Right ventricular fibrosis in adults with uncorrected secundum atrial septal defect and pulmonary hypertension: a cardiovascular magnetic resonance study with late gadolinium enhancement, native T1 and extracellular volume.

Introduction: Right ventricular (RV) fibrosis represents both adaptive and maladaptive responses to the overloaded RV condition. Its role in pulmonary hypertension (PH) associated with secundum atrial septal defect (ASD), which is the most common adult congenital heart disease (CHD), remains poorly understood. Methods: We enrolled 65 participants aged ≥18 years old with uncorrected secundum ASD who had undergone clinically indicated right heart catheterization (RHC), divided into the non-PH group (n = 7), PH group (n = 42), and Eisenmenger syndrome (ES) group (n = 16). We conducted cardiovascular magnetic resonance (CMR) studies with late gadolinium enhancement (LGE) imaging, native T1 mapping, and extracellular volume (ECV) measurement to evaluate the extent and clinical correlates of RV fibrosis. Results: LGE was present in 94% of the population and 86% of the non-PH group, mostly located at the right ventricular insertion point (RVIP) regions. LGE in the septal and inferior RV region was predominantly observed in the ES group compared to the other groups (p = 0.031 and p < 0.001, respectively). The mean LGE scores in the ES and PH groups were significantly higher than those in the non-PH group (3.38 ± 0.96 vs. 2.74 ± 1.04 vs. 1.57 ± 0.79; p = 0.001). The ES and PH groups had significantly higher degrees of interstitial RV fibrosis compared to those in the non-PH group, indicated by native T1 (1,199.9 ± 68.9 ms vs. 1,131.4 ± 47.8 ms vs. 1,105.4 ± 44.0 ms; p < 0.001) and ECV (43.6 ± 6.6% vs. 39.5 ± 4.9% vs. 39.4 ± 5.8%; p = 0.037). Additionally, native T1 significantly correlated with pulmonary vascular resistance (r = 0.708, p < 0.001), RV ejection fraction (r = -0.468, p < 0.001) and peripheral oxygen saturation (r = -0.410, p = 0.001). Conclusion: In patients with uncorrected secundum ASD, RV fibrosis may occur before the development of PH and progressively intensify alongside the progression of PH severity. A higher degree of RV fibrosis, derived from CMR imaging, correlates with worse hemodynamics, RV dysfunction, and poorer clinical conditions.

Ductal stenting vs. surgical shunting in late presenting duct-dependent pulmonary circulation: a single-center experience.

Introduction: PDA stenting is an option to mBTT shunt for younger patients; nevertheless, few reports of this palliative approach have been made for the late presenter population, especially for patients who are older than 30 days but under 5 years. This study aimed to evaluate the clinical result and intra-hospital costs of ductal stenting in late-presenting patients in comparison to surgical shunting. Methods: A single-center, retrospective cohort study was conducted from August 2016 to August 2022. This study included patients with pulmonary duct dependent CHD who were hospitalized for palliative therapy. The extracted data were baseline characteristics, clinical findings, supportive examination findings, complications, outcomes, and length of stay of the patients. Monitoring was carried out during treatment up to 30 days after the procedure. Results: A total of 143 patients were included in the analysis; 43 patients underwent PDA stent and 100 patients underwent mBTT shunt with median age of PDA stent group 110 (31-1,498) days and mBTT shunt group 174.5 (30-1,651) days. Primary outcome composite was not significant in both groups including 30 days mortality [6 (14%) vs. 14 (14%), p = 1.000], reintervention [1 (2.3%) vs. 7 (7%), p = 0.436], and 30 days rehospitalization [0 (0%) vs. 2 (2%), p = 0.319]. Secondary outcome analysis showed shorter ICU length of stay in the PDA stent group [2 (0-16) days vs. 4 (1-63) days, p = 0.002]. Conclusions: PDA stent has an outcome that is non inferior from the mBTT shunt procedure in the composite outcome including 30 days mortality, reintervention, and 30 days rehospitalization but significantly lower in ICU length of stay.

Adherence to penicillin treatment is essential for effective secondary prevention of rheumatic heart disease: a systematic review and meta-analysis.

Background: Penicillin is essential for secondary prevention of acute rheumatic fever (ARF) and rheumatic heart disease (RHD). However, the incidences of ARF recurrence and RHD progression remain high, particularly in endemic countries. This meta-analysis evaluated the effectiveness of penicillin adherence in secondary prevention of ARF recurrence and RHD progression. Methods: The authors included original articles employing an observational study design in which the study population included patients with ARF or RHD and documented adherence to secondary prophylaxis with penicillin for secondary prevention. Systematic searches of the PubMed, Scopus, and Cochrane databases were performed. Moreover, the authors also conducted a snowballing literature search from Europe PMC to expand the included studies. The quality of each study was assessed using the National Institute of Health Quality Assessment Tool. The statistical analyses were conducted using Review Manager 5.4.1 software developed by Cochrane. In addition, the authors utilized pooled odds ratios (ORs) to compare the adherence techniques. Results: A total of 310 studies were identified, of which 57 full-text articles were assessed for eligibility. The authors included six studies with 1364 patients for the qualitative synthesis and meta-analysis. Good adherence to penicillin for the secondary prophylaxis of ARF and RHD, significantly reduced the odds of ARF recurrence or RHD progression by up to 71% compared to that associated with poor adherence [pooled OR 0.29 (0.21-0.40); I²=0% (p=0.56); Z=7.64 (p <0.00001)]. Conclusion: Good adherence to penicillin for secondary prophylaxis in patients with ARF or RHD is essential for reducing the risk of ARF recurrence or RHD progression.

Clinical Outcome of Rheumatic Mitral Valve Repair and Replacement Surgery in Indonesia; A Comparison with Non-Rheumatic Aetiology.

Introduction: Mitral valve repair (MVr) has been shown to achieve better outcomes than mitral valve replacement (MVR) in degenerative aetiology. However, that cannot be applied in rheumatic mitral valve disease. Therefore, this study aims to evaluate early and late clinical outcomes and mid-term survival in RHD compared to the non-RHD group and whether mitral valve repair is a better surgical approach in RHD patients. Methods: Patients who underwent mitral valve surgery with or without coronary artery bypass grafting were included in this study. All patients were divided into the RHD and non-RHD group by the type of mitral surgery performed. Early and late outcomes were evaluated, and mid-term cumulative survival was reported. Results: A total of 1382 patients post MV surgeries were included. The 30-day mortality was significantly higher in the RHD group compared to the non-RHD group (8.7% vs. 4.4%, p = 0.003). There was no difference in 30-day mortality between repair and replacement in each respective group. During follow-up (12-54 months), all-cause mortality between RHD and non-RHD groups (16.7% vs. 16.2%) was not different. In the RHD group, the survival of MVr was 85.6% (95% CI 82.0%-88.5%), and MVR was 78.3% (95% CI 75.8%-80.6%), p-value log rank 0.26 However, in the non-RHD group, patients who underwent MVr had better survival than MVR, with cumulative survival of 81.7% (95% CI 72.3%-88.2%) vs. 71.1% (95% CI 56.3%-81.7%) p-value log rank 0.007. Conclusion: Early mortality rate in rheumatic mitral valve surgery was higher than in non-rheumatic valve surgery. Although in rheumatic MV disease MV repair did not show a significant survival advantage over MV replacement, a trend towards more favourable survival in the repair group was observed.

Global Left Ventricular Myocardial Work Efficiency in Patients With Severe Rheumatic Mitral Stenosis and Preserved Left Ventricular Ejection Fraction.

BACKGROUND: Assessment of left ventricular (LV) function plays a pivotal role in the management of patients with valvular heart disease, including those caused by rheumatic heart disease. Noninvasive LV pressure-strain loop analysis is emerging as a new echocardiographic method to evaluate global LV systolic function, integrating longitudinal strain by speckle-tracking analysis and noninvasively measured blood pressure to estimate myocardial work. The aim of this study was to characterize global LV myocardial work efficiency in patients with severe rheumatic mitral stenosis (MS) with preserved ejection fraction (EF). METHODS: We retrospectively included adult patients with severe rheumatic MS with preserved EF (> 50%) and sinus rhythm. Healthy individuals without structural heart disease were included as a control group. Global LV myocardial work efficiency was estimated with a proprietary algorithm from speckle-tracking strain analyses, as well as noninvasive blood pressure measurements. RESULTS: A total of 45 individuals with isolated severe rheumatic MS with sinus rhythm and 45 healthy individuals were included. In healthy individuals without structural heart disease, the mean global LV myocardial work efficiency was 96% (standard deviation [SD], 2), Compared with healthy individuals, median global LV myocardial work efficiency was significantly worse in MS patients (89%; SD, 4; p < 0.001) although the LVEF was similar. CONCLUSIONS: Individuals with isolated severe rheumatic MS and preserved EF, had global LV myocardial work efficiencies lower than normal controls.

Comparison between Visual Assessment and Longitudinal Strain during Dobutamine Stress Echocardiography.

Background: The relationship between visual assessment and longitudinal strain during dobutamine stress echocardiography (DSE) remains poorly investigated. This study assessed wall motion segments visually graded as normokinetic, hypokinetic, and akinetic at baseline and the peak of DSE and compared with longitudinal strain between segments with and without induced impaired contractility and improved contractility during DSE. Methods: This study included 112 patients examined by DSE, consisting of 58 patients referred for diagnostic study and 54 patients referred for viability study. Regional left ventricular (LV) contractility was assessed visually and longitudinal strain was measured using echocardiography transthoracic. Results: At baseline, the strain of LV segments was -16.33 ± 6.26 in visually normokinetic, 13.05 ± 6.44 in visually hypokinetic, and -8.46 ± 5.69 in visually akinetic segments. During peak dose, the strain of LV segments was -15.37 ± 6.89 in visually normokinetic, -11.37 ± 5.11 in visually hypokinetic, and -7.37 ± 3.92 in visually akinetic segments. In segments with visually observed impaired contractility, the median longitudinal strain was significantly lower than in segments without impaired contractility. For segments with visually observed improved contractility, the median longitudinal strain was significantly higher than for segments without improved contractility. In diagnostic study, sensitivity of visual assessment for absolute decrease of >2% longitudinal strain was 77%, respectively. In the viability study, the sensitivity was 82% for an absolute decrease of ≥2% longitudinal strain. Conclusions: There is good association between strain analysis value and visually assessed wall motion contractility.

Impact of Rheumatic Process in Left and Right Ventricular Function in Patients with Mitral Regurgitation.

Background: Mitral regurgitation (MR) burdens the left and right ventricles with a volume or pressure overload that leads to a series of compensatory adaptations that eventually lead to ventricular dysfunction, and it is well known that in rheumatic heart disease (RHD) that the inflammatory process not only occurs in the valve but also involves the myocardial and pericardial layers. However, whether the inflammatory process in rheumatic MR is associated with ventricular function besides hemodynamic changes is not yet established. Purpose: Evaluate whether rheumatic etiology is associated with ventricular dysfunction in patients with chronic MR. Methods: The study population comprised patients aged 18 years or older included in the registry who had echocardiography performed at the National Cardiovascular Center Harapan Kita in Indonesia during the study period with isolated primary MR due to rheumatic etiology and degenerative process with at least moderate regurgitation. Results: The current study included 1,130 patients with significant isolated degenerative MR and 276 patients with rheumatic MR. Patients with rheumatic MR were younger and had a higher prevalence of atrial fibrillation and pulmonary hypertension, worse left ventricle (LV) ejection fraction and tricuspid annular plane systolic excursion (TAPSE) value, and larger left atrium (LA) dimension compared to patients with degenerative mitral regurgitation (MR). Gender, age, LV end-systolic diameter, rheumatic etiology, and TAPSE were independently associated with more impaired LV ejection fraction. Whereas low LV ejection fraction, LV end-systolic diameter, and tricuspid peak velocity (TR) peak velocity >3.4 m/s were independently associated with more reduced right ventricle (RV) systolic function (Table 3). Conclusions: Rheumatic etiology was independently associated with more impaired left ventricular function; however, rheumatic etiology was not associated with reduced right ventricular systolic function in a patient with significant chronic MR.

A Novel Peptide Elabela is Associated with Hypertension-Related Subclinical Atherosclerosis.

INTRODUCTION: Elabela is a newly identified peptide which, alongside apelin, acts as an endogenous ligand that activates the angiotensin receptor-like 1 receptor. Previous studies have shown the association of elabela with hypertension, but information about the role of elabela in hypertension-related subclinical atherosclerosis is scarce. AIM: We aimed to determine the elabela levels in hypertensive patients and explore its association with subclinical atherosclerosis. METHODS: A total of 104 subjects with hypertension were included in the study. Elabela levels were measured using an enzyme-linked immunosorbent assay, by first extracting the peptide following the manufacturer's instructions. Subclinical atherosclerosis was assessed by measuring the carotid intima-media thickness (IMT) using ultrasound. RESULTS: Compared to stage 1, elabela levels decreased in stage 2 hypertension (0.23 [0.13, 0.45] ng/ml vs. 0.14 [0.09, 0.23] ng/ml; P = 0.000), and in the group with increased carotid IMT compared to normal IMT (0.24 [0.13, 0.38] ng/ml vs. 0.15 [0.10, 0.23] ng/ml; P = 0.005). Additionally, a linear correlation analysis showed that elabela had a significant negative correlation with systolic blood pressure (r = - 0.340, P = 0.000) and carotid IMT (r = - 0.213; P = 0.030). In multivariate analysis, lower elabela levels were associated with a higher cardiovascular risk group in this study (OR 5.0, 95% CI 1.8-13.5, P < 0.001). CONCLUSIONS: This study demonstrated for the first time that circulating elabela declined in a higher stage of hypertension and hypertensive patients with increased carotid IMT, implicating that elabela may be involved in the pathogenesis of hypertension-associated subclinical atherosclerosis.