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PAH-CHD

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https://www.readbyqxmd.com/read/29136712/-long-term-outcome-and-prognostic-factors-in-pregnant-women-with-pulmonary-arterial-hypertension-associated-with-congenital-heart-disease
#1
Q T Ou, J K Lu, J Zhang, Y Chen, Q Li, J L Zhang
Objective: To investigate the perinatal outcome, risk factors and long-term outcome of pregnancy complicated with pulmonary arterial hypertension(PAH) and congenital heart diseases (CHD). Methods: Clinical data of 110 pregnant women who were diagnosed as PAH-CHD were retrospectively analyzed in the Department of Obstetrics and Gynecology and Surgical Intensive Care Unit at Beijing Anzhen Hospital from 2004 to 2013. The survival and treatment status were followed up. Results: 110 subjects consisted of 11 mild PAH, 33 moderate and 66 severe ones...
November 1, 2017: Zhonghua Nei Ke za Zhi [Chinese Journal of Internal Medicine]
https://www.readbyqxmd.com/read/28819713/plasma-growth-differentiation-factor-15-is-a-potential-biomarker-for-pediatric-pulmonary-arterial-hypertension-associated-with-congenital-heart-disease
#2
Gang Li, Yan Li, Xiao-Qiu Tan, Peng Jia, Jian Zhao, Dong Liu, Ting Wang, Bin Liu
We aimed to investigate plasma growth differentiation factor-15 (GDF-15) levels in pediatric pulmonary arterial hypertension secondary to congenital heart disease (PAH-CHD), and assess the association with hemodynamic parameters. Plasma GDF-15 levels were measured in children with PAH-CHD (n = 46) and compared to children with CHD without PAH (n = 39). Normal individuals (n = 30) served as health control group. Plasma GDF-15 levels were significantly elevated in patients with PAH-CHD compared with those with CHD without PAH (median 1415 ng/L, interquartile range [IQR] 926...
December 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28681131/hemodynamic-evaluation-of-children-with-persistent-or-recurrent-pulmonary-arterial-hypertension-following-complete-repair-of-congenital-heart-disease
#3
Heiner Latus, Inken Wagner, Stefan Ostermayer, Gunter Kerst, Joachim Kreuder, Dietmar Schranz, Christian Apitz
Persistent or recurrent pulmonary arterial hypertension (PAH) following complete surgical repair of congenital heart disease (CHD) represents one of the largest group of PAH associated with CHD (PAH-CHD) in recent registry studies and seems to have a particularly poor prognosis. However, little is known about this fourth clinical subclass of PAH-CHD, especially in children. The purpose of this study was to assess specific characteristics of invasive hemodynamics of this disease in children, including acute vasodilator testing (AVT) and pulmonary endothelial function (PEF) and to compare to patients with idiopathic PAH (IPAH), who usually present with a similar fatal clinical course...
July 5, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28608969/effect-of-iloprost-on-biomarkers-in-patients-with-congenital-heart-disease-pulmonary-arterial-hypertension
#4
Xiao-Ye Li, Yu Zheng, Yuliang Long, Xiaochun Zhang, Lei Zhang, Dan Tian, Daxin Zhou, Qian-Zhou Lv
Some biomarkers play important roles in the endothelial dysfunction of patients with pulmonary arterial hypertension (PAH), including nitric oxide (NO), endothelin-1 (ET-1), asymmetric dimethylarginine (ADMA), galectin-3 (Gal-3), B-type natriuretic peptide (BNP), and uric acid (UA). However, studies on these biomarkers in pulmonary artery blood in congenital heart disease-PAH (CHD-PAH) and the effect of iloprost on the regulation of biomarkers are lacking. This study investigated potential CHD-PAH biomarkers and their association with the severity of disease...
September 2017: Clinical and Experimental Pharmacology & Physiology
https://www.readbyqxmd.com/read/28606655/mortality-in-pulmonary-arterial-hypertension-due-to-congenital-heart-disease-serial-changes-improve-prognostication
#5
M T U Schuijt, I M Blok, A H Zwinderman, A C M J van Riel, M J Schuuring, R J de Winter, A L Duijnhouwer, A P J van Dijk, B J M Mulder, B J Bouma
BACKGROUND: Adult patients with pulmonary arterial hypertension due to congenital heart disease (PAH-CHD) suffer from high mortality. This underlines the importance of adequate risk stratification to guide treatment decisions. Several baseline parameters are associated with mortality, however, their prognostic value may weaken after years of follow-up. Therefore we investigated the prognostic value of serial changes in standard clinical parameters in PAH-CHD. METHODS: In this prospective observational cohort study we included consecutive PAH-CHD adults, between 2005 and 2016...
September 15, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28369399/pulmonary-arterial-hypertension-in-congenital-heart-disease-translational-opportunities-to-study-the-reversibility-of-pulmonary-vascular-disease
#6
Diederik E van der Feen, B Bartelds, Rudolf A de Boer, Rolf M F Berger
Pulmonary arterial hypertension (PAH) is a progressive and lethal pulmonary vascular disease (PVD). Although in recent years outcome has improved by new treatments that delay disease progression, a cure has not yet been achieved. In PAH associated with congenital heart disease (CHD), remodeling of the pulmonary vasculature reaches an irreversible phenotype similar to all forms of end-stage PAH. In PAH-CHD, however, also an early stage is recognised, which can be completely reversible. This reversible phase has never been recognised in other forms of PAH, most likely because these patients are only diagnosed once advanced disease has developed...
July 7, 2017: European Heart Journal
https://www.readbyqxmd.com/read/28358329/the-changing-landscape-of-pulmonary-arterial-hypertension-in-the-adult-with-congenital-heart-disease
#7
REVIEW
Alexandra C van Dissel, Barbara J M Mulder, Berto J Bouma
Pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) is a common type of pulmonary arterial hypertension (PAH) and a frequent complication of congenital heart disease (CHD). PAH-CHD represents a heterogeneous patient population and it is important to distinguish between the underlying cardiac defects considering the prognostic and therapeutic implications. Improved interventional techniques have enabled repair or palliation of most cardiac defects, though a substantial number of patients remain at high risk for PAH after closure...
March 30, 2017: Journal of Clinical Medicine
https://www.readbyqxmd.com/read/28283462/real-world-long-term-survival-of-incident-patients-with-pulmonary-arterial-hypertension
#8
P Marques-Alves, R Baptista, A Marinho da Silva, M Pêgo, G Castro
BACKGROUND: Pulmonary arterial hypertension (PAH) is a progressive, fatal disease. Long-term outcomes data are scarce in Portugal. We aimed to estimate survival of newly diagnosed PAH at a Portuguese referral center in the modern management era. METHODS: Between January 2009 and November 2015 all incident PAH cases were consecutively enrolled in a prospective cohort study. Sixty-five patients were followed up for a median of 3.1 [interquartile range 1.7-5.4] years...
May 2017: Revista Portuguesa de Pneumologia
https://www.readbyqxmd.com/read/28054902/novel-goal-directed-hemodynamic-optimization-therapy-based-on-major-vasopressor-during-corrective-cardiac-surgery-in-patients-with-severe-pulmonary-arterial-hypertension-a-pilot-study
#9
RANDOMIZED CONTROLLED TRIAL
Shuwen Li, Qing Ma, Yanwei Yang, Jiakai Lu, Zhiquan Zhang, Mu Jin, Weiping Cheng
INTRODUCTION: Pulmonary arterial hypertension (PAH) is a common and fatal complication of congenital heart disease (CHD). PAH-CHD increases the risk for postoperative complications. Recent evidence suggests that perioperative goal-directed hemodynamic optimization therapy (GDHOT) significantly improves outcomes in surgery patients. Standard GDHOT is based on major solution volume, vasodilators and inotropic therapy, while novel GDHOT is based on major vasopressor and inotropic therapy...
December 22, 2016: Heart Surgery Forum
https://www.readbyqxmd.com/read/27683607/clinical-classification-in-pediatric-pulmonary-arterial-hypertension-associated-with-congenital-heart-disease
#10
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/27642135/pulmonary-arterial-hypertension-in-congenital-heart-disease-current-perspectives-and-future-challenges
#11
George Giannakoulas, Michael A Gatzoulis
Medical and scientific research in the field of pulmonary arterial hypertension (PAH) in adults with congenital heart disease (ACHD) has gradually become globalized, inclusive and collaborative over the past few years. The education of physicians, health administrators and patients on congenital heart disease (CHD), specifically in the field of PAH, is of paramount importance. It is also crucial for ACHD patients with PAH to be followed in tertiary centers and to benefit from a multidisciplinary approach. Shared care models dictate a closer collaboration between tertiary expert centers and local non-specialist services, as well as networking between expert physicians in CHD and PAH and geneticists/epidemiologists, with the inclusion of PAH-CHD patients in national and international registries with a detailed genotypic/phenotypic characterization...
August 20, 2016: Hellenic Journal of Cardiology: HJC, Hellēnikē Kardiologikē Epitheōrēsē
https://www.readbyqxmd.com/read/27568461/elevated-serum-hmgb1-in-pulmonary-arterial-hypertension-secondary-to-congenital-heart-disease
#12
Yi-Yuan Huang, Wei Su, Zhao-Wei Zhu, Liang Tang, Xin-Qun Hu, Sheng-Hua Zhou, Zhen-Fei Fang, Jiang Li
AIMS: This study investigated the potential value of serum high mobility group box-1 (HMGB1) level in the diagnosis, staging and treatment response of patients with pulmonary arterial hypertension secondary to congenital heart disease (PAH-CHD). METHODS AND RESULTS: This was a single-center prospective study in 106 CHD patients. Serum HMGB1 levels were measured by enzymelinked immunosorbent assay. HMGB1 levels were significantly increased in patients with PAH compared to patients without PAH (P<0...
October 2016: Vascular Pharmacology
https://www.readbyqxmd.com/read/27511447/pregnancy-outcomes-in-patients-with-pulmonary-arterial-hypertension-associated-with-congenital-heart-disease
#13
MULTICENTER STUDY
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...
February 15, 2017: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/27443136/pulmonary-hypertension-and-congenital-heart-disease
#14
REVIEW
Todd S Roth, Jamil A Aboulhosn
Pulmonary arterial hypertension in congenital heart disease (PAH-CHD) is a frequent complication in adults with congenital heart disease. Regardless of etiology, the optimal treatment strategy for this difficult population is challenging. The new frontier of targeted PAH therapies has demonstrated improved functional capacity in the various phenotypes of PAH-CHD, with work currently in progress scrutinizing outcomes. In those who fail conventional medical therapy, heart and heart-lung (block) transplantation become the final therapeutic options, with the role of ventricular assist devices and the total artificial heart still under investigation in this group...
August 2016: Cardiology Clinics
https://www.readbyqxmd.com/read/27122915/insight-into-pulmonary-arterial-hypertension-associated-with-congenital-heart-disease-pah-chd-classification-and-pharmacological-management-from-a-pediatric-cardiological-point-of-view
#15
REVIEW
I-Chen Chen, Zen-Kong Dai
UNLABELLED: Compared with adult patients with pulmonary hypertension (PH), pulmonary vascular disease is characterized by complex heterogeneity in pediatric patients. The Nice PH classification does not completely characterize or individualize any subgroup of pediatric PH. This is in contrast to the Panama classification, in which prenatal and fetal origins of many pulmonary vascular diseases in neonates and children, perinatal pulmonary vascular maladaptation, prenatal and postnatal pulmonary vascular mal-development, and pulmonary vascular hypoplasia are included...
November 2015: Acta Cardiologica Sinica
https://www.readbyqxmd.com/read/27099763/effect-of-dual-pulmonary-vasodilator-therapy-in-pulmonary-arterial-hypertension-associated-with-congenital-heart-disease-a-retrospective-analysis
#16
Oliver Monfredi, Elliot Heward, Linda Griffiths, Robin Condliffe, Vaikom S Mahadevan
BACKGROUND: Patients with pulmonary arterial hypertension (PAH) are managed according to evidence-based treatment guidelines. METHODS AND RESULTS: In this single-centre retrospective analysis, we examined outcomes of patients with PAH caused by congenital heart disease (PAH-CHD) with respect to exercise capacity and survival of adults treated with either bosentan or sildenafil monotherapy or bosentan-sildenafil dual therapy between January 2007 and January 2014...
2016: Open Heart
https://www.readbyqxmd.com/read/27053697/pulmonary-hypertension-in-children-with-congenital-heart-disease-pah-chd-pphvd-chd-expert-consensus-statement-on-the-diagnosis-and-treatment-of-paediatric-pulmonary-hypertension-the-european-paediatric-pulmonary-vascular-disease-network-endorsed-by-ishlt-and
#17
Rainer Kozlik-Feldmann, Georg Hansmann, Damien Bonnet, Dietmar Schranz, Christian Apitz, Ina Michel-Behnke
Pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) is a complex disease that presents with a broad spectrum of morphological and haemodynamic findings of varying severity. Recently, the aspect of paediatric pulmonary hypertensive vascular disease (PPHVD) has been introduced to expand the understanding of the full spectrum of pulmonary hypertension and increased pulmonary vascular resistance. Evaluation and treatment of PAH-CHD/PPHVD-CHD can be divided into in different topics...
May 2016: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/26988953/acute-vasodilator-response-in-pediatric-pulmonary-arterial-hypertension-current-clinical-practice-from-the-topp-registry
#18
MULTICENTER STUDY
Johannes M Douwes, Tilman Humpl, Damien Bonnet, Maurice Beghetti, D Dunbar Ivy, Rolf M F Berger
BACKGROUND: In pulmonary arterial hypertension (PAH), acute vasodilator response testing (AVT) is considered important to identify adult patients with favorable prognosis using calcium-channel blocker (CCB) therapy. However, in pediatric PAH, criteria used to identify acute responders and CCB use are insufficiently studied. OBJECTIVES: This study sought to describe current clinical practice of AVT and subsequent treatment decisions in pediatric PAH. METHODS: From January 2008 to May 2013, 529 consecutive children with confirmed pulmonary hypertension were enrolled in an international registry...
March 22, 2016: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/26984567/treatment-of-pulmonary-arterial-hypertension-in-congenital-heart-disease-in-singapore-versus-the-netherlands-age-exceeds-ethnicity-in-influencing-clinical-outcome
#19
A C M J van Riel, M J Schuuring, I D van Hessen, A P J van Dijk, E S Hoendermis, J W Yip, B J M Mulder, B J Bouma
BACKGROUND: Advanced treatment of pulmonary arterial hypertension (PAH) in congenital heart disease (CHD) is increasingly applied worldwide following the-mainly Western world based-international PAH-CHD guidelines. However, studies comparing clinical presentation and outcome after the initiation of PAH-specific treatment are lacking. We aimed to analyse this in a Singaporean and Dutch cohort of PAH-CHD patients. METHODS: Adult CHD patients starting PAH-specific therapy, enrolled in two nationwide registries, were analysed...
June 2016: Netherlands Heart Journal
https://www.readbyqxmd.com/read/26897077/the-role-of-cystatin-c-as-a-biomarker-for-prognosis-in-pulmonary-arterial-hypertension-due-to-congenital-heart-disease
#20
MULTICENTER STUDY
Ilja M Blok, Annelieke C M J van Riel, Mark J Schuuring, Rianne H A C M de Bruin-Bon, Arie P J van Dijk, Elke S Hoendermis, Aeilko H Zwinderman, Barbara J M Mulder, Berto J Bouma
BACKGROUND: Adults with pulmonary arterial hypertension due to congenital heart disease (PAH-CHD) have a poor prognosis. Identifying patients with a high risk for clinical events and death is important because their prognosis can be improved by intensifying their treatment. Cystatin C, a novel cardiac biomarker, correlates with right ventricular dimensions in patients with idiopathic PAH, giving it potential to determine prognosis in PAH-CHD patients. We investigated the predictive value of cystatin C for long-term mortality and clinical events...
April 15, 2016: International Journal of Cardiology
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