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  • Open Access

    REVIEW

    Endothelial and Mitochondrial Dysfunction in COPD Pathophysiology: Focus on Homocysteine–L-Carnitine Interplay

    Eduard Belskikh1,*, Yuliya Marsyanova2, Denis Melnikov3, Oleg Uryasev1, Valentina Zvyagina2

    BIOCELL, Vol.49, No.11, pp. 2093-2123, 2025, DOI:10.32604/biocell.2025.069272 - 24 November 2025

    Abstract Elevated homocysteine is a clinically relevant metabolic signal in chronic obstructive pulmonary disease (COPD). Higher circulating levels track with oxidative stress, endothelial dysfunction, mitochondrial impairment, and pulmonary vascular remodeling, rise with disease severity, and may contribute to the excess cardiovascular risk—although effect sizes and causality remain uncertain. This review centers on the homocysteine–carnitine relationship in COPD pathophysiology. Carnitine deficiency, prevalent in COPD, can worsen mitochondrial bioenergetics, promote accumulation of acyl intermediates, and reduce nitric oxide bioavailability via endothelial nitric oxide synthase uncoupling (eNOS). Conversely, restoring carnitine status in experimental and early clinical settings has been… More >

  • Open Access

    REVIEW

    Pulmonary Hypertension Associated with Congenital Heart Disease: A Clinical Primer

    Lily M. Landry1,*, Christopher L. Jenks2

    Congenital Heart Disease, Vol.20, No.3, pp. 325-339, 2025, DOI:10.32604/chd.2025.066142 - 11 July 2025

    Abstract Pulmonary hypertension associated with congenital heart disease represents a significant challenge for clinicians due to its complex pathophysiology and diverse presentation. This patient population exhibits a broad spectrum of anatomical and hemodynamic abnormalities, with congenital heart disease-associated pulmonary arterial hypertension (PAH-CHD) comprising a significant proportion of pediatric pulmonary hypertension (PH) cases. Although progress in diagnostic methods and treatment options has been made, PH continues to be a major contributor to illness and death among affected pediatric patients, especially when diagnosis or treatment is postponed. This review aims to equip non-specialist clinicians with a better understanding More >

  • Open Access

    ARTICLE

    Hemodynamic Profile Based on Right Heart Catheterization in Adult Acyanotic Congenital Heart Disease with Pulmonary Hypertension

    Dina Anggraini1, Kurnia Wahyudi2, Melawati Hasan3, Sri Endah Rahayuningsih4,*, Charlotte Johanna Cool3

    Congenital Heart Disease, Vol.20, No.2, pp. 133-141, 2025, DOI:10.32604/chd.2025.064164 - 30 April 2025

    Abstract Background: Congenital heart disease (CHD) occurs in 9 out of 100 births and is the leading cause of birth defects, with acyanotic CHD being more common. The incidence of adult CHD is rising faster than pediatric CHD. Pulmonary hypertension is the most common complication in untreated CHD patients. Methods: This study is retrospective descriptive research based on medical record data and the results of right heart catheterization examinations in adult acyanotic CHD aged ≥18 years and free from other organ disorders. Results: A total of 103 patients met the inclusion criteria, the majority were young… More >

  • Open Access

    REVIEW

    Reverse Potts Shunt in Children with Suprasystemic Pulmonary Arterial Hypertension: A Systematic Review and Meta-Analysis

    Yong An1,2, Gang Wang1,2, Jiangtao Dai1,2, Zhengxia Pan1,2, Yuhao Wu1,2,*

    Congenital Heart Disease, Vol.20, No.1, pp. 1-12, 2025, DOI:10.32604/chd.2025.063152 - 18 March 2025

    Abstract Background Pulmonary arterial hypertension (PAH) is a progressive condition with a poor prognosis in children. Lung transplantation (Ltx) remains the ultimate option when patients are refractory to PAH-specific therapy. Reverse Potts shunt (RPS) has been introduced to treat suprasystemic PAH. This study aims to investigate the clinical outcomes of suprasystemic PAH in children. Methods Embase, Pubmed, and the Cochrane Library databases were searched for related studies that reported the clinical outcomes of suprasystemic PAH following RPS in children. To investigate the clinical outcomes of RPS, meta-analyses of the early and overall mortalities were performed. Results Nine studies… More >

  • Open Access

    REVIEW

    Side effect management algorithms for niraparib/abiraterone acetate in prostate cancer

    Jean-Baptiste Lattouf1, Jenny J. Ko2, Margot K. Davis3, Christian Constance4, Geoffrey T. Gotto5

    Canadian Journal of Urology, Vol.31, No.5, pp. 11977-11985, 2024

    Abstract Introduction: Niraparib, a PARP1/2 inhibitor, is newly approved in combination with abiraterone acetate (AA) plus prednisone or prednisolone (niraparib/AA+P) for the treatment of adult patients with BRCA-mutated, treatment-naïve metastatic castration resistant prostate cancer (mCRPC). Detailed guidance beyond the prescribing information may be helpful in managing the side effect profile and dosing practicalities of this combination therapy.
    Materials and methods: A panel of specialists convened to design management algorithms for four common niraparib/ AA+P treatment-related adverse events (AEs) in mCRPC; anemia, thrombocytopenia, hypertension, and nausea. The algorithms build on Health Canada-approved prescribing information to highlight practical considerations related… More >

  • Open Access

    ARTICLE

    Efficacy of Intravenous Treprostinil in High-Risk Single Ventricle Patients Undergoing Glenn Procedure

    Xiaofeng Wang1,#, Xingwei Chen2,#, Shilin Wang1, Xia Li1, Zhongyuan Lu1, Wenlong Wang1, Xu Wang1,*

    Congenital Heart Disease, Vol.19, No.5, pp. 489-498, 2024, DOI:10.32604/chd.2024.054441 - 31 December 2024

    Abstract Objective: Pulmonary hypertension is a crucial factor affecting the recovery after Glenn procedure. This study explores the effects of intravenous treprostinil on hemodynamic status and hospital postoperative recovery under different administration strategies. Methods: We retrospectively included pediatric patients admitted to Fuwai Hospital from 2019 to 2022 who underwent the Glenn procedure and had intraoperative measurements of mean pulmonary artery pressure (mPAP) >15 mmHg postoperatively. Patients with non-anatomical single ventricle physiology undergoing the Glenn procedure and those requiring postoperative extracorporeal membrane oxygenation were excluded. Due to the standardized use of treprostinil in our center starting in… More >

  • Open Access

    REVIEW

    The Potential of Circular RNAs as Biomarkers in Pulmonary Arterial Hypertension Related to Congenital Heart Disease

    Fajri Marindra Siregar1,2, Sofia Mubarika Haryana3, Dyah Wulan Anggrahini4, Lucia Kris Dinarti4, Anggoro Budi Hartopo4,*

    Congenital Heart Disease, Vol.19, No.4, pp. 375-388, 2024, DOI:10.32604/chd.2024.054742 - 31 October 2024

    Abstract A particular type of endogenous noncoding RNAs known as circular RNAs (circRNAs) has now become possible biomarkers for several diseases because of their stability and tissue-specific expression patterns. CircRNAs might play a role in various of biological processes. The identification of particular circRNAs dysregulated in pulmonary arterial hypertension (PAH) raises the possibility of these molecules serving as biomarkers for the disease’s early diagnosis and treatment. This review mainly summarizes the role and potential of circRNA as a future biomarker in PAH related to congenital heart disease. This study presented several potential circRNA targets as diagnostic More > Graphic Abstract

    The Potential of Circular RNAs as Biomarkers in Pulmonary Arterial Hypertension Related to Congenital Heart Disease

  • Open Access

    ARTICLE

    Risk Stratification and Prognosis of Pulmonary Arterial Hypertension Associated with Congenital Heart Disease

    Wenjie Dong1,2,#, Zhibin Hong1,#, Aqian Wang2, Kaiyu Jiang2, Hai Zhu2, Fu zhang2, Zhaoxia Guo2, Hongling Su2,*, Yunshan Cao3,*

    Congenital Heart Disease, Vol.19, No.3, pp. 325-339, 2024, DOI:10.32604/chd.2024.052267 - 26 July 2024

    Abstract Background: Current guidelines for managing pulmonary arterial hypertension (PAH) recommend a risk stratification approach. However, the applicability and accuracy of these strategies for PAH associated with congenital heart disease (PAH-CHD) require further validation. This study aims to validate the reliability and predictive accuracy of a simplified stratification strategy for PAH-CHD patients over a three-year follow-up. Additionally, new prognostic variables are identified and novel risk stratification methods are developed for assessing and managing PAH-CHD patients. Methods: This retrospective study included 126 PAH-CHD patients. Clinical and biochemical variables across risk groups were assessed using Kruskal-Wallis and Fisher’s… More > Graphic Abstract

    Risk Stratification and Prognosis of Pulmonary Arterial Hypertension Associated with Congenital Heart Disease

  • Open Access

    ARTICLE

    Reliability of Echocardiographic Pulmonary Vascular Resistance to Screen for the New Definition of Precapillary Pulmonary Hypertension in Uncorrected Secundum Atrial Septal Defect

    Risalina Myrtha1,2,*, Hasanah Mumpuni3,4, Real Kusumanjaya Marsam3,4, Dyah Wulan Anggrahini3,4, Anggoro Budi Hartopo3,4, Lucia Kris Dinarti3,4

    Congenital Heart Disease, Vol.19, No.3, pp. 315-324, 2024, DOI:10.32604/chd.2024.051587 - 26 July 2024

    Abstract Background and Objective: The most feared complication of uncorrected secundum Atrial Septal Defect (ASD) is pulmonary arterial hypertension (PAH). Pulmonary vascular resistance (PVR) is crucial in detecting precapillary pulmonary hypertension (PH) to guide the need for PAH-specific therapy. There is a change in the cut-off value of PVR according to the recently updated PH guideline. How echocardiographic PVR (PVR) correlates to PVR by right heart catheterization (RHC) (PVR) according to the new guidelines has not been known. The aim of this study is to determine the reliability of PVR in detecting PAH in Uncorrected Ostium… More > Graphic Abstract

    Reliability of Echocardiographic Pulmonary Vascular Resistance to Screen for the New Definition of Precapillary Pulmonary Hypertension in Uncorrected Secundum Atrial Septal Defect

  • Open Access

    REVIEW

    Treatment and Clinical Management of Chronic Thromboembolic Pulmonary Hypertension: An Update of Literature Review

    Yuan Ren1, Yingxian Sun1, Zhiguang Yang2, Yanli Chen1,*

    Congenital Heart Disease, Vol.19, No.2, pp. 157-176, 2024, DOI:10.32604/chd.2024.047930 - 16 May 2024

    Abstract Chronic thromboembolic pulmonary hypertension (CTEPH) is a chronic, progressive, debilitating, and life-threatening complication of pulmonary embolism (PE). Recent technological advances have permitted various treatment options for the treatment of CTEPH, including surgery, angioplasty, and medical treatment, depending on the location and characteristics of lesions. Pulmonary endarterectomy (PEA) is the treatment of choice for CTEPH, as it offers excellent long-term outcomes and a high probability of recovery. Moreover, various medical and interventional therapies are currently being developed for patients with inoperable CTEPH. This review mainly summarizes the current treatment approaches of CTEPH, offering more options for More >

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