Echocardiographic Assessment of Ebstein's Anomaly in a 60-Year-Old Man

We present an echocardiographic evaluation of an elderly man affected with Ebstein's anomaly. In the natural history of this congenital disease only 5% of patients survive beyond the fifth decade. The patient presented severe right heart failure and he was refered to our institution for heart transplantation

RV longitudinal deformation correlates with myocardial fibrosis in patients with end-stage heart failure

Objectives This study was performed to determine the accuracy of right ventricular (RV) longitudinal strain (LS) in predicting myocardial fibrosis in patients with severe heart failure (HF) undergoing heart transplantation. Background RVLS plays a key role in the evaluation of its systolic performance and clinical outcome in patients with refractory HF. Methods We studied 27 patients with severe systolic HF (ejection fraction 25% and New York Heart Association functional class III to IV, despite full medical therapy and cardiac resynchronization therapy) using echocardiography before heart transplantation. RV free wall LS, right atrial LS, sphericity index (SI), and tricuspid annular plane systolic excursion (TAPSE) were all measured. Upon removal of the heart, from the myocardial histologic analysis, the ratio of the fibrotic to the total sample area determined the extent of fibrosis (%). Results RV myocardial fibrosis correlated with RV free wall LS (r = 0.80; p < 0.0001), SI (r = 0.42; p = 0.01) and VO max (r = -0.41; p = 0.03), with a poor correlation with TAPSE (r = -0.34; p = 0.05) and right atrial LS (r = -0.37; p = 0.03). Stepwise multivariate analysis showed that RV free wall LS (β = 0.701, p < 0.0001) was independently associated with RV fibrosis (overall model R= 0.64, p < 0.0001). RV free wall LS was the main determinant of myocardial fibrosis. In the subgroup of patients with severe RV fibrosis, RV free wall LS had the highest diagnostic accuracy for detecting severe myocardial fibrosis (area under the curve = 0.87; 95% confidence interval: 0.80 to 0.94). Conclusions In late-stage HF patients, the right ventricle is enlarged, with reduced systolic function due to significant myocardial fibrosis. RV free wall myocardial deformation is the most accurate functional measure that correlates with the extent of RV myocardial fibrosis and functional capacity

A multidisciplinary approach for the emergency care of patients with left ventricular assist devices: A practical guide

The use of a left ventricular assist device (LVAD) as a bridge-to-transplantation or destination therapy to support cardiac function in patients with end-stage heart failure (HF) is increasing in all developed countries. However, the expertise needed to implant and manage patients referred for LVAD treatment is limited to a few reference centers, which are often located far from the patient's home. Although patients undergoing LVAD implantation should be permanently referred to the LVAD center for the management and follow-up of the device also after implantation, they would refer to the local healthcare service for routine assistance and urgent health issues related to the device or generic devices. Therefore, every clinician, from a bigger to a smaller center, should be prepared to manage LVAD carriers and the possible risks associated with LVAD management. Particularly, emergency treatment of patients with LVAD differs slightly from conventional emergency protocols and requires specific knowledge and a multidisciplinary approach to avoid ineffective treatment or dangerous consequences. This review aims to provide a standard protocol for managing emergency and urgency in patients with LVAD, elucidating the role of each healthcare professional and emphasizing the importance of collaboration between the emergency department, in-hospital ward, and LVAD reference center, as well as algorithms designed to ensure timely, adequate, and effective treatment to patients with LVAD. Copyright © 2022 Cameli, Pastore, Mandoli, Landra, Lisi, Cavigli, D'Ascenzi, Focardi, Carrucola, Dokollari, Bisleri, Tsioulpas, Bernazzali, Maccherini and Valente

Correlation of Left Ventricular Myocardial Work Indices with Invasive Measurement of Stroke Work in Patients with Advanced Heart Failure

ObjectivesThis study aimed to explore the correlation between left ventricular (LV) myocardial work (MW) indices and invasively-derived LV stroke work index (SWI) in a cohort of patients with advanced heart failure (AHF) considered for heart transplantation. BackgroundLeft ventricular MW has emerged as a promising tool for diagnostic and prognostic purposes in heart failure (HF) but its relationship with hemodynamic data derived from right heart catheterization (RHC) has not been assessed in patients with advanced heart failure yet. Materials and methodsConsecutive patients with AHF considered for heart transplantation from 2016 to 2021 performing RHC and echocardiography as part of the workup were included. Conventional LV functional parameters and LV MW indices, including LV global work index (GWI), LV global constructive work (GCW), LV global wasted work (GWW), LV global work efficiency (GWE), and other were calculated and compared with invasively-measured LV SWI. ResultsThe population included 44 patients. Median time between RHC and echocardiography was 0 days (IQR: 0-24). Median age was 60 years (IQR: 54-63). For the most part, etiology of HF was non-ischemic (61.4%) and all patients were either on class NYHA II (61.4%) or III (27.3%). Median left ventricular ejection fraction was 25% (IQR: 22.3-32.3), median NT-proBNP 1,377 pg/ml (IQR: 646-2570). LV global longitudinal strain (GLS) significantly correlated with LV SWI (r = -0.337; p = 0.031), whereas, LV ejection fraction (EF) did not (r = 0.308; p = 0.050). With regard to LV MW indices, some of them demonstrated correlation with LV SWI, particularly LV GWI (r = 0.425; p = 0.006), LV GCW (r = 0.506; p = 0.001), LV global positive work (LV GPW; r = 0.464; p = 0.003) and LV global systolic constructive work (GSCW; r = 0.471; p = 0.002). ConclusionAmong LV MW indices, LV GCW correlated better with invasively-derived SWI, potentially representing a powerful tool for a more comprehensive evaluation of myocardial function

Technology and technique for left ventricular assist device optimization: A Bi-Tech solution

Background We investigated the synergistic effect of the new cone-bearing design of Jarvik 2000 (Jarvik Heart Inc., NY) together with a minimally-invasive approach to outcomes of LVAD patients. Methods We retrospectively reviewed all patients from 5 institutions involved in the Jarvik 2000 Italian Registry, from October 2008 to October 2016. Patients were divided into three groups according to pump design and implantation technique: pin-bearing design and conventional approach (Group 1); cone-bearing and conventional approach (Group 2); cone-bearing and minimally-invasive implantation (Group 3). Results A total of 150 adult patients with end-stage heart failure were enrolled: 26 subjects in Group 1, 74 in Group 2, and 50 in Group 3. Nineteen patients (73%) in Group 1, 51 (69%) in Group 2, and 36 (72%) in Group 3 were discharged. During follow-up, 22 patients underwent transplantation, while in 3 patients the LVAD was explanted. The overall 1-year survival was 58 +/- 10%, 64 +/- 6%, and 74% +/- 7% in Groups 1, 2, and 3, respectively (p = 0.034). The competing-risks-adjusted cumulative incidence rate for adverse events was 42.1 [27-62.7] per 100 patient-years in Group 1, 35.4 [25.3-48.2] in Group 2, and 22.1 [12.4-36.4] in Group 3 (p = 0.046 for Group 1 vs. 3). Conclusions The association of the modern cone-bearing configuration of Jarvik 2000 and minimally invasive surgery improved survival and minimized the risk for cardiovascular events, as a result of combining technology and technique

Effect of bosentan on pulmonary hypertension secondary to systolic heart failure

Objectives: The dual endothelin receptor antagonist bosentan improves pulmonary vascular resistance (PVR) in patients with primary pulmonary hypertension (PH). The effects of bosentan on secondary PH due to systolic heart failure (HF) are not well defined. This study evaluates the effect and tolerability of bosentan in patients with PH secondary to HF. Methods: Seventeen adult HF patients with PH and New York Heart Association class III-IV symptoms were treated with bosentan, 62.5 mg twice daily, for 1 month, which was gradually increased to 125 mg twice daily thereafter. Right heart catheterization (RHC), a clinical evaluation and echocardiographic measurements were performed at baseline and at 4 ± 3 (mean ± SD) months of follow-up. Response to bosentan was defined as an improvement in clinical, echocardiographic and RHC parameters. Results: Six patients did not complete the study (therapy was discontinued due to hypotension, elevated liver enzymes or acute decompensation of HF), 11 patients completed the follow-up; 9 patients responded to therapy. Systemic arterial pressures, pulmonary pressures, PVR and the transpulmonary gradient significantly decreased compared with baseline levels in 9 responders (p = 0.05, 0.05, 0.01 and 0.004, respectively), and 4 became eligible for heart transplantation and 3 for left ventricular assist device implantation. Conclusions: Bosentan decreased pulmonary pressures and PVR in the majority of patients with PH secondary to systolic HF, thereby allowing them to be considered candidates for heart transplantation