Poor right ventricular function is associated with impaired exercise capacity and ventilatory efficiency in transthyretin cardiac amyloid patients.

CardioPulmonary Exercise Test (CPET) is the gold standard to evaluate functional capacity in patients at high risk of heart failure (HF). Few studies with a limited number of subjects and conflicting results, analyzed the role of CPET in patients with systemic amyloidosis. Aims of our study were the assessment of the response to exercise in patients with Transthyretin amyloid (ATTR) cardiomyopathy (CA), and the correlation of clinical, biohumoral and echocardiographic parameters with CPET parameters, such as VO2 peak and VE/VCO2 slope. From February 2018 to March 2019, 72 cardiac ATTR patients were prospectively enrolled and underwent a complete clinical, biohumoral, echocardiographic and CPET assessment. All patients completed the exercise stress test protocol, without any adverse event. At CPET, they achieved a mean VO2 peak of 14 mL/Kg/min and a mean VE/VCO2 slope of 31. The blood pressure response to exercise was inadequate in 26 (36%) patients (flat in 25 and hypotensive in 1), while 49/72 patients (69%) showed an inadequate heart rate recovery. In multivariate analysis, s' tricuspidalic was the only independent predictor of VO2 peak, while in the two test models performed to avoid collinearity, both TAPSE and s' tricuspidalic were the strongest independent predictors of VE/VCO2 slope. Our data demonstrate the role of right ventricular function as an independent predictor of exercise capacity and ventilatory efficiency in ATTR. In CPET evaluation, a significant proportion of patients presented an abnormal arterial pressure response and heart rate variation to exercise.

Long-Term Mortality Comparison of Patients With Acute Myocardial Infarction Complicated by Cardiogenic Shock and Treated With Culprit-Only or Multivessel Percutaneous Coronary Intervention.

OBJECTIVES: We sought to determine whether, in a real word context of patients with Acute Myocardial Infarction (AMI), multivessel disease (MVD) and cardiogenic shock (CS), the successful treatment with primary percutaneous coronary intervention (p-PCI) of only culprit lesions (OC-PCI) is associated with better long-term mortality rates than multivessel PCI (MV-PCI) of all significant lesions. METHODS: From our registry of all consecutive patients admitted for AMI between January 1995 and December 2016 we selected those presenting with CS and MVD successfully treated with p-PCI, and compared those who underwent OC-PCI against MV-PCI, either during the p-PCI (MV-pPCI) or by staged revascularization (Staged-PCI) during hospitalization. The primary endpoint was 2-year all-cause death. RESULTS: Among 4210 patients with AMI, 406 (9.6%) presented CS (Killip class IV). A total of 292 patients had MVD. Of them, 252 (86.3%) were successfully treated with p-PCI, 159 patients with OC-PCI and 93 with MV-PCI, either in the same (n = 29) or staged procedure (n = 64). At 2-year follow-up the overall mortality was 47.6%, lower in MV-PCI group (37.6% vs 53.5% in OC-PCI, p = 0.019). Diabetes (HR = 1.50, 1.01-2.22), three vessel disease (HR = 1.49, 1.02-2.17) and basal left ventricular ejection fraction <15% (HR = 3.39, 2.41-6.27) were independent predictors of mortality, while MV-PCI was the only variable associated with improved survival (HR = 0.54, 0.36-0.81). CONCLUSIONS: In this real world registry of AMI patients with MVD presenting CS, MV-PCI was associated with better long-term survival.

Electromechanical dissociation of left atrium in patients with Cardiac Amyloidosis by Magnetic Resonance: Prognostic and clinical correlates.

OBJECTIVES: Left atrial (LA) function is an important marker of hemodynamic status in cardiac amyloidosis (CA), and its characterization may provide relevant prognostic information. We sought to assess the prevalence and prognostic impact of LA dysfunction by cardiac magnetic resonance (CMR) in patients with CA. METHODS: We performed CMR in 80 consecutive patients with CA, including 38 with AL (47%) and 42 with ATTR (53%). LA function was assessed by acquiring short axis cine steady-state free precession (SSFP) covering the entire chamber. The atrial emptying fraction (AEF) was calculated as the ratio between the difference of LA maximal and minimal volume to LA maximal volume, expressed as percentage. Severe atrial dysfunction was defined as AEF ≤ 14%. RESULTS: Mean AEF was 18% (13-35%). Overall, AEF ≤ 14% was present in 19 patients (24%), including 21% of those in sinus rhythm (SR) with no history of atrial fibrillation (AF). After a median of 3 years (IQR 2-4), 36 patients (44%) died of cardiac causes. Patients with AEF ≤ 14% showed increased cardiac mortality, with an independent OR of 4.2 (95 IC 2.1-8.2, P < 0.0001). Of note, AEF ≤ 14% was the stronger independent predictor of cardiac death. Patients in SR with AEF ≤ 14% had worse outcome than those with AF. CONCLUSIONS: Severe impairment of LA contractile function was present in three-quarters of patients with CA, and was prevalent irrespective of CA etiology, both in the presence and absence of AF. Severe LA dysfunction was associated with an independent 4-fold increase in risk for cardiac death at three years.

Prevalence, causes and predictors of cardiovascular hospitalization in patients with hypertrophic cardiomyopathy.

BACKGROUND: Despite numerous studies assessing the natural history of patients with hypertrophic cardiomyopathy (HCM), there is lack of data regarding the burden of hospitalization. Aim of this study was to describe prevalence, causes and predictors of cardiovascular hospitalization in patients with HCM. METHODS: We retrospectively included 253 patients with HCM undergoing first evaluation at our center. Enrolment criteria included cardiac magnetic resonance imaging (CMRI) at baseline and > 1-year follow-up. All hospital admissions were recorded during follow-up and adjudicated as acute vs elective and cardiovascular (CV) vs non-cardiovascular (non-CV). RESULTS: During 6.4 ± 4.0 years there were 187 hospitalizations in 92 patients (36%, at a rate of 5.7%/year). Most were CV-related (158/187,84.5%; 4.8%/year) while non-CV admissions were 29/187 (15.5%, 0.88%/year). There was a slight predominance of elective (n = 96, 58%, 2.8%/year) vs acute (n = 62, 41.8%, 2.0%/year) CV hospitalizations. Independent predictors of CV hospitalization were baseline symptoms (NYHA class II vs I: HR 2.06; 95% CI 1.24-3.43, NYHA III-IV vs I: HR 3.05; 95% CI 1.40-6.65, p = .004), indexed left atrial (LA) volume (HR 1.03; 95% CI 1.01-1.04, p < .001), and lower indexed right ventricular end-diastolic volume iRVEDV) at cardiac magnetic resonance (HR 0.99; 95% CI 0.97-0.99, p = .03). CONCLUSIONS: In little over 6 years, CV hospitalization was required in over one-in-three of our HCM patients, often unplanned and due to acute disease-related complications. Symptomatic status, larger LA volume and reduced iRVEDV at baseline were independently associated with CV admissions. Strategies aimed at preventing hospitalizations are an important target to reduce the burden of disease in HCM patients.

Anticoagulation resumption after intracranial hemorrhage in patients treated with VKA and DOACs.

BACKGROUND: Intracranial hemorrhage (ICH) is associated with severe prognosis and recurrent risk. This impacts on the decision to resume anticoagulation in atrial fibrillation (AF) or venous thromboembolism (VTE) patients. Purpose of our study is to evaluate the incidence rate of recurrent ICH in patients with AF or VTE resuming anticoagulation after a first ICH episode. METHODS: We report data of two cohorts of AF or VTE after a first ICH. The Vitamin K antagonist (VKA) cohort (166 patients) derives from CHIRONE Study, the direct oral anticoagulant (DOAC) cohort (178 patients) derives from START2-Register RESULTS: The clinical characteristics of the two cohort are similar with the exception of more prevalence of history of previous stroke/TIA in DOAC patients with respect to VKA (p = 0.02) and serum creatinine levels>1.5 mg/dL in VKA patients with respect to DOAC(p = 0.0001). The index ICH was spontaneous in 66.4% and in 33.7% among DOAC and VKAs cohort respectively (p = 0.0001). During follow-up, 14 recurrent ICH were recorded; 9 (rate 2.5 × 100 patient-years) in VKA and 5 (rate 1.3 × 100 patient-years) in DOAC (Relative Risk 1.9; 95% CI 0.6-7.4; p = 0.2). The univariate logistic regression analysis showed that patients with recurrent ICH were more frequently males, hypertensive, with a history of previous Stroke/TIA and older than patients without recurrence. VKA patients showed a higher risk of recurrence with respect to DOAC patients (OR 1.9;95% CI 0.7-6.7). CONCLUSIONS: A trend toward fewer ICH recurrences was detected among DOACs patients in comparison to the previously reported rate of patients on warfarin.

Biomarkers and Prediction of Prognosis in Transthyretin-Related Cardiac Amyloidosis: Direct Comparison of Two Staging Systems.

BACKGROUND: The severity of heart disease varies widely among patients with transthyretin-related cardiac amyloidosis (ATTR-CA) at presentation, and availability of tools able to predict prognosis is essential for clinical and research purposes. Currently, two biomarker-based staging systems are available. The aim of this study was to compare their predictive performance. METHODS: A total of 175 patients diagnosed with ATTR-CA (133 wild-type and 42 hereditary) were stratified into different stages based on 2 systems: the first system included N-terminal pro-B-type natriuretic peptide (NT-proBNP) and estimated glomerular filtration rate (eGFR), and the second one included NT-proBNP and troponin I (TnI). Survival estimates and age-adjusted survival for all-cause mortality were analysed over a median follow-up of 27 months (interquartile range 16-43 months). RESULTS: Predictive performance was more accurate when NT-proBNP and eGFR were used, resulting in effective survival stratification: 64.4 months for stage 1, 44.6 months for stage 2, and 20.5 months for stage 3 (P < 0.01 for stages 1 vs 2; P < 0.0001 for stages 1 vs 3; P < 0.0001 stages 2 vs 3). The combination of NT-proBNP and TnI was unable to effectively differentiate survival: 64.5 months for stage 1, 50.9 months for stage 2, and 27.3 months for stage 3 (P = 0.223 for stages 1 vs 2; P < 0.0001 for stages 1 vs 3; P < 0.0001 for stages 2 vs 3). The same results were seen after age adjustment. CONCLUSIONS: A staging system using NT-proBNP and eGFR had better prognostic accuracy for ATTR-CA patients compared with one using NTproBNP and TnI.

Very elderly patients with venous thromboembolism on oral anticoagulation with VKAs or DOACs: Results from the prospective multicenter START2-Register Study.

INTRODUCTION: Few data are available on the safety of anticoagulation in very elderly patients treated with Vitamin K antagonists (VKAs) and direct oral anticoagulants (DOACs) for venous thromboembolism (VTE). METHODS: We carried out a prospective cohort study on VTE patients aged ≥85 years enrolled in the Survey on anticoagulaTed pAtients RegisTer (START2-Register) on treatment with VKAs or DOACs, with the aim to evaluate mortality, bleeding and thrombotic rates (venous and arterial). RESULTS: We enrolled 272 patients, 58.7% on VKA and 41.3% on DOACs. Baseline characteristics were similar between treatment groups, with a higher prevalence of renal failure in VKAs patients and of a history of bleeding and previous stroke/TIA in DOACs patients. During follow-up of 429 patient-years, 15 major and non-major clinically relevant bleedings were recorded (rate 3.5 × 100 pt-yrs), 5 were major bleeds (rate 1.2 × 100 pt-yrs), 1 in a patient on aspirin (rate 4.3 × 100 pt-yrs). Bleeding rate was higher in patients on DOACs (crude HR 4.7; 95%CI 1.5-15.01). Eight thrombotic events were recorded (rate 1.9 × 100 pt-yrs), 3 recurrent VTE and 5 stroke/TIA. Overall, the incidence of thrombotic events was higher in DOACs patients (crude HR 4.5; 95% CI 1.5; 13.3). The rate of recurrent VTE was similar in the two group. Mortality rate was significantly lower in DOACs patients (crude HR 0.30; 95% CI 0.1;0.9). CONCLUSION: A higher bleeding risk was found in very elderly VTE patients on DOACs despite the wide use of low-dosages. Similarly a higher thrombotic risk was found while the incidence of recurrent VTE was low and similar between the groups. Mortality rate were significantly lower in DOACs patients.

[Ventricular storm in a young man with primary hypokinetic dilated cardiomyopathy: why thinking of sarcoidosis?] / Storm ventricolare in un giovane con cardiomiopatia ipocinetico-dilatativa primitiva: perché pensare alla sarcoidosi.

The diagnostic approach to patients with cardiac sarcoidosis is challenging, as the disease may occur as a subclinical entity or have heterogeneous clinical manifestations ranging from ventricular arrhythmias to advanced cardiac failure. Therefore, while clinical suspicion remains key, imaging techniques such as nuclear magnetic resonance imaging and myocardial scintigraphy play an important confirmatory role. Final diagnosis requires histological proof on cardiac or extracardiac biopsy. A multidisciplinary context is essential for appropriate diagnosis, staging and management. We present the case of a young man with dilated cardiomyopathy in whom, following the onset of malignant and recurrent ventricular arrhythmias, a final diagnosis of cardiac sarcoidosis was reached based on a host of invasive and non-invasive diagnostic techniques, allowing tailored treatment.