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Simple sensor-based procedures, including auscultation and electrocardiography (ECG), can facilitate early diagnosis of valvular diseases, resulting in timely treatment. This study assessed the impact of combining these sensor-based procedures with machine learning on diagnosing valvular abnormalities and ventricular dysfunction. Data from auscultation at three distinct locations and 12-lead ECGs were collected from 1052 patients undergoing echocardiography. An independent cohort of 103 patients was used for clinical validation. These patients were screened for severe aortic stenosis (AS), severe mitral regurgitation (MR), and left ventricular dysfunction (LVD) with ejection fractions ≤ 40%. Optimal neural networks were identified by a fourfold cross-validation training process using heart sounds and various ECG leads, and their outputs were combined using a stacking technique. This composite sensor model had high diagnostic efficiency (area under the receiver operating characteristic curve (AUC) values: AS, 0.93; MR, 0.80; LVD, 0.75). Notably, the contribution of individual sensors to disease detection was found to be disease-specific, underscoring the synergistic potential of the sensor fusion approach. Thus, machine learning models that integrate auscultation and ECG can efficiently detect conditions typically diagnosed via imaging. Moreover, this study highlights the potential of multimodal artificial intelligence applications.

Atrial cardiomyopathy is a condition that causes electrical and contractile dysfunction of the atria, often along with structural and functional changes. Atrial cardiomyopathy most commonly occurs in conjunction with ventricular dysfunction, in which case it is difficult to discern the atrial features that are secondary to ventricular dysfunction from those that arise as a result of primary atrial abnormalities. Isolated atrial cardiomyopathy (atrial-selective cardiomyopathy [ASCM], with minimal or no ventricular function disturbance) is relatively uncommon and has most frequently been reported in association with deleterious rare genetic variants. The genes involved can affect proteins responsible for various biological functions, not necessarily limited to the heart but also involving extracardiac tissues. Atrial enlargement and atrial fibrillation are common complications of ASCM and are often the predominant clinical features. Despite progress in identifying disease-causing rare variants, an overarching understanding and approach to the molecular pathogenesis, phenotypic spectrum, and treatment of genetic ASCM is still lacking. In this review, we aim to analyze the literature relevant to genetic ASCM to understand the key features of this rather rare condition, as well as to identify distinct characteristics of ASCM and its arrhythmic complications that are related to specific genotypes. We outline the insights that have been gained using basic research models of genetic ASCM in vitro and in vivo and correlate these with patient outcomes. Finally, we provide suggestions for the future investigation of patients with genetic ASCM and improvements to basic scientific models and systems. Overall, a better understanding of the genetic underpinnings of ASCM will not only provide a better understanding of this condition but also promises to clarify our appreciation of the more commonly occurring forms of atrial cardiomyopathy associated with ventricular dysfunction., Competing Interests: Disclosures None.

Objectives: The aim of this study was to evaluate the relationship between atrioventricular valve and ventricular function in Fontan survivors, including the effect of atrioventricular valve surgery., Methods: Analysis focused on transplant-free survival and the need for atrioventricular valve surgery in single ventricle patients after Fontan completion. Longitudinal echocardiographic examination of long-term valve and ventricular function was performed., Results: Fontan completion was performed in 113 patients, having a right univentricular morphology in 33.6%, a left ventricle morphology in 62.8% and ambiguous in 3.6%. Perioperative mortality was 2.7% (n = 3). Within a median follow-up of 16.3 years (interquartile range 10.6-23.6), transplant-free survival was 96.1 ± 1.9% and 90.4 ± 5.8% at 10-25 years. Twenty AV valve procedures were performed in 14 (12.4%) children, respectively, pre-Fontan (n = 10), per-Fontan (n = 8) and post-Fontan (n = 2), resulting in a cumulative incidence of AV valve surgery is 5.7 ± 2.2% and 12.3 ± 3.2% at 1-5 years. Atrio-ventricular valve function deteriorated over time [hazard ratio (HR) 1.112, 95% confidence interval (CI) 1.089-1.138, P < 0.001], without difference for valve morphology (P = 0.736) or ventricular dominance (P = 0.484). AV valve dysfunction was greater in patients requiring AV valve surgery (HR 20.383, 95% CI 6.223-36.762, P < 0.001) but showed a comparable evolution since repair to those without valve surgery (HR 1.070, 95% CI 0.987-1.160, P = 0.099). Progressive time-related ventricular dysfunction was observed (HR 1.141, 95% CI 1.097-1.182, P < 0.001), significantly less in left ventricle-dominance (HR 0.927, 95% CI 0.860-0.999, P = 0.047) but more after AV valve surgery (HR 1.103, 95% CI 1.014-1.167, P = 0.022)., Conclusions: In a homogeneously treated Fontan population, 25-year transplant-free survival is encouraging. Atrio-ventricular valve surgery was necessary in 12.4%, resulting mostly in a durable valve function. However, a slow time-related decline of atrioventricular valve function as of ventricular function is worrisome, evoking a role for additional heart failure therapy., (© The Author(s) 2023. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Background: There is a significant incidence of pre-Fontan attrition-defined as failure to undergo Fontan completion-after superior cavopulmonary connection. This study investigated the impact of at least moderate ventricular dysfunction (VD) and atrioventricular valve regurgitation (AVVR) on pre-Fontan attrition., Methods: This single-center retrospective cohort study included all infants who underwent Norwood palliation from 2008 to 2020 and subsequently underwent superior cavopulmonary connection. Pre-Fontan attrition was defined as death, listing for heart transplantation before Fontan completion, or unsuitability for Fontan completion. The study's secondary outcome was transplant-free survival., Results: Pre-Fontan attrition occurred in 34 of 267 patients (12.7%). Isolated VD was not associated with attrition. However, patients with isolated AVVR had 5 times the odds of attrition (odds ratio, 5.4; 95% CI 1.8-16.2), and patients with both VD and AVVR had 20 times the odds of attrition (odds ratio, 20.1; 95% CI 7.7-52.8) compared with patients without VD or AVVR. Only patients with both VD and AVVR had significantly worse transplant-free survival compared with patients without VD or AVVR (hazard ratio, 7.7; 95% CI 2.8-21.6)., Conclusions: The additive effect of VD and AVVR is a powerful contributor to pre-Fontan attrition. Future research investigating therapies that can mitigate the degree of AVVR may help improve Fontan completion rates and long-term outcomes., (Copyright © 2023 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Competing Interests: Competing interests: None declared.

Objectives: This study aimed to determine the longitudinal change of systemic ventricular function and atrioventricular valve (AVV) regurgitation after total cavopulmonary connection (TCPC)., Methods: In 620 patients who underwent TCPC between 1994 and 2021, 4219 longitudinal echocardiographic examinations of systemic ventricular function and AVV regurgitation were evaluated retrospectively., Results: The most frequent primary diagnosis was hypoplastic left heart syndrome in 172, followed by single ventricle in 131, tricuspid atresia in 95 and double inlet left ventricle (LV) in 91 patients. Dominant right ventricle (RV) was observed in 329 (53%) and dominant LV in 291 (47%). The median age at TCPC was 2.3 (1.8-3.4) years. Transplant-free survival at 5, 10 and 15 years after TCPC was 96.3%, 94.7% and 93.6%, respectively, in patients with dominant RV and 97.3%, 94.6% and 94.6%, respectively, in those with dominant LV (P = 0.987). Longitudinal analysis of systemic ventricular function was similar in both groups during the first 10 years postoperatively. Thereafter, systemic ventricular function worsened significantly in patients with dominant RV, compared with those with dominant LV (15 years: P = 0.007, 20 years: P = 0.03). AVV regurgitation was more frequent after TCPC in patients with dominant RV compared with those with dominant LV (P < 0.001 at 3 months, 3 years, 5 years, 10 years and 15 years, P = 0.023 at 20 years). There was a significant correlation between postoperative systemic ventricular dysfunction and AVV regurgitation (P < 0.001)., Conclusions: There were no transplant-free survival difference and no difference in ventricular function between dominant RV and dominant LV for the first 10 years after TCPC. Thereafter, ventricular function in dominant RV was inferior to that in dominant LV. The degree of AVV regurgitation was significantly higher in dominant RV, compared with dominant LV, and it was positively associated with ventricular dysfunction, especially in dominant RV., (© The Author(s) 2023. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

The dominant ventricular morphology affects both the early and late outcomes of the Fontan procedure, but its impact on the patients' status immediately following the Glenn procedure is unknown. This study aims to evaluate the effect of the infants' dominant ventricular morphology on the immediate course after undergoing the Glenn procedure. This single-center, retrospective study included all patients who underwent the Glenn procedure between October 2003 and May 2016. The patients were divided into two groups according to their dominant ventricular morphology. Their postoperative records were reviewed and compared. Out of the 89 patients who underwent the Glenn procedure during the study period, 40 (44.9%) had dominant right ventricular morphology and 49 (55.1%) had left ventricular morphology. There were no significant group differences in baseline characteristics or operative data. The maximal postoperative vasoactive-inotropic score was significantly higher and the extent of ventricular dysfunction was significantly more severe in the dominant right ventricle group (P < 0.05). The length of hospitalization was slightly but not significantly longer in the hypoplastic LV group. It is concluded that patients with a dominant LV morphology had a superior ventricular function and required less inotropic support compared to that of a dominant RV morphology in the immediate postoperative course following the Glenn procedure. Survival was not affected by these differences. Further study to determine the pathophysiologic basis for these differences is warranted., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Objective: The aim of our study was to investigate the role of tissue Doppler and Myocardial Performance Index (MPI) in evaluating cardiac involvement in patients with rheumatoid arthritis (RA) with no cardiac symptoms, to determine whether these measurements differ between healthy controls and RA patients, and whether they can be used to determine the risk of cardiovascular disease and predict prognosis., Patients and Methods: 50 RA patients fulfilling the 2010 American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) RA criteria and 50 healthy volunteering controls were included in the study. All patients and controls were assessed using electrocardiography (ECG), echocardiography, conventional Doppler echocardiography and tissue Doppler echocardiography. MPI values were calculated. In addition, RA patients were compared after being divided into two subgroups: seropositive and seronegative RA. Disease activity levels of the patients were determined based on Disease Activity Score in 28 Joints (DAS28)., Results: The control group and RA group were compared in terms of PR interval, left atrial diameter, E/A, E/e', and MPI values. Comparisons between the groups yielded statistically significant differences in left atrial diameter, E/A, E/e', and MPI values and no significant difference in PR intervals. These parameters were also compared between seropositive and seronegative patients. Left atrial diameter was significantly higher in seronegative patients than in seropositive patients. There was no significant difference in the other values. DAS28 scores had no correlation with cardiac parameters., Conclusions: Early detection of ventricular dysfunction in RA may be useful in clinical practice when predicting prognosis and optimizing treatment. The present study found that RA patients had impaired tissue Doppler measurements and MPI results compared to controls. MPI and tissue Doppler may be useful in early detection of ventricular dysfunction.

Background: Mitral regurgitation (MR) burdens the left and right ventricles with a volume or pressure overload that leads to a series of compensatory adaptations that eventually lead to ventricular dysfunction, and it is well known that in rheumatic heart disease (RHD) that the inflammatory process not only occurs in the valve but also involves the myocardial and pericardial layers. However, whether the inflammatory process in rheumatic MR is associated with ventricular function besides hemodynamic changes is not yet established., Purpose: Evaluate whether rheumatic etiology is associated with ventricular dysfunction in patients with chronic MR., Methods: The study population comprised patients aged 18 years or older included in the registry who had echocardiography performed at the National Cardiovascular Center Harapan Kita in Indonesia during the study period with isolated primary MR due to rheumatic etiology and degenerative process with at least moderate regurgitation., Results: The current study included 1,130 patients with significant isolated degenerative MR and 276 patients with rheumatic MR. Patients with rheumatic MR were younger and had a higher prevalence of atrial fibrillation and pulmonary hypertension, worse left ventricle (LV) ejection fraction and tricuspid annular plane systolic excursion (TAPSE) value, and larger left atrium (LA) dimension compared to patients with degenerative mitral regurgitation (MR). Gender, age, LV end-systolic diameter, rheumatic etiology, and TAPSE were independently associated with more impaired LV ejection fraction. Whereas low LV ejection fraction, LV end-systolic diameter, and tricuspid peak velocity (TR) peak velocity >3.4 m/s were independently associated with more reduced right ventricle (RV) systolic function (Table 3)., Conclusions: Rheumatic etiology was independently associated with more impaired left ventricular function; however, rheumatic etiology was not associated with reduced right ventricular systolic function in a patient with significant chronic MR., Competing Interests: The authors have no competing interests to declare., (Copyright: © 2023 The Author(s).)

Individuals with Fontan circulation are at risk of late mortality from both cardiac and noncardiac causes. Despite the known risk of mortality, referral indications for advanced heart failure care vary between centers, and many individuals die from Fontan circulation-related complications either after late consideration for advanced heart failure therapies or having never seen a heart failure specialist. There is a critical need for guidelines to direct appropriately timed referral for advanced heart failure consultation. The Advanced Cardiac Therapies Improving Outcomes Network (ACTION) Fontan Committee has developed recommended thresholds for advanced heart failure referral to guide primary cardiologists. These recommendations are divided into 4 categories of clinical Fontan circulatory dysfunction including (1) cardiac/systemic ventricular dysfunction, (2) Fontan pathway dysfunction, (3) lymphatic dysfunction, and (4) extracardiac dysfunction.

Arterial pulse waves contain clinically useful information about cardiac performance, arterial stiffness and vessel tone. Here we describe a novel method for non-invasively assessing wave properties, based on measuring changes in blood flow velocity and arterial wall diameter during the cardiac cycle. Velocity and diameter were determined by tracking speckles in successive B-mode images acquired with an ultrafast scanner and plane-wave transmission. Blood speckle was separated from tissue by singular value decomposition and processed to correct biases in ultrasound imaging velocimetry. Results obtained in the rabbit aorta were compared with a conventional analysis based on blood velocity and pressure, employing measurements obtained with a clinical intra-arterial catheter system. This system had a poorer frequency response and greater lags but the pattern of net forward-traveling and backward-traveling waves was consistent between the two methods. Errors in wave speed were also similar in magnitude, and comparable reductions in wave intensity and delays in wave arrival were detected during ventricular dysfunction. The non-invasive method was applied to the carotid artery of a healthy human participant and gave a wave speed and patterns of wave intensity consistent with earlier measurements. The new system may have clinical utility in screening for heart failure., Competing Interests: Conflict of interest disclosure P.D.W. is the named inventor on patents filed by Imperial College Innovations, Ltd that describe some of the underlying technology., (Copyright © 2022 The Authors. Published by Elsevier Inc. All rights reserved.)

Objectives: Critically ill neonates with a history of pulmonary hypertension (PH) or ventricular dysfunction are at risk to experience an extubation failure (EF) after liberation from mechanical ventilation (MV). Due to insufficient data from neonatal cohorts, it remains unclear whether NT-proBNP is an appropriate biomarker to predict EF in this cohort. The Zlog-transformation of NT-proBNP (further named NT-proBNP Zlog ) is an additional tool to optimize the interpretation of NT-proBNP since absolute NT-proBNP values are varying with the age of these infants., Patients and Methods: This was a retrospective single-center analysis at the University Children's Hospital, Bonn, Germany, during the study period from January 2020 until December 2021. Forty-three neonates met the inclusion criteria and were screened for study participation., Inclusion Criteria: prolonged (>24 h) MV with at least one extubation attempt, with a history of PH and/or ventricular dysfunction in the echocardiographic assessment at admission to the neonatal intensive care unit or during the period of MV, NT-proBNP measurements before (max. 24 h, baseline) and after (max. 24 h, follow-up) the first extubation attempt. The primary clinical endpoint was defined as EF with need for reintubation (0-72 h). Neonates with an EF were allocated to group A and neonates with successful liberation from MV to group B., Main Results: The primary clinical endpoint (EF) was reached in 21% (nine infants). Absolute mean NT-proBNP values (NT-proBNP abs ) at baseline did not differ significantly in infants of group A and B (6931 vs. 7136 pg/ml, p = 0.227). NT-proBNP Zlog values at baseline (2.35 vs. 1.57, p = 0.073) tended to higher values in group A. NT-proBNP abs values measured at follow-up were significantly higher in infants allocated to group A (11120 vs. 7570 pg/ml, p = 0.027). Likewise, NT-proBNP Zlog values at follow-up were significantly higher in infants allocated to group A (3.05 vs. 1.93, p = 0.009). NT-proBNP abs values at follow-up and NT-proBNP Zlog values at baseline correlated significantly with the severity of PH. Regarding the receiver operating characteristic-analysis, a NT-proBNP abs value at follow-up of ≥4622 pg/ml was calculated as optimal cut-off value for the prediction of EF (area under the curve [AUC] 0.742, p = 0.001). A NT-proBNP Zlog value at baseline of ≥1.63 and at follow-up of ≥2.14 was calculated as optimal cut-off for the prediction of EF (AUC: 0.690/p = 0.027, and 0.781/p = 0.000, respectively)., Conclusion: NT-proBNP abs and NT-proBNP Zlog might be valuable biomarkers for the prediction of EF in critically ill neonates. The Zlog-transformation of NT-proBNP allows an age-independent interpretation of NT-proBNP and should be considered for clinical routine., (© 2022 The Authors. Pediatric Pulmonology published by Wiley Periodicals LLC.)

Background: Nicorandil, an adenosine triphosphate-sensitive potassium channel agonist and nitric oxide donor, is a coronary vasodilator used to treat ischemia-induced chest pain, but it's potential cardioprotective benefits during open heart surgery have not been thoroughly investigated. The study objective was to assess the impact of nicorandil on postoperative ventricular dysfunction and end-organ injury in an established experimental model of open-heart surgery with cardiopulmonary bypass (CPB) and cardioplegic arrest. We hypothesized that nicorandil would attenuate myocardial ischemia-reperfusion (IR) injury, preserve ventricular function, and reduce end-organ injury., Methods: Rabbits were cannulated for CPB, followed by 60 min of aortic cross-clamp (ACC) with cold cardioplegic arrest, and 120 min of recovery after ACC removal. Nicorandil (or normal saline vehicle) was given intravenously 5 min before ACC and continued throughout the recovery period. Left ventricular developed pressure (LVDP), systolic contractility (LV + dP/dt), and diastolic relaxation (LV -dP/dt) were continuously recorded, and blood and tissue samples were collected for measurement of oxidant stress (OS), inflammation, apoptosis, and organ injury., Results: Nicorandil significantly attenuated IR-induced LV dysfunction compared to saline control (R-120: LV + dP/dt: 1596 ± 397 vs. 514 ± 269 mmHg/s, p = 0.010; LV -dP/dt: -1524 ± 432 vs. -432 ± 243 mmHg/s, p &lt; 0.001; LVDP: 55 ± 11 vs. 22 ± 5 mmHg, p = 0.046). Furthermore, nicorandil inhibited IR-induced increases in OS, inflammation, apoptosis, and organ injury., Conclusions: Nicorandil exhibits myocardial protection by attenuation of IR-induced LV dysfunction associated with OS, inflammation, apoptosis, and organ injury. Nicorandil should be explored further as a potential therapeutic strategy for limiting global IR injury during open-heart surgery in humans., Competing Interests: Declaration of Competing Interest The authors declare no conflicts of interest., (Copyright © 2022 Elsevier B.V. All rights reserved.)

Precapillary pulmonary hypertension (PH precap ) is a condition with elevated pulmonary vascular pressure and resistance. Patients have a poor prognosis and understanding the underlying pathophysiological mechanisms is crucial to guide and improve treatment. Ventricular hemodynamic forces (HDF) are a potential early marker of cardiac dysfunction, which may improve evaluation of treatment effect. Therefore, we aimed to investigate if HDF differ in patients with PH precap compared to healthy controls. Patients with PH precap (n = 20) and age- and sex-matched healthy controls (n = 12) underwent cardiac magnetic resonance imaging including 4D flow. Biventricular HDF were computed in three spatial directions throughout the cardiac cycle using the Navier-Stokes equations. Biventricular HDF (N) indexed to stroke volume (l) were larger in patients than controls in all three directions. Data is presented as median N/l for patients vs controls. In the RV, systolic HDF diaphragm-outflow tract were 2.1 vs 1.4 (p = 0.003), and septum-free wall 0.64 vs 0.42 (p = 0.007). Diastolic RV HDF apex-base were 1.4 vs 0.87 (p < 0.0001), diaphragm-outflow tract 0.80 vs 0.47 (p = 0.005), and septum-free wall 0.60 vs 0.38 (p = 0.003). In the LV, systolic HDF apex-base were 2.1 vs 1.5 (p = 0.005), and lateral wall-septum 1.5 vs 1.2 (p = 0.02). Diastolic LV HDF apex-base were 1.6 vs 1.2 (p = 0.008), and inferior-anterior 0.46 vs 0.24 (p = 0.02). Hemodynamic force analysis conveys information of pathological cardiac pumping mechanisms complementary to more established volumetric and functional parameters in precapillary pulmonary hypertension. The right ventricle compensates for the increased afterload in part by augmenting transverse forces, and left ventricular hemodynamic abnormalities are mainly a result of underfilling rather than intrinsic ventricular dysfunction., (© 2022. The Author(s).)

Objective: This study aimed to describe the clinical characteristics and the different phenotypes of children with multisystem inflammatory syndrome in children (MIS-C) temporally related to COVID-19 and to evaluate the risk conditions that favored a greater severity of the disease during a 12-month period at a pediatric reference hospital in Colombia., Methods: A 12-month retrospective observational study of children under the age of 18 years who met criteria for MIS-C., Results: A total of 28 children presented MIS-C criteria. The median age was 7 years. Other than fever (100%) (onset 4 days prior to admission), the most frequent clinical features were gastrointestinal (86%) and mucocutaneous (61%). Notably, 14 (50%) children had Kawasaki-like symptoms. The most frequent echocardiographic abnormalities were pericardial effusion (64%), valvular involvement (68%), ventricular dysfunction (39%), and coronary artery abnormalities (29%). In addition, 75% had lymphopenia. All had at least one abnormal coagulation test. Most received intravenous immunoglobulin (89%), glucocorticoids (82%), vasopressors (54%), and antibiotics (64%). Notably, 61% had a more severe form of the disease and were admitted to an intensive care unit (median 4 days, mean 6 days); the severity predictors were patients with the inflammatory/MIS-C phenotype (OR 26.5; 95%CI 1.40-503.7; p=0.029) and rash (OR 14.7; 95%CI 1.2-178.7; p=0.034). Two patients had macrophage activation syndrome., Conclusions: Coronary artery abnormalities, ventricular dysfunction, and intensive care unit admission were frequent, which needs to highlight the importance of early clinical suspicion.

Background: Heart involvement is frequent although often clinically silent in systemic sclerosis (SSc) patients. Early identification of cardiac involvement can be improved by noninvasive methods such as MRI, in addition to transthoracic echocardiography (TTE)., Purpose: To assess the ability of phase-contrast (PC)-MRI to detect subclinical left (LV) and right (RV) ventricular diastolic dysfunction in SSc patients., Study Type: Prospective., Population: Thirty-five consecutive SSc patients (49 ± 14 years) and 35 sex- and age-matched healthy controls (48.6 ± 13.5 years) who underwent TTE and MRI in the same week., Field Strength/sequence: 5 T/PC-MRI using a breath-hold velocity-encoded gradient echo sequence., Assessment: LV TTE (E/E') and LV and RV PC-MRI indices of diastolic function (LV early and late transmitral [E M , Ef M , A M , Af M ] and RV transtricuspid [E T , Ef T , A T , Af T ] peak filling flow velocities and flow rates, as well as LV [ E M ' ] and RV [ E T ' ] peak longitudinal myocardial velocities during diastole) were measured., Statistical Tests: Two-tailed t-test, Wilcoxon test, or Fischer test for comparison of variables between SSc and healthy control groups; sensitivity, specificity, receiver-operating-characteristic (ROC) area under the curve (AUC) to assess discriminative ability of variables. A P-value <0.05 was considered statistically significant., Results: TTE LV E/E' and MRI E M / E M ' and E T / E T ' were significantly higher in SSc patients than in controls (8.27 ± 1.25 vs. 6.70 ± 1.66; 9.43 ± 2.7 vs. 6.51 ± 1.50; 6.51 [4.70-10.40] vs. 4.13 [3.22-5.75], respectively) and separated SSc patients and healthy controls with good sensitivity (68%, 71%, and 80%), specificity (85%, 94%, and 62%), and AUC (0.787, 0.807, and 0.765). LV Ef M was significantly higher in SSc patients than in controls (347.1 ± 113.7 vs. 284.7 ± 94.6) as RVAf T (277 [231-355] vs. 220 [154-253] mL/sec) with impaired relaxation pattern (Ef T /Af T , 0.95 [0.87-1.21] vs. 1.12 [0.93-1.47])., Data Conclusion: MRI was able to detect LV and RV diastolic dysfunction in SSc patients with good accuracy in the absence of LV systolic dysfunction at echocardiography. Use of MRI can allow to better assess the early impact of myocardial fibrosis related to SSc., Level of Evidence: 1 TECHNICAL EFFICACY STAGE: 2., (© 2022 International Society for Magnetic Resonance in Medicine.)

Objective: In our study, we aimed at evaluating the change in biventricular functions and pulmonary arterial stiffness (PAS) in patients with subclinical hypothyroidism (SH) in whom euthyroidism was achieved with L-thyroxine therapy., Patients and Methods: 70 SH patients and 75 healthy volunteers were included in our study consecutively. Baseline demographic and echocardiographic data of the participants were recorded. The data obtained in the control evaluation 6 months after the euthyroidism were achieved in the SH group patients started on L-thyroxine treatment and then compared with the baseline measurements., Results: The mean age of patients in the SH group was 44.1 ± 9.4 years and 47.1% were women. Euthyroidism in SH patients was achieved with a mean daily L-thyroxine treatment of 59 µg/day for a mean of 16.1 ± 4.5 weeks. Positive changes in metabolic and hormonal profiles were achieved after L-thyroxine treatment in SH patients. It was determined that left ventricular and right ventricular isovolumetric relaxation and myocardial performance index were higher in SH patients compared to the control group, and these measurements were observed to decrease significantly with L-thyroxine treatment (p < 0.05 for each). While PAS was 16.9 ± 3.1 kHz/ms in the control group, it was 25.2 ± 5.3 kHz/ms in the SH group (p < 0.05). After L-thyroxine treatment, PAS measurements decreased to 17.2 ± 3.2 kHz/ms (p < 0.05) in the SH group and showed a positive change. Thyroid-stimulating hormone (TSH) change (Δ TSH) with Δ E/A ratio (r: -0.407, p < 0.001), right ventricular myocardial performance index (Δ RV MPI) change (r: 0.404, p < 0.001) and PAS change (Δ PAS) (r: 0.458, p < 0.001) found to be correlated., Conclusions: SH is associated with dysfunction in the biventricular and pulmonary vascular bed. Biventricular functions and PAS change positively in SH patients with L-thyroxine treatment.

Background: Advances in surgical technique and medical surveillance have improved outcomes of single ventricle (SV) palliation, particularly during the first interstage period. However, there remains a considerable mortality risk beyond this period., Methods: Patients born between January 2004 and December 2011 who required SV palliation were retrospectively identified. Patients who survived stage 1 palliation, were discharged home, and then were evaluated for Glenn candidacy, and continued care at our institution were included. Perioperative echocardiographic, hemodynamic, and operative data were analyzed at each surgical stage. The primary outcome was death or need for transplant. Univariate and multivariate analysis was completed using Cox proportional-hazards modeling., Results: A total of 175 patients were included. Three patients died after pre-operative evaluation before Glenn. Glenn was completed in 168 patients, 16 died before Fontan. Fontan was completed in 149 patients; 117 were alive without need for transplant, 17 died post-Fontan, and 1 required transplantation. Twenty-one patients were lost to follow-up throughout the study period and were censored at time of last follow-up. Pre-Glenn moderate or severe atrioventricular valve regurgitation (AVVR) was an independent risk factor for death/transplant (HR 2.41; p-value .026). Pre-Glenn moderate ventricular dysfunction was also an independent risk factor (HR 5.29; p-value .012). Other risk factors included right ventricular (RV) dominant morphology and perinatal acidosis., Conclusions: Despite advances in SV palliation, a subset of these children remains at increased risk for poor outcomes. Early risk factors include RV dominant morphology and perinatal acidosis. Patients with substantial AVVR or ventricular dysfunction before Glenn palliation are also at significantly higher risk for death or requirement of transplantation later in childhood., Competing Interests: Disclosure statement Dr John Wood is the recipient of an Additional Ventures Single Ventricle grant. This grant was not used for this study. The other authors have no financial disclosures or conflicts of interest., (Copyright © 2022 International Society for Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved.)

Global longitudinal strain (GLS) can identify subclinical myocardial dysfunction in patients with cirrhosis. This systematic review aims to provide evidence of a possible difference in GLS values between patients with cirrhosis and patients without cirrhosis. Studies from inception to August 11, 2021, were screened and included based on the inclusion criteria. The Newcastle Ottawa Scale was used to assess the quality of nonrandomized studies. Meta-analyses were conducted with subsequent sensitivity and subgroup analyses according to age, sex, cirrhosis etiology, and severity. Publication bias was evaluated using Begg's funnel plot, Egger's test, and rank correlation test with subsequent trim-and-fill analysis. The systematic database search yielded 20 eligible studies. Random effect showed a significant reduction of left ventricular (LV) GLS (MD:-1.43;95%; 95%CI,-2.79 to -0.07; p = 0.04; I2 = 95% p<0.00001) and right ventricular (RV) GLS (MD:-1.95; 95%CI,-3.86 to -0.05, p = 0.04; I2 = 90%, p<0.00001) in the group with cirrhosis. A sensitivity test on subgroup analysis based on the study design showed a -1.78% lower LV-GLS in the group with cirrhosis (I2 = 70%, p = 0.0003). Meta-regression analysis showed that the severity of cirrhosis was significantly related to GLS reduction. This research received no specific grants from any funding agency in the public, commercial, or not-for-profit sectors. The study protocol was registered at PROSPERO (CRD42020201630). We followed the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) 2020 statement guidelines., Competing Interests: The authors have declared that no competing interests exist.

Aortopulmonary window (APW) seen in association with anomalous origin of left coronary artery from pulmonary artery (ALCAPA) is extremely uncommon. When faced with this combination, ALCAPA usually goes undetected since most of the clinical and echocardiographic features of ALCAPA, including ventricular dysfunction are absent due to co-existent pulmonary hypertension. Herein we report a 5-month-old child with a large APW in whom a preoperative computed tomography angiogram facilitated the diagnosis of ALCAPA. The case is described for its rarity and the challenges faced during management. Also, it is extremely crucial that this coronary anomaly is identified and corrected during surgery, since failure to do so results in unforeseen postoperative ventricular dysfunction.

Objectives: To evaluate any association between non-sustained ventricular tachycardia (NSVT) detected by intra-cardiac device and clinical outcomes in repaired adult congenital heart disease (ACHD) without tetralogy of Fallot (TOF)., Background: NSVT portends a higher risk of serious ventricular tachyarrhythmia in TOF. However its clinical significance when incidentally detected by implantable cardiac device is not well elucidated in non-TOF ACHD cohort., Methods: We performed a single center, retrospective, longitudinal follow-up study in repaired ACHD (≥18 years) patients without TOF who hosted a pacemaker or automatic implantable cardiac defibrillator (AICD). The cohort was divided based on presence/absence of device detected NSVT. The primary end-point was a composite of sustained ventricular tachycardia (VT), ventricular fibrillation (VF), or sudden cardiac death (SCD)., Results: One hundred fifty eight patients (male 56.3%, median [IQR] age of 35 [28-43] years at last follow-up] with longitudinal post-implant follow-up duration of 8 (5-12) years were included. NSVT was detected in 52 (33%) patients. The primary composite end-point was more frequent in NSVT group [11.5% vs. 2.8%; p = .04]. Patients with NSVT were (i) older at the time of initial implant (age 25 vs. 18 years, p = .011) and more frequently demonstrated (ii) systemic ventricular dysfunction (44% vs. 26%; p = .015), as well as (iii) history of ventriculotomy (38% vs. 21%; p = .017)., Conclusions: In our repaired ACHD cohort, we noted a significant association between device-detected-NSVT and the primary composite end-point of sustained VT/VF or SCD. Systemic ventricular dysfunction and history of ventriculotomy were more frequent in the NSVT group and likely constituted the clinical milieu., (© 2021 Wiley Periodicals LLC.)

Introduction: Heart failure (HF) is a chronic disease that acutely and progressively reduces physical functionality. The patient commonly suffers from intermittent relapses that increase the likelihood of comorbidities such as chronic insomnia, cognitive impairment, alterations in sexual response, psychological distress, symptoms of anxiety and depression disorder, and decreased self-care behaviors. The objective of this study was to identify the main needs for psychological support in patients with HF., Methodology: A descriptive, cross-sectional and analytical study was carried out. Participants were selected through consecutive non-probability samples. 150 patients with a diagnosis of HF who attended the Specialized Center for Heart Failure at the National Institute of Cardiology “Ignacio Chávez” were evaluated from November 2018 to April 2019, applying the inventories: Quality of life in patients with HF (Minnesota), Hospital Anxiety and Depression Scale (HADS), psychological distress thermometer (visual analog scale) and the HF Patient Information Needs Inventory., Results: 33% of the patients perceive a high level of psychological distress that requires immediate assistance and 26.6% report a medium level that requires monitoring. The main information needs reported by the patients were information on taking medications (53.7%) and managing psychological distress (43.9%)., Conclusion: Active assessment of psychological distress and unmet information needs is relevant in patients with heart failure, thus highlighting the importance of their identification and the correct design and application of evidence-based interventions aimed at their resolution.

The Fontan operation has been the final palliation for patients born with congenital heart defects with a functional single ventricle for more than 4 decades. The "normal" Fontan physiology is characterized by the loss of the sub-pulmonary ventricle with consequent elevated pressure in the caval system, non-pulsatile blood flow in the pulmonary circulation and at least mild reduction of the systemic output. When successful, this procedure is associated with a range of benefits including improved arterial saturation and abolishment of chronic volume overload, allowing a fairly normal life to the majority of patients through early adulthood. As we enter the 5th decade of caring for patients palliated with the Fontan procedure, it is evident that adult survivors face significant morbidity due to multiorgan dysfunction, early mortality and need for heart transplantation. Several late complications may occur: ventricular dysfunction, arrhythmia, cyanosis, exercise intolerance, elevated pulmonary vascular resistance, protein-losing enteropathy, plastic bronchitis, hepatic and renal complications. The mechanism of late Fontan failure is multifactorial and not completely understood, it depends on interactions between the ventricle, the pulmonary vascular bed, the venous and lymphatic compartments. Conclusions: the aim of this review is to describe the pathophysiology of Fontan circulation and the clinical and hemodynamic characteristics of early and late failing Fontan survivors, their association with morbidity and mortality, and the strategies for their management.

Heart failure is a significant health problem worldwide. Despite all the new therapies available nowadays, many patients will reach advanced stages of the disease. Diuretic resistance, kidney dysfunction, and refractory congestion, all highly prevalent in advanced heart failure, frequently complicate the situation, making it more challenging to manage. Ultrafiltration through hemodialysis or peritoneal dialysis can be alternative options to treat fluid overload. Peritoneal dialysis has gained increased interest in the last decades due to several benefits such as functional class improvement, reduction in hospital admissions, improvement in quality of life, and even a reduction in mortality shown by numerous cohort studies. However, the majority of the studies were observational and with a limited number of patients. In addition, the optimal timing for the initiation of this type of therapy and the subgroup of patients who would benefit the most from it is unknown. Hence, randomized controlled trials in this subject are urgently needed. We aim to review the contemporary evidence of peritoneal dialysis in patients with heart failure and diuretic resistance across the spectrum of ventricular dysfunction and degree of renal dysfunction., Competing Interests: The authors declare no conflict of interest., (© 2021 The Author(s). Published by IMR Press.)

Background & Aims: Intrahepatic cholestasis of pregnancy (ICP) is associated with an increased risk of stillbirth. This study aimed to assess the relationship between bile acid concentrations and fetal cardiac dysfunction in patients with ICP who were or were not treated with ursodeoxycholic acid (UDCA)., Methods: Bile acid profiles and NT-proBNP, a marker of ventricular dysfunction, were assayed in umbilical venous serum from 15 controls and 76 ICP cases (36 untreated, 40 UDCA-treated). Fetal electrocardiogram traces were obtained from 43 controls and 48 ICP cases (26 untreated, 22 UDCA-treated). PR interval length and heart rate variability (HRV) parameters were measured in 2 behavioral states (quiet and active sleep)., Results: In untreated ICP, fetal total serum bile acid (TSBA) concentrations (r = 0.49, p = 0.019), hydrophobicity index (r = 0.20, p = 0.039), glycocholate concentrations (r = 0.56, p = 0.007) and taurocholate concentrations (r = 0.44, p = 0.039) positively correlated with fetal NT-proBNP. Maternal TSBA (r = 0.40, p = 0.026) and alanine aminotransferase (r = 0.40, p = 0.046) also positively correlated with fetal NT-proBNP. There were no significant correlations between maternal or fetal serum bile acid concentrations and fetal HRV parameters or NT-proBNP concentrations in the UDCA-treated cohort. Fetal PR interval length positively correlated with maternal TSBA in untreated (r = 0.46, p = 0.027) and UDCA-treated ICP (r = 0.54, p = 0.026). Measures of HRV in active sleep and quiet sleep were significantly higher in untreated ICP cases than controls. HRV values in UDCA-treated cases did not differ from controls., Conclusions: Elevated fetal and maternal serum bile acid concentrations in untreated ICP are associated with an abnormal fetal cardiac phenotype characterized by increased NT-proBNP concentration, PR interval length and HRV. UDCA treatment partially attenuates this phenotype., Lay Summary: The risk of stillbirth in intrahepatic cholestasis of pregnancy (ICP) is linked to the level of bile acids in the mother which are thought to disrupt the baby's heart rhythm. We found that babies of women with untreated ICP have abnormally functioning hearts compared to those without ICP, and the degree of abnormality is closely linked to the level of harmful bile acids in the mother and baby's blood. Babies of women with ICP who received treatment with the drug UDCA do not have the same level of abnormality in their hearts, suggesting that UDCA could be a beneficial treatment in some ICP cases, although further clinical trials are needed to confirm this., Competing Interests: Conflict of interest BHG has previously served as a director for Monica Healthcare Limited and has no commercial or financial connections in the company. CW and HUM are consultants with Mirum Pharmaceuticals and CW is a consultant for GlaxoSmithKline. The remaining authors have no conflicts of interest to disclose. Please refer to the accompanying ICMJE disclosure forms for further details., (Copyright © 2021 European Association for the Study of the Liver. Published by Elsevier B.V. All rights reserved.)

Objectives: In patients with anatomically repaired congenitally corrected transposition of the great arteries, the impact of electrophysiological features on postoperative ventricular dysfunction remains less well known. Our goal was to investigate the role of fragmented QRS and QRS duration in mortality and systemic ventricular dysfunction after anatomical repair of corrected transposed great arteries., Methods: Consecutive patients who underwent anatomical repair in our institution from January 2005 to December 2017 were enrolled in this retrospective analysis. Fragmented QRS was defined as ≥1 discontinuous deflections in narrow QRS complexes, and ≥2 in wide QRS complexes, in 2 contiguous electrocardiogram leads. The primary end point was a composite of all-cause mortality and systemic ventricular dysfunction., Results: A total of 74 patients were included. Among them, 30, 15 and 29 underwent the Senning arterial switch, the Senning Rastelli and the hemi-Mustard/bidirectional Glenn/Rastelli procedures, respectively. The primary end point occurred in 9 (12.2%) patients and included 7 late deaths and 2 cases of late-onset systemic ventricular dysfunction. Fragmented QRS and QRS prolongation were noted in 19 (25.7%) and 21 (28.4%) patients, respectively. In patients with the primary end point, QRS fragmentation (6/9 vs 10/65; P < 0.001) and QRS prolongation (6/9 vs 15/65; P = 0.013) were noted more frequently than in patients without the primary end point. No statistical differences in these electrocardiogram findings were found among patients treated with 3 surgical strategies., Conclusions: Appearance of QRS fragmentation or QRS prolongation is associated with death or ventricular dysfunction in anatomically repaired corrected transposition of the great arteries. Although there is a trend that QRS fragmentation and QRS prolongation appear more frequently in patients who had the Senning-arterial switch operation, there is no statistically significant difference associated with these electrocardiogram features among varied procedures., (© The Author(s) 2020. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Introduction: Treatment of hypoplastic left heart syndrome varies across institutions. This study examined the impact of introducing a standardised programme., Methods: This retrospective cohort study evaluated the effects of a comprehensive strategy on 1-year transplant-free survival with preserved ventricular and atrioventricular valve (AVV) function following a Norwood operation. This strategy included standardised operative and perioperative management and dedicated interstage monitoring. The post-implementation cohort (C2) was compared to historic controls (C1). Outcomes were assessed using logistic regression and Kaplan-Meier analysis., Results: The study included 105 patients, 76 in C1 and 29 in C2. Groups had similar baseline characteristics, including percentage with preserved ventricular (96% C1 versus 100% C2, p = 0.28) and AVV function (97% C1 versus 93% C2, p = 0.31). Perioperatively, C2 had higher indexed oxygen delivery (348 ± 67 ml/minute/m2 C1 versus 402 ± 102ml/minute/m2 C2, p = 0.015) and lower renal injury (47% C1 versus 3% C2, p = 0.004). The primary outcome was similar in both groups (49% C1 and 52% C2, p = 0.78), with comparable rates of death and transplantation (36% C1 versus 38% C2, p = 0.89) and ventricular (2% C1 versus 0% C2, p = 0.53) and AVV dysfunction (11% C1 versus 11% C2, p = 0.96) at 1-year. When accounting for cohort and 100-day freedom from hospitalisation, female gender (OR 3.7, p = 0.01) increased and ventricular dysfunction (OR 0.21, p = 0.02) and CPR (OR 0.11, p = 0.002) or ECMO use (OR 0.15, p = 001) decreased the likelihood of 1-year transplant-free survival., Conclusions: Standardised perioperative management was not associated with improved 1-year transplant-free survival. Post-operative ventricular or AVV dysfunction was the strongest predictor of 1-year mortality.

Background: Nephropathic cystinosis is a rare autosomal recessive lysosomal storage disorder that initially affects the kidney progressing to multi-organ failure due to accumulation of cystine in all tissue compartments., Objective: The main objective of this study is the evaluation of cardiac function in cystinosis patients using non-conventional echocardiographic modalities like pulsed wave tissue Doppler imaging (PW-TDI) and 2D speckle tracking echocardiography (2D-STE)., Methods: This is a case control study conducted on fifteen patients with cystinosis and 15 normal controls. Echocardiography was done for all participants and PW-TDI was performed for measurement of S', E', A' velocities and myocardial performance index (MPI) at basal parts of septal, left ventricle (LV), and right ventricle (RV) free walls. 2D-STE was done for evaluation of global longitudinal strain (GLS), global circumferential strain (GCS), and global radial strain (GRS) of LV. Mitral E and A velocities and tricuspid annular plane systolic excursion (TAPSE) were also measured., Results: The GLS, GRS, and S' velocity at basal septum and LV lateral wall were significantly lower in patients denoting LV systolic dysfunction (p = 0.005, p < 0.0001, p = 0.001, p = 0.006, respectively), while E/E' were significantly higher in patients group denoting LV diastolic dysfunction (p < 0.001). For RV function, TAPSE, S', and E' velocity were significantly lower in patients group (p 0.013, p < 0.01, p = 0.05, respectively) indicating RV systolic and diastolic dysfunction. The TDI-derived MPI for both LV and RV were significantly higher in patients group (p < 0.0001, p < 0.01, respectively) indicating both ventricular systolic and diastolic dysfunction. For prediction of cardiac dysfunction among patients, the receiver operating characteristic (ROC) curve showed that GRS ≤ 29% had sensitivity 93.3% and specificity 100%, GLS > - 20.1% had sensitivity 66.7% and specificity 93.3%, LV-E/E' >7.87 had sensitivity 73.3% and specificity 93.3%, and MPI-LV > 0.36 had sensitivity 100% and specificity 93.3% while MPI-RV > 0.29 had sensitivity 80% and specificity 93.3% and TAPSE ≤ 19 mm had sensitivity 80% and specificity 73.3%., Conclusions: Patients with cystinosis have significant both left and right ventricular dysfunction, which can be better evaluated using the non-conventional echocardiographic modalities like TDI and 2D-STE for early detection of subtle cardiac dysfunction.

Introduction and Objectives: Adequate medication intake affects treatment effectiveness. The aim of this study was to establish the impact of prescription and secondary adherence to beta-blockers on medium- and long-term and long-term cardiovascular outcomes, after a first type 1 ST-elevation myocardial infarction (STEMI) episode without heart failure or left ventricular ejection fraction ≥ 40%., Methods: A retrospective observational study was conducted in a cohort of patients admitted from 2008 to 2013 to the University Clinical Hospital in Valencia. Competing risk analysis assessed the relationship between cardiovascular mortality or new vascular event with beta-blocker prescription and secondary adherence, defined as a proportion of days covered., Results: During after the first year following discharge, beta-blocker prescription was not significantly associated with better health outcomes in the 460 patients included. However, cardiovascular mortality was lower in adherent patients compared to non-adherent patients, at 0.6% vs. 6.6% (HR = 0.083; 95% CI, 0.015-0.448; p = 0.003), and in adherent patients compared to those who did not receive the treatment due to lack of prescription or lack of adherence, with 0.6% vs. 4.8% (HR = 0.115; 95% CI, 0.022-0.587; p = 0.009). These results were not observed when the complete follow-up period was analysed (median 46.7 months)., Conclusions: Secondary adherence to beta-blockers improves 1-year prognosis after STEMI with preserved left ventricular function., (Copyright © 2019 Elsevier España, S.L.U. All rights reserved.)

Background: del Nido (DN) cardioplegia is commonly used during robotic mitral valve surgery. Poor venous drainage during surgery may result in venous backpressure and washout of this one-shot cardioplegia, limiting its cardioprotective effects., Methods: One hundred eighty-seven patients undergoing isolated robotic mitral valve surgery, from January 2015 to July 2017, were retrospectively reviewed. Intraoperative central venous pressure (CVP) tracings were reviewed and venous drainage was categorized as good or poor and the relationship of the quality of venous drainage to postoperative ventricular dysfunction (operationalized as the need for inotropic support during and after weaning from cardiopulmonary bypass [CPB]) was assessed., Results: Drainage was judged to be good in 107 patients and poor in 79 patients. On univariate analysis, 23 patients (41%) with good drainage required inotropic support whereas 33 patients (59%) with poor drainage required inotropic support (P = .0025). On multivariable analysis, poor venous drainage remained significantly associated with inotropic use even after adjusting for cross-clamp and CPB time. Inotrope use was associated with significantly longer intensive care unit length of stay (P = .027)., Conclusion: Maintenance of excellent venous drainage, as assessed by CVP monitoring, should be a high priority in isolated robotic mitral valve surgery undertaken with DN cardioplegia., (© 2020 Wiley Periodicals, Inc.)

Vectorcardiogram (VCG) represents the trajectory of the tip of cardiac vectors in three dimensional space with varying time. It is a recurring, near-periodic pattern of cardiac dynamics that is constructed by drawing the instantaneous vectors from a zero reference point according to direction, magnitude and polarity in the space. Being a three dimensional entity, it is more informative and more sensitive than conventional ECG as an evaluation tool of the physiology of cardiac dynamics, because of its extra degree of freedom. Accordingly, it is possible to find out even a minute and early electrophysiological alteration in diseases. Each cardiac cycle primarily consists of three loops in VCG corresponding to P, QRS, and T wave activities. The morphological assessment of the QRS loop was carried out in three dimensional space in order to analyze the spatial vectors of the ventricles and their patho-physiological correlation in various cardiac diseases. Spatial Velocity (SV) is a virtual velocity that represents the rate of movement of the tip of the cardiac vector through space, coordinated by three orthogonal leads, and can be estimated by using simple mathematical formula. It is the rate of change in amplitude and the directionality of instantaneous vectors in seriatim in the three dimensional space to quantify their the temporo-spatial characteristic pattern. We propose to evaluate this novel VCG descriptor SV, in normal individuals and patients of ventricular dysfunction. The possible mechanisms consistent with the patho-physiological basis of ventricular dysfunction or heart failure with altered SV would enrich the current understanding of the disease. Heart failure is the final common pathway of multitude of cardiac pathologies. Despite etiological heterogeneity, there are common mechanisms involved in the complex electrophysiological alteration of the failing myocardium. The changes observed as a consequence of ventricular dysfunction involve ion channel remodeling, intercellular uncoupling, myocardial ischemia, alterations in calcium handling, remodeling of the extracellular matrix, the presence of scars, activation of the sympathetic & the renin-angiotensin-aldosterone system, dilatation as well as stretch of viscous etc. The source modulation of the depolarization wave and its propagation in the heart and body fluid volume conductor also influence the visual pattern of cardiac vectors. In addition, patients with heart failure receive pharmacological or non-pharmacological therapies that also influence the electrophysiological changes. We hypothesize that the spatial velocity of ventricular depolarization and repolarization vectors of the VCG loop alters with a characteristic pattern in the patients with ventricular dysfunction and can differentiate healthy individuals from patients with ventricular dysfunction and also differentiate various categories, gradations and severity stratifications of the patients with ventricular dysfunction., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2019 Elsevier Ltd. All rights reserved.)

Lysosomes are at the epicenter of cellular processes critical for inflammasome activation in macrophages. Inflammasome activation and IL-1β secretion are implicated in myocardial infarction (MI) and resultant heart failure; however, little is known about how macrophage lysosomes regulate these processes. In mice subjected to cardiac ischemia/reperfusion (IR) injury and humans with ischemic cardiomyopathy, we observed evidence of lysosomal impairment in macrophages. Inducible macrophage-specific overexpression of transcription factor EB (TFEB), a master regulator of lysosome biogenesis (Mϕ-TFEB), attenuated postinfarction remodeling, decreased abundance of proinflammatory macrophages, and reduced levels of myocardial IL-1β compared with controls. Surprisingly, neither inflammasome suppression nor Mϕ-TFEB-mediated attenuation of postinfarction myocardial dysfunction required intact ATG5-dependent macroautophagy (hereafter termed "autophagy"). RNA-seq of flow-sorted macrophages postinfarction revealed that Mϕ-TFEB upregulated key targets involved in lysosomal lipid metabolism. Specifically, inhibition of the TFEB target, lysosomal acid lipase, in vivo abrogated the beneficial effect of Mϕ-TFEB on postinfarction ventricular function. Thus, TFEB reprograms macrophage lysosomal lipid metabolism to attenuate remodeling after IR, suggesting an alternative paradigm whereby lysosome function affects inflammation.

Infants with a single ventricle can develop systemic ventricular dysfunction (SVD) after stage 1 operation, but available information is sparse. We reviewed our patients having Norwood, Sano, or hybrid procedures to better understand this problem. We conducted a retrospective, case-controlled cohort study of 267 patients having stage1 operation, examining outcomes between stages 1 and 2 (survival and subsequent cardiac surgeries), predictor variables, and histology of hearts explanted at transplantation. SVD developed in 32 (12%) patients and resolved in 13 (41%); mean age of onset was 3.0 ± 1.63 months; median = 2.79. SVD was not associated with cardiac anatomy, type of stage 1 procedure, weight, coronary abnormality, or atrioventricular valve regurgitation. The mean age of resolution = 12.1 ± 9.6 months; median = 6.3, and resolution may have been more likely with a systemic LV than RV (p = 0.067). Outcomes for the entire SVD group were less favorable than for those without, but patients with resolution of SVD had outcomes at least as good those without SVD. Myocardial histology (n = 4) suggested chronic ischemia. The risk of SVD after stage 1, while low, may be a fundamental feature of this patient population. SVD occurs with either a systemic RV or LV, although patients with a systemic LV may be more likely to have resolution than those with an RV. We identified no predictor variables, but histologic findings suggest chronic ischemia may be involved. Given the low incidence of SVD, multi-center studies will be required to better define predictors of onset and resolution.

The aim of the present study is to systematically evaluate the impact of RDN on cardiac structure and function in patients with resistant hypertension (RH) or diastolic dysfunction. We retrieved Pubmed, Embase and Cocharane Library databases, from inception to April 1 st , 2016. Studies reporting left ventricular mass (LVMI) or left ventricular (LV) diastolic function (reflected by the ratio of mitral inflow velocity to annular relaxation velocity [E/e']) responses to RDN were included. Two randomized controlled trials (RCTs), 3 controlled studies and 11 uncontrolled studies were finally identified. In observational studies, there was a reduction in LVMI, E/e' and N-terminal pro B-type natriuretic peptide (BNP) at 6 months, compared with pre-RDN values. LV ejection fraction (LVEF) elevated at 6 months following RDN. In RCTs, however, no significant change in LVMI, E/e', BNP, left atrial volume index or LVEF was observed at 12 months, compared with pharmaceutical therapy. In summary, both LV hypertrophy and cardiac function improved at 6 months after RDN. Nonetheless, current evidence failed to show that RDN was superior to intensive (optimal) drug therapy in improving cardiac remodeling and function.

Restrictive cardiomyopathy (RCM) is characterized by nondilated left or right ventricle with diastolic dysfunction. The restrictive cardiomyopathies are a heterogenous group of myocardial diseases that vary according to pathogenesis, clinical presentation, diagnostic evaluation and criteria, treatment, and prognosis. In this review, an overview of RCMs will be presented followed by a detailed discussion on 3 major causes of RCM, for which tailored interventions are available: cardiac amyloidosis, cardiac sarcoidosis, and cardiac hemochromatosis. Each of these 3 RCMs is challenging to diagnose, and recognition of each disease entity is frequently delayed. Clinical clues to promote recognition of cardiac amyloidosis, cardiac sarcoidosis, and cardiac hemochromatosis and imaging techniques used to facilitate diagnosis are discussed. Disease-specific therapies are reviewed. Early recognition remains a key barrier to improving survival in all RCMs., (© 2017 American Heart Association, Inc.)

We hypothesised that infants with ventricular dysfunction after cardiac surgery have impaired haemodynamic response to arginine-vasopressin therapy. We retrospectively reviewed the medical records of neonates and infants treated with arginine-vasopressin within 48 hours of corrective or palliative cardiac surgery who underwent echocardiographic assessment of ventricular function before initiation of therapy. Patients were classified as "responders" if their systolic blood pressure increased by ⩾10% without increase in catecholamine score or if it was maintained with decreased catecholamine score. Response was assessed 1 hour after maximum upward titration of arginine-vasopressin. A total of 36 children (15 neonates) were reviewed (17 male). The median (interquartile) age was 10.4 weeks (1.1-26.9), and the median weight was 4.3 kg (3.2-5.8). Diagnoses included single ventricle (eight), arch abnormalities (five), atrioventricular septal defect (four), double-outlet right ventricle (three), tetralogy of Fallot (three), and others (13). In all, 12 patients (33%) had ventricular dysfunction. Only 15 (42%) responded favourably according to our definition 1 hour after the "target" arginine-vasopressin dose was achieved. Ventricular dysfunction was not associated with poor response. The overall mortality was 25%, but mortality in patients with ventricular dysfunction was 42%. Favourable response was associated with shorter ICU stay (9.5 days versus 19.5 days, p=0.01). We conclude that arginine-vasopressin fails to increase blood pressure in ~50% of hypotensive children after cardiac surgery. The response rate does not increase with duration of therapy. Ventricular function does not predict haemodynamic response. The mortality in this group is very high. Prospective comparison of vasopressin with other vasoactive agents and/or inotropes is warranted.