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A 72-year-old man presented for routine dual chamber pacemaker interrogation 13 years following insertion for sick sinus syndrome. Increased noise, impedance and threshold of the right ventricular (RV) lead were identified. RV capture was maintained with an overall RV pacing burden of 47%. A routine generator replacement was scheduled alongside RV lead replacement. Fluoroscopy at the start of the procedure revealed an unexpected striking fracture of the RV pacing lead with complete separation of the proximal and distal portions within the RV. The patient was asymptomatic and described no predisposing factors. He underwent implantation of a new ventricular lead and generator and has remained well. This case demonstrates clear RV lead fracture as a late complication of pacemaker implantation despite maintained capture. This emphasises the need for a chest X-ray when a change in device parameters is noted at device interrogation even in the absence of symptoms.

Background Patients with congenitally corrected transposition of the great arteries (ccTGA) with intact ventricular septum (IVS) or d-looped transposition of the great arteries (DTGA) with IVS post atrial switch operation often develop left ventricular dysfunction after anatomical repair despite prior retraining of the morphologically left ventricle (mLV) using pulmonary artery banding (PAB). We examined histopathological changes in such mLV. Methods Capillary density, myocyte diameter, and interstitial fibrous area in the mLV were retrospectively evaluated in postmortem or explanted heart specimens obtained from patients with ccTGA/IVS or DTGA/IVS post atrial switch operation after PAB for retraining and compared with those of patients with normal cardiac anatomy, ccTGA/IVS or DTGA/IVS without PAB, and ccTGA or DTGA with high mLV pressure using generalized estimating equations models. Results Adjusting for age, capillary density in four patients with ccTGA/IVS or DTGA/IVS after PAB was ∼20% lower than that in eight patients with normal cardiac anatomy (3149 ± 863 / um2 vs 3978 ± 1206 /um2 (mean, SD); p = 0.039), while myocyte diameter was ∼50% larger (16.2 ± 4.0 um vs 11.7 ± 2.4 um (mean, SD); p Conclusions We observed significant cardiomyocyte hypertrophy but lower capillary density in patients with ccTGA/IVS or DTGA/IVS after PAB for retraining compared to normal controls. This suggests inadequate capillary growth is a potential pathological basis for mLV dysfunction occurring after retraining or anatomical repair.

BACKGROUND This study sought to determine long-term outcomes of a primary Damus-Kaye-Stansel (DKS) procedure in patients with a functional single ventricle and to compare the results with those of our historical control subjects who underwent pulmonary artery banding before the DKS procedure. METHODS The study reviewed the medical records of all patients who underwent the DKS procedure at the German Heart Center of Munich, Germany between December 1994 and December 2019. RESULTS The DKS procedure was performed as initial palliation in 52 patients (primary DKS group) and as staged palliation after pulmonary artery banding in 24 patients (staged DKS group). The median follow-up period after the DKS procedure was 8.9 years in the primary DKS group and 8.0 years in the staged DKS group. The survival rates at 10 years after the DKS procedure were 89% in the primary DKS group and 68% in the staged DKS group (log-rank P = 0.04). Before total cavopulmonary connection, the pressure gradient through the systemic ventricular outflow tract was significantly lower in the primary DKS group than in the staged DKS group (P < .001). At last follow-up echocardiography, reduced ventricular function was observed in 1 patient in the primary DKS group and in 7 patients in the staged DKS group (P < .001). The degree of neoaortic regurgitation was significantly higher in the staged DKS group than in the primary DKS group (P < .001). CONCLUSIONS A primary DKS procedure in patients with a functional single ventricle and potential systemic ventricular outflow tract obstruction is recommended to obtain favorable long-term survival with preserved ventricular function and competent semilunar valve function.

A 29-year-old woman was referred to our hospital for catheter ablation (CA) due to symptomatic atrial fibrillation (AF), after two failed CA attempts at another hospital. She had presented with left ventricular dysfunction at the age of 1 year, with a suspected diagnosis of endocardial

Paediatric cardiac tumours are rare, often benign and carry associations with genetic conditions. Cardiac fibromas are mainly composed of fibroblast and connective tissue . They can lead to symptoms due to obstruction of blood flow or arrythmias. In this case, we report an 8-month-old girl child who presented to paediatric cardiology office for cardiac evaluation given a family history of Gorlin syndrome, also known as nevoid basal cell carcinoma syndrome, found to have a large 4×4×6 cm fibroma in the apical lateral free wall of the left ventricle and later presented to the emergency department with cardiac arrest.

A 60-year-old man was referred to our hospital because of chest tightness. CT scans showed no specific findings except a right ventricular (RV) mass. Echocardiogram revealed that the large cardiac mass was compressing the right ventricular outflow tract, and the patient thus underwent an emergency operation. As the tumour on the inlet side of the right ventricle was tightly adhered to the entire tricuspid chordae, a complete resection was impossible. Pathological findings revealed that the tumour was metastatic squamous cell carcinoma. Four cycles of chemotherapy were administered. Further investigations were conducted to identify the primary cancer focus, but there were no specific findings. Eight months after the operation, the patient returned to the hospital. Echocardiogram showed a severely occupying mass once again, and the patient died as a result. Autopsy revealed no findings relating to the primary origin of the cardiac metastases.

Background Differences in left ventricular mass regression (LVMR) between transcatheter aortic valve replacement (TAVR) and surgical aortic valve replacement (SAVR) have not been studied. We present clinical and echocardiographic data from veterans who underwent TAVR and SAVR, evaluating the degree of LVMR and its association with survival. Methods We retrospectively reviewed TAVR (n = 194) and SAVR (n = 365) procedures performed in veterans from 2011 to 2019. After 1:1 propensity matching, we evaluated mortality and secondary outcomes. Echocardiographic data (median follow-up 957 days, interquartile range 483-1652 days) were used to evaluate LVMR, its association with survival, and predictors of LVMR. Results There was no difference between SAVR and TAVR patients in mortality (for up to 8 years), stroke at 30 days, myocardial infarction, renal failure, prolonged ventilation, reoperation, or structural valve deterioration. SAVR patients (67.3% [101/150]) were more likely to have LVMR than TAVR patients (55.7% [44/79], p = 0.11). The magnitude of LVMR was greater for the SAVR patients (median = −23.3%) than for the TAVR patients (median = −17.8%, p = 0.062). SAVR patients with LVMR had a survival advantage over SAVR patients without LVMR (p = 0.016). However, LVMR was not associated with greater survival in TAVR patients (p = 0.248). Conclusions SAVR patients were more likely to have LVMR and had a greater magnitude of LVMR than TAVR patients. LVMR was associated with better survival in SAVR patients, but not in TAVR patients.

[Background] To describe long-term survival and cardiovascular events in adult patients with single ventricle physiology (SVP) without Fontan palliation, focusing on predictors of mortality and comparing groups according to their cardiovascular physiology., [Methods] Multicentre observational and retrospective study including adult patients with SVP without Fontan palliation since their first adult clinic visit. The cohort was subdivided into 3 groups: Eisenmenger, restricted pulmonary flow, and aortopulmonary shunt. Death was considered as the main end point. Other clinical outcomes occurring during follow-up were considered as secondary end points., [Results] A total of 146 patients, mean age 32.5 ± 11.1 years, were analysed. Over a mean follow-up of 7.3 ± 4.1 years, 33 patients (22.6%) died. Survival was 86% and 74% at 5 and 10 years, respectively. Right ventricular morphology was not associated with higher mortality. Four variables at baseline were related to a higher mortality: at least moderate atrioventricular valve regurgitation, platelet count < 150 × 103/mm3, GFR < 60 mL/min/1.73 m2, and QRS > 120 ms). A total of 34.2% of patients were admitted to the hospital due to heart failure, and 7.5% received a heart transplant. Other cardiovascular outcomes were also frequent: atrial arrhythmias in 19.2%, stroke in 15.1%, and pacemaker/implantable cardioverter-defibrillator in 6.2%/2.7%., [Conclusions] Adult patients with SVP who had not undergone Fontan exhibit a high mortality rate and frequent major cardiovascular events. At least moderate atrioventricular valve regurgitation, thrombocytopenia, renal dysfunction, and QRS duration > 120 ms at baseline visit allow identification of a cohort of patients at higher risk of mortality., [Objectif] Décrire la survie à long terme et les événements cardiovasculaires chez les patients adultes présentant une physiologie à ventricule unique (PVU) sans intervention de Fontan, en se concentrant sur les prédicteurs de mortalité et en comparant les groupes en fonction de leur physiologie cardiovasculaire., [Méthodes] Étude observationnelle et rétrospective, multicentrique, incluant des patients adultes atteints de PVU sans intervention de Fontan depuis leur première visite en clinique adulte. La cohorte a été subdivisée en trois groupes : syndrome d'Eisenmenger, flux pulmonaire restreint ou fenêtre aorto-pulmonaire. Le décès a été considéré comme le critère principal d'évaluation. Les autres observations cliniques survenues au cours du suivi ont été considérées comme des critères secondaires., [Résultats] Un total de 146 patients, d'un âge moyen de 32,5 ± 11,1 ans, a été considéré pour l’analyse. Sur un suivi moyen de 7,3 ± 4,1 ans, 33 patients (22,6 %) sont décédés. La survie était de 86 % et 74 % à 5 et 10 ans, respectivement. La morphologie du ventricule droit n'était pas associée à une mortalité plus élevée. Quatre variables initiales étaient liées à une mortalité plus élevée : régurgitation au moins modérée de la valve auriculo-ventriculaire, numération plaquettaire < 150 × 103/mm3, DFG < 60 ml/min/1,73 m2 et QRS > 120 ms). Au total, 34,2 % des patients ont été admis à l'hôpital en raison d'une insuffisance cardiaque, et 7,5 % ont reçu une transplantation cardiaque. D'autres conséquences cardiovasculaires étaient également fréquentes : arythmies auriculaires dans 19,2 % des cas, accident vasculaire cérébral dans 15,1 % des cas et stimulateur cardiaque/ défibrillateur cardioverteur implantable dans 6,2 % / 2,7 % des cas., [Conclusions] Les patients adultes atteints de PVU qui n'ont pas subi d'intervention de Fontan présentent un taux de mortalité élevé et des événements cardiovasculaires majeurs fréquents. Une régurgitation au moins modérée de la valve auriculo-ventriculaire, une thrombocytopénie, une dysfonction rénale et une durée du QRS > 120 ms lors de la visite initiale permettent de distinguer une cohorte de patients présentant un risque de mortalité plus élevé.

Background The Fontan procedure, the last of a series of palliative operations for patients born with single ventricles, is associated with a significant late burden of complications. There are other strategies for patients who are suboptimal candidates for Fontan completion; however, the long-term outcomes of these different surgical options have not been clearly elucidated. We performed a systematic literature review to establish the current role of other treatment approaches besides the Fontan procedure. Methods The MEDLINE and Embase databases were systematically searched for articles describing the long-term outcomes of patients with single ventricles who have not received the Fontan procedure. Results A total of 36 articles met all inclusion criteria. There is a scarcity of contemporary data on the non-Fontan cohort. Historical studies provided a significant contribution. Conclusions Long-term survival of unoperated patients with single ventricles is possible under the rare conditions of having balanced hemodynamics. As many as half of patients may survive on only a systemic-to-pulmonary artery shunt or bidirectional cavopulmonary shunt for more than 20 years with reasonable functional status. In patients with a failing single ventricle, the bidirectional cavopulmonary shunt is an excellent bridge to heart transplantation and may provide better posttransplant survival than patients with a Fontan circulation. Currently, the Fontan procedure continues to be the best definitive palliation for patients born with single ventricle lesions. However, for those with borderline indications, other strategies should be carefully considered.

BACKGROUND Patients with chronic obstructive pulmonary disease (COPD) can develop left ventricular (LV) systolic dysfunction and geometric changes due to several reasons. We investigated subclinical LV systolic dysfunction and structural features in patients with COPD, and its correlation with the severity of airway obstruction, identified by GOLD classification. METHODS We studied 52 patients with COPD and 29 age and sex-matched controls, without any cardiac disease. In addition to conventional echocardiographic evaluation speckle tracking echocardiography (STE) based strain imaging were performed to analyze sub-clinical LV systolic dysfunction. Also LV volumes were measured by using three dimensional real time echocardiography (3DRTE). All patients underwent spirometry. RESULTS Conventional echocardiographic parameters (LV wall thickness and diameters, LV EF) and LV volume measurements were similar between the groups. LV global longitudinal peak systolic strain (-14.76 ± 2.69% to -20.27 ± 1.41%, p

The Single Ventricle Reconstruction Trial demonstrated increased risk of death or heart transplant 1 year after Norwood among subjects randomly assigned to modified Blalock-Taussig shunts compared with right ventricle to pulmonary artery (RV-PA) shunts. We used the Single Ventricle Reconstruction Trial public use database to evaluate incidence and risk factors for postoperative renal failure and relationships between renal failure, shunt type, and outcomes after Norwood.Postoperative renal failure was defined a priori as a threefold rise in creatinine from baseline, or dialysis use, within 7 days of Norwood. We used multivariate logistic regression to evaluate risk factors for postoperative renal failure and Cox hazard regression to determine the association between postoperative renal failure and 1 year after Norwood mortality.Overall, postoperative renal failure occurred in 8.4% (46 of 544) patients with risk factors including receipt of a modified Blalock-Taussig shunt (adjusted odds ratio [aOR] 3.3, P = .02), low center volume (aOR 2.7, P = .005), two or more preoperative complications (aOR 4.0, P.001), low birth weight (aOR 3.2, P = .002), postoperative heart block (aOR 8.5, P = .001), and delayed sternal closure (aOR 5.3, P = .026). Renal failure was an independent risk factor for 1-year mortality (aHR 1.9, P = .019). Assessing interaction by shunt type, mortality risk associated with renal failure was greatest in the RV-PA shunt group (aHR 3.3 vs RV-PA shunt without renal failure, P = .001), but was also increased in the modified Blalock-Taussig shunt group (aHR 1.9, P = .03).Postoperative renal failure is common after Norwood and is independently associated with mortality. Although renal failure is more common after modified Blalock-Taussig shunt, the highest mortality risk with renal failure occurs after RV-PA shunt.

Background: In case of long-term and physiological loads (e.g. during pregnancy or regular athletics training), reversible morphological changes occur in the heart - cardiomyocytes undergo hypertrophy, however, this is not accompanied by impairment of left ventricular function or myocyte metabolism. However, in the course of various pathological processes, as time goes by, gradually permanent morphological changes occur. These changes are referred to as remodeling of the heart muscle, which, regardless of the primary cause, can lead to the development of chronic heart failure. Materials and methods: The study was performed on post-mortem material of 35 human hearts obtained from forensic sections and anatomopathological sections of people who died of non-cardiac causes (mainly traffic accidents, suicide attempts, strokes, acute infections); material was fixed in a 4% formalin solution. The hearts were subjected to macro- and microscopic assessment. During microscopic assessment the features of remodeling were evaluated. Results and conclusions: In vivo and echocardiographic tests, as well as macroscopic evaluation of post-mortem material, suggest the presence of some kind of right ventricular muscle remodeling, however classic microscopic observations, presented in this study do not provide such unambiguous evidence. Thus, the question arises: why and how the right ventricular function is disturbed, sometimes at early stages of arterial hypertension.

For diagnosing cardiovascular disease, an accurate segmentation method is needed. There are several unresolved issues in the complex field of cardiac magnetic resonance imaging, some of which have been partially addressed by using deep neural networks. To solve two problems of over-segmentation and under-segmentation of anatomical shapes in the short-axis view from different cardiac magnetic resonance sequences, we propose a novel two-stage framework with a weighted decision map based on convolutional neural networks to segment the myocardium (Myo), left ventricle (LV), and right ventricle (RV) simultaneously. The framework comprises a decision map extractor and a cardiac segmenter. A cascaded U-Net++ is used as a decision map extractor to acquire the decision map that decides the category of each pixel. Cardiac segmenter is a multiscale dual-path feature aggregation network (MDFA-Net) which consists of a densely connected network and an asymmetric encoding and decoding network. The input to the cardiac segmenter is derived from processed original images weighted by the output of the decision map extractor. We conducted experiments on two datasets of multi-sequence cardiac magnetic resonance segmentation challenge 2019 (MS-CMRSeg 2019) and myocardial pathology segmentation challenge 2020 (MyoPS 2020). Test results obtained on MyoPS 2020 show that the average Dice coefficients of the proposed method on the segmentation tasks of Myo, LV and RV are 84.70%, 86.00%, and 86.31%, respectively.

There are two borderline left-heart phenotypes in the fetus: (1) severe aortic stenosis (AS), which is associated with a 'short, fat', globular left ventricle (LV), LV systolic dysfunction and LV growth arrest; and (2) severe left-heart hypoplasia (LHH), which is associated with a 'long, skinny' LV, laminar flow across hypoplastic mitral and aortic valves and arch hypoplasia. Both phenotypes may be counseled for possible single-ventricle palliation. We aimed to compare the rates of left-sided cardiac structure growth and Z-score change over gestation and to describe the postnatal outcomes associated with these two phenotypes. We hypothesized that the left-sided structures would grow faster in fetuses with LHH compared to those with AS, and that those with LHH would be more likely to achieve biventricular circulation.This was a retrospective cohort study using data collected in an institutional cardiology database between April 2001 and April 2018. We included fetuses with severe AS or severe LHH, and with at least two fetal echocardiograms. Inclusion criteria for the AS group included: aortic-annulus Z-score -2.0, severe AS, depressed LV function, retrograde systolic flow in the aortic arch and endocardial fibroelastosis. Inclusion criteria for the LHH group included: aortic-annulus Z-score -2.0, hypoplastic but apex-forming LV, normal LV function and retrograde systolic flow in the aortic arch. Exclusion criteria were: abnormal cardiac connections, other forms of structural congenital heart disease, cardiomyopathy, history of fetal aortic valvuloplasty and participation in a maternal hyperoxygenation study. Measurements and respective Z-scores for the aortic-valve annulus, mitral-valve annulus, LV long- and short-axis dimensions, along with aortic-arch measurements, were collected longitudinally for each fetus and plotted over time for both cohorts. Mean slopes of change in dimension and Z-scores over gestation were calculated and compared between the two groups using mixed generalized linear regression accounting for repeated measures. A subanalysis was performed, matching six fetuses from each group for initial aortic-annulus Z-score and gestational age, due to the significant differences in these two variables between the original cohorts.In total, 53 fetuses with 158 echocardiograms were included. In the AS cohort, there were 20 (38%) fetuses with 65 echocardiograms, and in the LHH cohort there were 33 (62%) fetuses with 93 echocardiograms. On the first echocardiogram, LHH fetuses had a later gestational age and a larger aortic-annulus diameter. The rate of aortic-annulus growth was greater in the LHH group compared with the AS group (mean ± SD, 0.15 ± 0.01 mm/week for LHH vs 0.07 ± 0.01 mm/week for AS (P 0.001)). While the LHH group had a decrease in aortic-annulus Z-score over time, this was at a slower rate than the decrease in the AS group (mean ± SD, -0.04 ± 0.02/week for LHH vs -0.13 ± 0.02/week for AS (P 0.001)). A similar pattern was seen for the mitral-valve and LV short-axis-dimension Z-scores. Subanalysis of six fetuses from each group matched for initial aortic-annulus Z-score and gestational age demonstrated similar findings, with the LHH group Z-scores decreasing at a slower rate than those in the AS group. Fifty-two of the 53 fetuses were liveborn, one LHH fetus dying in utero. Of the 20 liveborn in the AS cohort, 15 (75%) infants underwent single-ventricle palliation, two (10%) underwent biventricular repair and three (15%) died prior to intervention. Of the 32 liveborn in the LHH cohort, three (9.4%) underwent single-ventricle palliation, 28 (87.5%) achieved biventricular circulation, of which six required no surgery, and one (3.1%) died prior to intervention.The left-sided cardiac structures grow at a faster rate in fetuses with severe LHH than they do in fetuses with severe AS, and the Z-scores decrease at a slower rate in fetuses with severe LHH than they do in those with severe AS. The majority of infants in the LHH group did not undergo single-ventricle palliation. This information can be useful in counseling families on the expected growth potential of the fetus's aortic valve, mitral valve and LV, depending on the cardiac phenotype. © 2022 International Society of Ultrasound in Obstetrics and Gynecology.

Neonatal myocardial infarction due to coronary thrombus is a rare cause of acute heart failure and is associated with high morbidity and mortality. We present a rare case of a full-term newborn who developed coronary artery thrombus treated with intracoronary recombinant tissue plasminogen activator infusion while undergoing therapeutic hypothermia. Also, we describe a unique treatment strategy to support systemic circulation sparing the patient from neonatal extracorporeal membrane oxygenation and its complications. Neonatal myocardial infarction should be suspected and ruled out in sick newborns.

Background: Although the assessment of right ventricular (RV) diastolic function is feasible, it has garnered far less momentum for use compared with its left ventricular counterpart. The scarcity of data defining normative RV diastolic function and the fact that implications of RV diastolic dysfunction in different disease states on outcomes are less well known both hinder integration into routine clinical assessment. The aim of this study was to establish normal values of RV diastolic parameters stratified by sex, age, and race using data from the World Alliance Societies of Echocardiography study. Methods: A subset of 888 normal subjects from the World Alliance Societies of Echocardiography database were analyzed, including measurements of tricuspid valve (TV) inflow E- and A-wave velocities, E-wave deceleration time, and TV annular tissue Doppler e′ and a′ velocities. Additionally, right atrial (RA) maximal volume and RA peak reservoir strain were measured. Patients were grouped by age ([removed]65 years) and stratified by sex and race. Differences were analyzed using unpaired t tests. Results: Compared with men, women had significantly higher TV e′ and E-wave and A-wave velocities, though differences were modest. Increasing age was associated with stepwise lower TV E wave, e′ velocity, and TV E/A ratio and higher a′ velocity and E/e′ ratio. RA peak reservoir strain was also lower, and RA end-systolic volume trended toward being smaller for older age groups. Asian subjects demonstrated significantly higher a′ velocities, lower E wave, the smallest RA end-systolic volumes, and the lowest RA peak strain values compared with white subjects of both sexes. Conclusions: This study provides normal values for parameters used in the assessment of RV diastolic function stratified by race, sex, and age. The results demonstrate significant differences in RV diastolic parameters between age groups, which manifest in both individual parameters and composite ratios of TV inflow and annular velocities. Although limited sex- and race-related differences were also noted, age appears to have the most significant impact on RV diastolic parameters. These findings may aid in refining current normative values.

Acute myocarditis (AM) and hypertensive heart disease (HHD) have different pathophysiological backgrounds, thus potentially showing distinct patterns of altered myocardial deformation. Therefore, CMR left ventricular (LV) feature tracking (FT)- based strain parameters were indexed to myocardial mass index (LVMi) in order to evaluate potential additional value in the differentiation among AM, HHD, and healthy volunteers (HV) compared to non-indexed conventional strain.Patients with AM (n = 43) and HHD (n = 28) underwent CMR at 3T. 61 HV served as controls. Cine imaging-based FT-strain analysis was performed and natural strain (nStrain) values were evaluated for gender and age specific differences in HV. Strain parameters were indexed to LVMi yielding ratio Strain (rStrain). These were evaluated for their discriminatory accuracy compared to nStrain values.There were significant differences in nStrain between genders (p0.05), but not between age groups in HV. Circumferential strains differentiated best between HV and AM, reaching an area under the curve (AUC) of 0.86 (female) and 0.81 (male), yielding 93 (72) % sensitivity and 55 (75) % specificity. In discriminating between HV and HHD as well as AM and HHD, longitudinal strains outperformed all other parameters with AUCs of 1.00 (female)/ 0.92 (male) and 0.90 (female)/ 0.74 (male), respectively. Sensitivity and specificity levels of 100 %/ 100 % (female) and 91 %/ 72 % (male) for HV versus AM as well as 82 %/ 71 % (female) and 91%/ 57 % (male) for AM versus HHD could be demonstrated. The usage of rStrains significantly increased the AUC for circumferential and radial strains in male patients.rStrain provided additional value in the differentiation of diseases with increased LVM. As rStrain is derived from standard native cine imaging, such parameters can be time efficiently and reliably calculated, giving them the potential to be a powerful addition to the currently developing multiparametric native diagnostic approaches.

Although patients with systemic right ventricle (SRV) represent a significant proportion of patients with congenital heart disease (CHD) implanted with cardiac resynchronization therapy (CRT), there are limited and conflicting data in this specific patient group.We aimed to analyze outcomes of patients with SRV implanted with a CRT device.Data were analyzed from an observational, retrospective, multicenter cohort study including all patients with CHD implanted with a CRT device from 6 French centers from 2004 to 2020. Response to CRT was defined as an increase in systemic ventricular ejection fraction of ≥10% and/or an improvement in New York Heart Association functional class by at least 1 grade.A total of 85 patients with CHD were enrolled (mean age 39.8 ± 20.0 years; 55 [64.7%] males; 25 defibrillators [29.4%]), including 31 patients with SRV (36.5%) (mean age 43.9 ± 19.8 years; 16 [51.6%] males). The mean change in QRS duration after implantation was similar as compared with patients with systemic left ventricle (-46 ± 26 ms vs -35 ± 32 ms; P = .16). During a mean follow-up of 5.1 ± 3.5 years, late complications included 2 lead dysfunctions (6.5%), 3 CRT-related infections (9.7%), and 1 inappropriate implantable cardioverter-defibrillator shock (3.2%). The proportion of CRT responders at 6, 12, and 24 months were 82.6%, 80.0%, and 77.8% in patients with SRV vs 66.7%, 64.3%, and 69.6% in patients with systemic left ventricle (P = NS).In this multicenter cohort, one-third of patients with CHD implanted with a CRT device had SRV. CRT in patients with SRV was associated with a high rate of responders, comparable to that of patients with systemic left ventricle.

Background:Surgical repair for postinfarction ventricular septal rupture (VSR) was still a challenging operation, especially when required in the acute phase or posterior VSR. The extended sandwich patch technique is characterized by large double patches fixed by circumferential transmural large mattress sutures via right ventricle incision. In the present study, we report early and midterm clinical and echocardiographic outcomes of this technique., Methods:Of 33 consecutive patients undergoing VSR repair using the extended sandwich patch technique between 2008 and 2018, 25 received surgery within 1 week from the onset of myocardial infarction (early group) and 8 received surgery after 1 week (late group), including 12 patients with posterior VSR. All patients underwent surgery within 24 hours of VSR diagnosis., Results:Intraoperative transesophageal echocardiography identified no residual shunt, but 2 patients received reoperative VSR repair at 9 days and 2 months after the first VSR repair. The 30-day mortality and 1-year survival in the early and late groups were 20% and 12.5%, and 58% and 88%, respectively. After VSR repair, right and left heart chamber sizes were significantly decreased, and these improvements were stable throughout the subsequent follow-up period (median 4.0 years). Tricuspid annular plane systolic excursion and right ventricular fractional area change remained constant throughout the observation period without changing after VSR repair., Conclusions:The extended sandwich patch technique through a right ventriculotomy offered safe and simple, leak-free repair even in technically demanding acute phase or posterior VSR. No significant decline was found in the right heart function after surgery.

Background Ventricular interdependence may account for altered ventricular mechanics in congenital heart disease. The present study aimed to identify differences in load-dependent right ventricular (RV)–left ventricular (LV) interactions in porcine models of pulmonary stenosis (PS) and pulmonary insufficiency (PI) by invasive admittance-derived hemodynamics in conjunction with noninvasive cardiovascular magnetic resonance (CMR). Methods Seventeen pigs were used in the study (7 with PS, 7 with PI, and 3 controls). Progressive PS was created by tightening a Teflon tape around the pulmonary artery, and PI was created by excising 2 leaflets of the pulmonary valve. Admittance catheterization data were obtained for the RV and LV at 10 to 12 weeks after model creation, with the animal ventilated under temporary diaphragm paralysis. CMR was performed in all animals immediately prior to pressure–volume catheterization. Results In the PS group, RV contractility was increased, manifested by increased end-systolic elastance (mean difference, 1.29 mm Hg/mL; 95% confidence interval [CI], 0.57-2.00 mm Hg/mL). However, in the PI group, no significant changes were observed in RV systolic function despite significant changes in RV diastolic function. In the PS group, LV end-systolic volume was significantly lower compared with controls (mean difference, 25.1 mL; 95% CI, -40.5 to -90.7 mL), whereas in the PI group, the LV showed diastolic dysfunction, demonstrated by an elevated isovolumic relaxation constant and ventricular stiffness (mean difference, 0.03 mL−1; 95% CI, -0.02 to 0.09 mL−1). Conclusions The LV exhibits systolic dysfunction and noncompliance with PI. PS is associated with preserved LV systolic function and evidence of some LV diastolic dysfunction. Interventricular interactions influence LV filling and likely account for differential effects of RV pressure and volume overload on LV function.

Background COVID-19 infection is known to cause a wide array of clinical chronic sequelae, but little is known regarding the long-term cardiac complications. We aim to report echocardiographic follow-up findings and describe the changes in left (LV) and right ventricular (RV) function that occur following acute infection. Methods Patients enrolled in the World Alliance Societies of Echocardiography-COVID study with acute COVID-19 infection were asked to return for a follow-up transthoracic echocardiogram. Overall, 198 returned at a mean of 129 days of follow-up, of which 153 had paired baseline and follow-up images that were analyzable, including LV volumes, ejection fraction (LVEF), and longitudinal strain (LVLS). Right-sided echocardiographic parameters included RV global longitudinal strain, RV free wall strain, and RV basal diameter. Paired echocardiographic parameters at baseline and follow-up were compared for the entire cohort and for subgroups based on the baseline LV and RV function. Results For the entire cohort, echocardiographic markers of LV and RV function at follow-up were not significantly different from baseline (all P > .05). Patients with hyperdynamic LVEF at baseline (>70%), had a significant reduction of LVEF at follow-up (74.3% ± 3.1% vs 64.4% ± 8.1%, P < .001), while patients with reduced LVEF at baseline (−20%) at baseline had significant improvement at follow-up (−15.2% ± 3.4% vs −17.4% ± 4.9%, P = .004). Patients with abnormal RV basal diameter (>4.5 cm) at baseline had significant improvement at follow-up (4.9 ± 0.7 cm vs 4.6 ± 0.6 cm, P = .019). Conclusions Overall, there were no significant changes over time in the LV and RV function of patients recovering from COVID-19 infection. However, differences were observed according to baseline LV and RV function, which may reflect recovery from the acute myocardial injury occurring in the acutely ill. Left ventricular and RV function tends to improve in those with impaired baseline function, while it tends to decrease in those with hyperdynamic LV or normal RV function.

Background The objective of this study is to assess the safety and early impact of intramyocardial delivery of autologous bone marrow–derived mononuclear cells (BM-MNC) at time of surgical Ebstein repair. Methods Patients with Ebstein anomaly (ages 6 months to 30 years) scheduled to undergo repair of the tricuspid valve were eligible to participate in this open-label, non-randomized phase I clinical trial. BM-MNC target dose was 1-3 million cells/kg. Ten patients have undergone surgical intervention and cell delivery to the right ventricle (RV) and completed 6-month follow-up. Results All patients underwent surgical tricuspid valve repair and uneventful BM-MNC delivery; there were no ventricular arrhythmias and no adverse events related to study product or delivery. Echocardiographic RV myocardial performance index improved and RV fractional area change showed an initial decline and then through study follow-up. There was no evidence of delayed myocardial enhancement or regional wall motion abnormalities at injection sites on 6-month follow-up magnetic resonance imaging. Conclusions Intramyocardial delivery of BM-MNC after surgical repair in Ebstein anomaly can be performed safely. Echocardiography variables suggest a positive impact of cell delivery on the RV myocardium with improvements in both RV size and wall motion over time. Additional follow-up and comparison to control groups are required to better characterize the impact of cell therapy on the myopathic RV in Ebstein anomaly.

Objective The study objective was to evaluate the short- and long-term outcomes of patients with ischemic cardiomyopathy after surgical ventricular restoration and to identify risk factors related to poor results. Methods Between August 2002 and April 2016, 62 patients affected by ischemic cardiomyopathy underwent surgical left ventricular restoration at our unit. Patients' mean age at operation was 63 years (39-79 years). Mean ejection fraction was 29.6%. The Surgical Treatment for Ischemic Heart Failure trial criteria have been used as indications for surgery. Fifty-seven patients (91%) received surgical myocardial revascularization. Mitral valve repair was performed in 39 patients (63%). The surgical technique consisted of the classic Dor operation or a different approach reducing the equatorial diameter of the left ventricle and avoiding the use of a patch. The data were analyzed retrospectively for perioperative results and short- and long-term clinical outcomes. Results One patient died of noncardiac causes within 30 days (1.6%). All-cause death occurred in 36 patients (58%) during follow-up (0.6-14.7 years; median follow-up time, 7.02 years), of whom 15 died of cardiac causes. Age, need for preoperative intra-aortic balloon pump, reduction less than 35% of postoperative left ventricular end-diastolic and end-systolic volumes, type of surgical technique, and ejection fraction less than 25% were identified as risk factors for late cardiac mortality. Perioperative levosimendan administration and presence of preoperative moderate to severe mitral regurgitation influenced early and intermediate-term outcomes, but no statistical relevance on long-term results was demonstrated. Conclusions Patients with ischemic dilative cardiomyopathy have favorable short- and long-term outcomes after ventricular restoration. Age, preoperative ejection fraction less than 25%, inadequate left ventricular surgical reverse remodeling, and type of surgical technique negatively affect long-term survival.

Background Obesity is considered a major cardiovascular risk factor. The excess of pericardial fat (PF) in patients with obesity has been associated with a variety of electrocardiographic alterations. In previous studies, we demonstrated that rapid weight loss and bariatric interventions result in decreased PF. Objectives The aim of this study is to report the changes in PF after bariatric surgery and its effect on ventricular conduction. Setting US hospital, academic institution. Methods A linear measurement of PF thickness on computed tomography scans was obtained for 81 patients, as well as a retrospective review of electrocardiographic changes before and after bariatric surgery. We compared the changes in PF thickness and electrocardiographic components before and after procedures. Common demographics and co-morbidities were collected along with lipid profiles preoperative and postoperative. Results A total of 81 patients had electrocardiograms done before and 1 year after bariatric surgery. Females comprised 67.9% (n = 55), and the average age for our population was 55.07 ± 14.17 years. Pericardial fat thickness before surgery was 5.6 ± 1.84 and 4.5 ± 1.62 mm after surgery (P = .0001). Ventricular conduction (QT and QT corrected [QTc] intervals) showed a significant improvement from 438.7 + 29 before to 426.8 + 25.3 after bariatric surgery (P = .006). We found a statistically significant association between the decrease in PF and the decrease in QTc intervals (P = .002). Conclusion Obesity is a risk factor for arrhythmias and sudden cardiac death. Bariatric surgery and its effect on PF produce an improvement in ventricular conduction, which may reduce the ventricular electrical instability in patients with obesity.

Pulmonary arterial hypertension is characterized by obliteration and obstruction of the pulmonary arterioles that in turn results in high right ventricular afterload and right heart failure. The pathobiology of pulmonary arterial hypertension is complex, with contributions from multiple pathophysiologic processes that are regulated by a variety of molecular mechanisms. This nature likely explains the limited efficacy of our current therapies, which only target a small portion of the pathobiological mechanisms that underlie advanced disease. Here we review the pathobiology of pulmonary arterial hypertension, focusing on the systemic, cellular, and molecular mechanisms that underlie the disease.

BACKGROUND There is little insight into which patients can be weaned off right ventricular (RV) acute mechanical circulatory support (AMCS) after left ventricular assist device (LVAD) implantation. We hypothesize that concomitant RV AMCS insertion instead of postoperative implantation will improve 1-year survival and increase the likelihood of RV AMCS weaning. METHODS A multicenter retrospective database of 826 consecutive patients who received a HeartMate II or HVAD between January 2007 and December 2016 was analyzed. We identified 91 patients who had early RV AMCS on index admission. Cox proportional-hazards model was constructed to identify predictors of 1-year mortality post-RV AMCS implantation and competing risk modeling identified RV AMCS weaning predictors. RESULTS There were 91 of 826 patients (11%) who required RV AMCS after CF-LVAD implantation with 51 (56%) receiving a concomitant RV AMCS and 40 (44%) implanted with a postoperative RV AMCS during their ICU stay; 48 (53%) patients were weaned from RV AMCS support. Concomitant RV AMCS with CF-LVAD insertion was associated with lower mortality (HR 0.45 [95% CI 0.26-0.80], p = 0.01) in multivariable model (which included age, BMI, angiotensin-converting enzyme inhibitor use, and heart transplantation as a time-varying covariate). In the multivariate competing risk analysis, a TPG < 12 (SHR 2.19 [95% CI 1.02-4.70], p = 0.04) and concomitant RV AMCS insertion (SHR 3.35 [95% CI 1.73-6.48], p < 0.001) were associated with a successful wean. CONCLUSIONS In patients with RVF after LVAD implantation, concomitant RV AMCS insertion at the time of LVAD was associated with improved 1-year survival and increased chances of RV support weaning compared to postoperative insertion.

Mitral annular disjunction (MAD) is an abnormal insertion of the hinge line of the posterior mitral leaflet on the atrial wall: the mitral annulus shows a separation or "disjunction" between the leaflet-atrial wall junction and the crest of the left ventricle myocardium. This anomaly is often observed in patients with myxomatous mitral valve prolapse. The anatomical substrate of MAD remains unclear for the following reasons: (1) most studies are focused on the association between MAD and arrhythmias, rather than on pathomorphological aspects of MAD; and (2) the complex anatomic architecture of the posterior mitral annulus is often simply described as the posterior segment of a fibrous ring. The aims of this paper are to review the pertinent normal anatomy of the mitral valve and to propose new hypotheses on the morphological nature of MAD.

Cardiac resynchronization therapy (CRT) is an established treatment for heart failure patients with myocardial dysfunction and delayed ventricular activation, but approximately 25% to 40% of patients do not respond to CRT. Left ventricular (LV) multisite pacing (MSP) has been proposed as a tool to improve CRT response. The goal of this study is to examine the safety and efficacy of LV MSP in CRT nonresponders. Between January 2018, and September 2019, the Strategic Management to Improve CRT Using Multi-Site Pacing trial prospectively enrolled 584 CRT-defibrillator recipients for established indications at 52 sites across the United States and evaluated their response at 6 months using the clinical composite score (CCS). Of the nonresponders, 102 patients had the LV MSP feature turned on and 78 patients completed the 12-month CCS evaluation. The LV MSP feature-related complication-free rate was 99.0% with a lower 95% confidence interval limit of 94.9%, which was higher than the performance goal of 90%. The proportion of nonresponders with an improved CCS from 6 to 12 months was 51.3% with a lower 95% confidence interval limit of 41.4%, which was higher than the performance goal of 5%. The estimated mean reduction in battery longevity with the LV MSP feature was about 3.6 months (estimated battery longevity of 8.87 ± 2.08 years at 6 months and 8.07 ± 2.23 years at 12 months). In conclusion, in CRT nonresponders, the use of the LV MSP feature is safe and associated with a ∼50% conversion rate with a small projected reduction in CRT-defibrillator battery longevity. LV MSP should be considered in the management of CRT nonresponders.

This study aimed to compare the efficacy and safety of ablation with high and low power settings using either a flexible tip or straight SF tip irrigated catheter in the left ventricle (LV) using a peripheral microemboli monitoring system.The microemboli risk of flexible and straight SF tip irrigated catheters in creating ablative lesions in the LV at variable power settings has not been adequately assessed.Six pigs underwent catheter ablation in the LV using a flexible tip or straight SF tip catheter with 2 energy settings (30 or 50 W, 30 seconds, irrigation saline 17 mL/min).A total of 79 radiofrequency (RF) applications were assessed. High power settings via a flexible tip formed a significantly higher arterial microbubble volume in the extracorporeal circulation (P = 0.005). Notably, RF applications with a steam pop induced an exponential increase of microbubble volume with both catheters. A higher power setting induced a significantly higher number of microembolic signals on carotid artery Doppler ultrasound with a flexible tip irrigated catheter (P 0.001). Similarly, the straight SF tip irrigated catheter tended to increase the number of microembolic signals with 50 W (P = 0.091).RF ablation at high power settings in the LV carries a risk of microembolic events compared with lower power settings. When high power settings are used for creating ablative lesions for deep intramural foci in the LV, the risk of microembolic events induced by RF ablation should be carefully monitored.

We studied conduit-related risk factors for mortality, conduit reintervention, conduit replacement, and pulmonary artery (PA) reinterventions after truncus repair. Patients who underwent truncus repair at our institution between 1995 and 2019 were studied. Cox proportional hazards modeling evaluated variables for association with mortality, time to conduit reintervention, time to conduit replacement, and time to PA reintervention. Truncus was repaired in 107 patients at median age of 17 days (IQR 9-45). Median follow-up time was 7 years. Aortic homografts were implanted in 57 (53%) patients, pulmonary homograft in 40 (37%), and bovine jugular conduit in 10 (9%). Median conduit size was 11 mm (IQR 10-12) and median conduit Z-score was 1.71 (IQR 1.08-2.34). At 5 years, there was 87% survival, 21% freedom from conduit reinterventions, 37% freedom from conduit replacements, and 55% freedom from PA reinterventions. Conduit size (HR 0.7, 95%CI 0.4-1.4, p=.41) and type (aortic homograft reference; bovine jugular vein graft HR 0.6, 95% CI 0.08-5.2, p=.69; pulmonary homograft HR 0.7, 95% CI 0.2-2.3, p=.58) were not associated with mortality. On multivariate analysis, the hazard for conduit reintervention, conduit replacement, and PA reintervention decreased with increasing conduit Z-score values of 1 to 2.5 (non-linear relationship, p.01), with little additional reduction in hazard beyond this range. Implantation of a larger conduit within Z-score values of 1 and 2.5 is associated with a decreased hazard for conduit reintervention, conduit replacement, and PA reintervention after truncus repair. The type and size of the conduits did not impact mortality.

Patients with borderline hypoplastic right or left ventricle and VSD may be candidates for either single ventricle palliation or staged ventricular recruitment (SVR) followed by eventual biventricular conversion. Components of SVR include restriction of atrial septal defects (ASD) without ventricular septal defects (VSD) closure and addition of accessory pulmonary blood flow. This study evaluated the impact of ASD restriction on ventricular growth and function. We retrospectively reviewed patients with borderline ventricular hypoplasia and VSD who underwent a staged ventricular recruitment (SVR) procedure from 2012 to June 2019. Pre- and post-recruitment MRI and echocardiogram data were compared and analyzed. We excluded cases in which we intentionally restricted VSD with simultaneous ASD restriction. Forty-six patients (41 with right-dominant ventricle, 25 with risk factors for Fontan procedure) underwent SVR at a median age of 15.1 months' (interquartile range (IQR), 7.2-37.2 months'). The median indexed ventricular end-diastolic volume, end-systolic volume, and stroke volume according to cardiac MRI significantly increased at median 11.0 months' (IQR:7.8~14.1 months') after recruitment. Among them, except 2 operative mortalities after SVR, 26 patients underwent bi-ventricular repair (56.5% including one and a half ventricle repair) at a median of 8.0 months' (IQR: 6.2-12.2 months') after recruitment. Fifteen patients await biventricular completion, and 3 patients underwent single ventricle palliation. Pulmonary blood flow (Qp) tended to increase after recruitment regardless of type of pulmonary blood flow modification without statistical significance. Six patients died at a median duration of 6.5 months' (IQR: 2.9-11.7) after SVR; 3 patients died after biventricular completion, 2 after recruitment, and 1 after returning to single ventricle palliation. All of them were considered poor Fontan candidates due to severe atrioventricular valve regurgitation, pulmonary hypertension, pulmonary vein stenosis, or airway stenosis. Restriction of the atrial septum leads to the growth of hypoplastic ventricle in patients with ventricular septal defects who undergo SVR regardless of the preoperative characteristics, and eventual biventricular repair can be performed in a subgroup of these patients. Future work is necessary to optimize timing of SVR and method of accessory pulmonary blood flow.

Prestenting right ventricular outflow tracts (RVOTs) before transcatheter pulmonary valve replacement (TPVR) is essential. Optimus-XXL (AndraTec GmbH, Koblenz, Germany) is a new extra-large, balloon-expandable cobalt-chrome stent with promising technologies.From June 2020 to November 2020, 15 patients with congenital heart disease, dysfunctional RVOTs and target TPVR diameter ≥ 23 mm received Optimus-XXL stents before proceeding to TPVR using the SAPIEN valve (Edwards Lifesciences, Irvine, CA). Standard safety and outcomes were prospectively assessed.Patients' median age and weight were 25.8 years (range: 10.5-63.1 years) and 58 kg (range: 43.8-101 kg), respectively. Underlying diagnosis was tetralogy of Fallot (66.7%), and RVOTs were patched (80%). Fifteen bare-metal stents were implanted using femoral (n = 14) and jugular approaches (n = 1). One conduit rupture was immediately controlled with a covered Optimus-XXL. Median stent length was 43 mm (range: 33-57 mm), and median target expansion diameter was 28 mm (range: 23-30 mm). Two procedural incidents occurred during stent delivery and were percutaneously treated. Stent stability was documented during TPVRs immediately performed in 14 patients. Median stent shortening was 13.7%, and median percentage of intended stent expansion was 95.9%. There was no stent fracture on the short-term follow-up (median: 4.5 months).We report the first implantations of Optimus-XXL stents in dysfunctional RVOTs with excellent preliminary results. Optimus-XXL should be considered as a valuable adjunct in the armamentarium for routine and complex TPVR procedures.

Patients with pulmonary atresia and intact ventricular septum (PA-IVS) require intervention early in life, and most survive to a definitive procedure of either Fontan circulation or right ventricle to pulmonary artery (RV-PA) repair. It remains unknown how surgical strategy impacts hemodynamics and comorbidities in adults. Retrospective analysis of adults (age ≥18 years) with PA-IVS undergoing hemodynamic catheterization at Mayo Clinic, MN between January 2000 through January 2020 was performed. Fourteen patients in the RV-PA group (71% biventricular, 29% 1.5 ventricle repair) and 19 post-Fontan patients [9 lateral tunnel (48%), 6 atriopulmonary (32%), and 4 extracardiac (21%)] were identified. Median age was 29 (21, 34) years. There were no differences in demographics and laboratory data (including MELD-XI) between groups. All patients assessed for liver disease had evidence of hepatic congestion or cirrhosis (14 in the Fontan group and 4 in the RV-PA group). Invasive hemodynamics were comparable between groups with the Fontan and RV-PA groups having similar systemic venous pressure (15.7±4.4 vs. 14.3±6.2, p=0.44) and cardiac output (2.2±0.6 vs. 2.0±0.4 L/min/m2, p=0.23). There was no difference in transplant-free survival (p=0.92; 5-year transplant-free survival RV-PA 84%, Fontan 80%). Hemodynamic derangements, namely elevated systemic venous pressure and low cardiac output, are prevalent in patients with PA-IVS undergoing cardiac catheterization regardless of surgical strategy.

Aims We aim to assess changes in routine echocardiographic and longitudinal strain parameters in patients recovering from Coronavirus disease 2019 during hospitalization and at 3-month follow-up. Methods and results Routine comprehensive echocardiography and STE of both ventricles were performed during hospitalization for acute coronavirus disease 2019 (COVID-19) infection as part of a prospective pre-designed protocol and compared with echocardiography performed ∼3 months after recovery in 80 patients, using a similar protocol. Significantly improved right ventricle (RV) fractional area change, longer pulmonary acceleration time, lower right atrial pressure, and smaller RV end-diastolic and end-systolic area were observed at the recovery assessment (P < 0.05 for all). RV global longitudinal strain improved at the follow-up evaluation (23.2 ± 5 vs. 21.7 ± 4, P = 0.03), mostly due to improvement in septal segments. Only eight (10%) patients recovering from COVID-19 infection had abnormal ejection fraction (EF) at follow-up. However, LV related routine (E, E/e′, stroke volume, LV size), or STE parameters did not change significantly from the assessment during hospitalization. A significant proportion [36 (45%)] of patients had some deterioration of longitudinal strain at follow-up, and 20 patients (25%) still had abnormal LV STE ∼3 months after COVID-19 acute infection. Conclusion In patients previously discharged from hospitalization due to COVID-19 infection, RV routine echocardiographic and RV STE parameters improve significantly concurrently with improved RV haemodynamics. In contrast, a quarter of patients still have LV systolic dysfunction based on STE cut-offs. Moreover, LV STE does not improve significantly, implying subclinical LV dysfunction may be part and parcel of recovering from COVID-19 infection., Graphical Abstract Graphical Abstract

Background Cardiovascular disease is an important driver of the increased mortality associated with chronic kidney disease (CKD). Higher left ventricular mass (LVM) predicts increased risk of adverse cardiovascular outcomes and total mortality, but previous reviews have shown no clear association between intervention-induced LVM change and all-cause or cardiovascular mortality in CKD. Methods The primary objective of this meta-analysis was to investigate whether treatment-induced reductions in LVM over periods ≥12 months were associated with all-cause mortality in patients with CKD. Cardiovascular mortality was investigated as a secondary outcome. Measures of association in the form of relative risks (RRs) with associated variability and precision (95% confidence intervals [CIs]) were extracted directly from each study, when reported, or were calculated based on the published data, if possible, and pooled RR estimates were determined. Results The meta-analysis included 42 trials with duration ≥12 months: 6 of erythropoietin stimulating agents treating to higher vs. lower hemoglobin targets, 10 of renin-angiotensin-aldosterone system inhibitors vs. placebo or another blood pressure lowering agent, 14 of modified hemodialysis regimens, and 12 of other types of interventions. All-cause mortality was reported in 121/2584 (4.86%) subjects in intervention groups and 168/2606 (6.45%) subjects in control groups. The pooled RR estimate of the 27 trials ≥12 months with ≥1 event in ≥1 group was 0.72 (95% CI 0.57 to 0.90, p = 0.005), with little heterogeneity across studies. Directionalities of the associations in intervention subgroups were the same. Sensitivity analyses of ≥6 months (34 trials), ≥9 months (29 trials), and >12 months (10 trials), and including studies with no events in either group, demonstrated similar risk reductions to the primary analysis. The point estimate for cardiovascular mortality was similar to all-cause mortality, but not statistically significant: RR 0.67, 95% CI 0.39 to 1.16. Conclusions These results suggest that LVM regression may be a useful surrogate marker for benefits of interventions intended to reduce mortality risk in patients with CKD.

The correlation between the Z-score of the left ventricular (LV) diameter and the LV volume-overload due to pulmonary over-circulation in children with ventricular septal defect (VSD) or patent ductus arteriosus (PDA) remains unclear.Methods and Results:The present, retrospective study enrolled 70 children (aged 0.3-16.8 years; 33 males, 37 females) with a diagnosis of isolated VSD and/or PDA who underwent cardiac catheterization (CC) between 2015 and 2019. Patients with chromosomal/genetic anomalies, growth disorder, right-ventricular enlargement or other conditions causing LV enlargement were excluded. Echocardiographic parameters were retrospectively evaluated from the medical records, converted to a Z-score, then compared with CC data. The pulmonary-systemic flow ratio on CC (cQp/Qs) correlated significantly with the Z-score of both the LV end-diastolic diameter (Zd) (r=0.698, P0.0001) and LV end-systolic diameter (r=0.593, P0.0001). Regression analysis and curve-fitting were used to predict the cQp/Qs based on the Zd, and a significant regression equation was found on cubic regression (RThe Z-score of the LV diameter can be a useful, non-invasive marker for evaluating LV volume overload and determining the surgical indications in children with VSD or PDA because of its strong correlation with the cQp/Qs.

Purpose-Prognosis of Duchenne muscular dystrophy (DMD) is related to cardiac dysfunction. Two dimensional-speckle tracking echocardiography (2D-STE) has recently emerged as a non-invasive functional biomarker for early detection of DMD-related cardiomyopathy. This study aimed to determine, in DMD children, the existence of a left ventricle (LV) dyssynchrony using 2D-STE analysis.Methods-This prospective controlled study enrolled 25 boys with DMD (mean age 11.0±3.5 years) with normal LV ejection fraction and 50 age-matched controls. Three measures were performed to assess LV mechanical dyssynchrony: the opposing-wall delays (longitudinal and radial analyses), the modified Yu index, and the time-to-peak delays of each segment. Feasibility and reproducibility of 2D-STE dyssynchrony were evaluated. Results-All three mechanical dyssynchrony criteria were significantly higher in the DMD group than in healthy subjects: (1) opposing-wall delays in basal inferoseptal to basal anterolateral segments (61.4±45.3 msec vs. 18.3±50.4 msec, Prespectively) and in mid inferoseptal to mid anterolateral segments (58.6±35.3 msec vs. 42.4±36.4 msec, Prespectively), (2) modified Yu index (33.3±10.1 msec vs. 28.5±8.1 msec, P, respectively), and (3) most of time-to-peak values, especially in basal and mid anterolateral segments. Feasibility was excellent and reliability was moderate to excellent, with ICC values ranging from 0.49 to 0.97.Conclusion-Detection of LV mechanical dyssynchrony using 2D-STE analysis is an easily and reproducible method in pediatrics. The existence of an early LV mechanical dyssynchrony visualized using 2D-STE analysis in children with DMD before the onset of cardiomyopathy represents a perspective for future pediatric drug trials in the DMD-related cardiomyopathy prevention. Clinical Trial Registration-Clinicaltrials.gov NCT02418338. Post-hoc study, registered on April 16, 2015.

Supplemental Digital Content is available in the text., Background: Many heart diseases can result in reduced pumping capacity of the heart muscle. A mismatch between ATP demand and ATP production of cardiomyocytes is one of the possible causes. Assessment of the relation between myocardial ATP production (MVATP) and cardiac workload is important for better understanding disease development and choice of nutritional or pharmacologic treatment strategies. Because there is no method for measuring MVATP in vivo, the use of physiology-based metabolic models in conjunction with protein abundance data is an attractive approach. METHOD: We developed a comprehensive kinetic model of cardiac energy metabolism (CARDIOKIN1) that recapitulates numerous experimental findings on cardiac metabolism obtained with isolated cardiomyocytes, perfused animal hearts, and in vivo studies with humans. We used the model to assess the energy status of the left ventricle of healthy participants and patients with aortic stenosis and mitral valve insufficiency. Maximal enzyme activities were individually scaled by means of protein abundances in left ventricle tissue samples. The energy status of the left ventricle was quantified by the ATP consumption at rest (MVATP[rest]), at maximal workload (MVATP[max]), and by the myocardial ATP production reserve, representing the span between MVATP(rest) and MVATP(max). Results: Compared with controls, in both groups of patients, MVATP(rest) was increased and MVATP(max) was decreased, resulting in a decreased myocardial ATP production reserve, although all patients had preserved ejection fraction. The variance of the energetic status was high, ranging from decreased to normal values. In both patient groups, the energetic status was tightly associated with mechanic energy demand. A decrease of MVATP(max) was associated with a decrease of the cardiac output, indicating that cardiac functionality and energetic performance of the ventricle are closely coupled. Conclusions: Our analysis suggests that the ATP-producing capacity of the left ventricle of patients with valvular dysfunction is generally diminished and correlates positively with mechanical energy demand and cardiac output. However, large differences exist in the energetic state of the myocardium even in patients with similar clinical or image-based markers of hypertrophy and pump function. Registration: URL: https://www.clinicaltrials.gov; Unique identifiers: NCT03172338 and NCT04068740.

Introduction Electromagnetic interference (EMI) from left ventricular assist devices (LVADs) can cause implantable cardioverter-defibrillator (ICD) oversensing. We sought to assess the frequency of inappropriate shocks/oversensing due to LVAD-related EMI and prospectively compare integrated (IB) versus dedicated bipolar (DB) sensing in patients with LVADs. Methods Single-center study in LVAD patients with Medtronic or Abbott ICDs between September 2017 and March 2020. We excluded patients that were pacemaker dependent. Measurements were obtained of IB and DB sensing and noise to calculate a signal-to-noise ratio (SNR). Device checks were reviewed to assess appropriate and inappropriate sensing events. Results Forty patients (age 52 ± 14 years, 75% men, 38% ischemic cardiomyopathy) were included with the median time between LVAD implantation and enrollment of 6.7 months [2.3, 11.4 months]. LVAD subtypes included: HeartWare (n=22, 55%), Heartmate II (n=10, 25%), and Heartmate III (n=8, 20%). Over a follow-up duration of 21.6 ± 12.9 months after LVAD implantation, 5% of patients (n=2) had oversensing of EMI from the LVAD (both with HeartWare LVADs and Abbott ICDs) at 4 days and 10.8 months after LVAD implantation. Both patients underwent adjustment of ventricular sensing with resolution of oversensing and no further events over 5 and 15 months of further follow-up. The SNR was similar between IB and DB sensing (50 [29-67] and 57 [41-69], p=0.89). Conclusion ICD oversensing of EMI from LVADs is infrequent and can be managed with reprogramming the sensitivity. There was no significant difference in the R-wave SNR with IB versus DB ICD leads. This article is protected by copyright. All rights reserved.

Aims Sleep apnoea and congestive heart failure (CHF) commonly co‐exist, but their interaction is unclear. Metabolomics may clarify their interaction and relationships to outcome. Methods and results We assayed 372 circulating metabolites and lipids in 1919 and 1524 participants of the Framingham Heart Study (FHS) (mean age 54 ± 10 years, 53% women) and Women's Health Initiative (WHI) (mean age 67 ± 7 years), respectively. We used linear and Cox regression to relate plasma concentrations of metabolites and lipids to echocardiographic parameters; CHF and its subtypes heart failure with reduced ejection fraction (HFrEF) and heart failure with preserved ejection fraction (HFpEF); and sleep indices. Adenine dinucleotide phosphate (ADP) associated with left ventricular (LV) fractional shortening; phosphocreatine with LV wall thickness; lysosomal storage molecule sphingomyelin 18:2 with LV mass; and nicotine metabolite cotinine with time spent with an oxygen saturation less than 90% (β = 2.3 min, P = 2.3 × 10−5). Pro‐hypertrophic metabolite hydroxyglutarate partly mediated the association between LV wall thickness and HFpEF. Central sleep apnoea was significantly associated with HFpEF (P = 0.03) but not HFrEF (P = 0.5). There were three significant metabolite canonical variates, one of which conferred protection from cardiovascular death [hazard ratio = 0.3 (0.11, 0.81), P = 0.02]. Conclusions Energetic metabolites were associated with cardiac function; energy‐ and lipid‐storage metabolites with LV wall thickness and mass; plasma levels of nicotine metabolite cotinine were associated with increased time spent with a sleep oxygen saturation less than 90%, a clinically significant marker of outcome, indicating a significant hazard for smokers who have sleep apnoea.

Objectives To assess the impact of pregnancy on cardiac function and fibrosis by cardiovascular magnetic resonance (CMR) in patients with repaired Tetralogy of Fallot (rToF). Background CMR T1 mapping can assess diffuse myocardial fibrosis which is associated to adverse clinical outcomes. Right ventricular (RV) accelerated remodeling is reported in rToF women with experienced pregnancy. Methods We included rToF women from the national registry of congenital heart disease to perform CMR, assessing functional data, T1 mapping/ extracellular volume fraction (ECV). The results including clinical data were compared between women with experienced pregnancy vs non-experienced pregnancy and healthy individuals. Results Fifty rToF women performed CMR, median age 36 (range 21–67) years. Fifteen were nulliparous. T1 mapping was compared to 30 controls, (14 women) median age 42 (24–64) years. In the left ventricle (LV), T1 times and ECV in all rToF women vs female controls were 1248 ± 61 ms/ 25.8 ± 2.9% vs 1255 ± 40 ms/ 26.8 ± 3.1%, p = 0.7 and p = 0.3, respectively. In rToF, RV T1 times was 1385 ± 124 ms and ECV 37.7 ± 5.4%. There was no association to parity or age in rToF LV T1/ ECV, p = 0.9 for both, or RV T1/ECV, p = 0.4 and p = 0.6, respectively. Indexed LV mass was higher in the rToF pregnancy group, 43 ± 10 vs 38 ± 6 g/m2, p = 0.03 while RV ejection fraction was lower, 49 ± 7% vs 53 ± 6%, p = 0.04. Conclusion Women with rTOF showed evidence of increased RV CMR markers suggestive of diffuse fibrosis while LV CMR markers were within normal values. Having experienced pregnancy might affect RV function, however without association to CMR biomarkers.

Aims To distinguish between constrictive pericarditis (CP) and restrictive cardiomyopathy (RCM) using cardiac magnetic resonance feature tracking (CMR‐FT) left ventricle (LV) diastolic time–strain curve patterns and myocardial strain. Methods and Results A total of 32 CP patients, 27 RCM patients, and 25 control subjects were examined by CMR‐FT and analysed for global strain, segmental strain, and LV time–strain curve patterns in the longitudinal, circumferential, and radial directions. Speckle tracking echocardiography (STE) strain imaging was performed in some cases. The peak global longitudinal strain (GLS) and global circumferential strain (GCS) of the RCM group were lower than those of the CP group. GLS [median (interquartile range) CP vs. RCM: −11.15 (−12.85, −9.35) vs. −6.5 (−8.75, −4.85), P

A 4-year-old boy with left intralobar pulmonary sequestration associated with left main coronary artery obstruction (LMCAO) and severe mitral regurgitation (MR) was admitted to our hospital. Since the patient presented with dyskinesia of the cardiac apex and increased left ventricular end-diastolic volume (LVEDV), left main coronary artery reconstruction and mitral annuloplasty were performed. The enlargement of the left ventricle was improved after sequential surgeries. There was a risk of deterioration of MR and regrowth of LVEDV due to shunt blood flow; therefore, left lower lobectomy and aberrant artery division were performed. This is a very rare case of a patient with pulmonary sequestration associated with LMCAO and severe MR.

Aims There is limited information on the association between left ventricular (LV) dimensions and cardiovascular (CV) outcomes in patients with heart failure (HF) with reduced LV ejection fraction (HFrEF) receiving recommended HF treatment. We investigated the association between LV dimensions and CV outcomes in HFrEF patients receiving recommended HF treatment. Methods and results We investigated the association between LV echocardiographic dimensions and CV outcomes using conventional Cox models in 1138 HFrEF patients in sinus rhythm randomized to warfarin or aspirin treatment in the Warfarin vs. Aspirin in Reduced Cardiac Ejection Fraction (WARCEF) trial. LV enlargement, whether by diameter [LV end‐diastolic diameter index (LVEDDI) and LV end‐systolic diameter index (LVESDI)] or volume [LV end‐diastolic volume index (LVEDVI) and LV end‐systolic volume index (LVESVI)], was independently associated with all‐cause death [LVEDDI: hazard ratio (HR) per cm/m2 1.53, LVESDI: HR per cm/m2 1.65, LVEDVI: HR per 10 mL/m2 1.07, and LVESVI: HR per 10 mL/m2 1.10; all P values

Background The Fontan operation is a palliative procedure and a substantial number of patients eventually experiences late Fontan circulation failure. Previous concepts of Fontan failure implicate increasing pulmonary vascular resistance (PVR) as a key contributor to late circulatory failure. However, data to support this assumption are sparse. We sought to characterize longitudinal hemodynamic and echocardiographic findings in adult failing Fontan patients. Methods We performed a retrospective cohort study in adult Fontan patients, identifying patients with Fontan failure. Hemodynamic, echocardiographic and clinical data were recorded. Results Of 173 adult patients (median follow-up after Fontan 20.2 years [IQR 15.7-24.3]), 48 (28%) showed signs of clinical Fontan failure. Thirty-seven patients (77.1%) exhibited ventricular dysfunction (systolic dysfunction defined by ejection fraction ≤45%, n = 22, or diastolic dysfunction defined by systemic ventricular end-diastolic pressure (SVEDP) ≥12 mmHg, n = 15). Elevated indexed PVR (≥2.5 WU*m2) was only observed in 9 (18.8%) patients. Ejection fraction declined from 60% [IQR 55-65] to 47% [IQR 35-55] during follow-up (p Conclusions Systolic and diastolic ventricular dysfunction are frequent features in late Fontan failure in adults, while increases in PVR were rarely observed. The intricate interplay between hemodynamic compromises in Fontan failure deserves further research to optimize treatment strategies and outcome.

Right ventricular (RV) pacing-induced cardiomyopathy (PICM) is typically defined as left ventricular systolic dysfunction resulting from electrical and mechanical dyssynchrony caused by RV pacing. RV PICM is common, occurring in 10-20% of individuals exposed to frequent RV pacing. Multiple risk factors for PICM have been identified, including male sex, wider native and paced QRS durations, and higher RV pacing percentage, but the ability to predict which individuals will develop PICM remains modest. Biventricular and conduction system pacing, which better preserve electrical and mechanical synchrony, typically prevent the development of PICM and reverse left ventricular systolic dysfunction after PICM has occurred.

Aims The management of heart failure (HF) in young children is challenging. The present study aimed to clarify the effect of left univentricular epicardial pacing on dilated cardiomyopathy with left bundle branch block (LBBB) in children. Methods and results A total of five cases (30.86 ± 16.39 months, three female) of children weighing 5.8–15 kg with dilated cardiomyopathy and LBBB were included in this study. LBBB in one child occurred after device closure of peri‐membranous ventricular septal defects, and the remaining four were idiopathically discovered early after birth. Before implantation, all children suffered from refractory HF and cardiac dilatation; the left ventricular ejection fraction was 33.48 ± 5.84% with Ross Heart Failure Classification III–IV. Electrical and mechanical dyssynchrony were observed in all children with QRS duration >140 ms and prolonged septal‐to‐left posterior wall motion delay. Left univentricular epicardial pacing was successfully implanted via left axillary minithoracotomy in the five children. Sensed atrioventricular delays (83 ± 15 ms) were optimized by velocity time integral of aortic blood flow before discharge. During the follow‐up period (10.8 ± 2.68 months), the dilated failing heart was reversed significantly in terms of decreased left ventricular dimension (55.62 ± 3.46 vs. 38.94 ± 3.69 mm, P = 0.005), while the left ventricular ejection fraction improved to 60.18 ± 8.78% (P = 0.006). Conclusions In young children with low body weight, if HF is caused by or related to LBBB, left ventricular epicardial pacing still has an excellent effect.

BACKGROUND: Assessment of subclinical myocardial dysfunction by using feature tracking has shown promise in prognosis evaluation of heart failure with preserved ejection fraction (HFpEF). Global early diastolic longitudinal strain rate (eGLSR) can identify earlier diastolic dysfunction; however, limited data are available on its prognostic value in HFpEF. PURPOSE: To evaluate the association between left ventricular (LV) eGLSR and primary composite outcomes (all-cause death or heart failure hospitalization) in patients with HFpEF. MATERIALS AND METHODS: In this retrospective study, consecutive patients with HFpEF (included from January 2010 to March 2013) underwent cardiovascular MRI. The correlation between eGLSR and variables was assessed by using linear regression. The association between eGLSR (obtained with use of feature tracking) and outcomes was analyzed by using Cox proportional regression. RESULTS: A total of 186 patients with HFpEF (mean age ± standard deviation, 59 years ± 12; 77 women) were included. The eGLSR was weakly correlated with LV end-diastole volume index (Pearson correlation coefficient [r] = –0.35; P < .001), heart rate (r = 0.35; P < .001), and LV ejection fraction (r = 0.30; P < .001) and moderately correlated with LV end-systole volume index (r = –0.41; P < .001). At a median follow-up of 9.2 years (interquartile range, 8.7–10.0 years), 72 patients experienced primary composite outcomes. Impaired eGLSR, defined as an eGLSR of less than 0.57 per second, was associated with a greater rate of heart failure hospitalization or all-cause death (hazard ratio, 2.0 [95% CI: 1.1, 3.7]; P = .02) after adjusting for multiple clinical and imaging-based variables. CONCLUSION: Left ventricular global early diastolic longitudinal strain rate obtained from cardiovascular MRI feature tracking was independently associated with adverse outcomes in patients with heart failure with preserved ejection fraction. © RSNA, 2021 Online supplemental material is available for this article. An earlier incorrect version appeared online. This article was corrected on October 22, 2021.

This study examines the feasibility of blood speckle tracking for vector flow imaging in healthy adults and describes the physiologic flow pattern and vortex formation in relation to the wall motion in the left ventricle. The study included 21 healthy volunteers and quantified and visualized flow patterns with high temporal resolution down to a depth of 10-12 cm without the use of contrast agents. Intraventricular flow seems to originate during the isovolumetric relaxation with a propagation of blood from base to apex. With the E-wave, rapid inflow and vortex formation occurred on both sides of the valve basally. During diastasis the flow gathers in a large vortex before the pattern from the E-wave repeats during the A-wave. In isovolumetric contraction, the flow again gathers in a large vortex that seems to facilitate the flow out in the aorta during systole. No signs of a persistent systolic vortex were visualized. The geometry of the left ventricle and the movement of the AV-plane is important in creating vortices that are favorable for the blood flow and facilitate outflow. The quantitative measurements are in concordance with these findings, but the clinical interpretation must be evaluated in future clinical studies.