Some patients with Takotsubo cardiomyopathy (TTC) develop cardiogenic shock due to left ventricular outflow tract (LVOT) obstruction – there is, however, a paucity of data regarding this condition. Prevalence, associated factors and management implications of LVOT obstruction in TTC was explored, based on two-year data from two Belgian heart centres. A total of 32 patients with TTC were identified out of 3,272 patients presenting with troponin-positive acute coronary syndrome. In six patients diagnosed with TTC (19%), a significant LVOT obstruction was detected by transthoracic echocardiography. Patients with LVOT obstruction were older and had more often septal bulging, and presented more frequently in cardiogenic shock as compared to those without LVOT obstruction (P < 0.05). Moreover, all patients with LVOT obstruction showed systolic anterior motion (SAM) of the anterior mitral valve leaflet, which was associated with a higher grade of mitral regurgitation (2.2±0.7 vs. 1.0±0.6, P<0.001). Adequate therapeutic management including fluid resuscitation, cessation of inotropic therapy, intravenous β-blocker, and the use of intra-aortic balloon pump resulted in non-inferior survival in TTC patients with LVOT obstruction as compared to those without LVOT obstruction. TTC is complicated by LVOT obstruction in approximately 20% of cases. Older age, septal bulging, SAM-induced mitral regurgitation and hemodynamic instability are associated with this condition. Timely and accurate diagnosis of LVOT obstruction by echocardiography is key to successful management of these TTC patients with LVOT obstruction and results in a non-inferior outcome as compared to those patients without LVOT obstruction.

Left ventricular (LV) systolic dysfunction in cardiac amyloidosis (CA) is associated with poor prognosis. This study aimed to investigate the prognostic implications of right ventricular (RV) systolic dysfunction in CA. A total of 93 patients diagnosed with CA who underwent standard and speckle-tracking echocardiography were included. During a median follow-up of 17 (5 to 38) months, 42 patients (45%) died. Nonsurvivors were more likely to present with immunoglobulin light-chain amyloidosis and New York Heart Association class III to IV heart failure symptoms. Regarding the echocardiographic characteristics, nonsurvivors had a higher LV apical ratio, worse LV diastolic function, and worse RV systolic function (evaluated with both tricuspid annular plane systolic excursion and RV free wall strain). RV free wall strain was independently associated with all-cause mortality in several multivariable Cox regression models and had incremental prognostic value over conventional parameters of RV function when added to a basal model (including heart failure symptoms, amyloidosis phenotype, and LV global longitudinal strain). Based on spline curve analysis and Youden index, a value of 16% for RV free wall strain was identified as the optimal cutoff to predict outcome and patients with RV free wall strain <16% had a significantly worse short- and long-term survival during follow-up (1- and 3-year cumulative survival: 81% vs 31% and 67% vs 20%, respectively, p <0.001). In conclusion, RV systolic dysfunction is independently associated with poor outcome in patients with CA and the use of advanced echocardiographic parameters, such as RV free wall strain, may be of aid for better risk stratification.

Background and Aims: In real-world, wild-type transthyretin cardiomyopathy is increasingly diagnosed in patients ≥ 80 years old (octogenarians), although being underrepresented in randomized clinical trials. Specific data on natural course and outcome under tafamidis treatment in octogenarians are therefore scarce. The impact of tafamidis treatment on mortality in real-world wild-type transthyretin cardiomyopathy octogenarians was studied. Methods: An international, multicentre cohort study of 710 consecutive wild-type transthyretin cardiomyopathy patients with mean follow-up of 2.2 ± 1.8 years for all-cause mortality endpoint was performed. Results: The cohort consisted of 58.5% (415/710) octogenarians (85 ± 4 years, 74.2% male). Before tafamidis availability, natural course in octogenarians (148/257) vs. non-octogenarians (109/257) was poor, with 16% 1-year and 71% 5-year mortality vs. 8% and 47%, respectively (P < .001). Since tafamidis availability, 70.1% (253/361) octogenarians were initiated on tafamidis vs. 83.7% (231/276) non-octogenarians (P < .001). Tafamidis discontinuation was similar (octogenarians 10.3% and non-octogenarians 7.4%; P = .260). Overall tafamidis treated vs. untreated octogenarians had better unadjusted survival (P < .001), with 5% 1-year and 24% 3-year mortality. Tafamidis treatment associated with lower mortality after propensity score matching on baseline variables, including age, National Amyloidosis Centre stage, and New York Heart Association class in on average 394 subjects [hazard ratio (HR) = 0.53, 95% confidence interval (CI) 0.34–0.84, P = .007], also in octogenarians (HR = 0.57, 95% CI 0.33–1.01, P = .053). Neither age at diagnosis (P = .217) nor at treatment initiation (P = .154) interacted with tafamidis mortality benefit. Octogenarians had poorer survival despite tafamidis, when initiated at ≥90 years (HR = 3.3, 95% CI 1.10–9.53, P = .033) and National Amyloidosis Centre Stage ≥3 (HR = 2.4, 95% CI 0.87–6.46, P = .090). Conclusions: Real-world tafamidis treatment improves survival without age affecting treatment efficacy, although mortality remains considerable in octogenarians.

Cardiac amyloidosis (CA) is often overlooked or misdiagnosed. Effects of growing disease awareness, diagnostic ameliorations and novel treatment options on CA diagnosis and management are scarcely ...

BACKGROUND: A formal prognostic staging system in wild-type transthyretin amyloid cardiomyopathy (ATTRwt-CM), based on echocardiographic imaging, is lacking. We evaluated the prognostic performance of global longitudinal strain (GLS) staging in a large cohort of patients with ATTRwt-CM, including under tafamidis treatment and relative to National Amyloidosis Center (NAC) biomarker staging. METHODS: A multicentric, international ATTRwt-CM patient cohort with baseline GLS (distribution quartiles), evaluated by echocardiography, was studied, related to all-cause mortality. RESULTS: The study comprised 816 patients with ATTRwt-CM, median age of 81.5 years, 83% males, and 72% tafamidis initiated. During a 2.2-year median follow-up, 29.7% of patients died. GLS worsened with increasing NAC disease stage (I: −14.3%, II: −11.6%, III: −11.4%; P <0.001). Median survival per baseline GLS quartile stage 1 (<−15.8%), 2 (−15.8 to −12.9%), 3 (−12.8 to −10.0%), and 4 (GLS >−10.0%) was not met, 6.7, 4.6, and 3.4 years, respectively ( P <0.001). The median GLS −12.8% cutoff predicted 1-year mortality with 74% sensitivity, 52% specificity (area under the curve, 0.73 [95% CI, 0.66–0.80]; P <0.001). GLS was the only independent echocardiographic and strong mortality predictor, independent of other predictors, including age, New York Heart Association class symptoms, NAC stage, and tafamidis treatment (hazard ratio, 1.08 [95% CI, 1.04–1.12]; P <0.001), also when restricted to 591 tafamidis-treated subjects (hazard ratio, 1.15 [95% CI, 1.08–1.22]; P <0.001). Baseline GLS −12.8% cutoff value provided further prognostic discriminative ability for mortality within each NAC disease stage stratum (all P <0.050). Likelihood ratio test indicated incremental prognostic value of GLS (staging) over baseline NAC staging ( P <0.001). CONCLUSIONS: GLS is a strong, independent mortality predictor in ATTRwt-CM, irrespective of tafamidis treatment, that may be an adjunct or complementary to biomarker staging.