Eduard Belskikh^1,*, 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 >

Lily M. Landry^1,*, 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 >

Dina Anggraini¹, Kurnia Wahyudi², Melawati Hasan³, Sri Endah Rahayuningsih^4,*, 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 >

Xiaofeng Wang^1,#, Xingwei Chen^2,#, Shilin Wang¹, Xia Li¹, Zhongyuan Lu¹, Wenlong Wang¹, Xu Wang^1,*

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 >

Wenjie Dong^1,2,#, Zhibin Hong^1,#, Aqian Wang², Kaiyu Jiang², Hai Zhu², Fu zhang², Zhaoxia Guo², Hongling Su^2,*, Yunshan Cao^3,*

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

Risalina Myrtha^1,2,*, Hasanah Mumpuni^3,4, Real Kusumanjaya Marsam^3,4, Dyah Wulan Anggrahini^3,4, Anggoro Budi Hartopo^3,4, Lucia Kris Dinarti^3,4

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

Yuan Ren¹, Yingxian Sun¹, Zhiguang Yang², Yanli Chen^1,*

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 >

Dian Kesumarini^1,2, Yunita Widyastuti³, Cindy Elfira Boom¹, Lucia Kris Dinarti^4,*

Congenital Heart Disease, Vol.18, No.6, pp. 671-678, 2023, DOI:10.32604/chd.2023.044746 - 19 January 2024

Abstract Tetralogy of Fallot (TOF) with total anomalous pulmonary vein connections (TAPVC) is a rare type of complex congenital heart disease among all TOF cases. Co-presentation of major aortopulmonary collateral arteries (MAPCAs) compensates for the lack of central pulmonary blood flow and decreases the severity of right-to-left shunting in TOF. We present a case of a 2-year-old child with complex diagnoses of TOF, TAPVC, a large secundum atrial septal defect (ASD), and intraoperatively identified MAPCAs. She underwent surgery to repair the TAPVC, valve-sparing reconstruction of the right ventricular outflow tract, interventricular defect closure, and the creation… More >

Andrei George Iosifescu^1,2,*, Roxana Enache^1,3, Ioana Marinică⁴, Corina Radu², Vlad Anton Iliescu^1,2

Congenital Heart Disease, Vol.18, No.1, pp. 67-72, 2023, DOI:10.32604/chd.2023.026598 - 09 January 2023

Abstract Left main coronary compression syndrome (LMCS) may complicate pulmonary artery aneurysms (PAA), usually developed in the context of pulmonary arterial hypertension (PAH). We report the case of a 51-year-old female patient with an atrial septal defect (unsuitable for device closure) complicated by a PAA generating a 90% left main stenosis. The significant PAH held us back from immediate surgery. After specific dual PAH-targeted therapy (sildenafil and bosentan), the atrial septal defect could be closed with a unidirectional valved patch; the PAA-induced LMCS was treated by reductive arterioplasty. The postoperative course was uneventful. Follow-up showed clinical More > Graphic Abstract

Huan Zhou^1,#, Jin Kang^2,#, Jun Gao^2,*, Xiaoyuan Feng¹, Li Zhou², Xia Xiao², Zhengliang Meng², Chengwen Guo²

Congenital Heart Disease, Vol.17, No.6, pp. 697-707, 2022, DOI:10.32604/chd.2022.022648 - 11 October 2022

Abstract Background: Real-time three-dimensional echocardiography (RT-3DE) could obtain ventricular volume and ejection fraction rapidly and non-invasively without relying on ventricular morphology. This study aims to use RT-3DE to evaluate the changes in biventricular volume and systolic function in children with ventricular septal defect (VSD) and moderate to severe pulmonary hypertension (PH) before surgery. Methods: In this study 18 children with VSD and moderate to severe PH (VSD + PH Group) and 18 healthy children of the same age (Control Group) were recruited. Biventricular volume and systolic function were evaluated by RT-3DE. The measurements included: left and right… More > Graphic Abstract