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pulmonary arterial hypertension associated with congenital heart disease

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https://www.readbyqxmd.com/read/29906223/new-mouse-model-of-pulmonary-hypertension-induced-by-respiratory-syncytial-virus-bronchiolitis
#1
Dai Kimura, Jordy Saravia, Sridhar Jaligama, Isabella McNamara, Luan D Vu, Ryan D Sullivan, Salvatore Mancarella, Dahui You, Stephania A Cormier
Pulmonary hypertension (PH) has been observed in up to 75% of infants with moderate to severe respiratory syncytial virus (RSV) bronchiolitis and is associated with significant morbidity and mortality in infants with congenital heart disease. The purpose of this study was to establish a mouse model of PH secondary to RSV bronchiolitis that mimics the disease etiology as it occurs in infants. Neonatal mice were infected with RSV at 5 days of age, and then re-infected 4 weeks later. Serum free medium was administered to age-matched mice as control...
June 15, 2018: American Journal of Physiology. Heart and Circulatory Physiology
https://www.readbyqxmd.com/read/29891239/prevalence-and-prognostic-significance-of-pulmonary-artery-aneurysms-in-adults-with-congenital-heart-disease
#2
Pastora Gallego, María José Rodríguez-Puras, Pilar Serrano Gotarredona, Israel Valverde, Begoña Manso, Antonio González-Calle, Alejandro Adsuar, Jose M Cubero, Luis Díaz de la Llera, Antonio Ordoñez, Amir-Reza Hosseinpour
BACKGROUND: Prevalence and prognostic significance of pulmonary artery (PA) dilatation in congenital heart disease (CHD) have never been studied systematically. METHODS: Chest X-rays of 1192 consecutive adults with CHD were reviewed. Major diameter of the PA was determined by imaging techniques in those with PA dilatation. A value >29 mm was considered abnormal. Data on anatomy, hemodynamics, residual lesions and outcomes were retrospectively collected. RESULTS: Overall prevalence of PA dilatation was 18%...
June 2, 2018: International Journal of Cardiology
https://www.readbyqxmd.com/read/29864909/mir-23a-regulates-the-proliferation-and-migration-of-human-pulmonary-artery-smooth-muscle-cells-hpasmcs-through-targeting-bmpr2-smad1-signaling
#3
Yanwei Zhang, Bangtian Peng, Yu Han
Pulmonary arterial hypertension (PAH) associated with congenital heart disease (CHD) (PAH-CHD) is a severe and progressive disease with a poor prognosis. MiR-23a, a member of miR-23a/24/27a cluster, has been reported to function as an important player in PAH. However, the detailed functions and molecular mechanisms of miR-23a in PAH-CHD are still not fully elucidated. Due to hypoxia is an important stimulus for pulmonary artery smooth muscle cells (PASMCs) proliferation and vascular remodeling, we assessed the expression and functions of miR-23a in hypoxia-induced HPASMCs...
July 2018: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
https://www.readbyqxmd.com/read/29843651/clinical-and-genetic-characteristics-of-pulmonary-arterial-hypertension-in-lebanon
#4
Ossama K Abou Hassan, Wiam Haidar, Georges Nemer, Hadi Skouri, Fadi Haddad, Imad BouAkl
BACKGROUND: Pulmonary arterial hypertension (PAH) is a rare disease with an incidence rate of 2-6 cases per million per year. Our knowledge of the disease in the Middle East and North Africa (MENA) region is limited by the small number of clinical studies and the complete absence of genetic studies. METHODS: Our aim was to shed light on the clinical and genetic characteristics of PAH in Lebanon and the region by using exome sequencing on PAH patients referred to the American University of Beirut Medical Center (AUBMC)...
May 30, 2018: BMC Medical Genetics
https://www.readbyqxmd.com/read/29776964/arrhythmias-in-adult-patients-with-congenital-heart-disease-and-pulmonary-arterial-hypertension
#5
Maria Drakopoulou, Heba Nashat, Aleksander Kempny, Rafael Alonso-Gonzalez, Lorna Swan, Stephen J Wort, Laura C Price, Colm McCabe, Tom Wong, Michael A Gatzoulis, Sabine Ernst, Konstantinos Dimopoulos
OBJECTIVES: Approximately 5%-10% of adults with congenital heart disease (CHD) develop pulmonary arterial hypertension (PAH), which affects life expectancy and quality of life. Arrhythmias are common among these patients, but their incidence and impact on outcome remains uncertain. METHODS: All adult patients with PAH associated with CHD (PAH-CHD) seen in a tertiary centre between 2007 and 2015 were followed for new-onset atrial or ventricular arrhythmia. Clinical variables associated with arrhythmia and their relation to mortality were assessed using Cox analysis...
May 18, 2018: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/29720395/pulmonary-arterial-hypertension-in-adult-congenital-heart-disease
#6
REVIEW
Margarita Brida, Michael A Gatzoulis
Pulmonary arterial hypertension (PAH) is commonly associated with congenital heart disease (CHD) and relates to type of the underlying cardiac defects and repair history. Large systemic to pulmonary shunts may develop PAH if untreated or repaired late. PAH, when present, markedly increases morbidity and mortality in patients with CHD. Significant progress has been made for patients with Eisenmenger syndrome in pathophysiology, prognostication and disease-targeting therapy (DTT), which needs to be applied to routine patient care...
May 2, 2018: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/29707567/hotair-is-a-potential-novel-biomarker-in-patients-with-congenital-heart-diseases
#7
Yu Jiang, Hongdan Mo, Jing Luo, Suhong Zhao, Shuang Liang, Maomao Zhang, Jie Yuan
Objective: To investigate the expression of HOX transcript antisense RNA (HOTAIR) in cardiac tissues and plasma of patients with congenital heart diseases (CHDs). Methods: qRT-PCR was used to detect the expression of HOTAIR in right atrial appendage tissues of 16 patients with CHDs and 14 patients with rheumatic valvular heart diseases (RVHDs), as well as in plasma of 36 normal people and 90 patients with CHDs including 36 cases of ASD, 23 cases of VSD, and 31 cases of PDA...
2018: BioMed Research International
https://www.readbyqxmd.com/read/29701349/pulmonary-hypertension-in-valvular-heart-disease-surgery-risk-and-prognosis
#8
Márcio Madeira, Sara Ranchordás, Paulo Oliveira, Tiago Nolasco, Marta Marques, Luis Bruges, José Calquinha, Miguel Sousa Uva, Miguel Abecasis, José Pedro Neves
INTRODUCTION: Left heart disease is the most common cause of pulmonary hypertension (PH), and when present is associated with higher surgical risk. OBJECTIVES: Analyze the effect of PH severity on morbidity, early and late mortality in patients with pulmonary artery systolic pressure (PASP) over 30mmHg that underwent valvular heart surgery. METHODS: Retrospective observational study including all patients with PH, defined as PASP>30 mmHg that underwent isolated valvular heart surgery, between 2007 and 2016...
July 2017: Revista Portuguesa de Cirurgia Cardio-torácica e Vascular
https://www.readbyqxmd.com/read/29664959/contemporary-survival-of-patients-with-pulmonary-arterial-hypertension-and-congenital-systemic-to-pulmonary-shunts
#9
Chodchanok Vijarnsorn, Kritvikrom Durongpisitkul, Paweena Chungsomprasong, Densiri Bositthipichet, Salisa Ketsara, Yuttapon Titaram, Prakul Chanthong, Supaluck Kanjanauthai, Jarupim Soongswang
OBJECTIVE: To compare survival of patients with newly diagnosed pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) according to various clinical classifications with classifications of anatomical-pathophysiological systemic to pulmonary shunts in a single-center cohort. METHODS: All prevalent cases of PAH-CHD with hemodynamic confirmation by cardiac catheterization in 1995-2015 were retrospectively reviewed. Patients who were younger than three months of age, or with single ventricle following surgery were excluded...
2018: PloS One
https://www.readbyqxmd.com/read/29655882/incidence-and-mortality-of-adults-with-pulmonary-hypertension-and-congenital-heart-disease
#10
Sara Søndergaard Schwartz, Nicolas Madsen, Henning Bækgaard Laursen, Russel Hirsch, Morten Smærup Olsen
Reports on pulmonary hypertension (PH) in the aging congenital heart disease (CHD) population are few and focused on arterial PH and patients with systemic-to-pulmonary shunts. Our objective was to estimate incidence and mortality of adult-onset PH in the CHD population. Using Danish nationwide registries, we identified all patients diagnosed with CHD from 1963 to 1974 and 1977 to 2012. Patients were matched 1:10 by birth year and gender with general population subjects. Between 1977 and 2013 adults >18 years of age were followed up until PH diagnosis, death, or emigration, whichever came first, using data from the Danish National Registry of Patients...
June 15, 2018: American Journal of Cardiology
https://www.readbyqxmd.com/read/29650544/evaluation-and-management-of-right-sided-heart-failure-a-scientific-statement-from-the-american-heart-association
#11
REVIEW
Marvin A Konstam, Michael S Kiernan, Daniel Bernstein, Biykem Bozkurt, Miriam Jacob, Navin K Kapur, Robb D Kociol, Eldrin F Lewis, Mandeep R Mehra, Francis D Pagani, Amish N Raval, Carey Ward
BACKGROUND AND PURPOSE: The diverse causes of right-sided heart failure (RHF) include, among others, primary cardiomyopathies with right ventricular (RV) involvement, RV ischemia and infarction, volume loading caused by cardiac lesions associated with congenital heart disease and valvular pathologies, and pressure loading resulting from pulmonic stenosis or pulmonary hypertension from a variety of causes, including left-sided heart disease. Progressive RV dysfunction in these disease states is associated with increased morbidity and mortality...
May 15, 2018: Circulation
https://www.readbyqxmd.com/read/29554080/characteristics-of-pulmonary-vascular-remodeling-in-a-novel-model-of-shunt-associated-pulmonary-arterial-hypertension
#12
Mingjie Zhang, Zhiyu Feng, Rui Huang, Chongrui Sun, Zhuoming Xu
BACKGROUND Establishing a shunt-induced pulmonary arterial hypertension (PAH) model in mice would be of great scientific value, but no such models have been reported to date. Here, we established a shunt-associated PAH in mice to investigate the characteristics of pulmonary vascular remodeling, which provides a new platform for the in-depth study of PAH associated with congenital heart disease (CHD). MATERIAL AND METHODS Eighty mice were randomly divided into the heavy shunt group (n=32), the small shunt group (n=32), the sham operation group (n=8), and the control group (n=8)...
March 19, 2018: Medical Science Monitor: International Medical Journal of Experimental and Clinical Research
https://www.readbyqxmd.com/read/29520672/features-associated-with-pulmonary-arterial-hypertension-in-chinese-hospitalized-systemic-lupus-erythematosus-patients
#13
Shu-Zhen Xu, Yan Liang, Xiang-Pei Li, Xiao-Mei Li, Zong-Wen Shuai, Rui-Xue Leng, Hai-Feng Pan, Dong-Qing Ye
Pulmonary arterial hypertension (PAH) is an increasingly recognized complication of systemic lupus erythematosus (SLE). This study aims to estimate the point prevalence of PAH and identify risk factors for PAH in a large cohort of hospitalized SLE patients. We have collected the medical records of patients hospitalized with SLE at the First Affiliated Hospital of Anhui Medical University and Anhui Provincial Hospital. Resting transthoracic echocardiography (TTE) was used to estimate pulmonary artery pressure (PAP) and PAH was defined as systolic PAP (PASP) > 30 mmHg...
June 2018: Clinical Rheumatology
https://www.readbyqxmd.com/read/29517668/the-efficiency-of-endothelin-receptor-antagonist-bosentan-for-pulmonary-arterial-hypertension-associated-with-congenital-heart-disease-a-systematic-review-and-meta-analysis
#14
REVIEW
Hong-Yu Kuang, Yu-Hao Wu, Qi-Jian Yi, Jie Tian, Chun Wu, We Nian Shou, Tie-Wei Lu
BACKGROUND: Oral bosentan has been widely applied in pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD). A systemic review and meta-analysis was conducted for a therapeutic evaluation of oral bosentan in both adult and pediatric patients with PAH-CHD. The acute responses and a long-term effect were respectively assessed in a comparison with baseline characteristics, and the improvement of exercise tolerance was analyzed. METHODS: PubMed, Medline, Embase, and Cochrane Central Register of clinical controlled trails or observational studies have been searched for a recording of bosentan effects on the PAH-CHD participants...
March 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29444773/lung-function-inflammation-and-endothelin-1-in-congenital-heart-disease-associated-pulmonary-arterial-hypertension
#15
Andrew Low, Sarah George, Luke Howard, Nicholas Bell, Ann Millar, Robert M R Tulloh
BACKGROUND: Breathlessness is the most common symptom in people with pulmonary arterial hypertension and congenital heart disease (CHD-APAH), previously thought to be caused by worsening PAH, but perhaps also by inflammation and abnormalities of lung function. We studied lung function and airway inflammation in patients with CHD-APAH and compared the results with controls. METHODS AND RESULTS: Sixty people were recruited into the study: 20 CHD-APAH, 20 CHD controls, and 20 healthy controls...
February 14, 2018: Journal of the American Heart Association
https://www.readbyqxmd.com/read/29436381/subcutaneous-treprostinil-in-congenital-heart-disease-related-pulmonary-arterial-hypertension
#16
Nika Skoro-Sajer, Christian Gerges, Olga Hajnalka Balint, Dora Kohalmi, Monika Kaldararova, Iveta Simkova, Johannes Jakowitsch, Harald Gabriel, Helmut Baumgartner, Mario Gerges, Roela Sadushi-Kolici, David S Celermajer, Irene Marthe Lang
OBJECTIVE: To assess the efficacy and safety of subcutaneous treprostinil in adult patients with congenital heart disease (CHD)-associated pulmonary arterial hypertension (PAH) after 12 months of treatment. METHODS: Consecutive adult patients with CHD-PAH received subcutaneous treprostinil to maximum tolerated doses in an observational study. RESULTS: Advanced CHD-PAH patients with WHO class III or IV disease (n=32, age 40±10 years, 20 females) received treprostinil for suboptimal response to bosentan (n=12), WHO functional class IV disease (FC, n=7) or prior to bosentan approval (n=13)...
February 7, 2018: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/29426959/acute-effect-of-inhaled-iloprost-in-children-with-pulmonary-arterial-hypertension-associated-with-simple-congenital-heart-defects
#17
Qiangqiang Li, Konstantinos Dimopoulos, Chen Zhang, Yan Zhu, Qian Liu, Hong Gu
Inhaled prostacyclin analogue iloprost is currently utilized in adult patients with pulmonary arterial hypertension (PAH), but little information is available on its use in the pediatric population. This study evaluated the safety and acute haemodynamic effects of inhaled iloprost in children with PAH associated with congenital heart disease (CHD). Children with PAH-CHD who underwent cardiac catheterization and iloprost administration in our catheter laboratory between June 2007 and October 2015 were included...
April 2018: Pediatric Cardiology
https://www.readbyqxmd.com/read/29340731/sensitive-cardiac-troponins-could-they-be-new-biomarkers-in-pediatric-pulmonary-hypertension-due-to-congenital-heart-disease
#18
Seyma Kayali, Ilker Ertugrul, Tamer Yoldas, Ozkan Kaya, Senem Ozgür, Utku A Orün, Selmin Karademir
To analyze the role of sensitive cardiac troponin I (scTnI) and high-sensitive troponin T (hscTnT) in the determination of myocardial injury caused by volume and pressure load due to pulmonary hypertension (PH) and to investigate if these markers may be useful in the management of PH in childhood. Twenty-eight patients with congenital heart disease (CHD) with left to right shunt and PH, 29 patients with CHD with left to right shunt but without PH, and 18 healthy children, in total 75 individuals, were included in the study...
April 2018: Pediatric Cardiology
https://www.readbyqxmd.com/read/29332925/pulmonary-artery-pseudoaneurysm-secondary-to-lung-inf-lammation
#19
Shinichirou Ishimoto, Hiroyuki Sakurai, Ryouta Higure, Riken Kawachi, Mie Shimamura
Pulmonary artery aneurysms (PAA) and pseudoaneurysms (PAP) are caused by infections, vasculitis, trauma, pulmonary hypertension, congenital heart disease, and connective tissue disease. Most cases of such aneurysm occur in the trunk or major branches of the pulmonary artery, while the peripheral type is less common. The treatment modalities are medical therapy, surgery, and percutaneous catheter embolization. The mortality rate associated with rupture is approximately 50%. We encountered a case of a 53-year-old man with a pulmonary artery pseudoaneurysm secondary to pneumonia and cavity formation during chemotherapy for acute myeloid leukemia (AML)...
January 15, 2018: Annals of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29298686/a-single-center-experience-with-12-consecutive-cases-of-pregnancy-among-patients-with-membranous-ventricular-septal-aneurysm
#20
Kana Wang, Xiaodong Wang, Haiyan Yu, Xinghui Liu, Aiyun Xing, Yong You
BACKGROUND: Membranous ventricular septal aneurysm (MVSA) is a rare cardiac anomaly that can occur as an isolated entity or being associated with other cardiac malformations. Complications of MVSA include thromboembolism, arrhythmia, rupture, bacterial endocarditis, right ventricular outflow tract obstruction, and atrioventricular valve diseases.The success rate of pregnancy and delivery in patients with MVSA has not been reported in the literature. This study was to assess the clinical implications of this condition from our center's experience...
January 3, 2018: BMC Pregnancy and Childbirth
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