358 Clinical characteristic and natural history of chemotherapy induced dilated cardiomyopathy

Abstract Chemotherapy can lead to chemotherapy-induced dilated cardiomyopathy (CI-DCM), recognized as one of the Non-ischaemic Dilated Cardiomyopathy (DCM) phenotypes characterized by worse outcome. Evidences on a direct comparison between idiopathic-DCM (iDCM) and CI-DCM still lack. We included all the consecutive patients enrolled in the Trieste Muscle Heart Disease Registry. C-DCM was defined according to current recommendations. Uni- and multivariable analysis and Kaplan-Meier were performed. The primary outcome was all-cause death and the secondary outcomes were cardiac death and a composite of heart failure hospitalization, heart transplantation, ventricular assist-device implantation and major ventricular arrhythmias. The study included 511 patients (499 patients affected by iDCM and 52 patients affected by CI-DCM). Compared to iDCM, CI-DCM patients were older (51 ± 14 years vs. 58 ± 3 years respectively, P < 0.001) and had a higher LVEF (35%±10 vs. 32%±9, P = 0.03). CI-DCM patients had a higher incidence of all-cause of death compared to iDCM (36.5% vs. 8.4%, P < 0.001), while the incidence of cardiac death (7% in the CI-DCM group vs. 4% in the iDCM group, P = 0. 232) and of the composite secondary outcome was comparable amongst the two groups. At multivariable analysis, the diagnosis of CI-DCM was an independent predictor of primary outcome incidence (HR: 5.79, 95% CI: 1.83–18.27), P = 0.003, together atrial fibrillation. In a well-selected DCM cohort, patients with a chemotherapic etiology had a higher incidence of all-cause mortality compared to iDCM, while the incidence of cardiac adverse events was comparable among CI-DCM and iDCM

Reshaping care in the aftermath of the pandemic. Implications for cardiology health systems

In the last two years, the COVID-19 pandemic has undeniably changed everyday life and significantly reshaped the healthcare systems. Besides the direct effect on daily care leading to significant excess mortality, several collateral damages have been observed during the pandemic. The impact of the pandemic led to staff shortages, disrupted education, worse healthcare professional well-being, and a lack of proper clinical training and research. In this review we highlight the results of these important changes and how can the healthcare systems can adapt to prevent unprecedented events in case of future catastrophes

A case report of isolated cardiac light chain amyloidosis without clinically overt heart failure: an under-recognized presentation

Background: Cardiac involvement in amyloid light-chain (AL) amyloidosis usually represents a brick in the wall of a multi-system disease. The presence of cardiac deposition of free light chains (FLCs) is the main determinant of survival. Isolated cardiac AL is an uncommon scenario characterized by a challenging diagnostic and therapeutic workup. Case summary: A 57-year-old asymptomatic man was presented for an incidental finding of myocardial necrosis at the electrocardiogram (ECG) performed for newly diagnosed arterial hypertension. Alongside signs of previous myocardial infarction, transthoracic echocardiography showed a severely increased left ventricular (LV) wall thickness not consistent with ECG voltages, segmental akinaesia with normal LV systolic function with 'apical sparing' pattern. Laboratory assessment showed an unexpectedly high level of natriuretic peptide and persistently abnormal troponin in the absence of symptoms or signs of heart failure or ongoing ischaemia. Coronary angiogram confirmed the coronary artery disease. Before revascularization, a complete diagnostic workup was carried. Serum electrophoresis detected a monoclonal gammopathy that was further investigated by serum immunofixation, revealing high lambda FLCs concentration. Fat pad, bone marrow, and salivary glands biopsies resulted negative for amyloid deposition. Finally, endomyocardial biopsy was consistent with AL amyloidosis. Urgent percutaneous revascularization was performed, and the patients was timely started on chemotherapy. Discussion: The diagnosis of isolated cardiac AL amyloidosis is challenging and carries important therapeutic implications. As the short-term prognosis might be severely compromised, an accurate diagnostic flowchart has to be systematically pursued to obtain a precise diagnosis and address the optimal, tailored management

Transient versus persistent improved ejection fraction in non-ischaemic dilated cardiomyopathy

Aims The recent definition of heart failure with improved ejection fraction outlined the importance of the longitudinal assessment of left ventricular ejection fraction (LVEF). However, long-term progression and outcomes of this subgroup are poorly explored. We sought to assess the LVEF trajectories and their correlations with outcome in non-ischaemic dilated cardiomyopathy (NICM) with improved ejection fraction (impEF). Methods and results Consecutive NICM patients with baseline LVEF = 1 LVEF assessment after baseline were included. ImpEF was defined as a baseline LVEF = 10% point increase from baseline LVEF and LVEF >40%. Transient impEF was defined by the documentation of recurrent LVEF <= 40% during follow-up. The primary endpoint was a composite of all-cause death, heart transplantation and left ventricular assist device (D/HT/LVAD). Among 800 patients, 460 (57%) had impEF (median time to improvement 13 months). Transient impEF was observed in 189 patients (41% of the overall impEF group) and was associated with higher risk of D/HT/LVAD compared with persistent impEF at multivariable analysis (hazard ratio 2.54; 95% confidence interval 1.60-4.04). The association of declining LVEF with the risk of D/HT/LVAD was non-linear, with a steep increase up to 8% points reduction, then remaining stable. Conclusions In NICM, a 57% rate of impEF was observed. However, recurrent decline in LVEF was observed in approximate to 40% of impEF patients and it was associated with an increased risk of D/HT/LVAD

Correction: Diuretic dose trajectories in dilated cardiomyopathy: prognostic implications

Within the abstract, the following phrase in the ‘Methods’ section “According to FED trajectory, patients were classified as (i) dose (FED increase by ≥ 50% or newly initiated);” was corrected to read “According to FED trajectory, patients were classified as (i) dose ↑ (FED increase by ≥ 50% or newly initiated);”. In the ‘Results’ section of the abstract, the sentence “Baseline FED was independently associated with outcome (HR per 20 mg increase: 1.12 [95% CI 1.04–1.22, p = 0.003].” was corrected to “Baseline FED was independently associated with outcome (HR per 20 mg increase: 1.12 [95% CI 1.04–1.22], p = 0.003).” Finally, in Table 1, the LVEF, % for Dose↓ patients was given incorrectly whenit should have been “28 (22-34)” and the N value has been corrected from “263” to “282”. The original article has been corrected

Contemporary etiology and prognosis of dilated non-ischemic cardiomyopathy

Non-ischemic dilated cardiomyopathy (NI-DCM) represents a specific etiology of systolic heart failure that usually affect young individuals with a genetic background in up to 40% of cases. Behind the term NI-DCM there is a spectrum of different diseases, and an accurate etiological classification appears pivotal for the clinical management and prognostic stratification of these patients

Structural and functional development in airways throughout childhood: Children are not small adults

Children are not small adults and this fact is particularly true when we consider the respiratory tract. The anatomic peculiarities of the upper airway make infants preferential nasal breathers between 2 and 6 months of life. The pediatric larynx has a more complex shape than previously believed, with the narrowest point located anatomically at the subglottic level and functionally at the cricoid cartilage. Alveolarization of the distal airways starts conventionally at 36-37 weeks of gestation, but occurs mainly after birth, continuing until adolescence. The pediatric chest wall has unique features that are particularly pronounced in infants. Neonates, infants, and toddlers have a higher metabolic rate, and consequently, their oxygen consumption at rest is more than double that of adults. The main anatomical and functional differences between pediatric and adult airways contribute to the understanding of various respiratory symptoms and disease conditions in childhood. Knowing the peculiarities of pediatric airways is helpful in the prevention, management, and treatment of acute and chronic diseases of the respiratory tract. Developmental modifications in the structure of the respiratory tract, in addition to immunological and neurological maturation, should be taken into consideration during childhood

Sacubitril/Valsartan Induces Global Cardiac Reverse Remodeling in Long-Lasting Heart Failure with Reduced Ejection Fraction: Standard and Advanced Echocardiographic Evidences

Sacubitril/valsartan reduces mortality in heart failure with reduced ejection fraction (HFrEF) patients, partially due to cardiac reverse remodeling (RR). Little is known about the RR rate in long-lasting HFrEF and the evolution of advanced echocardiographic parameters, despite their known prognostic impact in this setting. We sought to evaluate the rates of left ventricle (LV) and left atrial (LA) RR through standard and advanced echocardiographic imaging in a cohort of HFrEF patients, after the introduction of sacubitril/valsartan. A multi-parametric standard and advanced echocardiographic evaluation was performed at the moment of introduction of sacubitril/valsartan and at 3 to 18 months subsequent follow-up. LVRR was defined as an increase in the LV ejection fraction 6510 points associated with a decrease 6510% in indexed LV end-diastolic diameter; LARR was defined as a decrease >15% in the left atrium end-systolic volume. We analyzed 77 patients (65 \ub1 11 years old, 78% males, 40% ischemic etiology) with 76 (28-165) months since HFrEF diagnosis. After a median follow-up of 9 (interquartile range 6-14) months from the beginning of sacubitril/valsartan, LVRR occurred in 20 patients (26%) and LARR in 33 patients (43%). Moreover, left ventricular global longitudinal strain (LVGLS) improved from -8.3 \ub1 4% to -12 \ub1 4.7% (p 25% of cases, both at standard and advanced echocardiographic evaluations

Sex-Specific Prognostic Implications in Dilated Cardiomyopathy After Left Ventricular Reverse Remodeling

Background. Women affected by Dilated Cardiomyopathy (DCM) experience better outcomes compared to men. Whether a more pronounced Left Ventricular Reverse Remodelling (LVRR) might explain this is still unknown. Aim. We investigated the relationship between LVRR and sex and its long-term outcomes. Methods. A cohort of 605 DCM patients with available follow-up data was consecutively enrolled. LVRR was defined, at 24-month follow-up evaluation, as an increase in left ventricular ejection fraction (LVEF) 65 10% or a LVEF > 50% and a decrease 65 10% in indexed left ventricular end-diastolic diameter (LVEDDi) or an LVEDDi 64 33 mm/m2. Outcome measures were a composite of all-cause mortality/heart transplantation (HTx) or ventricular assist device (VAD) and a composite of Sudden Cardiac Death (SCD) or Major Ventricular Arrhythmias (MVA). Results. 181 patients (30%) experienced LVRR. The cumulative incidence of LVRR at 24-months evaluation was comparable between sexes (33% vs. 29%; p = 0.26). During a median follow-up of 149 months, women experiencing LVRR had the lowest rate of main outcome measure (global p = 0.03) with a 71% relative risk reduction compared to men with LVRR, without significant difference between women without LVRR and males. A trend towards the same results was found regarding SCD/MVA (global p = 0.06). Applying a multi-state model, male sex emerged as an independent adverse prognostic factor even after LVRR completion. Conclusions. Although the rate of LVRR was comparable between sexes, females experiencing LVRR showed the best outcomes in the long term follow up compared to males and females without LVRR. Further studies are advocated to explain this difference in outcomes between sexes