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  • Open Access

    CASE REPORT

    Pulmonary Hypertension Crisis in Patient with Tetralogy of Fallot and Mixed Total Anomalous Pulmonary Vein Connection after the Primary Correction: A Rare Case Report

    Dian Kesumarini1,2, Yunita Widyastuti3, Cindy Elfira Boom1, Lucia Kris Dinarti4,*

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

    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 of patent foramen ovale (PFO).… More >

  • Open Access

    CASE REPORT

    “Treat-Repair-Treat”: Management of Left Main Coronary Compression by a Pulmonary Artery Aneurysm in a Patient with Atrial Septal Defect and Significant Pulmonary Hypertension

    Andrei George Iosifescu1,2,*, Roxana Enache1,3, Ioana Marinică4, Corina Radu2, Vlad Anton Iliescu1,2

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

    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 improvement, but PAH treatment was… More > Graphic Abstract

    “Treat-Repair-Treat”: Management of Left Main Coronary Compression by a Pulmonary Artery Aneurysm in a Patient with Atrial Septal Defect and Significant Pulmonary Hypertension

  • Open Access

    ARTICLE

    Evaluation of Biventricular Volume and Systolic Function in Children with Ventricular Septal Defect and Moderate to Severe Pulmonary Hypertension Using Real-Time Three-Dimensional Echocardiography

    Huan Zhou1,#, Jin Kang2,#, Jun Gao2,*, 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

    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 ventricular volume indexed to… More > Graphic Abstract

    Evaluation of Biventricular Volume and Systolic Function in Children with Ventricular Septal Defect and Moderate to Severe Pulmonary Hypertension Using Real-Time Three-Dimensional Echocardiography

  • Open Access

    ARTICLE

    Prediction of Pulmonary Arterial Pressure Level after Repair of Congenital Cardiac Communications and Discharge from the Hospital: Role of Down Syndrome and Early Postoperative Hemodynamics

    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

    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 measuring pulmonary and systemic… More >

  • Open Access

    ARTICLE

    Multi-Institutional US Experience of the Occlutech© AFR Device in Congenital and Acquired Heart Disease

    Barry O’Callaghan1, Jenny Zablah1, Joseph Vettukattil2, Daniel Levi3,4, Morris Salem4, Allison Cabalka5, Jason Anderson6, Makram Ebeid6, Ryan Alexy7, Gareth J. Morgan1,*

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

    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 each patient based… More >

  • Open Access

    ARTICLE

    Assessment of Reversibility in Pulmonary Hypertension Related to Congenital Heart Disease by Using Biomarkers and Clinical Features

    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

    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 synthase (eNOS) were… More >

  • Open Access

    ARTICLE

    Clinical Effect of an Improved Post-Operative Feeding Protocol “in Transition” Infants of Congenital Heart Disease with Pulmonary Hypertension

    Huaying He1,2, Zhiyong Lin1,2, Yuelan Weng1,2, Jianjie Zhou1,2, Man Ye1,2, Xiaowei Luo1,2, Qifeng Zhao1,2,*

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

    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 underwent family-centered (FC) feeding… More >

  • Open Access

    ARTICLE

    Plasma HGF and OPN as Potential Biomarkers of Pulmonary Arterial Hypertension in Congenital Heart Disease

    Dongdong Zheng1,#, Chi Shen1,2,#, Wenshi Liu1, Wenjing Lv1, Xiaofei Li1,*

    Congenital Heart Disease, Vol.16, No.4, pp. 373-381, 2021, DOI:10.32604/CHD.2021.015260

    Abstract Objectives: Pulmonary arterial hypertension in congenital heart disease (PAH-CHD) is the most common type of PAH and increases morbidity and mortality in patients with CHD. Right heart catheterization (RHC) is the standard method to diagnose PAH. However, RHC is an invasive and complicated method with relatively high cost. Noninvasive, feasible, and cost-efficient methods are urgently needed. The objective of this study was to evaluate three potential biomarkers of PAH-CHD: Hepatocyte growth factor (HGF), osteopontin (OPN), and suppression of tumorigenicity 2 (ST2). Methods: Plasma samples were collected from patients with CHD (n = 46) and healthy individuals (n = 22) and… More >

  • Open Access

    REVIEW

    Important Newborn Cardiac Diagnostic Dilemmas for the Neonatologist and Cardiologist–A Clinical Perspective

    Samuel Menahem1,2,*, Arvind Sehgal3,4

    Congenital Heart Disease, Vol.16, No.2, pp. 189-196, 2021, DOI:10.32604/CHD.2021.014903

    Abstract Most congenital heart disease (CHD) is readily recognisable in the newborn. Forewarned by previous fetal scanning, the presence of a murmur, tachypnoea, cyanosis and/or differential pulses and saturations all point to a cardiac abnormality. Yet serious heart disease may be missed on a fetal scan. There may be no murmur or clinical cyanosis, and tachypneoa may be attributed to non-cardiac causes. Tachypnoea on day 1 is usually non-cardiac except arising from ventricular failure or a large systemic arteriovenous fistula. A patent ductus arteriosus (PDA) may support either pulmonary or systemic duct dependent circulations. The initially high pulmonary vascular resistance (PVR)… More >

  • Open Access

    ARTICLE

    Late-Onset Pulmonary Hypertension After the Atrial Switch Procedure for Transposition of the Great Arteries

    Masataka Ogiso1,2, Kei Inai1,*, Morio Shoda2, Nobuhisa Hagiwara2, Hisashi Sugiyama1

    Congenital Heart Disease, Vol.15, No.6, pp. 483-493, 2020, DOI:10.32604/CHD.2020.013058

    Abstract Background: Pulmonary hypertension (PH) is one of the complications that can occur after the atrial switch procedure for transposition of the great arteries (TGA). This study aimed to assess the characteristics and prognosis of late-onset PH after the atrial switch procedure using catheterization data. Methods and Results: We retrospectively identified 40 patients with TGA after the atrial switch procedure that underwent catheterization between April 2007 and March 2020. Eligible patients were divided into two groups based on PH presence (PH group, n = 13 [33%]; non-PH group, n = 27 [67%]). Adverse events were defined as cardiac death and heart… More >

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