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https://www.readbyqxmd.com/read/27683607/clinical-classification-in-pediatric-pulmonary-arterial-hypertension-associated-with-congenital-heart-disease
#1
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
#2
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
#3
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
#4
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/27443136/pulmonary-hypertension-and-congenital-heart-disease
#5
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
#6
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 Cardiol Sin
https://www.readbyqxmd.com/read/27099763/effect-of-dual-pulmonary-vasodilator-therapy-in-pulmonary-arterial-hypertension-associated-with-congenital-heart-disease-a-retrospective-analysis
#7
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
#8
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
#9
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
#10
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
#11
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
https://www.readbyqxmd.com/read/26880324/pulmonary-arterial-hypertension-associated-with-congenital-heart-disease-comparison-of-clinical-and-anatomic-pathophysiologic-classification
#12
Sheila Ramjug, Nehal Hussain, Judith Hurdman, Catherine Billings, Athanasios Charalampopoulos, Charlie A Elliot, Ian Sabroe, David G Kiely, Robin Condliffe
BACKGROUND: Between 5% and 10% of patients with congenital heart disease (CHD) develop pulmonary arterial hypertension (PAH-CHD). Patients can be classified using either a clinical or anatomic-pathophysiologic system. No study has previously utilized both systems in a large cohort of patients. METHODS: Two hundred forty consecutive PAH-CHD patients diagnosed at a pulmonary hypertension referral center during 1995 to 2014 were identified from our unit database. Baseline characteristics, treatment and survival data were retrieved and survival analyses was performed...
May 2016: Journal of Heart and Lung Transplantation
https://www.readbyqxmd.com/read/26774383/egr-1-identifies-neointimal-remodeling-and-relates-to-progression-in-human-pulmonary-arterial-hypertension
#13
Diederik E van der Feen, Michael G Dickinson, Beatrijs Bartelds, Marinus A J Borgdorff, Hannie Sietsma, Marilyne Lévy, Rolf M F Berger
BACKGROUND: Pulmonary arterial hypertension (PAH) is hallmarked by the development of neointimal lesions. The transcription factor Egr-1 seems to play a critical role in neointimal formation in experimental PAH and was identified as a putative target for intervention. In this study we investigated whether Egr-1 is also associated with neointimal-type vascular remodeling in different forms of human PAH or pulmonary hypertension. METHODS: Using immunohistochemistry, we studied Egr-1 expression specifically in a wide morphologic spectrum of pulmonary arteries in the lung tissue of 72 patients with different forms and stages of PAH, specifically idiopathic PAH (n = 18), advanced-stage congenital heart disease‒associated PAH (PAH-CHD) (n = 21), early-stage PAH-CHD (n = 19) and non-neointimal hypoxic pulmonary hypertension (PH) (n = 4), and controls (n = 10)...
April 2016: Journal of Heart and Lung Transplantation
https://www.readbyqxmd.com/read/26714814/elevated-plasma-connective-tissue-growth-factor-levels-in-children-with-pulmonary-arterial-hypertension-associated-with-congenital-heart-disease
#14
Gang Li, Li Tang, Peng Jia, Jian Zhao, Dong Liu, Bin Liu
We aimed to investigate plasma connective tissue growth factor (CTGF) levels in pulmonary arterial hypertension (PAH) associated with congenital heart disease (CHD) (PAH-CHD) in children and the relationships of CTGF with hemodynamic parameters. Plasma CTGF levels were calculated in 30 children with CHD, 30 children with PAH-CHD and 25 health volunteers, using the subtraction method. Cardiac catheterization was performed to measure clinical hemodynamic parameters. Plasma CTGF levels were significantly higher in PAH-CHD than in those with CHD and health volunteers (p < 0...
April 2016: Pediatric Cardiology
https://www.readbyqxmd.com/read/26645265/sequencing-of-mutations-in-the-serine-threonine-kinase-domain-of-the-bone-morphogenetic-protein-receptor-type-2-gene-causing-pulmonary-arterial-hypertension
#15
Zeynep Mutlu, Meral Kayıkçıoğlu, Sanem Nalbantgil, Özcan Vuran, Hatice Kemal, Nesrin Moğulkoç, Biray Ertürk, Hüseyin Onay, Zuhal Eroğlu, Hakan Kültürsay
OBJECTIVE: Germline mutations in the bone morphogenetic protein receptor type-2 (BMPR2) gene are considered to be a major risk factor for pulmonary arterial hypertension (PAH). BMPR2 mutations have been reported in 10%-20% of idiopathic PAH and in 80% of familial PAH cases. The aim of this study was to evaluate the frequency of mutations in the serine/threonine kinase domain of the BMPR2 gene in a group of patients from a single PAH referral center in Turkey. METHODS: This cross-sectional study used a DNA-sequencing method to investigate BMPR2 mutations in the serine-threonine-kinase domain in 43 patients diagnosed with PAH [8 with idiopathic PAH and 35 with congenital heart disease (CHD)] from a single PAH referral center...
September 15, 2015: Anatolian Journal of Cardiology
https://www.readbyqxmd.com/read/26381329/medium-term-follow-up-of-renal-function-in-hypoxaemic-congenital-heart-disease-patients
#16
Efrén Martínez-Quintana, Fayna Rodríguez-González
UNLABELLED: Introduction Hypoxaemic congenital heart disease (CHD) patients are at higher risk of complications. The aim of this study was to compare and follow-up blood and 24-hour urine analytical data in hypoxaemic and non-hypoxaemic CHD patients. METHODS: The inclusion criteria for this study were as follows: patients older than 14 years of age with a structural CHD with or without associated hypoxaemia. RESULTS: In total, 27 hypoxaemic and 48 non-hypoxaemic CHD patients were included in order to compare blood and 24-hour urine analytical data...
August 2016: Cardiology in the Young
https://www.readbyqxmd.com/read/26346500/some-neonatal-risk-factors-for-adult-pulmonary-arterial-hypertension-remain-unknown
#17
Estelle Naumburg, Inge Axelsson, Daniel Huber, Lars Söderström
AIM: Pulmonary hypertension has been linked to premature birth, chronic lung disease, bronchopulmonary dysplasia and congenital heart disease. This national population-based registry study assessed the risk of adult pulmonary hypertension following premature birth, adjusted for known risk factors. METHODS: We focused on adults in the Swedish Pulmonary Arterial Hypertension Registry, who were born prematurely, and controls randomly selected from the Swedish medical birth register and matched to each case by birth year and delivery hospital...
November 2015: Acta Paediatrica
https://www.readbyqxmd.com/read/26316767/pulmonary-hypertension-diagnostic-and-therapeutic-challenges
#18
REVIEW
Isabel S Bazan, Wassim H Fares
Pulmonary hypertension (PH) is a hemodynamic and pathophysiologic state that can be found in multiple conditions with associated symptoms of dyspnea, decreased exercise tolerance, and progression to right heart failure. The World Health Organization has classified PH into five groups. The first group is pulmonary arterial hypertension (PAH), which can be idiopathic, heritable, due to drugs and toxins, or associated with conditions such as connective tissue diseases, congenital heart disease, portal hypertension, and others...
2015: Therapeutics and Clinical Risk Management
https://www.readbyqxmd.com/read/26299333/echocardiographic-estimation-of-right-ventricular-stroke-work-in-children-with-pulmonary-arterial-hypertension-comparison-with-invasive-measurements
#19
COMPARATIVE STUDY
Michael V Di Maria, Dale A Burkett, Adel K Younoszai, Bruce F Landeck, Luc Mertens, D Dunbar Ivy, Mark K Friedberg, Kendall S Hunter
BACKGROUND: Right ventricular (RV) failure is a key determinant of mortality in children with pulmonary arterial hypertension (PAH). RV stroke work (RVSW) can be estimated as the product of RV systolic pressure and stroke volume. The authors have shown that RVSW predicts adverse outcomes in this population when derived from hemodynamic data; noninvasive assessment of RVSW may be advantageous but has not been assessed. There are few data validating noninvasive versus invasive measurements in children with PAH...
November 2015: Journal of the American Society of Echocardiography
https://www.readbyqxmd.com/read/26261613/p-selectin-gene-polymorphism-associates-with-pulmonary-hypertension-in-congenital-heart-disease
#20
Xiao-Fei Li, Chun-Hui Song, Hong-Zhuan Sheng, Dong-Dong Zhen, Min Pan, Jian-Hua Zhu
OBJECTIVE: To investigate the relationship between P-selectin gene polymorphism and congenital heart disease (CHD) with pulmonary hypertension (PAH). METHODS: 58 CHD patients with PAH (PAH-CHD), 43 CHD patients without PAH and 205 healthy subjects were included in this study. The concentration of plasma P-selectin was determined by ELISA kits; the direct sequencing of PCR products was used to analyze the P-selectin genotypes. RESULTS: The concentration of plasma P-selectin was markedly higher in PAH-CHD patients than that in CHD subjects and controls, while no difference was observed between CHD group and control...
2015: International Journal of Clinical and Experimental Pathology
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