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pulmonary arterial hypertension associated with congenital heart disease

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https://www.readbyqxmd.com/read/29444773/lung-function-inflammation-and-endothelin-1-in-congenital-heart-disease-associated-pulmonary-arterial-hypertension
#1
Andrew Low, Sarah George, Luke Howard, Nicholas Bell, Ann Millar, Robert M R Tulloh
BACKGROUND: Breathlessness is the most common symptom in people with pulmonary arterial hypertension and congenital heart disease (CHD-APAH), previously thought to be caused by worsening PAH, but perhaps also by inflammation and abnormalities of lung function. We studied lung function and airway inflammation in patients with CHD-APAH and compared the results with controls. METHODS AND RESULTS: Sixty people were recruited into the study: 20 CHD-APAH, 20 CHD controls, and 20 healthy controls...
February 14, 2018: Journal of the American Heart Association
https://www.readbyqxmd.com/read/29436381/subcutaneous-treprostinil-in-congenital-heart-disease-related-pulmonary-arterial-hypertension
#2
Nika Skoro-Sajer, Christian Gerges, Olga Hajnalka Balint, Dora Kohalmi, Monika Kaldararova, Iveta Simkova, Johannes Jakowitsch, Harald Gabriel, Helmut Baumgartner, Mario Gerges, Roela Sadushi-Kolici, David S Celermajer, Irene Marthe Lang
OBJECTIVE: To assess the efficacy and safety of subcutaneous treprostinil in adult patients with congenital heart disease (CHD)-associated pulmonary arterial hypertension (PAH) after 12 months of treatment. METHODS: Consecutive adult patients with CHD-PAH received subcutaneous treprostinil to maximum tolerated doses in an observational study. RESULTS: Advanced CHD-PAH patients with WHO class III or IV disease (n=32, age 40±10 years, 20 females) received treprostinil for suboptimal response to bosentan (n=12), WHO functional class IV disease (FC, n=7) or prior to bosentan approval (n=13)...
February 7, 2018: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/29426959/acute-effect-of-inhaled-iloprost-in-children-with-pulmonary-arterial-hypertension-associated-with-simple-congenital-heart-defects
#3
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
https://www.readbyqxmd.com/read/29340731/sensitive-cardiac-troponins-could-they-be-new-biomarkers-in-pediatric-pulmonary-hypertension-due-to-congenital-heart-disease
#4
Seyma Kayali, Ilker Ertugrul, Tamer Yoldas, Ozkan Kaya, Senem Ozgür, Utku A Orün, Selmin Karademir
To analyze the role of sensitive cardiac troponin I (scTnI) and high-sensitive troponin T (hscTnT) in the determination of myocardial injury caused by volume and pressure load due to pulmonary hypertension (PH) and to investigate if these markers may be useful in the management of PH in childhood. Twenty-eight patients with congenital heart disease (CHD) with left to right shunt and PH, 29 patients with CHD with left to right shunt but without PH, and 18 healthy children, in total 75 individuals, were included in the study...
January 16, 2018: Pediatric Cardiology
https://www.readbyqxmd.com/read/29332925/pulmonary-artery-pseudoaneurysm-secondary-to-lung-inf-lammation
#5
Shinichirou Ishimoto, Hiroyuki Sakurai, Ryouta Higure, Riken Kawachi, Mie Shimamura
Pulmonary artery aneurysms (PAA) and pseudoaneurysms (PAP) are caused by infections, vasculitis, trauma, pulmonary hypertension, congenital heart disease, and connective tissue disease. Most cases of such aneurysm occur in the trunk or major branches of the pulmonary artery, while the peripheral type is less common. The treatment modalities are medical therapy, surgery, and percutaneous catheter embolization. The mortality rate associated with rupture is approximately 50%. We encountered a case of a 53-year-old man with a pulmonary artery pseudoaneurysm secondary to pneumonia and cavity formation during chemotherapy for acute myeloid leukemia (AML)...
January 15, 2018: Annals of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29298686/a-single-center-experience-with-12-consecutive-cases-of-pregnancy-among-patients-with-membranous-ventricular-septal-aneurysm
#6
Kana Wang, Xiaodong Wang, Haiyan Yu, Xinghui Liu, Aiyun Xing, Yong You
BACKGROUND: Membranous ventricular septal aneurysm (MVSA) is a rare cardiac anomaly that can occur as an isolated entity or being associated with other cardiac malformations. Complications of MVSA include thromboembolism, arrhythmia, rupture, bacterial endocarditis, right ventricular outflow tract obstruction, and atrioventricular valve diseases.The success rate of pregnancy and delivery in patients with MVSA has not been reported in the literature. This study was to assess the clinical implications of this condition from our center's experience...
January 3, 2018: BMC Pregnancy and Childbirth
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
#7
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
#8
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...
September 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
#9
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
#10
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
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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