Search Results (228)

Background/Objectives: In rhegmatogenous retinal detachment (RRD), vitreous cortex remnants (VCRs) may contribute to the development and progression of proliferative vitreoretinopathy (PVR). This study aimed to evaluate potential toxicity and trauma secondary to VCRs visualization and removal during pars plana vitrectomy (PPV) for RRD. Methods: Prospective study on patients with primary RRD who underwent PPV. Imaging assessment included widefield OCT (WF-OCT), ultra-WF retinography and fundus autofluorescence (FAF). During PPV, a filtered and diluted triamcinolone acetonide (TA) solution (20 mg/mL) was used to evaluate the presence and extension of VCRs, removed using an extendible diamond-dusted sweeper (EDDS). After six months, retinal and retinal pigment epithelium toxicity and retinal trauma due to VCRs removal were investigated. Results: Retinal reattachment was achieved in 21/21 cases included in the study. No signs of retinal or RPE toxicity were detected and WF-OCT performed in the areas of VCRs removal revealed an intact inner retinal architecture in the majority of eyes, with minor and localized inner retinal indentations in 4 cases. Conclusions: VCRs visualization and removal using TA and EDDS appears to be safe, with no retinal toxicity and very limited and circumscribed mechanical trauma. This approach may contribute to reducing the risk of postoperative PVR. Full article

Background/Objectives: Sotatercept has demonstrated efficacy in pulmonary arterial hypertension (PAH), but its use has not been studied in patients with Group 3 pulmonary hypertension (PH). Additionally, patients with connective tissue disease-associated PAH (CTD-PAH) were underrepresented in the STELLAR trial. Given the limited treatment options for pulmonary hypertension in patients with interstitial lung disease (PH-ILD), this study aimed to evaluate the use of sotatercept in CTD-PAH patients with concomitant ILD. Methods: Eligible patients (n = 7) had a confirmed diagnosis of CTD-PAH with concomitant ILD. The patients were already receiving background PAH therapy. Baseline hemodynamic and clinical measurements were reassessed after 24 weeks of sotatercept therapy. The variables assessed included six-minute walk distance (6MWD), pulmonary vascular resistance (PVR), echocardiographic right ventricular systolic pressure (eRVSP), N-terminal pro-brain natriuretic peptide (NT-proBNP) levels, World Health Organization (WHO) functional class, and supplemental oxygen requirements. Results: The study included seven patients with a mean age of 57 years (range: 39–73 years). After 24 weeks, the mean 6MWT distance increased from 211 m to 348 m (p < 0.01). Mean PVR decreased from 7.77 WU at baseline to 4.53 WU (p < 0.01). Mean eRVSP decreased from 79.43 mmHg to 54.14 mmHg (p < 0.01). NT-proBNP decreased from 3056.86 pg/mL to 1404.29 pg/mL (p < 0.01). The WHO functional class and supplemental oxygen requirements improved in all patients. Conclusions: Sotatercept was tolerated in patients with CTD-PAH and ILD, with no evidence of adverse respiratory effects. When added to foundational PAH therapy, sotatercept resulted in significant improvements across multiple parameters. These findings suggest that sotatercept may be a promising therapeutic option as an adjunctive treatment in this patient population. Full article

Pulmonary hypertension (PH) is broadly defined as a mean pulmonary arterial pressure (mPAP) exceeding 20 mm Hg at rest. Pulmonary arterial hypertension (PAH) is a specific subset of PH characterized by a normal pulmonary arterial wedge pressure (PAWP), combined with elevated mPAP and increased pulmonary vascular resistance (PVR), without other causes of pre-capillary hypertension such as lung diseases or chronic thromboembolic pulmonary hypertension. The majority of PAH cases are idiopathic; other common etiologies include connective tissue disease-associated PAH, congenital heart disease, and portopulmonary hypertension. To a lesser extent, genetic and familial forms of PAH can also occur. The pathophysiology of PAH involves the following four primary pathways: nitric oxide, endothelin-1, prostacyclin, and activin/bone morphogenetic protein (BMP). Dysregulation of these pathways leads to a progressive vasculopathy marked by vasoconstriction, vascular proliferation, elevated right heart afterload, and ultimately right-sided heart failure. Diagnosing PAH is challenging and often occurs at advanced stages. The gold standard for diagnosis remains invasive right heart catheterization. Along with invasive hemodynamic measurements, several noninvasive imaging modalities such as echocardiography and ventilation-perfusion scanning are key adjunct techniques. Also, recent advancements in cardiac magnetic resonance (CMR) have opened a new era for PAH management. Additionally, CMR and echocardiography not only enable diagnosis but also aid in evaluating disease severity and monitoring treatment responses. Current PAH treatments focus on targeting molecular pathways, reducing inflammation, and inhibiting right-sided heart failure. Integrating imaging with basic science techniques is crucial for enhanced patient diagnosis, and precision medicine is emerging as a key strategy in PAH management. Additionally, the incorporation of artificial intelligence into both molecular and imaging approaches holds significant potential. There is a growing need to integrate new imaging modalities with high resolution and reduced radiation exposure into clinical practice. In this review, we discuss the molecular pathways involved in PAH, the imaging modalities utilized for diagnosis and monitoring, and current targeted therapies. Advances in molecular understanding and imaging technologies, coupled with precision medicine, could hold promise in improving patient outcomes and revolutionizing the management of PAH patients. Full article

Inhaled treprostinil is approved for the treatment of pulmonary hypertension-associated interstitial lung disease (PH-ILD); however, it has not shown significant benefit in patients with a pulmonary vascular resistance (PVR) < 4 WU. As such, treatment for non-severe PH-ILD remains controversial. A total of 16 patients with non-severe PH-ILD were divided into two groups based on changes in PVR during exercise: a dynamic PVR group (n = 10), characterized by an increase in PVR with exertion, and a static PVR group (n = 6), with no increase in PVR with exercise. The dynamic PVR group received inhaled treprostinil, while the static PVR group was monitored off therapy. Baseline and 16-week follow-up values were compared within each group. At 16 weeks, the dynamic PVR group demonstrated significant improvements in mean 6 min walk distance (6MWD) (+32.5 m, p < 0.05), resting PVR (−1.04 WU, p < 0.05), resting mean pulmonary arterial pressure (mPAP) (−5.8 mmHg, p < 0.05), exercise PVR (−1.7 WU, p < 0.05), exercise mPAP (−13 mmHg, p < 0.05), and estimated right ventricular systolic pressure (−9.2 mmHg, p < 0.05). In contrast, the static PVR group remained clinically stable. These observations suggest that an exercise-induced increase in PVR, identified through Level 3 CPET, may help select patients with non-severe PH-ILD who are more likely to benefit from early initiation of inhaled treprostinil. Full article

Purpose: To evaluate and describe the efficacy and safety of viscodissection in managing complex funnel-shaped retinal detachments, minimizing trauma and facilitating safer perfluorocarbon liquid (PFCL) application. Methods: A retrospective case series of five patients with funnel-shaped retinal detachments: three due to perforating trauma and two from recurrent detachments. Initial visual acuities ranged from light perception to hand motion. Viscodissection was used to separate adhered retinal tissues in the funnel-shaped retinal detachment in a controlled, minimally traumatic manner, allowing funnel opening and PFCL application. Data collected included demographics, visual acuities, surgical details, and complications. Results: Viscodissection enabled successful funnel opening and PFCL use in all cases, with one instance of subretinal migration of PFCL. No retinal detachment recurrences occurred, but three patients required reoperation for new premacular proliferative vitreoretinopathy (PVR). Postoperative visual acuities improved in four patients (up to 20/100), while one remained at hand motion. Conclusions: Viscodissection is a promising technique for complex funnel-shaped retinal detachments, allowing non-traumatic tissue separation and improving visualization and safety during PFCL application. This approach may enhance surgical outcomes and reduce complications. Full article

Background and Objectives: This study investigated the frequency and clinical significance of subclinical but substantial peripheral arterial disease (PAD), identified using PAD evaluation, including pulse volume recording/ankle–brachial index (PVR/ABI), transcutaneous oxygen pressure (TcpO2), and skin perfusion pressure (SPP) tests in patients with antineutrophil cytoplasmic antibody-associated vasculitis (AAV). Materials and Methods: This study included 54 patients with PAD evaluation results at or after AAV diagnosis. PVR/ABI and/or TcpO2 and/or SPP were performed on the same day. Abnormal PVR/ABI, TcpO2, and SPP were defined as PVR/ABI < 0.97, TcpO2 < 40 mmHg, and SPP < 50 mmHg, respectively. Poor outcomes included all-cause mortality, end-stage kidney disease (ESKD), cerebrovascular accidents, and acute coronary syndrome after PAD evaluation. Results: The median age of the 54 patients was 67 years, and 48.1% were male. In total, 3 of 54 patients (5.6%), 6 of 16 (37.5%), and 6 of 23 (26.1%) had abnormal PVR/ABI, TcpO2, and SPP, respectively. The concordance rate between abnormal PVR/ABI and abnormal TcpO2 or SPP was very low. Among the 54 patients, 5 (9.3%) died, and 2 (3.7%) progressed to ESKD. Abnormal SPP was significantly associated with cutaneous and renal manifestations at the time of PAD evaluation and had the potential to predict progression to ESKD during follow-up in patients with AAV. Conclusions: This study is the first to reveal the clinical usefulness of PAD evaluation: abnormal SPP may have the potential to identify subclinical but substantial PAD and can predict simultaneous kidney involvement as well as future progression to ESKD in patients with AAV. Full article

The retinal pigment epithelium (RPE) plays a crucial role in maintaining retinal homeostasis, and dysregulation of the transforming growth factor-beta (TGF-β) signaling pathways contributes to retinal fibrosis and inflammatory diseases, including proliferative vitreoretinopathy (PVR). Tacrolimus (FK506), an immunosuppressant, has shown potential antifibrotic properties, but its effects on TGF-β-related genes and microRNAs (miRNAs) in RPE cells remain unclear. Human RPE (H-RPE) cells were treated with lipopolysaccharide (LPS) to induce inflammation and subsequently exposed to tacrolimus. Gene and miRNA expression profiling related to TGF-β signaling pathways were conducted using microarrays, followed by Quantitative Reverse-Transcription Polymerase Chain Reaction (RT-qPCR) validation. Protein levels were assessed via enzyme-linked immunosorbent assay (ELISA), and interactions were analyzed using STRING database network analysis. Tacrolimus modulated key components of the TGF-β pathway, upregulating TGF-β2, TGF-β3, SMAD2, and SMAD4 while downregulating TGF-βR1 and SMAD7. JAK/STAT and MAPK pathways were also affected, indicating broad regulatory effects. miRNA profiling identified hsa-miR-200a-3p, hsa-miR-589-3p, hsa-miR-21, and hsa-miR-27a-5p as key regulators. STRING analysis confirmed strong functional interactions within the TGF-β network. In conclusion, tacrolimus modulates both canonical (upregulation of SMAD2/4 and downregulation of SMAD7) and non-canonical (JAK/STAT and MAPK) TGF-β signaling pathways in LPS-stimulated RPE cells. These changes collectively suggest a dual anti-inflammatory and anti-fibrotic effect. The increased TGF-β2 and decreased SMAD7 levels, alongside altered miRNA expression (e.g., downregulation of miR-200a-3p), indicate that tacrolimus may inhibit key profibrotic mechanisms underlying PVR. These findings support the potential therapeutic repurposing of tacrolimus in PVR and warrant further in vivo validation. Full article

Background: Vesicourethral anastomosis (VUA) is a critical step in radical prostatectomy (RP), with interrupted suture (IS) and running suture (RS) as common techniques. However, there is no conclusive evidence suggesting the superiority of one technique over the other regarding voiding function. This study compares their effects on voiding function and continence recovery after retropubic RP. Methods: A two-group, parallel-design study included 70 patients with localized prostate cancer (pT1-pT2) undergoing retropubic RP by a single surgical team. Patients were randomized to VUA with IS (n = 35) or RS (n = 35). The primary outcomes included uroflowmetry parameters—maximum flow rate (MFR), voiding volume (VV)—post-void residual volume (PVR), urinary function assessed by the International Prostate Symptom Score (IPSS), and continence recovery. These outcomes were measured preoperatively and at 1, 3, 6, and 12 months post-surgery. Secondary outcomes included surgical parameters, perioperative complications and one-year oncological outcomes. Results: Suturing time was shorter for RS than IS (21 vs. 33 min, p = 0.001). Minimal anastomotic leakage occurred more frequently in the IS group (23% vs. 9%), while long-term anastomotic stenosis rates were comparable between RS and IS groups (12% vs. 9%). IS demonstrated significantly higher MFR at 1-month post-surgery (23.3 vs. 17.2 mL/s, p = 0.003). In subsequent follow-ups (3, 6, and 12 months), the mean MFR remained higher in the IS group, though without statistical significance. Logistic regression favored IS for early MFR outcomes (OR 4.16; 95% CI, 1.22–14.18; p = 0.023). Continence recovery and IPSS scores were similar between groups. Conclusions: Both techniques are effective and safe. RS reduces suturing time and leakage risk, while IS improves early postoperative MFR. Full article

Introduction: Evidence for the diagnostic yield of noninvasive diagnostic assessment for the diagnosis of peripheral arterial disease (PAD) in diabetic foot syndrome (DFS) is poor. Pulse volume recordings (PVRs) at the forefoot level could be a valuable diagnostic tool in the presence of medial arterial calcification. Patients and methods: Patients with DFS who underwent invasive angiography between 01/2020 and 11/2024 and had corresponding PVRs performed within 30 days prior to the procedure were included. DFS was classified according to the Wagner–Armstrong classification. Clinical characteristics and hemodynamic parameters, including systolic ankle pressures and ankle–brachial index were recorded. PVRs were analyzed semiquantitatively by investigators blinded to the clinical information and quantitatively with determination of upstroke time (UST), upstroke ratio (USR), and maximum systolic amplitude (MSA). Angiographic PAD severity was graded according to the GLASS classification. Statistical analysis included univariate significance tests, 2 × 2 contingency tables, receiver–operator characteristic (ROC) analysis and determination of interobserver agreement. Results: In this study, 90 extremities of 70 patients were analyzed, 47 of whom had an ABI ≥ 1.3. Critical limb-threatening ischemia with non-pulsatile PVRs was evident in 6.7%. An abnormal PVR curve morphology (mildly or severely abnormal) yielded a sensitivity and specificity of 63.3% and 85.7% for detection of severe PAD (GLASS stages 2 and 3). Interobserver agreement of semiquantitative PVR rating was substantial (Cohen’s kappa 0.8) in 51 evaluated cases. For detection of any PAD (GLASS ≥ 1) or severe PAD (GLASS ≥ 2), we found the highest diagnostic accuracy for MSA (area under the curve [AUC] 0.89 and 0.82). With a cut-off value of 0.58 mmHg, MSA had a sensitivity of 91.4% and a specificity of 80.8% for detection of any PAD (GLASS ≥ 1). MSA with a cut-off of 0.27 mmHg had a sensitivity of 72.2% and a specificity of 77.1% for detection of severe PAD, whereas the sensitivity and specificity for detection of inframalleolar disease were 62.9% and 69.4%, respectively. Results were consistent in subgroup analyses. Conclusions: PVRs with extraction of quantitative features offer promising diagnostic yield for detection of PAD in the setting of DFS. MSA outperformed UST and USR but showed limited capability of detecting impaired inframalleolar outflow. Full article

Background: Holmium Laser Enucleation of the Prostate (HoLEP) is an established treatment for benign prostatic hyperplasia (BPH). Pulse-modulated lasers, like MOSES technology (MoLEP), may enhance the procedure’s efficiency and safety. Methods: A 3-year single-center retrospective comparative study was conducted on 1368 patients treated with HoLEP/MoLEP at MITERA Hospital. Results: A total of 688 patients were treated with HoLEP and 680 with MoLEP. Compared to HoLEP, MoLEP demonstrated shorter surgical (50.5 min [IQR 33–60] vs. 58 min [IQR 46–69], p < 0.01) and enucleation times (34 min [IQR 23–43] vs. 43 min [IQR 34–51], p < 0.001) and shorter hospital stay (8 h [IQR 6–19] vs. 12 h [IQR 9–24], p = 0.027), catheterization time (19 h [IQR 12–48] vs. 24 h [IQR 24–48], p < 0.001), and irrigation duration (5 h [IQR 2–8] vs. 7 h [IQR 3–10], p < 0.001), with similar morcellated tissue weight and morcellation time. At 1 month, MoLEP showed higher Qmax (27.3 mL/s [IQR 23.9–30.3] vs. 20 mL/s [IQR 17–23.6], p < 0.001), lower PVR (11.4 mL [IQR 7.7–15] vs. 12.5 mL [IQR 7–18], p = 0.005), better IPSS (4 [IQR 3–6] vs. 7 [IQR 5–11], p < 0.005), QoL (1 [IQR 1–2] vs. 2 [IQR 1–2], p < 0.001), lower PSA (1.8 ng/mL [IQR 1.1–2.6] vs. 2.4 ng/mL [IQR 1.3–3.5], p < 0.001), which were maintained at 6 months, and fewer Clavien-Dindo I (2.5% vs. 7.5%, p < 0.001) and II (16% vs. 25.7%, p < 0.001) complications. Conclusions: MoLEP offered significant advantages over HoLEP in this study. Full article

Introduction: Placebo-controlled studies are crucial in clinical trials, but the placebo effect can vary across conditions. We aimed to assess the placebo effect in chronic thromboembolic pulmonary hypertension (CTEPH) trials. Methods: We conducted a systematic review and included randomized placebo-controlled trials investigating CTEPH interventions. Primary outcomes were the pre–post changes in the 6 min walk test (6MWT) and quality of life in the placebo arms. Secondary outcomes included mean pulmonary artery pressure (mPAP), pulmonary vascular resistance (PVR), cardiac index, and NT-proBNP levels. Meta-analyses were performed using random-effects models. Results: Seven trials with 270 CTEPH patients in placebo arms were analyzed. The average 6MWT change was not significant (−1.31 m; 95%CI −12.49 to +9.79). Quality of life with EQ-5D was not significantly improved (−0.04; 95%CI −0.10 to +0.02). mPAP, PVR, cardiac index, and NT-proBNP also demonstrated non-significant changes with small magnitudes. Conclusions: The placebo effect in CTEPH trials was not statistically significant and had small magnitude but should not discourage the use of placebo-controlled trials where applicable and ethical. Full article

Pulmonary hypertension (PH) is a serious cardiovascular disease caused by a variety of pathogenic factors, which is characterized by increased pulmonary vascular resistance (PVR) and progressive elevation of mean pulmonary artery pressure (mPAP). This disease can lead to right ventricular hypertrophy and, in severe cases, right heart failure and even death. Vascular remodeling—a pathological modification involving aberrant vasoconstriction, cell proliferation, apoptosis resistance, and inflammation in the pulmonary vascular system—is a significant pathological hallmark of PH and a critical process in its progression. Recent studies have found that vascular remodeling involves the participation of a diversity of cellular pathological alterations, such as the dysfunction of pulmonary artery endothelial cells (PAECs), the proliferation and migration of pulmonary artery smooth muscle cells (PASMCs), the phenotypic differentiation of pulmonary artery fibroblasts, the inflammatory response of immune cells, and pericyte proliferation. This review focuses on the mechanisms and the intercellular crosstalk of these cells in the PH process, emphasizing recent advances in knowledge regarding cellular signaling pathways, inflammatory responses, apoptosis, and proliferation. To develop better treatments, a list of possible therapeutic approaches meant to slow down certain biological functions is provided, with the aim of providing new insights into the treatment of PH by simplifying the intricacies of these complex connections. In this review, comprehensive academic databases such as PubMed, Embase, Web of Science, and Google Scholar were systematically searched to discuss studies relevant to human and animal PH, with a focus on vascular remodeling in PH. Full article

Background and Objectives: We aimed to assess the outcomes of upfront Optilume drug-coated balloon (DCB) dilation in patients after failed treatment for complex recurrent urethral stricture disease. All patients presented with acute urinary retention and were treated with DCB dilation regardless of stricture site and length. Materials and Methods: We retrospectively evaluated patients with acute urinary retention and known complex recurrent urethral strictures. Patients presented at the urology emergency room of our tertiary centre with an inability to void or a post-void residual (PVR) volume exceeding 400 mL between August 2021 and February 2024. Urethrography and/or endoscopic imaging confirmed the diagnosis. Patients with urinary tract infection/sepsis and those with neurological disease were excluded. Urethral dilation to 20 Fr was performed, followed by DCB dilation (30 Fr, 10 bar, 10 min). The primary endpoints were anatomical success (≥14 Fr by cystoscopy/calibration) at 12 months and freedom from repeat interventions. Results: Thirty-one consecutive male patients were evaluated, with twenty-six patients followed for ≥12 months (mean age 65 ± 16.8 years). The stricture sites included seven bulbopenile, seven bulbomembranous, seven anastomotic, three bladder neck, one penile, and one panurethral stricture. The median number of prior urethral/surgical interventions was 2 [IQR: 1–3] (range: 1–31). The median stricture length was 3 [IQR: 2–4] cm (range: 1–8). At 12 months, 65.4% (17/26) of subjects voided satisfactorily and were free of recurrence and reoperation. Conclusions: Timely DCB dilation may offer a viable treatment option for patients with complex recurrent urethral strictures and urinary retention, particularly those who are unable or unwilling to undergo surgical reconstruction and prefer to avoid indwelling catheters. Full article

Background/Objectives: Heart transplantation (HTX) is the definitive treatment for advanced heart failure (AdHF). The angiotensin receptor neprilysin inhibitor (ARNI) sacubitril/valsartan (S/V) has been shown to reduce heart failure (HF) hospitalizations and mortality when compared to conventionally administered HF medications (i.e. angiotensin-converting enzyme inhibitors (ACEIs) and angiotensin II receptor blockers (ARBs)). Nevertheless, limited data are available on the hemodynamic (HD) effects of ARNI in patients with AdHF. Therefore, the aim of the present study was to compare echocardiographic, laboratory, and HD parameters relevant to HF before and after switching to ARNI in patients with AdHF awaiting HTX. Methods: A retrospective analysis was conducted utilizing available data on HD parameters, N-terminal pro-brain natriuretic peptide (NT-proBNP) levels, data on kidney function, HF therapy, and comorbidities. The study cohort comprised 13 AdHF patients (3 women, 10 men; mean age 56.4 ± 9 years) of whom 53.8% presented with non-ischemic and 46.2% with ischemic etiology. All patients were awaiting heart transplantation (HTX) and were transitioned to ARNI therapy between 2018 and 2021. Results: After switching to ARNI, we observed significant improvements: in left ventricular ejection fraction (LVEF: 27.27 ± 1.04% vs. 23.65 ± 1.02%, p = 0.03; data are given as mean ± SEM after vs. before ARNI therapy, respectively), cardiac output (CO: 4.90 ± 0.35 L/min vs. 3.83 ± 0.24 L/min, p = 0.013), and stroke volume (SV: 70.9 ± 5.9 mL vs. 55.5 ± 4.12 mL, p = 0.013). Significant reductions in systemic vascular resistance (SVR: 1188 ± 79.8 vs. 1600 ± 100 DS/cm⁵, p = 0.004) and pulmonary vascular resistance (PVR: 232.5 ± 34.8 vs. 278.9 ± 31.7 DS/cm⁵, p = 0.04) were also noted. Central venous pressure (CVP), pulmonary arterial systolic and diastolic pressures (PAPs and PAPd), pulmonary capillary wedge pressure (PCWP), and NT-proBNP levels did not exhibit significant changes upon ARNI administration. Conclusions: Early transition to ARNI therapy offers significant benefits for invasively measured hemodynamic parameters in patients with AdHF, potentially aiding in the stabilization and improvement of this vulnerable patient population. Full article

Pulmonary hypertension (PH) is a progressive disorder characterized by obstructive changes in the pulmonary vasculature, leading to increased pulmonary vascular resistance (PVR), right ventricular (RV) strain, and eventual RV failure (RVF). Despite advancements in medical therapy, PH remains associated with significant morbidity and mortality, particularly in children. RVF is a clinical syndrome resulting from complex structural and functional remodeling of the right heart, leading to inadequate pulmonary circulation, reduced cardiac output, and elevated venous pressure. Management paradigms for pediatric PH diverge significantly from those in adults, particularly due to the predominance of congenital heart disease (CHD) and the dynamic nature of pediatric cardiovascular and pulmonary development. CHD remains a principal driver of PH in children, and its associated pathophysiology demands a nuanced approach. In patients with unrepaired left-to-right shunts, elevated pulmonary blood flow can lead to progressive pulmonary vascular remodeling and increased PVR. The postoperative persistence or progression of PH may occur if irreversible vascular changes have already developed. Current PH treatments primarily focus on reducing PVR, yet distinguishing between therapeutic approaches that target the pulmonary vasculature and those aimed at improving RV function remain challenging. In pediatric patients with progressive PH despite optimal therapy, additional targeted interventions may be necessary to mitigate RV dysfunction and disease progression. This review provides a comprehensive analysis of the mechanisms underlying RVF in PH, incorporating insights from clinical studies in adults and experimental models, while highlighting the unique considerations in children. Furthermore, it explores current pharmacological and interventional treatment strategies, emphasizing the need for novel therapeutic approaches aimed at directly reversing RV remodeling. Given the complexities of RV adaptation in pediatric PH, further research into disease-modifying treatments and innovative interventions is crucial to improving long-term outcomes in affected children. Full article