Pulmonary Regurgitation Research Articles (Page 1)

Background: Cardiovascular magnetic resonance imaging (CMR) can estimate diffuse myocardial fibrosis using extracellular volume fraction (ECV) quantification. Objectives: This study aimed to explore associations between and prognostic value of myocardial and hepatic fibrosis after tetralogy of Fallot repair (rTOF). Methods: Adults with rTOF were prospectively enrolled along with healthy controls. Patients were categorized by hemodynamic load (pulmonary stenosis and/or pulmonary regurgitation). Available clinical data were recorded at study entry. Myocardial and hepatic ECV were calculated from pre/post-contrast T1 maps. The primary outcome was a composite of death, resuscitated sudden death, sustained ventricular tachycardia (VT) or VT requiring invasive therapy, and/or heart failure requiring hospital admission. Multivariable modelling was used to examine the association between CMR measures and adverse cardiovascular events. Results: We studied n=181 patients (56% male; median age 33 years [IQR 26–47]) and n=20 age/sex-matched controls. Hepatic and myocardial fibrosis measures were correlated (r=0.031, p<0.001). Diffuse hepatic fibrosis was higher in rTOF patients as compared with controls (586±69 versus 531±75 msec, p=0.006) with statistically significant differences between hemodynamic load type (p=0.04). Right ventricular (RV) native T1 in rTOF was increased as compared with controls (1081±76 versus 997±743 msec, p<0.001) with a trend towards significant differences when stratified by hemodynamic load (p=0.09). Late gadolinium enhancement (LGE) was seen in the RV in 46% (n=83) and in the left ventricle (LV) in 37% (n=66). At median follow-up 2.1 years (IQR 0.7-2.7), the composite outcome occurred in 9% (n=17). Independent predictors of adverse cardiovascular events on multivariable models, adjusted for age and sex, were RV end-systolic volume (HR 1.43, 95%CI 1.09-1.99, p=0.02), RV native T1 (HR 1.15, 95%CI 1.07-1.25, p<0.01) and LV LGE (HR 3.15, 95%CI 1.06-10.13, p<0.04); hepatic fibrosis was not associated with outcome. Conclusion: In rTOF, myocardial and hepatic fibrosis were modestly correlated. As compared with controls, myocardial and hepatic fibrosis was increased in rTOF. Diffuse myocardial fibrosis in the RV (native T1) and replacement fibrosis in the LV (LGE) were independently associated with cardiovascular outcomes, although hepatic fibrosis was not. These findings should be validated in larger populations with longer follow-up.

Introduction: Pulmonary regurgitation and ensuing volume load in repaired tetralogy of Fallot (rTOF) leads to remodeling of the right ventricle (RV) and which has been associated with adverse long-term outcomes. Pulmonary valve replacement (PVR) is associated with variable degrees of RV reverse remodeling. There is evidence that earlier age at PVR is associated with improved clinical outcomes. Research Question / Hypothesis: The aim was to determine whether age at PVR was associated with the degree of RV reverse remodeling in rTOF. We hypothesized that younger age at PVR would enable a greater degree of RV reverse remodeling. Materials and Methods: Retrospective single-center cohort study of rTOF patients from the SCOUT-TOF registry (Single Center Outcomes study using CMR – Tetralogy of Fallot) who underwent PVR for an indication of RV dilation and had cardiac magnetic resonance imaging (CMR) performed before and after PVR. Multivariable linear regression assessed the association of age at PVR with percent change of indexed RV end-diastolic volume (RVEDVi) and end-systolic volume (RVESVi) from pre-to post-PVR CMR, adjusted for time since PVR, surgical PVR (vs. transcatheter), sex, and presence of a pulmonary artery stent prior to PVR. For patients with multiple post-PVR CMR studies, the study with the lowest RVEDVi and RVESVi was used. Results: In total, 105 patients with rTOF were included, 71 (68%) were male. PVR dates ranged from 2005 to 2020. PVR was performed at median age 20 years (IQR 16, 28), with CMR 3.8 years (IQR 1.7, 5.7) post-PVR, with median age at CMR of 30.6 years (IQR). RVEDVi percent change from pre- to post-PVR measurement was -37% (IQR -46, -27) and RVESVi percent change was -38% (IQR -48, -21). There was no association between age at PVR and percent change in RVEDVi with an estimated mean percent change 0.09% (95% CI -0.28,0.47, p = 0.62) nor between age at PVR and percent change in RVESVi on adjusted models. Conclusion: In this retrospective study we did not observe an age advantage to volume-based RV reverse remodeling after PVR. Further study to determine whether timing of PVR impacts clinical outcomes and the role of reverse RV remodeling on outcomes is warranted.

Background: Advancements in congenital heart disease management have led to an increasing adult population with repaired Tetralogy of Fallot (rTOF). Serial imaging of Right Ventricle (RV) size and function have been proposed to guide decisions regarding pulmonary valve replacement (PVR). However, longitudinal data on RV volume changes in this cohort remain sparse. Thus, the optimal timing of surveillance cardiac MRI (CMR) studies in this population remains unknown. Objective: To evaluate serial changes in right ventricular end-diastolic volume index (RVEDVi) in patients with repaired TOF and identify factors contributing to progressive RV dilation over time. Methods: We performed a single-center, retrospective cohort study of 45 patients with rTOF who underwent at least two CMR between 2005 and 2023 at Montefiore Medical Center. All RV volumetric measurements were reassessed by a single observer for consistency. Change in RVEDVi was analyzed, and the associations between RV dilation, pulmonary regurgitation (PR), tricuspid regurgitation (TR), right ventricular ejection fraction (RVEF) and surgical approach were assessed. Results: The cohort included 45 patients (median age at surgery: 13 months, interquartile range [IQR]: 7–49). Of these, 78% had TOF with pulmonary stenosis, and 67% underwent transannular patch repair (Table 1). Serial CMRs revealed considerable variability in RVEDVi, with a general trend toward RV dilation (Figure 1a). Notably, 29% (95% confidence interval [CI] : 17%, 45%) demonstrated a >15% increase in RVEDVi (Figure 1b), with a median time to this change of 29.3 years from surgery (Figure 2) or 5.5 years between two CMR assessments. Of the patients with significant RV dilation, 12 were female. Interestingly, neither the type of surgery, PR fraction, RVEF nor residual outflow obstruction were significantly associated with the degree of RV dilation. Conclusion: Longitudinal CMR analysis of rTOF patients reveals heterogeneous RV remodeling with only 29% of patients showing clinically significant progression of RV dilation over 5.5 years from first CMR assessment. These findings suggest that while RV progression occurs, the majority of patients will have no clinically significant change in RVEDVi over 5.5 years’ time. Robustly assessing change over time, identifying specific risk factors for RV dilation and refining surveillance intervals through multicenter studies could enable more personalized MRI monitoring strategies.

Objective: Patients with congenital heart disease complicated by right ventricular outflow tract obstruction (RVOTO) often develop secondary pulmonary regurgitation (PR) after corrective surgery, leading to progressive right ventricular (RV) dysfunction that necessitates pulmonary valve replacement (PVR). This study employed proximity barcoding assay (PBA) to dynamically monitor changes in plasma exosome protein profiles and subpopulation characteristics during the perioperative period of transcatheter pulmonary valve replacement (TPVR). Methods: This study collected 120 human plasma samples from 10 patients undergoing TPVR at three time points, preoperative (T1 group), 1 day postoperative (T2 group), and 1 week postoperative (T3 group), as well as 10 healthy individuals (N group). Single-exosome analysis was performed using PBA. We performed combinatorial analysis and functional analysis of membrane proteins on single exosomes across different groups and evaluated their predictive performance as biomarkers using Receiver Operating Characteristic (ROC) curves. Finally, cluster analysis was performed on exosome subpopulations to identify subpopulation markers. Results: Compared to the control group, the disease group exhibited significantly reduced expression levels of the protein combination DSCAML1 + ALCAM + ITGA1 + CR1 (P<0.05; AUC=1), while showing significantly elevated expression levels of BCAM + CD36 + DSCAML1 + ITGB1 (P<0.05; AUC>0.82). Gene Ontology (GO) analysis indicated that the proteins are associated with pathways related to response to stress, membrane side, and protein binding, while Kyoto Encyclopedia of Genes and Genomes (KEGG) analysis revealed enrichment in the Hematopoietic cell lineage pathway. Subpopulation analysis revealed increased Cluster 1 and decreased Cluster 2 proportions in T1-T3 groups compared to the N group. Subpopulation markers showed CD8A dominance in Cluster 1, while DSCAML1 predominated in Cluster 2. Conclusion: This study was the first to implement PBA for the dynamic assessment of plasma exosome alterations in patients with TPVR. We identified downregulated protein combinations such as DSCAML1 + ALCAM + ITGA1 + CR1 and upregulated combinations such as BCAM + CD36 + DSCAML1 + ITGB1. Analysis of exosome subpopulations further demonstrated a predominance of CD8A protein expression in the disease group, whereas, in the control group, DSCAML1 expression was more prevalent.

Introduction: Complex congenital heart disease (CHD) patients with atrial arrhythmias have higher rates of morbidity and mortality compared to less complex CHD patients. This study aims to identify echocardiographic predictors of thromboembolism (TE) and major bleeding in patients with Tetralogy of Fallot (ToF) or Pulmonary Atresia with Intact Ventricular Septum (PA-IVS) and atrial fibrillation or flutter (AF). Methods: This cohort study included patients with ToF or PA-IVS who had a diagnosis of AF who underwent a TTE across three sites. Outcomes were incidence rate of major bleeding or TE, calculated using univariable and multivariable cox regression with interaction for anticoagulant choice and fractional area change (FAC). The HAS-BLED score was augmented with major predictors of bleeding, and receiver operator curves compared. Results: We included 300 patients (287 ToF [95.7%], 13 PA-IVS [4.3%]), mean age 45.6 years. Most received warfarin (227 [79.4%]), 40 (13%) used DOACs, and 5 (1.6%) used enoxaparin. TTE predictors of TE included large LVOT diameter ≥3cm (HR 8.92, p=0.002), small LVOT VTI (p=0.014), FAC <30% (HR 7.08, p=0.026) and moderate RV dysfunction (HR 10.75, p=0.026). Predictors of major bleeding included moderate tricuspid regurgitation (HR 2.40, p=0.022), right atrial pressure (1.12, p<0.001), severe RA enlargement (HR 2.30, p=0.048), RV mid diameter (HR 1.06, p=0.005), RV end diastolic area (HR 1.04, p=0.021), RV end systolic area (HR 1.05, p=0.016) elevated RVSP and FAC ≤45% (HR 0.37, p=0.016). Among warfarin users, impaired RV function (FAC ≤45%) was linked to reduced major bleeding events (HR 0.27, p=0.025). There was a nonsignificant trend towards higher bleeding in DOAC users with FAC >45% (HR 3.87, p=0.284), but significantly higher bleeding in DOAC users with FAC ≤45% (HR 12.41, p=0.008) vs. warfarin users with normal RV function (table 1). We developed an augmented HAS-BLED score: the standard score plus one point for moderate-to-severe pulmonic or tricuspid regurgitation and one for FAC ≤45%. This outperformed the standard HAS-BLED in predicting major bleeding (AUC 0.67 augmented vs. 0.57 un-augmented, p=0.008) (figure 1). Conclusion: Echocardiographic evidence of right heart disease is a strong predictor of major bleeding in patients with ToF or PA-IVS. FAC is a useful tool to determine excess major bleeding risk, particularly in DOAC users. Augmenting HAS-BLED with right heart parameters improves diagnostic accuracy.

Introduction: Carcinoid cancer is a rare neuroendocrine tumor (NET) secreting vasoactive substances. Case Presentation: A 66-year-old woman presented to cardiology clinic for evaluation of dyspnea with exertion and leg swelling. Comorbidities included chronic kidney disease and a history of jejunal neuroendocrine tumor (NET) with prior resection and recent recurrence complicated by peritoneal carcinomatosis. A transthoracic echocardiogram was obtained at time of recurrence (1 year prior to presentation) and showed mild mitral regurgitation (MR), mild bi-atrial enlargement, moderate tricuspid regurgitation (TR), and mild aortic regurgitation (AR) with preserved ejection fraction. In clinic, physical exam showed jugular venous distention with prominent V waves and a systolic and diastolic murmur. Transthoracic echocardiogram (TTE) demonstrated severe TR, moderate to severe AR, moderate MR, severe pulmonary valve regurgitation, and a right to left shunt with enlarged right ventricle and reduced function. Patient was diagnosed with left and right carcinoid heart disease involving all 4 cardiac valves in the setting of a patent foramen ovale (PFO). After multidisciplinary discussion, surgical intervention was recommended. She underwent successful cardiopulmonary bypass with mitral valve replacement, aortic valve, pulmonary valve replacement, and tricuspid valve replacement in addition to closure of PFO. She did well postoperatively and was stable for dismissal from the hospital after 12 days. Discussion: Rarely, carcinoid heart disease can involve the left side of the heart. Left sided involvement is typically seen in the presence of a right to left side cardiac shunt. In patients with severe valve dysfunction secondary to carcinoid heart syndrome, surgical intervention is the definitive treatment and has been found to improve quality of life and prolong survival

A 56-year-old woman presented to her primary care physician with a 1-year history of facial flushing and short-lived paroxysms of chest pain. An electrocardiogram was performed which showed normal sinus rhythm and p-mitrale. Transthoracic echocardiography (TTE) revealed normal left ventricle (LV) size and function, moderate to severe aortic regurgitation, moderate mitral regurgitation (MR), moderate tricuspid regurgitation (TR), and mild pulmonic regurgitation. An agitated saline study suggested an intrapulmonary shunt (positive after >10 beats). Cardiac magnetic resonance imaging (CMR) confirmed these findings but reclassified the TR and MR as severe and the LV as dilated. CMR also incidentally noted numerous complex hepatic masses, largest measuring 6cm. Dedicated liver MRI confirmed this, and biopsy showed strong, diffusely positive staining for neuroendocrine markers, consistent with a well-differentiated neuroendocrine tumor (carcinoid). Positron Emission Tomography/Computed Tomography (PET/CT) revealed high uptake in the pancreatic head, cecum, and right scapula in addition to known liver lesions. Treatment with octreotide was initiated after obtaining a baseline serum serotonin level 1489 ng/mL (normal 50-220 ng/mL). The patient reported mild symptoms and NT Pro BNP was elevated at 265 pg/mL (normal <125 pg/mL). Given LV dilation, severe valve disease, and likely concealed symptoms, cardiac surgery consultation was pursued. Transesophageal echocardiogram with moderate sedation was deferred due to risk of carcinoid crisis. In coordination with oncology, surgery was scheduled after initiating octreotide. Preoperative octreotide infusion was administered, and she underwent four-valve replacement with bioprostheses. Intraoperatively, all four valves had thickened, retracted leaflets; a 7 mm patent foramen ovale (PFO) was also identified and closed. Pathology of valve specimens showed extracellular plaque deposition and bland spindle cells, consistent with carcinoid valve disease. The patient had an uneventful postoperative course. Carcinoid heart disease is a rare, morbid manifestation of neuroendocrine tumors, mediated by elevated serotonin and other vasoactive substances causing fibrotic valve dysfunction. It usually affects right-sided valves, but left-sided involvement may occur with intracardiac shunt or pulmonary metastases. This case illustrates the multidisciplinary diagnosis and management of an exceedingly rare form of carcinoid heart disease.

Introduction: Athletic participation is increasing among individuals with congenital heart disease (CHD), but there are limited data to guide sports participation decision-making in this population. We conducted a survey to define practice variation in this area focusing on two complex populations post repair (Tetralogy of Fallot (TOF) and D-transposition of the great arteries s/p arterial switch (D-TGA)). Methods: An online survey was distributed by investigators to clinicians treating patients with CHD. Respondents were queried regarding their approach to specific complex congenital patient scenarios and sports participation. Results: A total of 165 cardiologists completed the survey, most (147/165, 89%) in academic practice. Respondents included 95/165 (58%) pediatric cardiologists, 59/165 (36%) adult congenital heart disease (ACHD) specialists, and 11/165 (7%) other subspecialties. TOF: Nearly all respondents (156/165, 95%) reported supporting full sport participation in individuals with well-repaired TOF (without residual lesion or cardiac dysfunction). With severe pulmonic regurgitation, support for full participation remained high (139/165, 84%), but 3/165 (2%) recommended complete restriction and 23/165 (14%) restriction based on sport type (primarily from high static and high dynamic sports, 13/23, 57%). With moderate right ventricular outflow tract obstruction, by contrast, 92/164 (56%) supported full participation and 10/164 (6%) complete restriction, with the remaining 62/164 (38%) recommending sport-specific guidance. D-TGA: A similar majority (154/163, 95%) supported full participation for athletes with repaired D-TGA, but this number was considerably lower when with neo-aortic root dilation (4.0 cm) was added to the patient scenario, with only 50/163 (31%) recommending full participation and a majority (104/163, 64%) recommending sport-specific restriction. Notably, only 52/163 (31.9%) supported participation in contact sports in this specific scenario, although willingness to do so was higher (26/59, 44%) among ACHD specialists compared to pediatric cardiologists (23/95, 25%) (p=0.02). Conclusion: While the vast majority of providers support full sports participation in repaired TOF and D-TGA without residual issues, there is heterogeneity in guidance regarding sports participation in those with post repair issues. This marks an important area for future quality initiatives to ensure alignment with expert guidelines.

Background: Percutaneous treatment of pulmonary valve disease is a safe and minimally invasive alternative with favorable mid-term outcomes, especially in congenital heart disease. However, feasibility depends on right ventricular outflow tract (RVOT) anatomy, with dilation being the main limitation. Infective endocarditis (IE) of the native pulmonary valve (PV) without predisposing factors is extremely rare. We present a case of severe pulmonary regurgitation secondary to prior native PV endocarditis, treated with two percutaneous valves implanted at the origin of the pulmonary artery branches. Case Description: A 57-year-old male, hypertensive and former smoker, with a history of native PV IE in 2015 (no predisposing factors), treated with antibiotics and good clinical outcome, presented in May 2024 with NYHA class II dyspnea and lower limb edema. Echocardiography showed right ventricular (RV) dilation (basal: 61 mm; mid: 58 mm), TAPSE 29 mm, thickened and partially calcified PV with severe regurgitation (PHT 90 ms; diastolic reflux in branches), and no pulmonary hypertension. Cardiac MRI confirmed severe RV dilation (EDV: 387 ml; iEDV: 150 ml/m 2 ) (Image 1), RV ejection fraction 30%, severe pulmonary regurgitation (regurgitant fraction 63%), and dilation of the pulmonary artery trunk and branches. Given symptomatic severe RV dysfunction and high surgical risk, a percutaneous approach was selected. Cardiac CT revealed a dilated RVOT (minimum diameter 35.4 x 37.7 mm; area 10.5 cm 2 ), trunk (43.8 x 45.5 mm; area 15.1 cm 2 ), right branch (28.6 x 29.7 mm; area 6.67 cm 2 ), and left branch (27.6 x 30.2 mm; area 6.55 cm 2 ). Due to unsuitable anatomy for RVOT or main trunk valve placement, two Myval N 32 valves were implanted percutaneously in each pulmonary artery branch (Image 2). The procedure was successful and uneventful. The patient improved clinically; follow-up echocardiogram showed well-functioning prostheses, minimal paravalvular regurgitation, and reduced RV dimensions (basal: 56 mm; mid: 47 mm). Discussion: This is a rare case of severe pulmonary regurgitation due to native PV endocarditis without predisposing factors, successfully treated with bilateral percutaneous valve implantation in the pulmonary artery branches. This novel strategy offers a viable option in cases with unfavorable RVOT anatomy and emphasizes the importance of tailored interventions in complex structural heart disease.

Left ventricular non-compaction cardiomyopathy (LVNC) is an infrequent cardiac disorder characterized by prominent trabeculations, deep intertrabecular recesses, and thinning of the compacted myocardial layer, predisposing to heart failure, arrhythmias, and thromboembolic complications. The relationship between myocardial inflammation and chordae tendineae rupture is clinically relevant, as prior inflammatory processes may compromise valvular structures, leading to acute mitral regurgitation and ventricular overload. We present the case of a 45-year-old male with a history of severe mitral regurgitation and heart failure with preserved ejection fraction, who presented with progressive dyspnea, oppressive chest pain, diaphoresis, and lower extremity edema. Clinical assessment revealed arterial hypertension, tachycardia, hypoxemia, a loud systolic mitral murmur, jugular venous distension, and peripheral edema. Complementary studies demonstrated LVNC, severe prolapse of the posterior mitral leaflet with chordae tendineae rupture, acute mitral regurgitation, and pulmonary hypertension. Thoracic computed tomography revealed chronic lesions compatible with a previous viral infection, although specific SARS-CoV-2 testing results were unavailable. The patient underwent mitral valve replacement with a mechanical prosthesis, with postoperative complications including acute pulmonary edema, pleural effusion, renal insufficiency, and left ventricular dysfunction, which resolved favorably during hospitalization. This case highlights the importance of early recognition of LVNC and its valvular complications, as well as a comprehensive approach and timely surgical intervention to optimize clinical outcomes in complex cardiovascular scenarios.

Tetralogy of Fallot (TOF) is one of the most common cyanotic congenital heart malformations, characterized by four pathological features: right ventricular outflow tract obstruction/pulmonic stenosis, a ventricular septal defect, an overriding aorta, and right ventricular hypertrophy. It was initially partially defined by Nicholas Steno in the 17th century and completely described by Étienne-Louis Arthur Fallot and Maude Abbott in the 19th and 20th centuries. The advances in multimodality imaging and innovative surgical and transcatheter techniques have led to advances in the management of TOF. While initial management in the mid-20th century favored palliative procedures in infancy followed by complete surgical repair, data now support an early complete surgical repair in infancy. The major post-repair complication is the development of significant pulmonary regurgitation, necessitating either surgical or transcatheter valve replacement. Multimodality imaging is essential to the initial identification of TOF, preoperative planning, and post-procedural complication assessment. In this review, we provide a historical perspective of the discovery and clinical management of TOF from the 1600s to the present day, as well as the role of multimodality imaging in TOF management.

BACKGROUND Isolated pulmonary regurgitation (PR) without right heart remodeling or pulmonary hypertension is a rare clinical entity, poorly characterized in the literature. Typically associated with conditions like pulmonary hypertension or congenital heart disease, moderate PR in their absence poses diagnostic and management challenges. This case report describes a rare presentation of isolated moderate PR, highlighting the clinical approach, diagnostic evaluation, and importance of long-term monitoring. CASE REPORT A 71-year-old woman with a history of paroxysmal supraventricular tachycardia, multiple myeloma, hypertension, and dyslipidemia presented with palpitations and a syncopal episode. Echocardiography revealed moderate pulmonary regurgitation, mild tricuspid regurgitation, normal right ventricular size and function, and a normal pulmonary artery diameter. No acute intervention was required, and management focused on arrhythmia control and blood pressure optimization. Follow-up included serial echocardiography planned at 6-month intervals to monitor pulmonary regurgitation progression and right ventricular function. CONCLUSIONS This case underscores the rarity of isolated moderate PR without right heart remodeling and the need for careful evaluation using advanced imaging, such as echocardiography and potential cardiac MRI. Long-term monitoring is critical to detect progression to RV dysfunction, which may necessitate interventions like pulmonary valve replacement. Clinicians should consider primary valvular degeneration or arrhythmia-related strain as potential etiologies, with follow-up guided by symptom onset and imaging findings. This case contributes to the limited literature on isolated PR.

Disclosure: K. Thammakosol: None. P. Vathesatogkit: None. S. Leelasithorn: None. C. Sriphrapradang: None.Background: Neuroendocrine neoplasms (NENs) typically arise in the midgut, lungs, or pancreas, while primary ovarian NENs are very rare. Unlike gastrointestinal NENs, ovarian NENs can uniquely present with carcinoid syndrome and carcinoid heart disease in the absence of liver metastases, making them a distinct clinical entity. Case Presentation: We report the case of a 70-year-old woman with a history of coronary artery disease, controlled hypertension, and hypercholesterolemia who presented with a 3-month history of exertional dyspnea, bilateral leg edema, diarrhea, and facial flushing. Examination revealed a plethoric face, jugular venous engorgement, a pansystolic murmur over the tricuspid valve, a diastolic blowing murmur over the pulmonic valve, bilateral leg edema, and a palpable 10 cm mass in the left lower abdomen. Laboratory investigations showed significantly elevated 24-hour urinary 5-hydroxyindoleacetic acid (5-HIAA; 106.0 mg/day, normal: 2-9 mg/day) and serum chromogranin A (468.6 ng/mL, normal: 0-76.3 ng/mL). Transthoracic echocardiography demonstrated thickening and restricted motion of the tricuspid valve and pulmonic valve leaflets, causing severe non-coaptation with significant tricuspid regurgitation and severe pulmonic regurgitation with relative pulmonic stenosis. Abdominal computed tomography revealed a 7.8 x 11.8 x 9.0 cm heterogeneous enhancing left adnexal mass, which was DOTATE-avid on Gallium-68 DOTATE PET/CT, confirming a primary ovarian NEN. The patient was diagnosed with a primary ovarian NEN complicated by carcinoid syndrome and carcinoid heart disease. Preoperative management included long-acting somatostatin analog therapy (octreotide long-acting release 30 mg intramuscularly every 4 weeks), niacin supplementation, and management of right-sided heart failure. Two months later, she underwent a successful hysterectomy with bilateral salpingo-oophorectomy for complete tumor resection. Pathology confirmed an insular-type ovarian NEN with a Ki-67 index < 1%, and no evidence of ovarian surface involvement, lymphovascular invasion, or lymph node metastasis. Postoperatively, the patient experienced significant symptom resolution, including improvement in right-sided heart failure, diarrhea, and flushing, with normalization of 24-hour urine 5-HIAA (3.3 mg) and serum chromogranin A (18.3 ng/mL). Conclusions: This case highlights the importance of recognizing ovarian NEN as a rare but significant cause of carcinoid syndrome and carcinoid heart disease, even in the absence of liver metastases. It also underscores the critical role of multidisciplinary management in achieving optimal outcomes.Presentation: Sunday, July 13, 2025

Disclosure: K. Komisarenko: None.Background: T2DM is a multifactorial metabolic disease with significant genetic contributions. Polymorphisms in folate metabolism genes, particularly MTHFR C677T and A1298C, are associated with impaired homocysteine clearance, endothelial dysfunction, and neurotransmitter imbalance. Post-COVID metabolic stress may amplify these effects, especially in individuals with T2DM, but the combined influence of clinical and genetic factors remains poorly defined. Objective: To evaluate the association of MTHFR C677T and A1298C polymorphisms with plasma levels of serotonin, ST-2, and homocysteine in post-COVID T2DM and to develop multivariate regression models for predicting biomarker variations based on genetic and clinical profiles. Methods: This study included 250 adults with previously diagnosed T2DM who had recovered from confirmed SARS-CoV-2 infection. Demographic and clinical data, including BMI, hypertension status, and COVID-19 treatment modalities, were collected. MTHFR genotyping was performed using PCR-RFLP. Plasma levels of homocysteine were quantified via LC-MS/MS, while serotonin and ST-2 were measured using ELISA. Predictive models were constructed through multiple linear regression. Statistical significance was set at p < 0.05. Results: Patients carrying the 677T or 1298C alleles had significantly higher homocysteine (+3 µmol/L; p < 0.01) and lower serotonin levels (p < 0.05) than wild-type individuals. Elevated BMI and hypertension were additional predictors of unfavorable biomarker changes. Age was negatively associated with ST-2 levels, while a history of pulmonary insufficiency predicted increased ST-2 (p < 0.05). Dexamethasone treatment during COVID-19 was linked to increased serotonin, and oxygen therapy to reduced ST-2. All models were statistically significant (ANOVA p < 0.001) with strong explanatory power (R² = 0.87-0.91). Conclusions:MTHFR gene variants, in conjunction with clinical variables such as BMI and hypertension, influence key biochemical markers in post-COVID T2DM. Integrating genetic and metabolic profiling may inform individualized strategies for folate supplementation and cardiometabolic control. These findings support the practical implementation of genetic screening and biomarker monitoring in post-COVID endocrine care to reduce vascular, neuropsychiatric, and inflammatory complications in high-risk diabetic patients.Presentation: Saturday, July 12, 2025

Takayasu arteritis (TA) is a rare systemic inflammatory disease affecting medium and large arteries, mainly in young Asian women, with a frequency of 1-3 cases per million inhabitants. Diagnosing this disease is challenging due to nonspecific early symptoms, leading to gradual clinical progression until vascular complications appear. A 21-year-old pregnant woman, experiencing progressive mild fatigue, referred for clinical examination. Over the past 3-4 years, she had reported weakness, headache, sweating, and dizziness. Imaging revealed significant vascular damage, including an ascending aortic aneurysm, aortic valve annuloaortic ectasia, aortic, mitral, tricuspid, and pulmonary regurgitation, as well as dilation of the left atrium and left ventricle. While available treatments alleviated symptoms and slowed the progression, late diagnosis resulted in lifelong illness. The diagnostic challenges associated with nonspecific early symptoms and the risk of serious vascular complications underscore the importance of early detection of TA in young women with symptoms of systemic inflammation. Women with TA are more likely to have an unfavourable pregnancy outcomes than the general population. Therefore, such patients need careful monitoring and a collaborative approach to achieve the best possible delivery results. This report aims to compile information on pregnancy in women with TA and how the disease impacts pregnancy progression.

In the past few decades, long-term basic research and clinical practice of surgical treatment for tetralogy of Fallot have been carried out at home and abroad, and the treatment effect has been improved, the complications and mortality have been gradually decreased. Pulmonary regurgitation is a common complication after traditional radical repair of tetralogy of Fallot, which is an important factor leading to many adverse outcomes. Valve-sparing repair has gradually become the first choice for the treatment of tetralogy of Fallot, which can effectively prevent the occurrence of postoperative pulmonary regurgitation, maintain right ventricular function, and improve the prognosis of children. However, there are controversies about the application indications and surgical strategies of valve-sparing repair, and a lack of clear clinical guidelines. This article reviews the research progress on the advantages, surgical indications, surgical techniques, prognosis and prospects of valve-sparing repair for tetralogy of Fallot, in order to provide evidence-based medical evidence for when to undergo valve-sparing repair for tetralogy of Fallot patients and to better improve the quality of life of patients.

Slim et al.'s paper provided an insight into the differences between repaired tetralogy of Fallot and isolated pulmonary regurgitation in their strain. Repaired tetralogy of Fallot had higher right ventricular circumferential strain, while isolated pulmonary regurgitation relied on longitudinal strain more. This allowed the authors to infer that repaired tetralogy of Fallot can withstand more chronic regurgitation before valve replacement is necessary. We highlighted new findings relevant to this paper. Arrhythmia in repaired tetralogy of Fallot is associated with a reduced global circumferential strain of the right ventricle. Specifically, a value of below -14% was associated with a 6.3 times increase in the risk for an arrhythmic event. We believe this would be beneficial for patients when considered for valve replacements, suggesting modification of current valve replacement guidelines to include strain thresholds alongside current volumetric thresholds.However, the data for isolated pulmonary regurgitation remains scarce. Further investigation is needed to provide clearer timelines for valve replacement. We emphasised the importance of exploring the underlying architecture of repaired tetralogy of Fallot patients' hearts and why they could generate more global circumferential strain. We acknowledged the broader effect of this paper and its specific benefit in our country, Egypt. This paper provided insights useful for broader global health impact, especially in low-income countries.

Recent introduction into clinical practice of large self-expanding valves addressees the issue of dilated dysfunctional right ventricular outflow tracts (RVOTs). The purpose of this study was to determine the safety and short-term efficacy of the VenusP-valve (Venus MedTech) implantation in the pulmonary position in the pediatric group of patients with emphasis on the evolving qualification criteria and implantation techniques. Over a 14-month period, 15 patients < 18years-old with severe pulmonary regurgitation underwent successful PPVI with the self-expanding VenusP-valve. All types of RVOT anatomies were represented in the study group. Desired valve position during the first attempt was achieved in 13 patients (87%). When compared between the first and second half of the study period, there was a significant difference toward less oversizing: 3.3 ± 1.4mm versus 1.8 ± 0.7mm, p = 0.039. On final angiography, trivial pulmonary regurgitation was present in 9 patients (60%). In the remaining 6 patients (40%), the valve was competent. Adverse events were limited to two patients (13.3%) with transient rhythm disturbances without any clinical sequelae. During a median follow-up of 11.5months (range 7.9-21months), all the valves remained normally functioning with no need for reinterventions and no episodes of infective endocarditis. The VenusP-valve implantation was safely and effectively performed in pediatric patients with all types of large dysfunctional RVOTs. With modified techniques of implantation, limited femoral vein access or stented pulmonary artery branches, the valves can be successfully implanted.

Abstract Background Assessment of the right ventricle (RV) using cardiac magnetic resonance (CMR) imaging is essential in children after tetralogy of Fallot (TOF) repair for the early detection of complications. We aimed to evaluate the effectiveness of transthoracic echocardiography (TTE) in assessing RV volume in post-TOF repair children. Methods A cross-sectional study was conducted on 36 post-TOF repair children. All participants underwent history taking and clinical examination. TTE was used to assess the RV end-diastolic volume index (RVEDVI), RV end-systolic volume index (RVESVI), RV ejection fraction (RVEF), and pulmonary regurgitation index (PRI). TTE results were compared to those of CMR imaging. Results Participants had a mean age of 10.1 ± 3.7 years and a male-to-female ratio of 2.3:1. The echocardiographic right ventricle’s end-diastolic volume (RVEDV) (146.5 ± 44.8 vs. 144.8 ± 45.1; P: 0.9), end-systolic volume (RVESV) (71.1 ± 24.8 vs. 63.3 ± 26.2; P: 0.2), RVEDVI (137.7 ± 29.9 vs. 138.6 ± 28.9; P: 0.9), and RVESVI (67 ± 17.9 vs. 58.8 ± 18.3; P: 0.1) were comparable to CMR’s, while the RVEF was significantly lower than CMR’s (51.5 ± 6.8 vs. 56.7 ± 8.8, P: 0.01). The RVESVI (r: 0.8; P: 0.000), RVEDVI (r = 0.9; P = 0.000), and RVEF (r = 0.5; P = 0.001) correlated positively between echocardiographic and CMR imaging. Kappa agreement testing showed a significant association between echocardiographic measurements and those of CMR. Three equations were successfully postulated based on a linear regression model to predict CMR parameters in comparable post-TOF repair children, i.e., RVEDVI, RVESVI, and PRF. Conclusion Conventional echocardiography effectively assessed RV functions and volumes and can predict the indications for CMR in post-TOF repair children.

In recent years, late pulmonary valve regurgitation after repair of Tetralogy of Fallot has gradually attracted the attention of scholars at home and abroad. Early pulmonary valve regurgitation may be asymptomatic and even survive for a long time without symptoms. However, long-term pulmonary valve regurgitation and chronic right ventricular volume overload may lead to decreased exercise tolerance, decreased right ventricular ejection fraction, arrhythmia, and sudden death. How to provide standardised, scientific, and individualised treatment strategies and life-cycle health services for repaired Tetralogy of Fallot patients with long-term pulmonary valve regurgitation has become a challenge for CHD surgeons around the world. This review summarises the pathophysiological mechanism of pulmonary valve regurgitation after repaired Tetralogy of Fallot, the preoperative diagnosis and evaluation of the severity of pulmonary valve regurgitation, the prevention of initial repair of Tetralogy of Fallot before pulmonary valve regurgitation, the determination of the intervention time of severe pulmonary valve regurgitation, the selection of the optimal intervention strategy, and the prospect of the future general clinical application of tissue engineering valved conduits, which are helpful to improve the long-term prognosis and quality of life of patients with repaired Tetralogy of Fallot.