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JOURNAL ARTICLE
Wenshi Liu, Ying Hua, Dongdong Zheng, Wenjing Lv, Wei Zhang, Qianqian Chen, Rong Huang, Xiaofei Li
BACKGROUND: Connective tissue growth factor (CTGF) has diagnostic value for pulmonary arterial hypertension (PAH) associated with congenital heart disease (CHD) in children; however, its value in adult patients remains unclear. This study evaluated CTGF as a biomarker in adult PAH-CHD patients.Methods and Results: Based on mean pulmonary artery pressure (mPAP), 56 CHD patients were divided into 3 groups: without PAH (W; mPAP <25 mmHg; n=28); mild PAH (M; mPAP 25-35 mmHg; n=18); and moderate and severe PAH (H; mPAP ≥35 mmHg; n=10)...
February 25, 2023: Circulation Journal: Official Journal of the Japanese Circulation Society
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JOURNAL ARTICLE
Jun Yasuhara, Kae Watanabe, Atsuyuki Watanabe, Takuro Shirasu, Yuichi Matsuzaki, Hirofumi Watanabe, Hisato Takagi, Naokata Sumitomo, Toshiki Kuno
The optimal treatment strategy using pulmonary vasodilators in pulmonary arterial hypertension associated with CHD (PAH-CHD) remains controversial. We aimed to compare the efficacy and safety of pulmonary vasodilators in PAH-CHD. PubMed and EMBASE databases were searched through May 2022 and a network meta-analysis was conducted. The primary outcomes were mean difference of changes in 6-minute walk distance, NYHA functional class, and N-terminal pro-brain natriuretic peptide. The secondary outcomes included pulmonary vascular resistance, mean pulmonary arterial pressure, and resting oxygen saturation...
November 2023: Cardiology in the Young
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JOURNAL ARTICLE
Hong-Da Zhang, Yi Yan, Yang-Yang He, Qian-Qian Liu, Fu-Hua Peng, Xin-Xin Yan, Zhi-Yan Han
BACKGROUND: Exercise intolerance is a major manifestation of pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD). We aimed to investigate the characteristics of exercise intolerance in different subgroups of PAH-CHD. METHODS: We retrospectively enrolled 171 adult patients with PAH-CHD and 30 age and sex-matched healthy subjects and performed cardiopulmonary exercise testing. Gas exchange parameters, including peak oxygen uptake (peak V̇o2 ), anaerobic threshold, and the slope of ventilatory equivalent for carbon dioxide (V̇e/V̇co2 slope), were recorded...
December 21, 2022: International Journal of Cardiology
#24
JOURNAL ARTICLE
Kellen Hirsch, Stephanie Nolley, David D Ralph, Ying Zheng, William A Altemeier, Christopher J Rhodes, Nicholas W Morrell, Martin R Wilkins, Peter J Leary, Samuel G Rayner
BACKGROUND: Subtypes of pulmonary arterial hypertension (PAH) differ in both fundamental disease features and clinical outcomes. Angiogenesis and inflammation represent disease features that may differ across subtypes and are of special interest in connective tissue disease-associated PAH (CTD-PAH). We compared inflammatory and angiogenic biomarker profiles across different etiologies of PAH and related them to clinical outcomes. METHODS: Participants with idiopathic PAH, CTD-PAH, toxin-associated PAH (tox-PAH), or congenital heart disease-associated PAH (CHD-PAH) were enrolled into a prospective observational cohort...
February 2023: Journal of Heart and Lung Transplantation
#25
JOURNAL ARTICLE
Katharina Strack, Sebastian Freilinger, Amely J Busse, Peter Ewert, Michael Hauser, Michael Huntgeburth, Ann-Sophie Kaemmerer, Nicole Nagdyman, Judith Schopen, Harald Kaemmerer, Fabian von Scheidt
BACKGROUND: Pulmonary arterial hypertension (PAH), a common complication in adults with congenital heart disease (CHD), leads to significant morbidity and mortality. Targeted PAH medication is available, but PAH-CHD patient data are limited. Several questions regarding indication, treatment escalation, and combination therapy remain unanswered. The aim of this study was therefore to evaluate PAH-specific treatment in adults with PAH-CHD to better understand PAH-specific therapy management...
October 2022: Cardiovascular Diagnosis and Therapy
#26
JOURNAL ARTICLE
Beizhu Xu, Caihua Huang, Caojin Zhang, Donghai Lin, Weifeng Wu
Patients with unrepaired congenital heart disease (CHD) are prone to pulmonary arterial hypertension (PAH). The ovine pulmonary arterial smooth muscle cells exposed to increased pulmonary blood flow (PBF) exhibited hyperproliferation and metabolic alterations, but the metabolic disorders of patients with CHD and associated PAH (PAH-CHD) have not yet been fully understood. Adult CHD patients were prospectively included and divided into the PAH-CHD group ( n = 24) and CHD group ( n = 38), while healthy adults were included as healthy control (HC) group ( n = 29)...
September 8, 2022: Metabolites
#27
REVIEW
Targeted Therapies in Patients with Pulmonary Arterial Hypertension Due to Congenital Heart Disease.
Adriana Mares, Debabrata Mukherjee, Richard A Lange, Nils P Nickel
Pulmonary arterial hypertension (PAH) is a devastating cardiovascular disease leading to right heart failure and death if untreated. Medical therapies for PAH have evolved substantially over the last decades and are associated with improvements in functional class, quality of life, and survival. PAH-targeted therapies now consist of multiple inhaled, oral, subcutaneous, and intravenous therapies targeting the phosphodiesterase, guanylate cyclase, endothelin and prostacyclin pathways. Patients with congenital heart disease (CHD) are at high risk of developing PAH and growing evidence exists that PAH-targeted therapy can be beneficial in PAH-CHD...
2022: Current Vascular Pharmacology
#28
JOURNAL ARTICLE
Qing-Fan Lin, Jing-Hong Rao, Shi-Mu Luo, Qing-Mu Wang, Li-Feng Deng, Xuan Chen, Chang-Di Chen, You-Fang Chen
OBJECTIVE: To investigate whether endothelial nitric oxide synthase ( eNOS ) rs1799983, rs2070744, and rs61722009 gene polymorphisms are associated with pulmonary arterial hypertension (PAH) in South Fujian newborns with congenital heart disease (CHD). METHODS: Genotyping for the eNOS rs1799983, rs2070744, and rs61722009 polymorphisms was performed using Sanger sequencing in 50 newborns with PAH secondary to CHD [CHD PAH (+)], 52 newborns with CHD without PAH [CHD PAH (-)], and 60 healthy controls...
August 18, 2022: Clinical and Experimental Hypertension: CHE
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JOURNAL ARTICLE
Yang Liu, Yanna Li, Jun Zhang, Dawei Zhang, Jiachen Li, Yichen Zhao, Kemin Liu, Xiaolong Ma, Chen Bai, Hong Gu, Xiangming Fan, Jiangang Wang
As pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) may increase maternal and fetal risk, this study explored the pregnancy outcomes of Chinese women with PAH-CHD. The clinical data of pregnant women with PAH-CHD admitted to the Beijing Anzhen Hospital from 2010 to 2019 were retrospectively analyzed; these patients and their offspring were followed up, with a mean period of 5.9 ± 2.7 years. Overall, 260 patients with PAH-CHD were included. The mean maternal age was 27...
April 2022: Pulmonary Circulation
#30
JOURNAL ARTICLE
YunTao Li, Mingwui Tung, Jianping Xu, Guangyu Pan, Rui Jiang
BACKGROUND: Vasopressin can constrict peripheral arteries without constricting the pulmonary artery. Theoretically, vasopressin is suitable for the perioperative treatment of pulmonary hypertension. Few studies have investigated the use of pituitrin (a substitute for vasopressin) after cardiac defect repair surgery. This study aimed to analyze the effect of pituitrin on hemodynamics and to determine whether pituitrin can be used after surgical repair in adult patients with pulmonary arterial hypertension-congenital heart disease (PAH-CHD)...
April 21, 2022: Heart Surgery Forum
#31
JOURNAL ARTICLE
Virginija Rudienė, Lina Kaplerienė, Dovilė Jančauskaitė, Emilija Meškėnė, Eglė Palevičiūtė, Monika Laukytė-Slėnienė, Diana Gasiūnaitė, Diana Ramašauskaitė, Elena Jurevičienė, Lina Gumbienė
Background and Objectives : Pregnancy and delivery in patients with congenital heart disease (CHD) and pulmonary arterial hypertension (PAH) carry a very high risk for maternal and foetal complications and are contraindicated according to the guidelines. In the last decades, when an available modern PAH-targeted medication therapy and a new management concept improved patients' well-being and survival, some PAH-CHD females decided to conceive. Of note, despite advanced treatment and modern healthcare system possibilities, dealing with pregnancy in a diverse PAH-CHD population is still challenging...
March 25, 2022: Medicina
#32
JOURNAL ARTICLE
Ann-Sophie Kaemmerer, Matthias Gorenflo, Dörte Huscher, David Pittrow, Peter Ewert, Christine Pausch, Marion Delcroix, Hossein A Ghofrani, Marius M Hoeper, Rainer Kozlik-Feldmann, Andris Skride, Gerd Stähler, Carmine Dario Vizza, Elena Jureviciene, Dovile Jancauskaite, Lina Gumbiene, Ralf Ewert, Ingo Dähnert, Matthias Held, Michael Halank, Dirk Skowasch, Hans Klose, Heinrike Wilkens, Katrin Milger, Christian Jux, Martin Koestenberger, Laura Scelsi, Eva Brunnemer, Michael Hofbeck, Silvia Ulrich, Anton Vonk Noordegraaf, Tobias J Lange, Leonhard Bruch, Stavros Konstantinides, Martin Claussen, Judith Löffler-Ragg, Hubert Wirtz, Christian Apitz, Rhoia Neidenbach, Sebastian Freilinger, Attila Nemes, Christian Opitz, Ekkehard Grünig, Stephan Rosenkranz
BACKGROUND: Pulmonary arterial hypertension (PAH) is common in congenital heart disease (CHD). Because clinical-trial data on PAH associated with CHD (PAH-CHD) remain limited, registry data on the long-term course are essential. This analysis aimed to update information from the COMPERA-CHD registry on management strategies based on real-world data. METHODS: The prospective international pulmonary hypertension registry COMPERA has since 2007 enrolled more than 10,000 patients...
December 2021: Cardiovascular Diagnosis and Therapy
#33
JOURNAL ARTICLE
Jingyuan Chen, Anandharajan Rathinasabapathy, Jun Luo, Xiaojie Yang, Peng Luo, Yusi Chen, Zilu Li, Jiang Li
Lipid homeostasis is dysregulated in pulmonary arterial hypertension (PAH). A decrease in serum high- and low-density lipoprotein cholesterol (HDL-C and LDL-C) is significantly associated with the worse prognosis of PAH. However, no study has investigated the differential distribution of lipids in various PAH subtypes. We enrolled 190 patients in this retrospective study, which includes 20 patients with congenital heart disease without PAH (CHD-nonPAH), 101 patients with PAH associated with congenital heart disease (CHD-PAH), 69 patients with idiopathic PAH (IPAH) and 81 healthy controls...
January 2022: Respiratory Medicine
#34
REVIEW
Sarah A Goldstein, Richard A Krasuski
Pulmonary arterial hypertension related to congenital heart disease (PAH-CHD) affects 5% to 10% of adults with CHD and is associated with significant morbidity and mortality. PAH-CHD develops as a consequence of intracardiac or extracardiac systemic-to-pulmonary shunts that lead to pulmonary vascular remodeling through a pathologic process that is similar to other causes of PAH. Eisenmenger syndrome is the most severe phenotype of PAH-CHD and is characterized by severe elevation in pulmonary vascular resistance, with shunt reversal causing hypoxemia and central cyanosis...
February 2022: Cardiology Clinics
#35
MULTICENTER STUDY
Amadeo-José Wals-Rodriguez, Maria-Jose Rodriguez-Puras, Rocío Garcia-Orta, Juan Robledo, Eduardo Moreno, Carmen Federero, Rocio Camacho, Begoña Manso, Nuria Hernandez, Jose María Cubero, Raquel Ladrón Abia, Pilar Cejudo Ramos, Antonio Ordoñez, Pastora Gallego
BACKGROUND: Mortality of pulmonary hypertension associated with congenital heart disease (PAH-CHD) in adults remains high. OBJECTIVES: To identify predictors of death and to assess the impact of treatment on outcome. METHODS: Retrospective, multicenter cohort study of 103 adults with PAH-CHD followed-up for 8.6 ± 4.6 years. Patients were grouped according to underlying shunt type into pre-tricuspid, post-tricuspid and complex...
2022: Heart & Lung: the Journal of Critical Care
#36
JOURNAL ARTICLE
Melody L Dong, Ingrid S Lan, Weiguang Yang, Marlene Rabinovitch, Jeffrey A Feinstein, Alison L Marsden
Untreated ventricular septal defects (VSDs) can lead to pulmonary arterial hypertension (PAH) characterized by elevated pulmonary artery (PA) pressure and vascular remodeling, known as PAH associated with congenital heart disease (PAH-CHD). Though previous studies have investigated hemodynamic effects on vascular mechanobiology in late-stage PAH, hemodynamics leading to PAH-CHD initiation have not been fully quantified. We hypothesize that abnormal hemodynamics from left-to-right shunting in early stage VSDs affects PA biomechanical properties leading to PAH initiation...
September 28, 2021: Biomechanics and Modeling in Mechanobiology
#37
REVIEW
Matthias Gorenflo, Victoria C Ziesenitz
Pulmonary arterial hypertension (PAH) is a devastating illness causing already significant morbidity in childhood. Currently approved treatment options for children comprise the endothelin receptor antagonist bosentan, as well as the phosphodiesterase-5 inhibitor sildenafil. But PAH treatment has advanced significantly over the past decade, and new classes of targeted drug therapies, such as stimulators of the soluble guanylate cyclase (riociguat) or prostacyclin receptor agonists (selexipag), are currently evaluated regarding their efficacy and safety in children, in order to limit off-label use...
August 2021: Cardiovascular Diagnosis and Therapy
#38
REVIEW
Katrijn Jansen, Andrew Constantine, Robin Condliffe, Robert Tulloh, Paul Clift, Shahin Moledina, S John Wort, Konstantinos Dimopoulos
INTRODUCTION: Pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) is a progressive, life-limiting disease. AREAS COVERED: In this paper, we review the classification and pathophysiology of PAH-CHD, including the mechanisms of disease progression and multisystem effects of disease. We evaluate current strategies of risk stratification and the use of biological markers of disease severity, and review principles of management of PAH-CHD...
September 2021: Expert Review of Cardiovascular Therapy
#39
REVIEW
Hassaan B Arshad, Valeria E Duarte
Pulmonary arterial hypertension is a common complication in patients with congenital heart disease (CHD), aggravating the natural course of the underlying defect. Pulmonary arterial hypertension (PAH) has a multifactorial etiology depending on the size and nature of the cardiac defect as well as environmental factors. Although progress has been made in disease-targeting therapy using pulmonary vasodilators to treat Eisenmenger syndrome, important gaps still exist in the evaluation and management of adult patients with CHD-associated PAH (PAH-CHD) who have systemic-to-pulmonary shunts...
2021: Methodist DeBakey Cardiovascular Journal
#40
JOURNAL ARTICLE
Nadya Arafuri, Indah K Murni, Nikmah S Idris, Cuno S P M Uiterwaal, Ary I Savitri, Sasmito Nugroho, Noormanto Noormanto
Background: In low-to-middle-income countries, repair of the left-to-right shunts congenital heart disease (CHD) are often done with existing pulmonary arterial hypertension (PAH). Long-term outcomes data of this condition in either low-to-middle or high-income countries are limited. We conducted a study to evaluate the outcomes of children with PAH related to left-to-right shunt CHD who underwent surgical or transcatheter repair. Methods: All cases of PAH related to left-to-right shunt CHD repairs from 2015-2018 were retrospectively reviewed with additional new patients who underwent repair within our study period (2019-2020)...
April 21, 2021: Global Heart
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