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pulmonary arterial hypertension associated with congenital heart disease

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https://www.readbyqxmd.com/read/29191503/management-of-adults-with-congenital-heart-disease-and-pulmonary-arterial-hypertension-in-the-uk-survey-of-current-practice-unmet-needs-and-expert-commentary
#1
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
https://www.readbyqxmd.com/read/29177254/semi-quantitative-assessment-of-pulmonary-arterial-hypertension-associated-with-congenital-heart-disease-through-myocardial-perfusion-imaging
#2
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 with 99mTc-methoxyisobutyl isonitrile (99mTc-MIBI) pre and 6 months post treatments...
November 27, 2017: Hellenic Journal of Nuclear Medicine
https://www.readbyqxmd.com/read/29136712/-long-term-outcome-and-prognostic-factors-in-pregnant-women-with-pulmonary-arterial-hypertension-associated-with-congenital-heart-disease
#3
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/29120872/a-rare-case-of-hemopericardium-from-a-ruptured-dissecting-pulmonary-artery-aneurysm-postmortem-computed-tomography-scan-and-autopsy-findings
#4
Rexson Tse, Jack Garland, Yvonne Triggs, Simon Stables
Nontraumatic hemopericardium is a potentially fatal pathology that is most commonly caused by myocardial wall rupture, ruptured aortic dissection, aortic aneurysm rupture, or a neoplastic process. A rare potential cause of hemopericardium is pulmonary artery dissection, with less than 100 reported cases in the literature. Pulmonary artery dissection is associated with pulmonary artery aneurysm, pulmonary artery hypertension, and congenital heart disease. We report a fatal case of nontraumatic hemopericardium caused by a ruptured dissecting pulmonary artery aneurysm in a 16-year-old girl with patent ductus arteriosus...
November 9, 2017: American Journal of Forensic Medicine and Pathology
https://www.readbyqxmd.com/read/29119450/surgical-treatment-for-adult-congenital-heart-disease-consideration-for-indications-and-procedures
#5
REVIEW
Kozo Matsuo, Masashi Kabasawa, Soichi Asano, Shigeru Tateno, Yasutaka Kawasoe, Yoshitomo Okajima, Naoki Hayashida, Hirokazu Murayama
The number of the adult patients with congenital heart diseases (ACHD) continues to grow owing to improvement of surgical results and medical management. Corrective surgery for complex CHD does not always mean complete cure. It is not rare that the patients will visit the cardiology institutes because of secondary lesions due to residua or sequela in adults. Some patients with CHD remain unrepairable with different degree of heart failure and pulmonary arterial hypertension. Association of arrhythmias is common in ACHD patients and sometimes critical...
November 8, 2017: General Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29076824/preliminary-results-from-a-nationwide-adult-cardiology-perspective-for-pulmonary-hypertension-registry-on-clinical-outcome-and-survival-in-pulmonary-hypertension-groups-simurg
#6
Cihangir Kaymaz, Bülent Mutlu, M Serdar Küçükoğlu, Barış Kaya, Bahri Akdeniz, Burçak Kılıçkıran Avcı, Enbiya Aksakal, Mehmet Akbulut, Zehra Atılgan Arıtürk, Sümeyye Güllülü, Gülten Aydoğdu Taçoy, Meral Kayıkçıoğlu, Sanem Nalbantgil, Cihan Örem, Hatice Betül Erer, Murat Yüce, Necip Ermiş, Omaç Tüfekçioğlu, Mesut Demir, Mehmet Birhan Yılmaz, Mehmet Güngör Kaya, Hakan Kültürsay, Zeki Öngen, Lale Tokgözoğlu
OBJECTIVE: The present study was designed to evaluate the characteristics of pulmonary hypertension (PH) and adult cardiology practice patterns for PH in our country. METHODS: We evaluated preliminary survey data of 1501 patients with PH (females, 69%; age, 44.8±5.45) from 20 adult cardiology centers (AdCCs). RESULTS: The average experience of AdCCs in diagnosing and treating patients with PH was 8.5±3.7 years. Pulmonary arterial hypertension (PAH) was the most frequent group (69%) followed by group 4 PH (19%), group 3 PH (8%), and combined pre- and post-capillary PH (4%)...
October 2017: Anatolian Journal of Cardiology
https://www.readbyqxmd.com/read/29041891/-functional-state-of-vascular-endothelium-in-patients-with-pulmonary-arterial-hypertension-associated-with-congenital-heart-diseases
#7
Y E Emelyanchik, G N Vol'f, A O Vesemiller, B A Salmina
In this article, we present results of the study of blebbing activity of membranes of peripheral blood lymphocytes, concentrations of soluble platelet/endothelial cell adhesion molecule 1, and vascular endothelial growth factor (VEGF) in patients with pulmonary arterial hypertension associated with congenital heart diseases. The research has shown that hypoxia and hemodynamic factors are the initiators of blebbing of membrane of peripheral blood lymphocytes. The activity of blebbing increases in parallel with the severity of the disease...
August 2017: Kardiologiia
https://www.readbyqxmd.com/read/28967497/pulmonary-artery-hypertension-in-childhood-the-transforming-growth-factor-%C3%AE-superfamily-related-genes
#8
REVIEW
Shi-Min Yuan
Pulmonary artery hypertension (PAH) is very rare in childhood, and it can be divided into heritable, idiopathic drug- and toxin-induced and other disease (connective tissue disease, human immunodeficiency virus infection, portal hypertension, congenital heart disease, or schistosomiasis)-associated types. PAH could not be interpreted solely by pathophysiological theories. The impact of the transforming growth factor-β superfamily-related genes on the development of PAH in children remains to be clarified. Pertinent literature on the transforming growth factor-β superfamily-related genes in relation to PAH in children published after the year 2000 was reviewed and analyzed...
August 12, 2017: Pediatrics and Neonatology
https://www.readbyqxmd.com/read/28966315/prognostic-value-of-pulmonary-artery-compliance-in-patients-with-pulmonary-arterial-hypertension-associated-with-adult-congenital-heart-disease
#9
Xiao-Ling Cheng, Zhi-Hong Liu, Qing Gu, Xin-Hai Ni, Qin Luo, Zhi-Hui Zhao, Jian-Guo He, Chang-Ming Xiong
In congenital heart disease (CHD), the presence of pulmonary arterial hypertension (PAH) is associated with a poor prognosis. In this study, we aim to investigate the role of pulmonary artery compliance (Cp) in predicting the mortality of PAH associated with adult congenital heart disease (APAH-CHD). One-hundred and seventy-five patients of APAH-CHD who underwent a comprehensive clinical evaluation were included in this study. All patients were followed up in a 6-month interval and the primary end point was all cause of death...
October 21, 2017: International Heart Journal
https://www.readbyqxmd.com/read/28962211/clinical-comparative-analysis-of-histidine-tryptophan-ketoglutarate-solution-and-st-thomas-crystalloid-cardioplegia-a-12-year-study-from-a-single-institution
#10
Ying-Zhong Lin, Jing-Bin Huang, Xiang-Wei Li, Xian-Ming Tang, Wei-Jun Lu, Zhao-Ke Wen, Jian Liang, Dian-Yuan Li, Hao Wang
Cardioplegic reperfusion during a long-term ischemic period interrupts cardiac surgery and increases cellular edema due to repeated administration. The present clinical study compared the protective effects of histidine-ketoglutarate-tryptophan (HTK) solution and St. Thomas crystalloid cardioplegia. Clinical experiences of the myocardial protection induced by one single perfusion with HTK were reviewed in high-risk patients with severe pulmonary arterial hypertension associated with complex congenital heart disease...
September 2017: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/28873355/pulmonary-interstitial-glycogenosis-associated-with-a-spectrum-of-neonatal-pulmonary-disorders
#11
Ernest Cutz, Rose Chami, Sharon Dell, Jacob Langer, David Manson
Primary or isolated pulmonary interstitial glycogenosis (PIG) is a rare disease presenting as tachypnea and hypoxemia during the perinatal period. A diffuse interstitial infiltrate with focal hyperinflation is visible on chest imaging. The biopsy findings include diffuse expansion of the interstitium by spindle-shaped cells with pale cytoplasm that, on electron microscopy (EM), are poorly differentiated mesenchymal cells containing abundant monoparticulate glycogen. This glycogenosis appears to be a transient abnormality, usually with a favorable prognosis...
October 2017: Human Pathology
https://www.readbyqxmd.com/read/28862067/pushing-the-envelope-a-treat-and-repair-strategy-for-patients-with-advanced-pulmonary-hypertension-associated-with-congenital-heart-disease
#12
Rebecca Johnson Kameny, Elizabeth Colglazier, Hythem Nawaytou, Phillip Moore, V Mohan Reddy, David Teitel, Jeffrey R Fineman
Pulmonary arterial hypertension (PAH) is a frequent complication of congenital heart disease as a consequence of altered pulmonary hemodynamics with increased pulmonary blood flow and pressure. The development of pulmonary vascular disease (PVD) in this patient population is an important concern in determining operative strategy. Early, definitive surgical repair, when possible, is the best therapy to prevent and treat PVD. However, this is not possible in some patients because they either presented late, after the development of PVD, or they have complex lesions not amenable to one-step surgical correction, including patients with single ventricle physiology, who have a continuing risk of developing PVD...
July 2017: Pulmonary Circulation
https://www.readbyqxmd.com/read/28828743/pregnancy-in-women-with-congenital-heart-disease
#13
REVIEW
Evin Yucel, Doreen DeFaria Yeh
Advances in cardiac surgical interventions in infancy and childhood have led to an increased number of women with congenital heart disease of childbearing age. For these women, individualized preconception counseling and pregnancy planning should be a vital component of their medical management, and presentation for obstetric care may even be an opportunity to re-establish cardiovascular care for patients who have been lost to follow-up. These patients have unique cardiovascular anatomy and physiology, which is dependent upon the surgical intervention they may have undergone during childhood or adolescence...
August 22, 2017: Current Treatment Options in Cardiovascular Medicine
https://www.readbyqxmd.com/read/28825767/clinical-and-parental-status-of-patients-with-congenital-heart-disease-associated-pulmonary-arterial-hypertension
#14
Amiram Nir, Neville Berkman, Sagui Gavri
BACKGROUND: Pulmonary arterial hypertension (PAH) is a significant consequence of congenital heart disease (CHD). Its presence and severity is associated with increased morbidity and mortality. OBJECTIVES: To evaluate the clinical and demographic characteristics of adults with congenital heart diseases (ADCHD) and PAH at a single center. METHODS: A prospective registry of all patients with PAH was conducted between 2009 and 2015. RESULTS: Thirty-two patients were identified...
August 2017: Israel Medical Association Journal: IMAJ
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
#15
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
#16
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/28643420/impact-on-survival-of-warfarin-in-patients-with-pulmonary-arterial-hypertension-receiving-subcutaneous-treprostinil
#17
Mona Ascha, Xuan Zhou, Youlan Rao, Omar A Minai, Adriano R Tonelli
INTRODUCTION: Anticoagulation is a common treatment modality in patients with pulmonary arterial hypertension (PAH). Further studies are needed to appropriately assess the risk/benefit ratio of anticoagulation, particularly in PAH patients receiving PAH-specific therapies. AIMS: We use observational long-term data on PAH patients treated with subcutaneous (SQ) treprostinil from a large open-label study. Patients were followed for up to 4 years. The use of warfarin and bleeding events were recorded...
October 2017: Cardiovascular Therapeutics
https://www.readbyqxmd.com/read/28637627/pulmonary-arterial-resistance-and-compliance-in-preterm-infants
#18
Seigo Okada, Jun Muneuchi, Yusaku Nagatomo, Mamie Watanabe, Chiaki Iida, Hiromitsu Shirouzu, Ryohei Matsuoka, Kunitaka Joo
BACKGROUND: Preterm birth is known to be associated with an increased risk of pulmonary arterial hypertension, although how preterm birth influences pulmonary hemodynamics has not been fully understood. Pulmonary arterial resistance (Rp) and compliance (Cp) are important factors to assess the pulmonary circulation. The purpose of this study is to clarify the relationship between Rp and Cp in preterm infants. METHODS: We performed cardiac catheterization in 96 infants (50 males) with ventricular septal defect, and compared pulmonary hemodynamic parameters including Rp and Cp between preterm and full-term infants...
June 16, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28616089/severe-pulmonary-arterial-hypertension-associated-with-congenital-cardiac-shunts-evolution-under-specific-treatment
#19
R I Negoi, I Ghiorghiu, F Filipoiu, M Hostiuc, I Negoi, C Ginghina
Objective: The aim of this study was to compare the effects of Sildenafil, Bosentan and combined therapy in patients with congenital cardiac shunts associated pulmonary artery hypertension (CCS-PAH). Design: Prospective observational study (February 2011 - January 2014) with a historical control group (January 2009 - January 2011). Setting: "CC Iliescu" Institute for Emergency Cardiovascular Diseases of Bucharest, a tertiary university-affiliated center. Patients: All cases with CCS-PAH. Interventions: Specific vasodilatory therapy: Sildenafil, Bosentan or combined therapy...
April 2017: Journal of Medicine and Life
https://www.readbyqxmd.com/read/28608969/effect-of-iloprost-on-biomarkers-in-patients-with-congenital-heart-disease-pulmonary-arterial-hypertension
#20
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
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