in PM/ DM patients, considerable progress has been made, and an overview will be provided in this review. The new concept of multimodality imaging, which involves an integrated approach of echocardiography (Echo), cardiac magnetic resonance and sometimes positron emission tomography (PET), can facilitate diagnosis. The development of ultrasound technology, including strain analysis, stress Echo and contrast-enhanced Echo, helps disclose early cardiac dysfunction more sensitively than conventional Echo. Cardiac magnetic resonance unveils silent, acute or chronic myocarditis in PM/DM and is used to monitor treatment efficacy due to its excellent tissue characterization. PET can be useful thanks to the appearance of new tracers that can eliminate the effects of glucose uptake by normal cardiomyocytes. The sensitivity of endomyocardial biopsy may be increased by targeted sampling with the guidance of cardiac imaging. Troponin I is specific to cardiac injury, and investigations into antibodies against cardiac tissue are being carried out. Disease-specific mechanisms and therapies are also discussed to give more insights into cardiac involvement in PM and DM.

Usefulness of Myocardial Strain and Twist for Early Detection of Myocardial Dysfunction in Patients With Autoimmune Diseases.

Cardiac involvement in autoimmune diseases (AD) is common but underdiagnosed due to a lack of sensitive imaging methods. We aim to evaluate the characteristics of left ventricular (LV) systolic dysfunction in patients with AD using deformational parameters from 2-dimensional speckle-tracking echocardiography (STE). We retrospectively enrolled 86 AD patients and 71 healthy controls. All subjects underwent transthoracic echocardiography and STE to analyze LV strain and twist. A twist-radial displacement loop was constructed to investigate the relation between LV contractility and dimension. In AD patients, 68 had preserved LV ejection fraction (EF ≥ 50%), and 18 had reduced LVEF (EF < 50%). The patients with preserved LVEF exhibited significantly lower values of global longitudinal, circumferential, and radial strain than controls (-19.11 ± 4.18 vs -21.49 ± 2.53%, -25.17 ± 5.04% vs -27.37 ± 2.87%, 17.68 ± 5.69% vs 21.17 ± 6.44%, respectively; all p <0.01) and a marked attenuation in peak twist (14.24 ± 5.57 vs 18.10 ± 5.97, p <0.01) attributed to impaired apical rotation (9.03 ± 5.17 vs 12.79 ± 5.99, p <0.01). AD patients were more likely to present with abnormal loop types with flat ascending slope and delayed peak twist time. In conclusion, abnormal strain and twist precede deterioration in LVEF, suggesting early myocardial involvement in AD. STE can be used as a good alternative for early detection of myocardial dysfunction in AD patients.

Left ventricular strain is associated with acute postoperative refractory hypotension in patients with constrictive pericarditis and preserved ejection fraction.

BACKGROUND: Pericardiectomy is an effective treatment for constrictive pericarditis (CP). Early postoperative complications such as refractory hypotension and congestive heart failure occur in these patients and are associated with increased morbidity and mortality. We hypothesized that left ventricular (LV) myocardial strain measured by two-dimensional speckle tracking echocardiography (2DSTE) could identify early cardiac dysfunction and relate to acute postoperative adverse events in CP patients. METHODS: Forty-four CP patients with preserved left ventricular ejection fraction (LVpEF, 64%±8%) and 44 age- and sex-matched controls were enrolled. Conventional 2DSTE was performed before pericardiectomy. Global and segmental peak systolic strain values were measured. The primary endpoint was a composite of postoperative refractory hypotension, congestive heart failure and cardiogenic death. Refractory hypotension was defined as hypotension requiring prolonged usage of intravenous inotropic medication (IVIM) (≥2 days). RESULTS: Postoperative refractory hypotension occurred in 26 cases, and no patients experienced congestive heart failure or cardiogenic death. Compared to controls, CP patients had decreased absolute global and segmental circumferential strain (CS), radial strain (RS), and longitudinal strain (LS) except septal LS. Patients with refractory hypotension exhibited lower epicardial CS (P=0.04). Epicardial CS was an independent risk factor correlated with postoperative adverse outcome [P=0.014, OR =1.236 (1.044-1.464)] while LVEF was not. Lower absolute value of epicardial CS was related to higher (P=0.02) and longer usage of intravenous furosemide (P=0.04) to keep negative fluid balance perioperatively. CONCLUSIONS: LV strain value is markedly reduced in patients with CP and LVpEF. Lower preoperative epicardial CS value is associated with greater risk of early refractory hypotension and more aggressive fluid management.

Short-term and 1-year outcomes after MitraClip therapy in functional versus degenerative mitral regurgitation patients: a systematic review and meta-analysis.

BACKGROUND: Differences in short-term and 1-year outcomes of percutaneous edge-to-edge mitral repair between patients with functional and degenerative mitral regurgitation (MR) remain unclear. We performed a systematic review and meta-analysis to investigate the safety and efficacy of MitraClip (MC) in patients with different MR etiologies. METHODS: This study systematically searched three common databases for studies on MC therapy until November 2017. The studies meeting the standard inclusion criteria were included. The data at baseline, short-term and 1-year clinical and echocardiographic outcomes were obtained and analyzed. All data were checked by another reviewer. RESULTS: Thirteen studies totalling 2,351 patients investigating the short-term and 1-year outcomes of MC in patients with functional MR (FMR) versus degenerative MR (DMR) were included for further analysis. FMR patients presented a higher risk profile at baseline. There was no difference in short-term outcomes between DMR and FMR for post-procedural MR grade 0-2 (76.8% vs. 77.1%; P=0.428), mean trans-mitral gradient (3.92 vs. 3.50 mmHg; P=0.098), 30-day mortality rate (0.05% vs. 0.03%; P=0.118) and 30-day NYHA I-II (85.3% vs. 78.7%; P=0.211). FMR patients had a higher rate of acute procedural success compared to the DMR patient group (91.2% vs. 95.2%; P=0.016). A greater portion of DMR patients implanted two or more MCs than the FMR patients (41.4% vs. 35.7%; P=0.043). For the 1-year outcomes, no difference was found in the mortality rate (13.0% vs. 15.2%; P=0.268) and proportion of patients with post-procedural MR grades 0-2 (75.0% vs. 80.7%; P=0.106). CONCLUSIONS: Despite a higher risk profile in FMR patients, the short-term and 1-year outcomes were not significantly different. We conclude that MC therapy is similar between FMR and DMR patients until 1-year follow-up. Large randomized trials are warranted to fully and further assess the clinical impact of the procedure in these two MR etiologies over a longer period of time.