Right Ventricle Research Articles

Pathogenesis and Surgical Treatment of Dextro-Transposition of the Great Arteries (D-TGA): Part II.

Dextro-transposition of the great arteries (D-TGA) is the second most common cyanotic heart disease, accounting for 5-7% of all congenital heart defects (CHDs). It is characterized by ventriculoarterial (VA) connection discordance, atrioventricular (AV) concordance, and a parallel relationship with D-TGA. As a result, the pulmonary and systemic circulations are separated [the morphological right ventricle (RV) is connected to the aorta and the morphological left ventricle (LV) is connected to the pulmonary artery]. This anomaly is included in the group of developmental disorders of embryonic heart conotruncal irregularities, and their pathogenesis is multifactorial. The anomaly's development is influenced by genetic, epigenetic, and environmental factors. It can occur either as an isolated anomaly, or in association with other cardiac defects. The typical concomitant cardiac anomalies that may occur in patients with D-TGA include ventriculoseptal defects, patent ductus arteriosus, left ventricular outflow tract obstruction (LVOTO), mitral and tricuspid valve abnormalities, and coronary artery variations. Correction of the defect during infancy is the preferred treatment for D-TGA. Balloon atrial septostomy (BAS) is necessary prior to the operation. The recommended surgical correction methods include arterial switch operation (ASO) and atrial switch operation (AtrSR), as well as the Rastelli and Nikaidoh procedures. The most common postoperative complications include coronary artery stenosis, neoaortic root dilation, neoaortic insufficiency and neopulmonic stenosis, right ventricular (RV) outflow tract obstruction (RVOTO), left ventricular (LV) dysfunction, arrhythmias, and heart failure. Early diagnosis and treatment of D-TGA is paramount to the prognosis of the patient. Improved surgical techniques have made it possible for patients with D-TGA to survive into adulthood.

Chronic systolic heart failure: association of remodeling of the right heart and renal dysfunction with cardiac arrhythmias

Introduction. Ischemic (ICMP) and dilated cardiomyopathy (DCMP) are the leading causes of chronic heart failure (CHF) with the most frequent indication for heart transplantation. The similarity of these diseases consists in the widespread and high mortality rate due to pathological remodeling of both the left and right chambers of the heart, which is accompanied by the progression of CHF, increasing the risk of sudden cardiac death.The purpose of the study. To study the relationship of systolic function of the left ventricle (LV) with remodeling of the right heart, renal dysfunction and their relationship with cardiac arrhythmia.Materials and methods. The study included 72 patients with CHF with reduced ejection fraction (CHFrEF), who were being treated at the N. V. Sklifosovsky Scientific Research Institute of EM in connection with worsening CHF. Group I consisted of 48 patients with DCMP; group II – 24 patients with ICMP.Results. LV ejection fraction (LV EF) in patients of both groups was <30 %, but it was significantly lower in group I (p=0.001). The EF of the right ventricle (RV) in group I was low and amounted to 24.6 [19.5; 28.5]%. In group I, the volume of the right atrium (PP) and the size of the pancreas correlated with LV EF (r= –0.489, p=0.015 and r= –0.410, p=0.005, respectively); each subsequent increase in the volume of right atrium (RA) by 1 ml increased the likelihood of atrial fibrillation (AF) by 1.018 times (OR: 1.018, 95 % CI: 1.0042–1.033, p=0.011). The estimated glomerular filtration rate (eGFR) <60 ml/min/1.73 m2 increased the chances of ventricular tachycardia (VT) by 9.5 times (OR: 9.5, 95 % CI: 2.32–38.87, p=0.002). Each subsequent decrease in eGFR by 1 ml/min/1.73 m2 increased the chances of developing VT by 1.03 times (OR: 0.967, 95 % CI: 0.937–0.988, p=0.036)].Conclusions. In patients with CHFrEF, regardless of the etiology, a decrease in LV EF leads to structural and functional changes in the right parts of the heart. In individuals with DCMP, an increase in RA volume by 1 ml increases the likelihood of AF by 1.018 times; a decrease in eGFR by 1 ml/min/1.73 m2 increases the chances of developing VT by 1.03 times.

Fetal Right Heart Strain in Systemic Right Ventricles and Impact on Post-surgical Outcomes.

Patients with hypoplastic left heart syndrome (HLHS) and its variants rely on the right ventricle (RV) to provide cardiac output. Diminished RV systolic function has been associated with poor clinical outcomes in this population. Echocardiographic strain has emerged as a useful method to quantify RV deformation. We aimed to describe fetal strain in the systemic RV and further investigate if there was any correlation with clinical outcomes. We conducted a retrospective, single center study evaluating strain in fetuses with systemic RV. We measured fetal RV global longitudinal strain (GLS) and segmental strain using Tomtec 2D speckle tracking software and compared these findings to controls. Fifty patients with systemic RV were included in the study group with controls matched one to one for each echocardiogram. Ten patients died after first-stage palliation. GLS was reproducible, with interobserver ICC 0.82. There was no statistically significant difference in GLS among different HLHS subtypes. Abnormal GLS did not correlate with worse clinical outcomes. GLS in systemic RVs in the 2nd and 3rd trimester did not vary significantly throughout gestation and did not correlate with clinical outcomes. Risk factors associated with poor outcome were mainly postnatal. Multi-centered studies are needed to determine if these findings hold true in a larger sample size.

Update of How Pediatric Cardiologists Noninvasively Evaluate Patients with Hypoplastic Left Heart Syndrome: 2013 vs. 2023.

Noninvasive functional assessment of systemic right ventricles (RV) in hypoplastic left heart syndrome (HLHS) is challenging. This study aimed to compare pediatric cardiologists' current noninvasive imaging practices to those reported in 2013. A web-based survey was distributed to pediatric cardiologists via various listservs which queried timing of echocardiograms during HLHS palliative stages and measurements of RV function. Demographics of 156 participants who completed the 2023 survey were similar to survey participants in 2013 (n = 222). Respondents were mostly male (62%), echocardiographers (48%), in university-based practice (67%) in North America (95%). Echocardiograms were predominantly obtained monthly during interstage I (41%), every 6months during interstage II (56%), and every year post-Fontan (68%), which is consistent from 2013. Routine cardiac magnetic resonance imaging (cMRI) significantly increased in interstage II (8.2%, 17%) and post-Fontan (24%, 56%) populations, respectively. Qualitative assessment (41%), ejection fraction (EF) by 3D (20%), fractional area change (16%), and RV strain/strain rate (13%) were preferred methods for systolic assessment, whereas a plurality of respondents (41%) did not believe RV diastolic measurements were valid. The largest gap between currently obtained and desired measurements existed for EF by 3D (46.5% points) and RV strain/strain rate (44.5% points). No differences existed between imagers compared to non-imagers. Variability in evaluating HLHS patients continues among pediatric cardiologists compared to 10years ago. Qualitative assessment remains the primary RV systolic functional evaluation. Providers do not rely on quantitative RV diastolic function assessments in HLHS patients. Use of cMRI is increasing for RV functional analysis.

Definition and management of right ventricular injury in adult patients receiving extracorporeal membrane oxygenation for respiratory support using the Delphi method: a PRORVnet study. Expert position statements.

Veno-venous extracorporeal membrane oxygenation (VV-ECMO) is an integral part of the management algorithm of patients with severe respiratory failure refractory to evidence-based conventional treatments. Right ventricular injury (RVI) pertaining to abnormalities in the dimensions and/or function of the right ventricle (RV) in the context of VV-ECMO significantly influences mortality. However, in the absence of a universally accepted RVI definition and evidence-based guidance for the management of RVI in this very high-risk patient cohort, variations in clinical practice continue to exist. Following a systematic search of the literature, an international Steering Committee consisting of eight healthcare professionals involved in the management of patients receiving ECMO identified domains and knowledge gaps pertaining to RVI definition and management where the evidence is limited or ambiguous. Using a Delphi process, an international panel of 52 Experts developed Expert position statements in those areas. The process also conferred RV-centric overarching open questions for future research. Consensus was defined as achieved when 70% or more of the Experts agreed or disagreed on a Likert-scale statement or when 80% or more of the Experts agreed on a particular option in multiple-choice questions. The Delphi process was conducted through four rounds and consensus was achieved on 31 (89%) of 35 statements from which 24 Expert position statements were derived. Expert position statements provided recommendations for RVI nomenclature in the setting of VV-ECMO, a multi-modal diagnostic approach to RVI, the timing and parameters of diagnostic echocardiography, and VV-ECMO settings during RVI assessment and management. Consensus was not reached on RV-protective driving pressure thresholds or the effect of prone positioning on patient-centric outcomes. The proposed definition of RVI in the context of VV-ECMO needs to be validated through a systematic aggregation of data across studies. Until further evidence emerges, the Expert position statements can guide informed decision-making in the management of these patients.

Impact of guideline directed medical therapy on myocardial function in adults with congenital heart disease

BackgroundGuideline-directed heart failure therapy with angiotensin receptor blocker/neprilysin inhibitor (ARNi) and sodium-glucose transporter inhibitors (SGLT2i) has been incrementally beneficial in improving outcomes in heart failure patients. ObjectiveEvaluate the feasibility and efficacy of guideline-directed medical therapy (GDMT) in adults congenital heart disease (ACHD) patients. MethodsIn a retrospective cohort study, ACHD patients with either New York Heart Association (NYHA) Class II symptoms or systemic ejection fraction (EF) <45%, optimized on a combination of beta-blocker (BB), ARNi, mineralocorticoid receptor antagonist (MRA) and SGLT2i were evaluated. ResultsForty-six patients with a mean age 42.6 ± 12.1 years prescribed GDMT were identified. Twenty-eight (61%) were male, 20 (43%) had a systemic right ventricle (RV) and 9 (20%) had single-ventricle physiology. Over the optimization period, 20 (43%) were sustained on ARNi and 42 (91%) on SGLT2i in addition to treatment with BB and MRA. Over a period of 45 weeks, echocardiography parameters for left ventricle (LV) ejection fraction (EF) (+7.5%, p = 0.006), systemic ventricle (SV) velocity time integral (VTI) (+1.9 cm, p = 0.012) and LV global longitudinal strain (GLS) (−2.5%, p = 0.005) improved when 3–4 medications were used versus 1–2 medications alone. The use of either ARNi or SGLT2i (+8.1%, p = 0.017) or in combination (+7.0%, p = 0.043) increased LVEF compared to the use of neither medication. ConclusionCombination GDMT is beneficial in improving myocardial characteristics in ACHD patients with systemic RV and LV.

Abstract Tu057: Renal denervation enhances right ventricular function, reduces myocardial damage, and restores myocardial sympathetic signaling in rats with heart failure induced by volume overload

Introduction: Heart failure (HF) is associated with chronically increased sympathetic nervous activity with depleted norepinephrine (NE) in the myocardium. Despite the known anti-hypertensive effect of renal denervation (RDN), its influence on the function of the right ventricle (RV) and the cardiac sympathetic nervous system in HF is not well comprehended. Hypothesis: RDN improves RV function by reducing central sympathetic drive and changes of a sympathetic nervous branch in the heart. Aims: To investigate the effect of RDN on RV function, HF markers, myocardial NE, and its metabolism in RV in HF induced by aorto-caval fistula (ACF). Methods: HF was induced by ACF in Ren-2 transgenic rats (TGR). One week later, the RDN was performed by phenol application. Two weeks after RDN, RV function was measured by pressure-volume analysis and RV tissue was collected. Echocardiography was done once a week, during the entire experiment. NE levels were measured using an ELISA kit, protein expression was assessed by western blotting, gene expression by TaqMan PCR gene expression assay, and ROS by oxidation of Amplex Red assay. Results: The ACF increased RV weight, RV dimensions (RVD1, RVD2, RVD3), end-systolic (ESP) and end-diastolic pressure (EDP), reduced RV systolic function (end-systolic elastance - Ees, fractional area change - FAC) and ventricular-arterial coupling (Ees/Ea ratio), increased gene expression of HF markers collagen I/III ratio, natriuretic peptide A (Nppa), myosine heavy chain 7/6 (Myh7/6) ratio, transglutaminase 2 (Tgm2), and decreased superoxide dismutase 2 (SOD2) protein expression. ACF increased plasmatic NE levels, depleted NE in RV, decreased tyrosine hydroxylase protein expression in RV, and increased gene, protein expression, and activity of monoamine oxidase A (MAO-A). RDN decreased RV hypertrophy and dilatation (RVD1, RVD2, RVD3), decreased RV ESP (-7 mmHg, p=0.004) and EDP (-2.2 mmHg, p=0.003), improved RV function (Ees +45%, p=0.03, FAC +20%, p=0.005, Ees/Ea ratio +46%, p=0.02), decreased HF markers collagen I/III ratio, Nppa, Tgm2, Myh7/6 ratio, and increased SOD2 protein expression. RDN decreased plasmatic NE levels, increased NE in RV (+32%, p=0.03), and decreased MAO-A in RV. Conclusions: RDN decreased RV ESP and EDP, improved RV systolic function, restored NE levels, and decreased RV gene expression of selected HF markers, probably by reduction of central sympathetic nerve drive and alterations of the cardiac sympathetic nervous system.

Abstract Tu080: Unique Physiology of Fibroblast and Immune Cells in the Right Ventricle

Introduction: Failure of the right ventricle (RV) is a main component of the morbidity and mortality of pulmonary hypertension (PH). There exists no specific treatment for RV dysfunction and failure and the only cure for many patients (especially in the case of pulmonary arterial hypertension) remains transplantation. This is partially due to the critical lack of understanding of RV biology; therapeutic strategies that are beneficial for LV failure have worse outcomes when applied to those with RV failure. RV dysfunction is characterized by exaggerated fibrosis and divergent contractile and hypertrophic responses to several drugs when compared to LV dysfunction. Thus, the RV may contain distinct cellular physiology compared to the LV that has high significance for the treatment of PH. Methods: Tissue digests from the RV and LV of Sprague Dawley rats were collected. We utilized digests to collect RNA for RNA-seq, and prepared cells for flow cytometry and fibroblast isolations. Fibroblasts were cultured for proliferation assays and cell signaling studies. Results: We found that the RV and LV have significant differences in expression of immune-related and fibrotic genes. The RV of control rats had an average of four-fold higher density of immune cells, namely macrophages/monocytes, compared to the LV (P&lt;0.05 via paired t-test). Fibroblasts from the RV of control rats, which had a higher proliferation rate and an alteration of metabolic genes than those from the LV (P&lt;0.05 via two-way ANOVA), also had a higher fold-change increase in periostin and CTGF expression upon treatment with TGFβ. Conclusions: The results demonstrate that in comparison to the LV, the RV is made up of a unique population of cells and has a unique capacity for fibrotic response that is reflected in its molecular makeup, which may contribute to its distinct response to PH. Future experiments will focus on determining the molecular mechanisms driving these differences. This knowledge is expected to yield new therapeutic approaches for RV dysfunction and failure occurring with PH, which currently have no specific treatment.

Abstract Tu065: The role of heavy metals in pulmonary arterial hypertension pathogenesis

Pulmonary arterial hypertension (PAH) is a disease where chronic high pulmonary arterial pressure causes inflammation, remodeling, and dysfunctions of lung endothelial/smooth muscle cells, and right ventricle (RV) cardiomyocytes. Pathogenesis is caused by a mixture of genetic, epigenetic, and environmental factors. Our clinical studies found PAH patients had elevated cadmium (Cd) and antimony (Sb) levels in the blood and/or urine, which correlated with increased severity of PAH parameters. Thus, we hypothesize that environmental factors, specifically chronic heavy metal exposure, could contribute to PAH pathogenesis either directly or indirectly. We tested if Cd could induce PAH or worsen PAH parameters in the mouse SU5416 and hypoxia (SuHx) PAH model. Forty male C57/6J mice were initially split in 2 groups: control and 5ppm Cd in drinking water (n=20/each) for 8 weeks (wks). Diastolic and systolic functions were evaluated with echocardiography (echo) at baseline, and 8ks. Then, half of these mice were subject to PAH induction, netting 4 subgroups (n=10/group): CTRL, Cd, PAH, and Cd+PAH. Echo was performed again 4 wks post-PAH induction, followed by RV systolic pressure (RVSP) measurement (n=3/group). The remaining 7 mice/group were euthanized for heart/lung histopathology focusing on fibrosis and hypertrophy, and protein western blot analyses. GraphPad Prism 8 software was used for statistical analysis with normality checked. When data did not follow normality, the non-parametric equivalence was used. Two-way and one-way ANOVA with Tukey were performed for echo/RVSP and histological/biochemical analyses respectively, with a p-value&lt;0.05 being significant. Results showed 12-wks Cd exposure alone caused: (1) significantly increased RV systolic function indicating systolic stimulation, and (2) significantly increased collagen contents in both ventricles and LV cardiomyocyte area when compared to CTRL. Twelve-wks Cd+PAH group demonstrated (1) significantly decreased RV cardiac output and RVSP measurements indicating RV failure; (2) no further RV changes in collagen contents or hypertrophy; (3) LV western blot analysis revealed significant upregulation of antioxidant proteins, including catalase and superoxide dismutase 2, when compared to PAH group. Further mechanistic experiments are being conducted, including whether Sb has similar results as Cd.

3D regional evaluation of right ventricular myocardial work from cineCT.

Regional myocardial work (MW) is not measured in the right ventricle (RV) due to a lack of high spatial resolution regional strain (RS) estimates throughout the ventricle. We present a cineCT-based approach to evaluate regional RV performance and demonstrate its ability to phenotype three complex populations: end-stage LV failure (HF), chronic thromboembolic pulmonary hypertension (CTEPH), and repaired tetralogy of Fallot (rTOF). 49 patients (19 HF, 11 CTEPH, 19 rTOF) underwent cineCT and right heart catheterization (RHC). RS was estimated from full-cycle ECG-gated cineCT and combined with RHC pressure waveforms to create regional pressure-strain loops; endocardial MW was measured as the loop area. Detailed, 3D mapping of RS and MW enabled spatial visualization of strain and work strength, and phenotyping of patients. HF patients demonstrated more overall impaired strain and work compared to the CTEPH and rTOF cohorts. For example, the HF patients had more akinetic areas (median: 9%) than CTEPH (median: <1%, p=0.02) and rTOF (median: 1%, p<0.01) and performed more low work (median: 69%) than the rTOF cohort (median: 38%, p<0.01). The CTEPH cohort had more impairment in the septal wall; <1% of the free wall and 16% of the septal wall performed negative work. The rTOF cohort demonstrated a wide distribution of strain and work, ranging from hypokinetic to hyperkinetic strain and low to medium-high work. Impaired strain (-0.15≤RS) and negative work were strongly-to-very strongly correlated with RVEF (R=-0.89, p<0.01; R=-0.70, p<0.01 respectively), while impaired work (MW≤5 mmHg) was moderately correlated with RVEF (R=-0.53, p<0.01). Regional RV MW maps can be derived from clinical CT and RHC studies and can provide patient-specific phenotyping of RV function in complex heart disease patients. Evaluating regional variations in right ventricular (RV) performance can be challenging, particularly in patients with significant impairments due to the need for 3D spatial coverage with high spatial resolution. ECG-gated cineCT can fully visualize the RV and be used to quantify regional strain with high spatial resolution. However, strain is influenced by loading conditions. Myocardial work (MW) - measured clinically derived as the ventricular pressure-strain loop area - is considered a more comprehensive metric due to its independence of preload and afterload. In this study, we sought to develop regional RV myocardial work (MW) assessments in 3D with high spatial resolution by combining cineCT-derived regional strain with RV pressure waveforms from right heart catheterization (RHC). We developed our method using data from three clinical cohorts who routinely undergo cineCT and RHC: patients in heart failure, patients with chronic thromboembolic pulmonary hypertension, and adults with repaired tetralogy of Fallot.We demonstrate that regional strain and work provide different perspectives on RV performance. While strain can be used to evaluate apparent function, similar profiles of RV strain can lead to different MW estimates. Specifically, MW integrates apparent strain with measures of afterload, and timing information helps to account for dyssynchrony. As a result, CT-based assessment of RV MW appears to be a useful new metric for the care of patients with dysfunction.

The Right Ventricle in Pulmonary Arterial Hypertension: An Organ at the “Heart of the Problem”

: Pulmonary Arterial Hypertension (PAH) is a progressive disease with no cure. A major determinant of outcome is the function of the right ventricle (RV). Unfortunately, progressive RV dysfunction and failure can occur despite PAH-specific therapies. While initial adaptive hypertrophic changes occur to maintain cardiac output and preserve contractile function and reserve, maladaptive changes occur in the RV muscle that contribute to RV systolic and diastolic dysfunction and failure. These include impaired angiogenesis / decreased capillary density with ischemia, fibrosis, cardiomyocyte apoptosis and impaired autophagy, inflammation, enhanced oxidative stress, altered metabolism, etc. Of note, there are no therapies currently approved that offset these changes and treatment of RV dysfunction is largely supportive only. Further patients often do not qualify for bilateral lung transplantation because of co-morbidities such as renal impairment. Thus, a dire unmet need exists regarding the management of RV dysfunction and failure in patients with PAH. In this State-of-the-Art review, we comprehensively outline the unique features of the RV compared to the left ventricle (LV) under normal circumstances and highlight the unique challenges faced by the RV when confronted with increased afterload as occurs in PAH. We provide detailed insights into the basis for the adaptive hypertrophic phase as well as detailed commentary into the pathophysiology of the maladapted dysfunctional state as well as the pathobiological aberrations occurring in the RV muscle that underlines the progressive dysfunction and failure that commonly ensues. We also review comprehensively the evaluation of RV function using all currently employed imaging, hemodynamic and other modalities and provide a balanced outline of strengths and limitations of such approaches with the treating clinician in mind. We outline the current approaches, albeit limited to chronic multi-modal management of RV dysfunction and failure. We further outline new possible approaches to treatment that include novel pharmacologic approaches, possible use of cellular/stem cell therapies and mechanical approaches. This review is directed to the treating clinician to provide comprehensive insights regarding the RV in patients with PAH.

Sparing of the Heart Facilitates Recovery From Cardiopulmonary Side Effects After Thoracic Irradiation

IntroductionWhen irradiating thoracic tumors, dose to the heart or lung has been associated with survival. We previously showed in a rat model that in addition to known side-effects such as pericarditis, pneumonitis and fibrosis, heart and/or lung irradiation also impaired diastolic function and increased pulmonary artery pressure. Simultaneous irradiation of both organs strongly intensified these effects. However, the long-term consequences of this interactions are not yet known. Therefore, here we investigate the long-term effects of combined heart and lung irradiation. Material and MethodsDifferent regions of the rat thorax containing the heart and/or 50% of the lungs were irradiated with protons. Respiratory rate (RR) was measured bi-weekly as an overall parameter for cardiopulmonary function. Echocardiography of the heart was performed at 8, 26 and 42 weeks after irradiation. Tissue remodeling and vascular changes were assessed using Masson Trichrome and Verhoeff-stained lung and LV tissue collected at 8 and 42 weeks after irradiation. ResultsDuring the entire experimental period RR was consistently increased after combined heart/lung irradiation. This coincided with persistent effects on lung vasculature and reduced right ventricle contraction. In contrast, recovery of RR, pulmonary remodeling and right ventricle contraction was observed after sparing of the heart. These corresponding temporal patterns suggest that the reduction of right ventricle (RV) function is related to vascular remodeling in the lung. ConclusionCombined irradiation of lung and heart leads to an intensified, persistent reduction of cardiopulmonary function. Recovery of the pulmonary vasculature and right ventricle function requires heart-sparing.

The cone repair allows right ventricle rehabilitation with excellent tricuspid valve function following the Starnes procedure

BackgroundWe present a case series of right ventricle (RV) rehabilitation after the Starnes procedure in patients with Ebstein anomaly (EA), applying the Cone repair of the tricuspid valve (TV) to achieve 2-ventricle or 1.5-ventricle physiology. MethodsThis is a retrospective database analysis from 2 institutions in North America. We included all consecutive cases of Cone repair after the Starnes procedure. The data are expressed as median and interquartile range (IQR). ResultsEleven patients underwent RV rehabilitation between 2019 and 2023 after initial Starnes palliation at a median age of 27 months (IQR, 20.5 months). All patients were critically ill before their Starnes procedure, and 4 were on extracorporeal membrane oxygenation. Before the Cone repair, the median preoperative regurgitant velocity at the Starnes patch was 1.65 m/s (IQR, 1.3 m/s). During the Cone procedure, 9 patients required a concomitant pulmonary valve repair, of whom 3 needed a transannular monocusp patch. Four patients were successfully rerouted to a 2-ventricle repair, and 7 patients with a previous Glenn achieved 1.5-ventricle circulation. There were no cases of heart block and no deaths. Seven patients had trivial, 3 patients had mild, and 1 patient had moderate tricuspid regurgitation (TR) at a median follow-up of 11 months (IQR, 21.5 months). There was no significant TV stenosis; all patients had good functional status at the last follow-up despite severe RV dysfunction in 1 patient. ConclusionsAfter the Starnes procedure, the Cone repair allowed RV rehabilitation, resulting in trivial or mild TR at a midterm follow-up. The Starnes procedure is a reproducible technique that no longer commits patients to lifetime single-ventricle physiology.

Correlation between worsening pneumonitis and right ventricular systolic function in critically ill patients with COVID-19

BackgroundThe pneumonitis associated with coronavirus disease 2019 (COVID-19) infection impacts the right ventricle (RV). However, the association between the disease severity and right ventricular systolic function needs elucidation.MethodWe conducted a retrospective study of 108 patients admitted to critical care with COVID-19 pneumonitis to examine the association between tricuspid annular plane systolic excursion (TAPSE) by transthoracic echocardiography as a surrogate for RV systolic function with PaO2/FiO2 ratio as a marker of disease severity and other respiratory parameters.ResultsThe median age was 59 years [51, 66], 33 (31%) were female, and 63 (58%) were mechanically ventilated. Echocardiography was performed at a median of 3 days [2, 12] following admission to critical care. The PaO2/FiO2 and TAPSE medians were 20.5 [14.4, 32.0] and 21 mm [18, 24]. There was a statistically significant, albeit weak, association between the increase in TAPSE and the worsening of the PaO2/FiO2 ratio (r2 = 0.041, p = 0.04). This association was more pronounced in the mechanically ventilated (r2 = 0.09, p = 0.02). TAPSE did not correlate significantly with FiO2, PaO2, PaCO2, pH, respiratory rate, or mechanical ventilation. Patients with a TAPSE ≥ 17 mm had a considerably worse PaO2/FiO2 ratio than a TAPSE < 17 mm (18.6 vs. 32.1, p = 0.005). The PaO2/FiO2 ratio predicted TAPSE (OR = 0.94, p = 0.004) with good area under the curve (0.72, p = 0.006). Moreover, a PaO2/FiO2 ratio < 26.7 (moderate pneumonitis) predicted TAPSE > 17 mm with reasonable sensitivity (67%) and specificity (68%).ConclusionIn patients admitted to critical care with COVID-19 pneumonitis, TAPSE increased as the disease severity worsened early in the course of the disease, especially in the mechanically ventilated. A TAPSE within the normal range is not necessarily reassuring in early COVID-19 pneumonitis.

Serial evaluation of biventricular function in COVID-19 recovered patients using speckle tracking echocardiography

ObjectivesThe persistence and outcomes following myocardial injury subsequent to coronavirus disease-2019 (COVID-19) infection has not been properly elucidated. We assessed sub-clinical bi-ventricular dysfunction using speckle tracking echocardiography (STE) in post COVID-19 patients. MethodsA total of 189 subjects following recovery from COVID-19 infection were enrolled. Detailed echocardiography including STE along with clinical, hematological, biochemical and inflammatory parameters were assessed for all. Patients were divided into four groups (asymptomatic, mild, moderate and severe) based on severity of COVID-19 infection. Additionally, 90 healthy individuals were enrolled as controls. All these patients were followed up for one year following enrolment. ResultsSubclinical LV and right ventricle (RV) dysfunction were seen in 58 (30.7 %) and 55 (29.1 %) patients respectively at baseline. Significant difference was observed in mean LVGLS values among the three groups (mild: −21.5 ± 2.8 %; moderate: −17 ± 7.1 %; severe: −12.1 ± 4 %; P < 0.0001). Over a year of follow-up, significant improvement in LVGLS from baseline (−19.1 ± 5.8 %) was observed (−19.9 ± 4.6 %; P < 0.0001). Similarly, RVFWS (−23.5 ± 6.3 % vs −23.8 ± 5.8 %; P = 0.03) had significant improvement from baseline to one year of follow-up. Reduced LVGLS was reported in 12 (6.3 %) subjects while impaired RVFWS was documented in 10 (5.3 %) subjects at one year of follow-up. ConclusionsSubclinical LV and RV dysfunction were seen in nearly a third of recovered COVID-19 patients. Over a year of follow-up, significant improvement in subclinical LV and RV dysfunction was noted.

Common atrial reservoir strain during the interstage period is a predictor of poor outcomes prior to Fontan completion in hypoplastic left heart syndrome.

The atrium augments ventricular function, but the significance of atrial function in hypoplastic left heart syndrome (HLHS) has not been well evaluated. We investigated the association of atrial reservoir strain (common atrial strain [CAS]) to death or need for transplantation in patients with HLHS. In this retrospective single-center study, echocardiograms from three timepoints (pre-stage 1 palliation [S1P], 4-8weeks post-S1P, and pre-Glenn) were analyzed in infants with classic HLHS. Patients were separated based on transplant-free survival to Fontan (survivors) versus death or heart transplant prior to Fontan (composite outcome). Echocardiographic parameters evaluated included CAS, right ventricle (RV) global longitudinal strain (RVGLS), RV fractional area change (FAC), and tricuspid annular plane systolic excursion (TAPSE). An equal variance t-test, regression, and receiver operating characteristic (ROC) analyses were performed. A total of 45 HLHS patients (25 survivors, 20 patients meeting endpoint) were included in this study. There were no significant differences in any of the functional parameters during the pre-stage 1 or post-stage 1 timepoints. Pre-Glenn CAS and RVGLS were significantly worse in those meeting composite endpoint compared to survivors. CAS was significantly correlated to RVGLS during the pre-S1P and pre-Glenn timepoints. A pre-Glenn CAS<19.5 had an area under the curve of .78 and a 75% sensitivity and 83% specificity for death or need for transplantation. Pre-Glenn CAS is significantly lower in patients with mortality or need for the transplantation prior to Fontan completion and may carry prognostic significance in patients with HLHS.

Unraveling the Impact of miR-146a in Pulmonary Arterial Hypertension Pathophysiology and Right Ventricular Function.

Pulmonary arterial hypertension (PAH) is a chronic disorder characterized by excessive pulmonary vascular remodeling, leading to elevated pulmonary vascular resistance and right ventricle (RV) overload and failure. MicroRNA-146a (miR-146a) promotes vascular smooth muscle cell proliferation and vascular neointimal hyperplasia, both hallmarks of PAH. This study aimed to investigate the effects of miR-146a through pharmacological or genetic inhibition on experimental PAH and RV pressure overload animal models. Additionally, we examined the overexpression of miR-146a on human pulmonary artery smooth muscle cells (hPASMCs). Here, we showed that miR-146a genic expression was increased in the lungs of patients with PAH and the plasma of monocrotaline (MCT) rats. Interestingly, genetic ablation of miR-146a improved RV hypertrophy and systolic pressures in Sugen 5415/hypoxia (SuHx) and pulmonary arterial banding (PAB) mice. Pharmacological inhibition of miR-146a improved RV remodeling in PAB-wild type mice and MCT rats, and enhanced exercise capacity in MCT rats. However, overexpression of miR-146a did not affect proliferation, migration, and apoptosis in control-hPASMCs. Our findings show that miR-146a may play a significant role in RV function and remodeling, representing a promising therapeutic target for RV hypertrophy and, consequently, PAH.

Comparison Between Histidine-Tryptophan-Ketoglutarate Cardioplegia and Cold Blood Cardioplegia for Myocardial Protection in Tetralogy of Fallot Patients Undergoing Surgical Repair: A Randomized Clinical Trial

ObjectivesThis study was conducted to compare the efficacy of histidine-tryptophan-ketoglutarate (HTK) cardioplegia and cold blood cardioplegia (CBC), especially for postoperative right ventricle (RV) function after Tetralogy of Fallot (TOF) repair. DesignRandomized, double-blinded, parallel group, controlled, clinical trial. SettingAin Shams University Hospitals.) ParticipantsA total of 100 children (1-5 years-old) scheduled for TOF repair. InterventionsPatients were allocated (double blinded one: one allocation ratio), to either HTK Group that received HTK cardioplegia (30 ml/kg by antegrade route) or CBC Group that received cold blood cardioplegia with blood to Ringer solution (4:1) in volume of 20 ml/kg. Measurements and Main ResultsThe HTK group showed a statistically significant reduction of the vasoactive inotropic score on admission to pediatric intensive care unit (13.0±4.1) in comparison to CBC (15.5±5.4) with P-value of 0.011 but after 24 hours Vasoactive-Inotropic Score was comparable. Lactate level during the first 24 hours in HTK group (6.2±0.7) mmol/l and (6.9±0.4) mmol/l in CBC group with P-value < 0.0001. Serial troponin measurements were lower in the HTK group (1.49 ± 0.45) in comparison to the CBC group (1.69 ± 0.18) with P-value of 0.005 at the first 72 hours postoperatively. Postoperative echocardiographic assessment of RV function by mean of tricuspid annular plane systolic excursion and myocardial performance index were better in HTK than CBC (P-value<0.05). ConclusionsHTK cardioplegia may offer better cardiac protection to pediatric patients undergoing TOF repair than our institutional standard CBC with better recovery for the hypertrophic RV.

Fatty infiltration and ventricular premature beats originating from right ventricular outflow tract: association or causality?

Anatomical evidence reveals heterogeneous fat distribution in both atrial and ventricular myocardium that are considered normal, but at the same time arrhythmogenic, and numerous cardiac pathophysiological conditions are associated with myocardial fat deposits. The relationship between fatty infiltration, especially in the epicardial layer and its pathophysiological implication is not completely understood. The aim of this study was to establish a positive or negative relationship between the ventricular burden and several parameters related to right ventricle (RV) adipose tissue - the RV thickness, RV indexed mass, body mass index (BMI), age, gender. Twenty-three patients with documented premature ventricular contractions (PVCs) originating from right ventricular outflow tract based on electrocardiography (ECG) evaluation were hospitalized between January 2018-November 2022 for electrophysiological study and PVCs ablation. Data obtained after collecting the clinical characteristics, ECG, RV measurements from transthoracic echocardiography (TTE), cardiac computed tomography (CT) and magnetic resonance imaging (MRI) were analyzed. A weak positive relationship between the ventricular burden and BMI (r=0.14, p=0.49), tricuspid annular plane systolic excursion (TAPSE) (r=0.07, p=0.7), the RV thickness (r=0.03, p=0.8), epicardial adipose tissue (r=0.13, p=0.55), RV mass indexed (r=0.05, p=0.82) was observed. No clear cut-off of the PVCs burden could be established in terms related to the increase in BMI, RV thickness, epicardial adipose tissue, RV mass indexed. No significant positive or negative relationship between the ventricular burden and the RV thickness, RV indexed mass were found in individuals with a high PVCs originating from right ventricular outflow tract (RVOT) burden.

Quantitative assessment of cardiac 123iodo-metaiodobenzylguanidine SPECT/CT in patients with arrhythmogenic right ventricular cardiomyopathy: Novel insight in disease monitoring

BackgroundThe heart-to-mediastinum ratio (H/M-Ratio) of 123iodo-metaiodobenzylguanidine (123I-MIBG) represents state-of-the-art assessment for sympathetic dysfunction in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC). This study aims to evaluate quantitative reconstruction of 123I-MIBG uptake and to demonstrate its correlation with echocardiographic parameters. MethodsCardiac innervation was assessed in 23 patients diagnosed with definite ARVC or borderline ARVC and 12 patients with other cardiac disease presenting arrhythmia, using quantitative 123I-MIBG Single Photon Emission Computed Tomography/Computed Tomography (SPECT/CT) imaging. Tracer uptake was evaluated in the left (LV) and right ventricle (RV) based on a CT scan after quantitative image reconstruction. The relationship between tracer uptake and echocardiographic parameter data was examined. ResultsAbsolute quantification of 123I-MIBG uptake in the LV and RV is feasible and correlates accurately with the gold standard H/M Ratio. When comparing sensitivity and specificity, the area under the curve (AUC) favors standardized uptake value (SUV) of the RV over the right-ventricle-to-mediastinum-ratio (RV/M-Ratio) for diagnosing ARVC. A reduced RV-SUV in patients with definite ARVC is associated with reduced RV function. RV polar maps revealed globally reduced 123I-MIBG uptake without segment-specific reduction in the RV. ConclusionsQuantitative 123I-MIBG SPECT in ARCV patients offers robust potential for clinical reporting and demonstrates a significant correlation with RV function. Segmental RV analysis needs to be evaluated in larger samples. In summary, cardiac 123I-MIBG imaging using SUV could facilitate image-guided therapy in patients diagnosed with ARVC.