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

Eloisa Sassá Carvalho^#, Maria Francilene S. Souza, Kelly Cristina O. Abud, Claudia R. P. Castro, Juliano G. Penha, Ana Maria Thomaz, Vanessa A. Guimarães, Antonio Augusto Lopes^*

Congenital Heart Disease, Vol.17, No.3, pp. 351-363, 2022, DOI:10.32604/chd.2022.019382 - 03 May 2022

Abstract Background: Postoperative pulmonary hypertension limits the success of surgical treatment in some patients with unrestrictive congenital cardiac communications. Identifying patients at risk of developing postoperative pulmonary hypertension is important to individualize follow-up strategies. Methods: We analyzed a prospective cohort of 52 pediatric patients (age 3 to 35 months) looking for perioperative predictors of mildly elevated pulmonary arterial pressure 6 months after surgery, defined as a systolic pressure greater than 30 mmHg by transthoracic echocardiography. This corresponds to a mean pulmonary arterial pressure of >20 mmHg. Clinical, echocardiographic and hemodynamic parameters were investigated. Perioperative hemodynamics was assessed by directly… More >

Barry O’Callaghan¹, Jenny Zablah¹, Joseph Vettukattil², Daniel Levi^3,4, Morris Salem⁴, Allison Cabalka⁵, Jason Anderson⁶, Makram Ebeid⁶, Ryan Alexy⁷, Gareth J. Morgan^1,*

Congenital Heart Disease, Vol.17, No.1, pp. 107-116, 2022, DOI:10.32604/CHD.2022.018590 - 26 October 2021

Abstract Objectives: To detail the US multi-institutional experience with the Occlutech^© (Occlutech International AB, Helsingborg, Sweden) atrial flow regulator (AFR) in children and adults with acquired or congenital heart disease. Background: The creation of a long-term atrial communication is desirable in several cardiovascular disease phenotypes, most notably pulmonary arterial hypertension, disorders of increased left ventricular filling and increased cavopulmonary pressures in patients with a Fontan type circulation. Methods: Patients were identified for inclusion from the AFR device manufacturer database. Data was collected using a RedCap database following IRB approval. 8 weeks of follow up data was sought for… More >

Liping Wang, Mingjie Zhang, Xi Chen, Yachang Pang, Jiaqi Liu, Zhuoming Xu^*

Congenital Heart Disease, Vol.17, No.1, pp. 87-97, 2022, DOI:10.32604/CHD.2022.018452 - 26 October 2021

Abstract Background: Reversibility of pulmonary hypertension (PH) is closely related to the treatment options for and prognosis of children with congenital heart disease. Objective: We combined patient-specific clinical features including diagnosis, age and echocardiographic results, and biomarkers of pulmonary vascular dysfunction to explore the noninvasive methods that can be used to accurately evaluate the reversibility of pulmonary hypertension in congenital heart disease (PH-CHD). Methods: Based on the preoperative systolic pulmonary arterial pressure (sPAP), 70 CHD patients were divided into normal, PH-CHD suspected, and confirmed groups. Additionally, biomarkers of circulating endothelial cells (CECs), endothelin-1 (ET-1), and endothelial nitric oxide… More >

Huaying He^1,2, Zhiyong Lin^1,2, Yuelan Weng^1,2, Jianjie Zhou^1,2, Man Ye^1,2, Xiaowei Luo^1,2, Qifeng Zhao^1,2,*

Congenital Heart Disease, Vol.16, No.6, pp. 655-673, 2021, DOI:10.32604/CHD.2021.016054 - 08 July 2021

Abstract Background: To achieve successful management of infants with congenital heart disease (CHD) together with pulmonary hypertension (PH), postoperative care, especially feeding care is vital in addition to surgery. Postoperative feeding is comprised of three stages: feeding in the intensive care unit, feeding in the general ward and family feeding, in which the general ward is considered as the “transitional stage”. At present, there is little research on the optimal mode of feeding care for the transitional stage, and there is no universally recognized and accepted protocol. Methods: We retrospectively analyzed 114 CHD infants with PH who… More >