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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)
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...
February 9, 2018: Pediatric Cardiology
Daniel L Varela, Mohamed Teleb, Wael El-Mallah
Pulmonary arterial hypertension (PAH) secondary to congenital heart disease (CHD) is the third most common cause of PAH, and it is becoming increasingly common as improvements in the management of CHD have led to increased life expectancy for these patients. The medical management of PAH due to CHD (PAH-CHD) is largely the same as what has been used for the treatment of idiopathic PAH, though the body of literature supporting this management decision is very small. There are currently few studies available which specifically focus on the treatment of PAH-CHD...
2018: Open Heart
Robert Tulloh, Kostas Dimopoulos, Robin Condliffe, Paul Clift
BACKGROUND: Pulmonary arterial hypertension (PAH) is a well-recognised complication of adult congenital heart disease (CHD). However, management is not currently standardised between centres and specific guidelines are lacking. In order to identify and understand the unmet needs related to PAH associated with CHD (PAH-CHD), a survey of physicians was performed. METHODS: An electronic survey was sent to two physician groups: (1) cardiologists registered in a UK cardiology directory; (2) specialist pulmonary hypertension (PH) physicians known to manage patients with adult PAH-CHD...
November 13, 2017: Heart, Lung & Circulation
Mei Liu, Chunxia Qin, Xiaotian Xia, Mengting Li, Yichun Wang, Lijun Wang, Xiaoke Shang, Xiaoli Lan
OBJECTIVE: The study aimed to use myocardial perfusion imaging (MPI) as a semi-quantitative method to assess the clinical severity of pulmonary arterial hypertension (PAH) in patients with congenital heart disease (CHD). SUBJECTS AND METHODS: A total of 24 patients with PAH related to CHD (PAH-CHD) who received interventional or medical treatment were included. All patients underwent physical examination, cardiac function evaluation, biochemical test, echocardiography, right heart catheterization (RHC), and MPI with99m Tc-methoxyisobutyl isonitrile (99m Tc-MIBI) pre and 6 months post treatments...
September 2017: Hellenic Journal of Nuclear Medicine
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]
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
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...
October 2017: Pediatric Cardiology
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
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
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
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
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
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
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
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
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
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
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
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
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