Biventricular Function Research Articles

Dual-energy computed tomography to detect early pulmonary vascular changes in children with sickle cell disease: a pilot study.

Pulmonary hypertension (PH) is a rare but fatal complication of sickle cell disease (SCD) that is possibly reversible if treated early. Dual-energy computed tomography (DECT) is a valuable tool for diagnosing PH. We attempted to determine if DECT can detect early signs of PH in children with SCD. This prospective observational pilot study was conducted at the Geneva University Hospitals and was approved by the local human ethics committee (CCER 2019-01975). A written informed consent was obtained from the patients and/or their legal guardian. Eight children (consisting of five girls and three boys) with homozygous SCD were included in the study. They underwent full cardiological workup using transthoracic echocardiography (TTE) and cardiopulmonary exercise test (CPET), as well as DECT. The median age of the children was 11 years old (range 8-12). All patients exhibited a normal biventricular systo-diastolic function using the TTE. The median tricuspid regurgitant jet velocity value was 2.24 m/s (range 1.96-2.98). Four children were found to have signs of vasculopathy detected on DECT. Of them, two had abnormal screening test results. They both had an increased VE/VCO2 slope during CPET and an increased TVR of >2.5 m/s on TTE. DECT is capable of identifying early signs of pulmonary vascular disease in children with SCD. Further studies are needed to understand the correlation between DECT abnormalities and hemodynamic pulmonary circulation better.

Acute lymphocytic myocarditis presenting as complete heart block in an adult: a case report

BackgroundComplete heart block (CHB) as a first presentation of acute viral myocarditis is a rare occurrence associated with increased morbidity and mortality. In such cases, an endomyocardial biopsy is recommended to make a clear histological diagnosis aiding to differentiate from other possible conditions such as sarcoiditic myocarditis, giant cell myocarditis, and eosinophilic myocarditis. Insertion of a permanent pacemaker may be considered on a case-to-case basis.Case presentationA previously healthy 21-year-old female presented to the emergency department after having suffered two episodes of syncope on a background of a few days’ history of myalgias, chills, and rigors. Electrocardiogram showed high-grade Mobitz II block with intermittent periods of CHB. A bedside echocardiogram upon admission demonstrated normal biventricular systolic function. Given the patient’s unstable haemodynamic status and lack of obvious reversible causes for the CHB, a permanent dual-chamber pacemaker was inserted urgently. Initial blood investigations indicated an ongoing inflammatory process highlighting the possibility of myocarditis as a cause of the CHB. Therefore, a troponin level was taken and was noted to be elevated confirming the suspicion of myocarditis. The left ventricular ejection fraction (LVEF) decreased over the following days to approximately 20%, clinically resulting in pulmonary oedema and acute shortness of breath. The patient required aggressive intravenous diuresis and anti-heart failure medication. An endomyocardial biopsy (EMB) confirmed the diagnosis of lymphocytic myocarditis. The patient’s condition improved secondary to an improvement in LVEF and resolution of the heart block. A cardiac magnetic resonance (CMR) imaging performed 6 weeks from admission reported an improved LVEF of 51% with no late gadolinium enhancement (LGE). Based on the reassuring CMR findings and the resolution of CHB on follow-up pacemaker checks, it was deemed safe to explant the pacemaker.ConclusionsAcute myocarditis may be complicated with high-degree AV block and cardiogenic shock necessitating close observation in a critical care unit. A permanent pacemaker may provide atrio-ventricular synchrony which helps stabilise the patient’s condition and protect from a prolonged period of heart block. Early myocardial fibrosis on EMB and degree of LGE on CMR are indicators of persistent atrioventricular block. Guideline-directed treatment of heart failure is essential.

436 Total Arterial Revascularisation Complicated by Unexpected Vasoconstrictor Requirements

Abstract Introduction The debate surrounding conduit selection in multi-vessel coronary artery bypass grafting (CABG) persists. Whilst combined arterial and venous conduits remains the gold standard approach, some studies report superior long-term outcomes with total arterial revascularisation (TAR). Case Description A 48-year-old male was admitted with acute coronary syndrome. Investigations demonstrated significant triple vessel coronary artery disease with preserved biventricular systolic function. He had no major comorbidities. He underwent uneventful urgent off-pump CABG x 3 with TAR. Conduits used were a skeletonised left internal mammary artery (LIMA) and a radial artery. They were anastomosed to make a Y configuration. The LIMA was anastomosed end-to-side to the mid-LAD and the radial artery was anastomosed side-to-side to the first obtuse marginal and end-to-side to the posterior descending artery. Post-operatively the patient had significant drain output, resulting in haemodynamic instability and a significant increase in vasoconstrictor requirements. This led to vasoconstrictive spasm of the arterial grafts and cardiac arrest ensued. The patient underwent emergency re-sternotomy and institution of cardiopulmonary bypass. The arterial grafts were removed, and the patient was re-grafted with three saphenous vein grafts. The patient was successfully discharged home in a stable condition on the tenth post-operative day. Discussion Careful management of post-operative complications in patients who underwent TAR is required in order to reduce the risk of arterial graft spasm. Pre-operative planning should include the consideration of alternative conduit strategies in patients where higher post-operative vasoconstrictor requirements are anticipated.

Feasibility of Interleukin-6 Receptor Blockade in Cardiac Antibody-mediated Rejection.

Antibody-mediated rejection (AMR) remains a significant cause of heart transplant mortality with few effective therapies. This study aimed to describe initial experience of using interleukin-6 receptor blockade with tocilizumab in the treatment of acute cardiac AMR at Barnes-Jewish Hospital/Washington University Transplant Center from July 2017 to May 2021 (n = 7). Clinical, echocardiographic, and serum alloantibody data were analyzed before and after treatment. All participants demonstrated marked improvement in functional status. Echocardiographic data following 4-6 mo of tocilizumab revealed significant improvements in biventricular systolic function for all participants. Consistent reductions in donor-specific HLA or angiotensin type I receptor antibodies were not observed, suggesting that tocilizumab may act downstream of antibody production. No patient experienced drug-related complications that necessitated discontinuation of therapy. These findings provide initial insights into the safety and efficacy of interleukin-6 receptor blockade in the treatment of cardiac AMR and support the design of larger prospective studies.

Echocardiographic findings in non-hospitalised children and adolescents following acute COVID-19.

Although COVID-19 is known to have cardiac effects in children, seen primarily in severe disease, more information is needed about the cardiac effects following COVID-19 in non-hospitalised children and adolescents during recovery. This study aims to compare echocardiographic markers of cardiac size and function of children following acute COVID-19 with those of healthy controls. This single-centre retrospective case-control study compared 71 cases seen in cardiology clinic following acute COVID-19 with 33 healthy controls. Apical left ventricle, apical right ventricle, and parasternal short axis at the level of the papillary muscles were analysed to measure ventricular size and systolic function. Strain was analysed on vendor-independent software. Statistical analysis was performed using t-test, chi-square, Wilcoxon rank sum, and regression modelling as appropriate (p < 0.05 significant). Compared to controls, COVID-19 cases had slightly higher left ventricular volumes and lower left ventricular ejection fraction and right ventricular fractional area change that remained within normal range. There were no differences in right or left ventricular longitudinal strain between the two groups. Neither initial severity nor persistence of symptoms after diagnosis predicted these differences. Echocardiographic findings in children and adolescents 6 weeks to 3 months following acute COVID-19 not requiring hospitalisation were overall reassuring. Compared to healthy controls, the COVID-19 group demonstrated mildly larger left ventricular size and lower conventional measures of biventricular systolic function that remained within the normal range, with no differences in biventricular longitudinal strain. Future studies focusing on longitudinal echocardiographic assessment of patients following acute COVID-19 are needed to better understand these subtle differences in ventricular size and function.

Cardiac Function Evaluation after SARS-CoV-2 mRNA Vaccination in Children and Adolescents: A Prospective Speckle-Tracking Echocardiography Study.

Possible cardiac impairment after SARS-CoV-2 mRNA vaccination is a common driver of parental vaccine hesitancy. We performed a comprehensive echocardiographic evaluation of biventricular function in vaccinated children with or without previous COVID-19 compared to healthy controls. We conducted a single-center, prospective, case-control study enrolling children and adolescents aged 5-18 years attending the pediatric clinic of the University Hospital of Padua from April to June 2022. Three months after receiving the primary mRNA vaccination or booster dose, the patients underwent a cardiac assessment, including standard echocardiography and speckle-tracking echocardiography (STE). A pre-pandemic historical cohort of age- and gender-matched healthy children were used as a control. A total of 39 post-VACCINE cases (24, 61% female), mean age 12.6 ± 2.6 years (range 8-17), were enrolled in the study. Ninety percent (N = 35) of patients were previously healthy. No differences in left ventricular diameters, left ventricular ejection fraction (LVEF), and tricuspid annular plane systolic excursion (TAPSE) were observed between cases and controls. Global longitudinal strain (GLS) was in the normal range in all individuals, with no differences between post-VACCINE cases and controls (-21.7 ± 2.3% vs. 21.2 ± 1.8%; p = 0.338). However, GLS was found to be slightly but significantly reduced in post-VACCINE children with a previous COVID-19 compared to naïve-vaccinated individuals (post-VACCINE+COVID-19: -19.9 ± 1.1% vs. post-VACCINE-only: -22.0 ± 2.3%; p = 0.002). We did not observe an impairment in GLS or in other indices of LV structure or function after mRNA COVID-19 vaccination.

Utility of Follow-Up Echocardiograms in Uncomplicated Surgical Secundum Atrial Septal Defect Closures: Preliminary Analysis.

Though less common in the current era, surgical closure of secundum atrial septal defects (ASD2) is still performed in certain clinical situations. Guidelines currently recommend lifelong follow-up with transthoracic echocardiograms (TTE) for patients who have undergone a surgical ASD2 closure. The goal of this study was to determine the utility of follow-up TTE in patients who underwent an uncomplicated ASD2 closure. Chart review was performed on patients who had a surgical ASD2 closure between April 1, 1996, and August 30, 2021. Patients were excluded if they had other congenital heart disease, had a diagnosis of a residual ASD2, atrial/ventricular arrhythmias, pulmonary hypertension, heart failure, or did not have a follow-up TTE > 6months after the procedure. The most recent TTEs and clinic notes were evaluated. A total of 30 patients met the criteria. The median age at ASD2 surgery was 4.0years (IQ; 1.9-10.5). ASD2 was closed via patch repair in 16 patients and primarily closed in 14 patients. The most recent TTE was performed a median of 9.5years (IQ; 4.0, 14.7) after ASD2 closure. Two patients had mild right atrial and ventricular dilation, one patient had mild right atrial dilation, and one patient had mild right ventricular dilation. All other patients had qualitatively normal right-sided chamber sizes. All patients had normal biventricular function (left ventricular fractional shortening (median 36% (IQ; 33, 42)), no evidence of residual atrial shunts, and no evidence of pulmonary hypertension. No patient was on any cardiac medications at last clinic visit. Four patients were discharged from cardiology clinic and 10 patients were lost to follow-up. There were no deaths. Twenty-four patients had 46 repeat echocardiograms > 1year after ASD2 with no change in clinical management. In patients who underwent an uncomplicated ASD2 closure, there were no significant abnormalities noted on follow-up TTEs. The need for repeat lifetime TTEs and their frequency, in this uncomplicated population, should be reassessed if larger studies with longer follow-up confirm these initial findings.

Impact of diabetes on remodelling, microvascular function and exercise capacity in aortic stenosis

ObjectiveTo characterise cardiac remodelling, exercise capacity and fibroinflammatory biomarkers in patients with aortic stenosis (AS) with and without diabetes, and assess the impact of diabetes on outcomes.MethodsPatients with moderate or...

Assessment of Myocardial Function in Infants of Mothers with Gestational Diabetes Mellitus Using Deformation Imaging over the First Year of Age

To assess serial myocardial performance and pulmonary vascular resistance (PVR) in infants of mothers with gestational diabetes mellitus (GDM) over the first year of life. This was a prospective, observational study. Echocardiography was performed at birth, 6months, and 1year of age. Pulmonary artery acceleration time and left ventricular (LV) eccentricity index provided surrogate measurements of PVR. Biventricular function was assessed by tissue Doppler imaging and deformation analysis. Fifty infants of mothers with GDM were compared with 50 controls with no difference in gestation (38.9±0.8 weeks vs 39.3±0.9weeks; P=.05) or birthweight (3.55±0.49 kg vs 3.56±0.41kg; P=.95). At 1 year of age, the pulmonary artery acceleration time was lower (70±11 vs 79±10; P=.01) in the GDM group. LV global longitudinal strain (24.7±1.9 vs 28.8±1.8 %; P<.01), LV systolic strain rate (1.8±0.2 vs 2.1±0.3 1/s; P<.01), and RV free wall strain (31.1±4.8 vs 34.6±3.9 %; P<.01) were lower in the GDM cohort at 1year of age (all P values adjusted for gestation, mode of delivery, and maternal body mass index). Our findings demonstrate higher indices of PVR and lower biventricular function in infants ofmothers with GDM compared with controls at each time point assessed in this study over the first year of life.

The Yasui operation: A single institutional experience over 30 years

ObjectiveThe Yasui operation was introduced in 1987 for patients with 2 adequate ventricles, a ventricular septal defect, and aortic atresia or interrupted aortic arch. Despite promising early outcomes, left ventricular outflow tract obstruction (LVOTO) remains a long-term concern. The purpose of this study is to report our institutional experience with the Yasui operation. MethodsWe retrospectively reviewed all patients undergoing the Yasui operation between 1989 and 2021. Results are reported as median with interquartile range (IQR). ResultsTwenty-five patients underwent a Yasui operation (19 primary), at 11 days (IQR, 7-218 days) of life and weight of 3 kg (IQR, 2.8-4.1 days). Fundamental diagnosis was ventricular septal defect/interrupted aortic arch in 11 patients and ventricular septal defect/aortic atresia in 14. Follow-up was 96% (24 out of 25) at 5 years (IQR, 1.4-14.7) with 92% survival. Freedom from LVOTO reoperation was 91% at late follow-up with 2 patients requiring baffle revision at 6 and 9 years. Latest echocardiogram showed 100% of patients had normal biventricular function and 87% (20 out of 23) less than mild LVOTO at 5 years (IQR, 2.3-14.9). Diagnosis, aortic valve morphology, and material used were not predictors of LVOTO. Freedom from right ventricle-to-pulmonary artery conduit reoperation was 48% at a median of 5 years (IQR, 1.4-14.7). Conduit type was not a predictor of reintervention. ConclusionsThe Yasui operation can be performed with low morbidity and mortality in patients with 2 acceptable-size ventricles and aortic atresia or interrupted aortic arch with severe LVOTO. Despite some burden of reoperation, midterm reoperation for LVOTO is not common and ventricular function is preserved.

Prognostic significance of subpulmonary left ventricular size and function in patients with a systemic right ventricle.

To assess the additional prognostic significance of echocardiographic parameters of subpulmonary left ventricular (LV) size and function in patients with a systemic right ventricle (SRV). All adults with an SRV who underwent transthoracic echocardiography in 2010-18 at a large tertiary centre were identified. Biventricular size and function were assessed at the most recent examination. The study endpoint was all-cause mortality or heart/heart-lung transplantation. We included 180 patients, with 100 (55.6%) males, with a mean age of 42.4 ± 12.3 years, of whom 103 (57.2%) had undergone Mustard/Senning operations and 77 (42.8%) had congenitally corrected transposition of great arteries. Over 4.9 (3.8-5.7) years, 28 (15.6%) patients died and 4 (2.2%) underwent heart or heart-lung transplantation. Univariable predictors of the study endpoint included age, New York Heart Association functional Class III or IV, history of atrial arrhythmias, presence of a pacemaker or cardioverter defibrillator, high B-type natriuretic peptide, and echocardiographic markers of SRV and subpulmonary LV size and function. On multivariable Cox analysis of echocardiographic variables, indexed LV end-systolic diameter [ESDi; hazard ratio (HR) 2.77 (95% confidence interval, CI) 1.35-5.68, P = 0.01], LV fractional area change [FAC; HR 0.7 (95% CI 0.57-0.85), P = 0.002), SRV basal diameter [HR 1.66 (95% CI 1.21-2.29), P = 0.005], and SRV FAC [HR 0.65 (95% CI 0.49-0.87), P = 0.008] remained predictive of mortality or transplantation. On receiver-operating characteristic analysis, subpulmonary LV parameters performed better than SRV markers in predicting adverse events. SRV basal diameter, SRV FAC, LV ESDi, and LV FAC are significantly and independently associated with mortality and transplantation in adults with an SRV. Accurate echocardiographic assessment of both SRV and subpulmonary LV is, therefore, essential to inform risk stratification and management.

From Compressed-Sensing to Deep Learning MR: Comparative Biventricular Cardiac Function Analysis in a Patient Cohort.

Conventional segmented, retrospectively gated cine (Conv-cine) is challenged in patients with breath-hold difficulties. Compressed sensing (CS) has shown values in cine imaging but generally requires long reconstruction time. Recent artificial intelligence (AI) has demonstrated potential in fast cine imaging. To compare CS-cine and AI-cine with Conv-cine in quantitative biventricular functions, image quality, and reconstruction time. Prospective human studies. 70 patients (age, 39 ± 15 years, 54.3% male). 3T; balanced steady state free precession gradient echo sequences. Biventricular functional parameters of CS-, AI-, and Conv-cine were measured by two radiologists independently and compared. The scan and reconstruction time were recorded. Subjective scores of image quality were compared by three radiologists. Paired t-test and two related-samples Wilcoxon sign test were used to compare biventricular functional parameters between CS-, AI-, and Conv-cine. Intraclass correlation coefficient (ICC), Bland-Altman analysis, and Kendall's W method were applied to evaluate agreement of biventricular functional parameters and image quality of these three sequences. A P-value <0.05 was considered statistically significant, and standardized mean difference (SMD) < 0. 100 was considered no significant difference. Compared to Conv-cine, no statistically significant differences were identified in CS- and AI-cine function results (all P > 0.05), except for very small differences in left ventricle end-diastole volumes of 2.5 mL (SMD = 0.082) and 4.1 mL (SMD = 0.096), respectively. Bland-Altman scatter plots revealed that biventricular function results were mostly distributed within the 95% confidence interval. All parameters had acceptable to excellent interobserver agreements (ICC: 0.748-0.989). Compared with Conv-cine (84 ± 13 sec), both CS (14 ± 2 sec) and AI (15 ± 2 sec) techniques reduced scan time. Compared with CS-cine (304 ± 17 sec), AI-cine (24 ± 4 sec) reduced reconstruction time. CS-cine demonstrated significantly lower quality scores than Conv-cine, while AI-cine demonstrated similar scores (P = 0.634). CS- and AI-cine can achieve whole-heart cardiac cine imaging in a single breath-hold. Both CS- and AI-cine have the potential to supplement the gold standard Conv-cine in studying biventricular functions and benefit patients having difficulties with breath-holds. 1 TECHNICAL EFFICACY STAGE: 1.

Multimodality imaging of myocardial tuberculosis presenting as right ventricular cardiomyopathy: A case report.

An 18-year male with no significant past medical history presented with features of right-sided heart failure. Haematological investigations revealed raised erythrocyte sedimentation rate (23 mm/h). Transthoracic echocardiography (TTE) demonstrated biventricular systolic dysfunction with thickened free wall of right ventricle (RV). Whole-body fluorodeoxyglucose (FDG)-positron emission tomography (PET) computed tomography (FDG-PET-CT) showed intensely increased FDG uptake in the thickened RV wall and an FDG avid lesion in the left medial temporal lobe. Cardiac magnetic resonance imaging (CMR) characterized thickening and suggested an infiltrative disease (isointense on TI, hyperintense on myocardial oedema sensitive sequence with transmural enhancement on late gadolinium sequence). Magnetic resonance imaging (MRI) brain showed a thick rim enhancing ring lesion in the body of hippocampus. Endomyocardial biopsy revealed moderately dense lymphomononuclear inflammation with the formation of granulomas. Since tuberculosis is endemic in our country and considering all laboratory, imaging and biopsy findings a working diagnosis of tuberculosis was considered, and standard anti-tubercular treatment was started. There was an improvement in clinical status and follow-up imaging (TTE, CMR, brain MRI and FDG-PET-CT) showed improvement in biventricular function with regression in myocardial thickening and resolution of brain lesion, confirming the diagnosis of tuberculosis.

Beneficial Effects of Ketone Ester in Patients With Cardiogenic Shock: A Randomized, Controlled, Double-Blind Trial

BackgroundCardiogenic shock (CS) is a life-threatening condition with sparse treatment options. The ketone body 3-hydroxybutyrate has favorable hemodynamic effects in patients with stable chronic heart failure. Yet, the hemodynamic effects of exogenous ketone ester (KE) in patients with CS remain unknown. ObjectivesThe authors aimed to assess the hemodynamic effects of single-dose enteral treatment with KE in patients with CS. MethodsIn a double-blind, crossover study, 12 patients with CS were randomized to an enteral bolus of KE and isocaloric, isovolumic placebo containing maltodextrin. Patients were assessed with pulmonary artery catheterization, arterial blood samples, echocardiography, and near-infrared spectroscopy for 3 hours following each intervention separated by a 3-hour washout period. ResultsKE increased circulating 3-hydroxybutyrate (2.9 ± 0.3 mmol/L vs 0.2 ± 0.3 mmol/L, P < 0.001) and was associated with augmented cardiac output (area under the curve of relative change: 61 ± 22 L vs 1 ± 18 L, P = 0.044). Also, KE increased cardiac power output (0.07 W [95% CI: 0.01-0.14]; P = 0.037), mixed venous saturation (3 percentage points [95% CI: 1-5 percentage points]; P = 0.010), and forearm perfusion (3 percentage points [95% CI: 0-6 percentage points]; P = 0.026). Right (P = 0.048) and left (P = 0.017) ventricular filling pressures were reduced whereas heart rate and mean arterial and pulmonary arterial pressures remained similar. Left ventricular ejection fraction improved by 4 percentage points (95% CI: 2-6 percentage points; P = 0.005). Glucose levels decreased by 2.6 mmol/L (95% CI: −5.2 to 0.0; P = 0.047) whereas insulin levels remained unaltered. ConclusionsTreatment with KE improved cardiac output, biventricular function, tissue oxygenation, and glycemic control in patients with CS (Treatment With the Ketone Body 3-hydroxybutyrate in Patients With Cardiogenic Shock [KETO-SHOCK1]; NCT04642768)

Clinical benefits of angiotensin receptor-Neprilysin inhibitor in adults with congenital heart disease

BackgroundThere are limited data about the clinical benefits of angiotensin receptor-neprilysin inhibitor (ARNI) in adults with congenital heart disease (CHD). The purpose of the study was to assess the clinical benefits (chamber function and heart failure indices) of ARNI in adults with CHD. MethodIn this retrospective cohort study, we compared the temporal change in chamber function and heart failure indices between 35 patients that received ARNI for >6 months, and a propensity matched control group (n = 70) of patients that received angiotensin converting enzyme inhibitor or angiotensin-II receptor blocker (ACEI/ARB) within the same period. ResultsOf the 35 patients in the ARNI group, 21 (60%) had systemic left ventricle (LV) while 14 (40%) had systemic right ventricle (RV). Compared to the ACEI/ARB group, the ARNI group had greater relative improvement in LV global longitudinal strain (GLS) (28% versus 11% increase from baseline, p < 0.001) and RV-GLS (11% versus 4% increase from baseline, p < 0.001), and greater relative improvement in New York Heart Association functional class (−14 versus −2% change from baseline, p = 0.006) and N-terminal pro-brain natriuretic peptide levels (−29% versus −13% change from baseline, p < 0.001). These results were consistent across different systemic ventricular morphologies. ConclusionsARNI was associated with improvement in biventricular systolic function, functional status, and neurohormonal activation, suggesting prognostic benefit. These results provide a foundation for a randomized clinical trial to empirically test the prognostic benefits of ARNI in adults with CHD, as the next step towards evidence-based recommendations for heart failure management in this population.

Establishing minimally important differences for cardiac MRI end-points in pulmonary arterial hypertension.

Cardiac magnetic resonance (CMR) is the gold standard technique to assess biventricular volumes and function, and is increasingly being considered as an end-point in clinical studies. Currently, with the exception of right ventricular (RV) stroke volume and RV end-diastolic volume, there is only limited data on minimally important differences (MIDs) reported for CMR metrics. Our study aimed to identify MIDs for CMR metrics based on US Food and Drug Administration recommendations for a clinical outcome measure that should reflect how a patient "feels, functions or survives". Consecutive treatment-naïve patients with pulmonary arterial hypertension (PAH) between 2010 and 2022 who had two CMR scans (at baseline prior to treatment and 12 months following treatment) were identified from the ASPIRE registry. All patients were followed up for 1 additional year after the second scan. For both scans, cardiac measurements were obtained from a validated fully automated segmentation tool. The MID in CMR metrics was determined using two distribution-based (0.5sd and minimal detectable change) and two anchor-based (change difference and generalised linear model regression) methods benchmarked to how a patient "feels" (emPHasis-10 quality of life questionnaire), "functions" (incremental shuttle walk test) or "survives" for 1-year mortality to changes in CMR measurements. 254 patients with PAH were included (mean±sd age 53±16 years, 79% female and 66% categorised as intermediate risk based on the 2022 European Society of Cardiology/European Respiratory Society risk score). We identified a 5% absolute increase in RV ejection fraction and a 17 mL decrease in RV end-diastolic or end-systolic volumes as the MIDs for improvement. Conversely, a 5% decrease in RV ejection fraction and a 10 mL increase in RV volumes were associated with worsening. This study establishes clinically relevant CMR MIDs for how a patient "feels, functions or survives" in response to PAH treatment. These findings provide further support for the use of CMR as a clinically relevant clinical outcome measure and will aid trial size calculations for studies using CMR.

The relationship of myocardial and liver T2* values with cardiac function and laboratory findings in transfusion-dependent thalassemia major patients: A retrospective cardiac MRI study.

Cardiac complications are the leading cause of death in thalassemia patients. It is assumed that progressive iron accumulation results in myocyte damage. Myocardial T2* measurement by cardiac MRI quantifies iron overload. We aimed to study the association between left and right ventricular (LV and RV) function and iron deposition estimation by cardiac MRI T2* in a sample of Iranian patients. Cardiac MRI exams of 118 transfusion-dependent thalassemia major patients were evaluated retrospectively. Biventricular function and volume and myocardial and liver T2* values were measured. The demographic and lab data were registered. Poisson and chi-square regression analyses investigated the correlation between the T2* value and ventricular dysfunction. The study participants' mean (SD) age was 32.7y (9.02), and 47.46% were female. Forty-nine cases (41.52%) revealed at least uni-ventricular dysfunction. LV dysfunction was noted in 20 cases, whereas 47 patients revealed RV dysfunction. The risk of LV dysfunction was 5.3-fold higher in patients with cardiac T2* value less than 10msec (RR=5.3, 95% CI=1.6, 17.1, P<0.05). No association was found between age, liver T2* value, serum ferritin level, and chelation therapy with the risk of LV and RV dysfunction. Cardiac MRI T2* measure is a good indicator of LV dysfunction. Moreover, MRI parameters, especially RV functional measures, may have a substantial role in patient management. Therefore, cardiac MRI should be included in beta-thalassemia patients' management strategies.

Bilateral lung transplantation for pediatric pulmonary arterial hypertension: perioperative management and one-year follow-up.

Bilateral lung transplantation (LuTx) remains the only established treatment for children with end-stage pulmonary arterial hypertension (PAH). Although PAH is the second most common indication for LuTx, little is known about optimal perioperative management and midterm clinical outcomes. Prospective observational study on consecutive children with PAH who underwent LuTx with scheduled postoperative VA-ECMO support at Hannover Medical School from December 2013 to June 2020. Twelve patients with PAH underwent LuTx (mean age 11.9 years; age range 1.9-17.8). Underlying diagnoses included idiopathic (n = 4) or heritable PAH (n = 4), PAH associated with congenital heart disease (n = 2), pulmonary veno-occlusive disease (n = 1), and pulmonary capillary hemangiomatosis (n = 1). The mean waiting time was 58.5 days (range 1-220d). Three patients were bridged to LuTx on VA-ECMO. Intraoperative VA-ECMO/cardiopulmonary bypass was applied and VA-ECMO was continued postoperatively in all patients (mean ECMO-duration 185 h; range 73-363 h; early extubation). The median postoperative ventilation time was 28 h (range 17-145 h). Echocardiographic conventional and strain analysis showed that 12 months after LuTx, all patients had normal biventricular systolic function. All PAH patients are alive 2 years after LuTx (median follow-up 53 months, range 26-104 months). LuTx in children with end-stage PAH resulted in excellent midterm outcomes (100% survival 2 years post-LuTx). Postoperative VA-ECMO facilitates early extubation with rapid gain of allograft function and sustained biventricular reverse-remodeling and systolic function after RV pressure unloading and LV volume loading.

Diastolic ventricular function in persistent pulmonary hypertension of the newborn.

Persistent pulmonary hypertension of the newborn (PPHN) is usually considered a consequence of impaired pulmonary circulation. However, little is known regarding the role of cardiac dysfunction in PPHN. In this study, we hypothesized that the tolerance for pulmonary hypertension in newborn infants depends on the biventricular function. The aim of this study is to evaluate biventricular cardiac performance by using Tissue Doppler Imaging (TDI) in an healthy newborn infants with asymptomatic pulmonary hypertension and in newborn infants with PPHN. Right and left cardiac function were investigated using conventional imaging and TDI in 10 newborn infants with PPHN ("PPHN") and 10 asymptomatic healthy newborn infants ("asymptomatic PH"). Systolic pulmonary artery pressure (PAP) as assessed by TDI and the mean systolic velocity of the right ventricular (RV) free wall were similar in both groups. The isovolumic relaxation time of the right ventricle at the tricuspid annulus was significantly longer in the "PPHN" than in the "asymptomatic PH" group (53 ± 14 ms vs. 14 ± 4 ms, respectively; p < 0.05). Left ventricular (LV) function was normal in both groups with a systolic velocity (S'LV) at the LV free wall groups (6 ± 0.5 cm/s vs. 8.3 ± 5.7 cm/s, p > 0.05). The present results suggest that high PAP with or without respiratory failure is not associated with altered right systolic ventricular function and does not affect LV function in newborn infants. PPHN is characterized by a marked right diastolic ventricular dysfunction. These data suggest that the hypoxic respiratory failure in PPHN results, at least in part, from diastolic RV dysfunction and right to left shunting across the foramen ovale. We propose that the severity of the respiratory failure is more related to the RV diastolic dysfunction than the pulmonary artery pressure.

Assessment of biventricular myocardial systolic function in COVID-19 patients: A Tissue Doppler Imaging echocardiography study

AbstractBackgroundThe 2019 novel coronavirus disease (COVID-19) has been reported as pandemy and the number of patients continues to rise. Based on recent data, cardiac injury is a prominent feature of the disease, leading to increased morbidity and mortality. In the present study we aimed to evaluate myocardial dysfunction using transthoracic echocardiography (TTE) and tissue Doppler imaging (TDI) in hospitalized COVID-19 patients.Methods and ResultsWe recruited 30 patients (56.7% male, 55.80 ± 14.949 years) who were hospitalized with the diagnosis COVID-19 infection. We analyzed left ventricular (LV) and right ventricular (RV) conventional and TDI parameters at the time of hospitalization and during the course of the disease. Patients without any cardiac disease and with preserved LV ejection fraction (EF) were included. TTE examination was performed and all the variables were recorded and analyzed retrospectively.We observed that both LV and RV conventional echocardiographic parameters were similar when the day of admission to the hospital was compared to the 5th day of the disease. Regarding TDI analysis, we demonstrated significant impairment in LV septal and lateral deformation (P &lt; 0.001). In the correlation analysis no marked correlation was observed between impairment in LV deformation and inflammation biomarkers.ConclusionCardiac involvement is an important feature of the COVID-19 infection but the exact mechanism is still undefined. Echocardiography is an essential technique to describe myocardial injury and provide new concepts for the possible definitions of cardiac dysfunction.