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pulmonary arterial hypertension associated with congenital heart disease

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https://www.readbyqxmd.com/read/27886187/plasma-proteomic-study-in-pulmonary-arterial-hypertension-associated-with-congenital-heart-diseases
#1
Xi Zhang, Hai-Tao Hou, Jun Wang, Xiao-Cheng Liu, Qin Yang, Guo-Wei He
Pulmonary arterial hypertension associated with congenital heart disease (CHD-PAH) has serious consequence and plasma protein profiles in CHD-PAH are unknown. We aimed to reveal the differential plasma proteins in 272 CHD patients with or without PAH. Various types of CHD-PAH were studied. Differential plasma proteins were first detected by iTRAQ proteomic technology and those with significant clinical relevance were selected for further ELISA validation in new cohort of patients. Among the 190 differential plasma proteins detected by iTRAQ, carbamoyl-phosphate synthetase I (CPSI, related to urea cycle and endogenous nitric oxide production) and complement factor H-related protein 2 (CFHR2, related to complement system and coagulant mechanism) were selected for further ELISA validation in new cohort of 152 patients...
November 25, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27866929/long-term-outcomes-of-pulmonary-arterial-hypertension-under-specific-drug-therapy-in-eisenmenger-syndrome
#2
Sébastien Hascoët, Alban-Elouen Baruteau, Marc Humbert, Gérald Simonneau, Xavier Jais, Jérôme Petit, Daniela Laux, Olivier Sitbon, Virginie Lambert, André Capderou
BACKGROUND: The long-term effectiveness of pulmonary arterial hypertension-specific drug therapy (PAH-SDT) in Eisenmenger syndrome is controversial. We investigated short-term and long-term hemodynamic changes under PAH-SDT and their associations with outcomes in a bicentric cohort. METHODS: Over 20 years, we included 69 patients with congenital heart disease, an indexed pulmonary vascular resistance (PVRi) >8 WU·m(2), and 292 standardized catheterizations at baseline and after PAH-SDT initiation or intensification...
October 15, 2016: Journal of Heart and Lung Transplantation
https://www.readbyqxmd.com/read/27858232/pulmonary-hypertension-in-heart-failure-patients-pathophysiology-and-prognostic-implications
#3
REVIEW
Marco Guazzi, Valentina Labate
Pulmonary hypertension (PH) due to left heart disease (LHD), i.e., group 2 PH, is the most common reason for increased pressures in the pulmonary circuit. Although recent guidelines incorporate congenital heart disease in this classification, left-sided heart diseases of diastolic and systolic origin including valvular etiology are the vast majority. In these patients, an increased left-sided filling pressure triggers a multistage hemodynamic evolution that ends into right ventricular failure through an initial passive increase in pulmonary artery pressure complicated over time by pulmonary vasoconstriction, endothelial dysfunction, and remodeling of the small-resistance pulmonary arteries...
November 17, 2016: Current Heart Failure Reports
https://www.readbyqxmd.com/read/27801982/risk-factors-for-pulmonary-arterial-hypertension-in-children-and-young-adults
#4
Estelle Naumburg, Lars Söderström, Daniel Huber, Inge Axelsson
OBJECTIVES: Pulmonary hypertension (PH) has been linked to preterm birth explained by congenital heart defects and pulmonary diseases. WORKING HYPOTHESIS: Other factors may influence the risk of PH among adolescences and children born premature. STUDY DESIGN: This national registry-based study assess risk of PH following premature birth adjusted for known risk factors. PATIENT-SUBJECT SELECTION AND METHODOLOGY: All cases born 1993-2010, identified by diagnostic codes applicable to PH and retrieved from the Swedish Registry of Congenital Heart Disease (N = 67)...
November 1, 2016: Pediatric Pulmonology
https://www.readbyqxmd.com/read/27773412/pulmonary-hypertension-registry-of-kerala-prokerala-rationale-design-and-methods
#5
S Harikrishnan, G Sanjay, M Ashishkumar, Jaideep Menon, G Rajesh, R Krishna Kumar
BACKGROUND: Pulmonary hypertension (PH) is a disease associated with a high morbidity and mortality. There is paucity of data regarding PH from the developing countries including India. Idiopathic pulmonary arterial hypertension is the most important etiological factor in the western world, but PH secondary to rheumatic heart disease, chronic obstructive pulmonary disease and untreated congenital heart disease could well be the predominant causes in developing countries like India. The main objective of the PROKERALA study - Pulmonary hypertension Registry Of Kerala is to collect data regarding the etiology, practice patterns and one-year outcomes of patients diagnosed to have PH...
September 2016: Indian Heart Journal
https://www.readbyqxmd.com/read/27770446/the-role-of-genetics-in-pulmonary-arterial-hypertension
#6
REVIEW
Lijiang Ma, Wendy K Chung
Group 1 pulmonary hypertension or pulmonary arterial hypertension (PAH) is a rare disease characterized by proliferation and occlusion of small pulmonary arterioles, leading to progressive elevation of pulmonary artery pressure and pulmonary vascular resistance, and right ventricular failure. Historically, it has been associated with a high mortality rate, although, over the last decade, treatment has improved survival. PAH includes idiopathic PAH (IPAH), heritable PAH (HPAH), and PAH associated with certain medical conditions...
October 22, 2016: Journal of Pathology
https://www.readbyqxmd.com/read/27753979/os-12-06-effect-of-long-term-iloprost-treatment-on-the-right-ventricular-function-in-patients-with-eisenmenger-syndrome
#7
Kyoung Im Cho, Min Gu Chon
OBJECTIVE: Inhaled ilroprost can improve clinical status and symptoms in Eisenmenger syndrome (ES). Right ventricular (RV) function is important prognostic factor of pulmonary arterial hypertension. But there has been insufficient data in echocardiographic parameters related to RV function after long term iloprost therapy. So we evaluated effect of long term iloprost treatment on RV function and other parameters measured by echocardiography in this study. DESIGN AND METHOD: Eleven consecutive patients with ES associated with congenital heart disease underwent echocardiographic measurements at baseline and 48 weeks after iloprost therapy...
September 2016: Journal of Hypertension
https://www.readbyqxmd.com/read/27711045/non-invasive-inhaled-nitric-oxide-in-the-treatment-of-hypoxemic-respiratory-failure-in-term-and-preterm-infants
#8
R Sahni, X Ameer, K Ohira-Kist, J-T Wung
OBJECTIVES: Inhaled nitric oxide (iNO) is effective in conjunction with tracheal intubation (TI) and mechanical ventilation (MV) for treating arterial pulmonary hypertension and hypoxemic respiratory failure (HRF) in near-term and term newborns. Non-invasive respiratory support with nasal continuous positive airway pressure (CPAP) is increasingly used to avoid morbidity associated with TI and MV, yet the effectiveness of iNO delivery via nasal CPAP remains unknown. To evaluate the effectiveness of iNO delivered via the bubble nasal CPAP system in term and preterm newborns with HRF...
October 6, 2016: Journal of Perinatology: Official Journal of the California Perinatal Association
https://www.readbyqxmd.com/read/27683607/clinical-classification-in-pediatric-pulmonary-arterial-hypertension-associated-with-congenital-heart-disease
#9
Willemijn M H Zijlstra, Johannes M Douwes, Mark-Jan Ploegstra, Usha Krishnan, Marcus T R Roofthooft, Hans L Hillege, D Dunbar Ivy, Erika B Rosenzweig, Rolf M F Berger
Congenital heart disease (CHD) is a frequent cause of pediatric pulmonary arterial hypertension (PAH), with diverse etiology and outcome. We aimed to describe phenotypic heterogeneity in pediatric PAH associated with CHD (PAH-CHD), assess the applicability of the Nice CHD classification, and explore whether this classification accurately reflects patient/disease characteristics and survival. All children with CHD from a contemporary cohort of consecutive pediatric PAH patients followed in three major referral centers (Denver, New York, the Netherlands) were characterized and classified on the basis of the latest proposed clinical classification for PAH-CHD (World Symposium on Pulmonary Hypertension, Nice, 2013)...
September 2016: Pulmonary Circulation
https://www.readbyqxmd.com/read/27643376/os-12-06-effect-of-long-term-iloprost-treatment-on-the-right-ventricular-function-in-patients-with-eisenmenger-syndrome
#10
Kyoung Im Cho, Min Gu Chon
OBJECTIVE: Inhaled ilroprost can improve clinical status and symptoms in Eisenmenger syndrome (ES). Right ventricular (RV) function is important prognostic factor of pulmonary arterial hypertension. But there has been insufficient data in echocardiographic parameters related to RV function after long term iloprost therapy. So we evaluated effect of long term iloprost treatment on RV function and other parameters measured by echocardiography in this study. DESIGN AND METHOD: Eleven consecutive patients with ES associated with congenital heart disease underwent echocardiographic measurements at baseline and 48 weeks after iloprost therapy...
September 2016: Journal of Hypertension
https://www.readbyqxmd.com/read/27587546/genetic-analyses-in-a-cohort-of-children-with-pulmonary-hypertension
#11
Marilyne Levy, Mélanie Eyries, Isabelle Szezepanski, Magalie Ladouceur, Sophie Nadaud, Damien Bonnet, Florent Soubrier
The prevalence of germline mutations in paediatric pulmonary hypertension (PH) is poorly documented. The objective of this study was to determine the mutation frequency in PH genes in a paediatric cohort and describe the clinical characteristics of mutation carriers.The study involved 66 index cases with PH: 35 children with idiopathic pulmonary arterial hypertension (IPAH); five children with familial PAH (FPAH); three children with pulmonary veno-occlusive disease (PVOD); and 23 children with PAH associated with congenital heart disease (APAH-CHD)...
October 2016: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/27583409/new-guidelines-for-managing-pulmonary-hypertension-what-the-pediatrician-needs-to-know
#12
Steven H Abman
PURPOSE OF REVIEW: Pediatric pulmonary vascular disease contributes to morbidities and death in diverse clinical settings, ranging from idiopathic or heritable forms of pediatric arterial hypertension, congenital heart disease, developmental lung disorders, chronic lung disease, left heart disease, sickle cell disease, oncologic disease, and systemic disorders. Despite its impact on the clinical courses in so many diseases, information is limited on how to best approach the diagnosis and evaluation of pediatric pulmonary hypertension...
October 2016: Current Opinion in Pediatrics
https://www.readbyqxmd.com/read/27575388/abnormal-muscularization-of-intra-acinar-pulmonary-arteries-in-two-cases-presenting-as-sudden-infant-death-sids
#13
Khairul Zainun, Kirsten Hope, Andrew G Nicholson, Marta Cecilia Cohen
Abnormal muscularization of intra acinar pulmonary arteries is a histological hallmark of persistent pulmonary hypertension of newborn (PPHN), an uncommon disease with high morbidity and mortality. PPHN presents with signs of respiratory distress immediately following birth. The disease is multi-factorial in origin and can be idiopathic as well associated with a variety of conditions such as congenital heart disease and both congenital and acquired lung diseases. We report two cases presenting as sudden unexpected death in late neonatal period (SUDI) showing abnormal muscularization of acinar pulmonary arteries reminiscent of PPHN...
August 30, 2016: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/27530086/gas-exchange-responses-during-6-min-walk-test-in-patients-with-pulmonary-arterial-hypertension
#14
Norman R Morris, Helen Seale, Julie Harris, Kathleen Hall, Aaron C W Lin, Fiona Kermeen
BACKGROUND AND OBJECTIVE: The 6-min walk test (6MWT) is the most widely utilized method of assessing exercise capacity in pulmonary arterial hypertension (PAH). Cardiopulmonary exercise testing has the advantage of providing additional physiological information over 6MWT. The goals of our study were to describe the addition of gas exchange measurements to 6MWT and to determine how these parameters were related to the severity of PAH in three major subgroups of PAH (idiopathic (IPAH), connective tissue disease-related (CTPAH) and congenital heart disease-related (CHPAH))...
August 16, 2016: Respirology: Official Journal of the Asian Pacific Society of Respirology
https://www.readbyqxmd.com/read/27511447/pregnancy-outcomes-in-patients-with-pulmonary-arterial-hypertension-associated-with-congenital-heart-disease
#15
Magalie Ladouceur, Louise Benoit, Jelena Radojevic, Adeline Basquin, Claire Dauphin, Sébastien Hascoet, Pamela Moceri, Charlene Bredy, Laurence Iserin, Marielle Gouton, Jacky Nizard
OBJECTIVE: There is growing evidence that maternal mortality in pregnant women with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) is lower than that in available data. In order to evaluate this hypothesis, we collected data of pregnancies in women with PAH-CHD. METHODS: Women with PAH-CHD followed in seven French referral centres were retrospectively included from 1997 to 2015. All pregnancies were recorded. We collected data on maternal, obstetrical and neonatal outcomes...
August 10, 2016: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/27505326/association-between-levels-of-anti-angiogenic-isoform-of-vascular-endothelial-growth-factor-a-and-pulmonary-hypertension
#16
Satoshi Suzuki, Akiomi Yoshihisa, Tetsuro Yokokawa, Tomofumi Misaka, Nobuo Sakamoto, Koichi Sugimoto, Takayoshi Yamaki, Hiroyuki Kunii, Kazuhiko Nakazato, Shu-Ichi Saitoh, Yasuchika Takeishi
BACKGROUNDS: Pulmonary hypertension (PH) is characterized by elevated pulmonary arterial pressure due to vasoconstriction and remodeling of the pulmonary microvasculature. Vascular endothelial growth factor (VEGF) is a key contributor for angiogenesis and vasculogenesis. VEGF165b is recently identified as an anti-angiogenic splicing variant of VEGF. The aim of this study was to examine the association between circulating levels of VEGF165b in PH patients under consideration with classifications of PH...
November 1, 2016: International Journal of Cardiology
https://www.readbyqxmd.com/read/27504381/illustrated-imaging-essay-on-congenital-heart-diseases-multimodality-approach-part-ii-acyanotic-congenital-heart-disease-and-extracardiac-abnormalities
#17
REVIEW
Venkatraman Bhat, Vinay Belaval, Karthik Gadabanahalli, Vimal Raj, Sejal Shah
Acyanotic heart disease constitutes a significant majority of patient who may present with non-cardiac symptoms. Either they are detected incidentally or present with respiratory complaints. Equipped with knowledge of anatomy by echocardiography and radiographic methods described in previous part of this presentation, diagnosis may be confidently attempted. On plain radiography acyanotic congenital heart diseases have variable appearance depending upon severity of disease. Cardiac size, chamber enlargement and pulmonary vascular pattern are key elements...
June 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/27492657/effects-of-long-term-iloprost-treatment-on-right-ventricular-function-in-patients-with-eisenmenger-syndrome
#18
Min Ku Chon, Kyoung Im Cho, Kwang Soo Cha, Jeong Sook Seo, Dong Soo Kim
BACKGROUND: Right ventricular (RV) function is an important prognostic factor of pulmonary arterial hypertension (PAH), but there is insufficient data regarding RV function after long-term inhaled iloprost treatment. We evaluated the effect of long-term iloprost treatment on RV function in patients with Eisenmenger syndrome (ES). METHODS: Eleven consecutive patients with ES associated with congenital heart disease underwent echocardiographic measurements at baseline and 48 weeks after iloprost therapy...
August 1, 2016: Journal of Cardiology
https://www.readbyqxmd.com/read/27404676/the-causes-treatment-and-outcome-of-pulmonary-hypertension-in-africa-insights-from-the-pan-african-pulmonary-hypertension-cohort-papuco-registry
#19
Friedrich Thienemann, Anastase Dzudie, Ana O Mocumbi, Lori Blauwet, Mahmoud U Sani, Kamilu M Karaye, Okechukwu S Ogah, Irina Mbanze, Amam Mbakwem, Patience Udo, Kemi Tibazarwa, Albertino Damasceno, Ashley K Keates, Simon Stewart, Karen Sliwa
BACKGROUND: Epidemiology, aetiology, management and outcome data for various forms of pulmonary hypertension (PH) in Africa are scarce. METHODS: A prospective, multinational cohort registry of 220 consecutive patients (97% of African descent) from 9 specialist centres in 4 African countries. The antecedents, characteristics and management of newly diagnosed PH plus 6-month survival were studied. RESULTS: There were 209 adults (median age 48years [IQR 35, 64]) and 11 children (age range 1 to 17years)...
October 15, 2016: International Journal of Cardiology
https://www.readbyqxmd.com/read/27384461/pulmonary-hypertension-and-pregnancy-outcomes-data-from-the-registry-of-pregnancy-and-cardiac-disease-ropac-of-the-european-society-of-cardiology
#20
Karen Sliwa, Iris M van Hagen, Werner Budts, Lorna Swan, Gianfranco Sinagra, Maryanne Caruana, Manuel Vazquez Blanco, Lodewijk J Wagenaar, Mark R Johnson, Gary Webb, Roger Hall, Jolien W Roos-Hesselink
AIMS: To describe the outcomes of pregnancy in women with pulmonary hypertension. METHODS AND RESULTS: In 2007 the European Registry on Pregnancy and Heart Disease was initiated by the European Society of Cardiology. Consecutive patients with all forms of cardiovascular disease, presenting with pregnancy, were enrolled with the aim of investigating the pregnancy outcomes. This subgroup of the cohort included 151 women with pulmonary hypertension (PH) either diagnosed by right heart catheterization or diagnosed as possible PH by echocardiographic signs, with 26% having pulmonary arterial hypertension (PAH), in three subgroups: idiopathic (iPAH), associated with congenital heart disease (CHD-PAH), or associated with other disease (oPAH), and 74% having PH caused by left heart disease (LHD-PH, n = 112)...
September 2016: European Journal of Heart Failure
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