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Pulmonary hypertension diagnosis and treatment

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https://www.readbyqxmd.com/read/29664959/contemporary-survival-of-patients-with-pulmonary-arterial-hypertension-and-congenital-systemic-to-pulmonary-shunts
#1
Chodchanok Vijarnsorn, Kritvikrom Durongpisitkul, Paweena Chungsomprasong, Densiri Bositthipichet, Salisa Ketsara, Yuttapon Titaram, Prakul Chanthong, Supaluck Kanjanauthai, Jarupim Soongswang
OBJECTIVE: To compare survival of patients with newly diagnosed pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) according to various clinical classifications with classifications of anatomical-pathophysiological systemic to pulmonary shunts in a single-center cohort. METHODS: All prevalent cases of PAH-CHD with hemodynamic confirmation by cardiac catheterization in 1995-2015 were retrospectively reviewed. Patients who were younger than three months of age, or with single ventricle following surgery were excluded...
2018: PloS One
https://www.readbyqxmd.com/read/29659150/chronic-obstructive-pulmonary-disease-in-patients-with-chronic-thromboembolic-pulmonary-hypertension-prevalence-and-implications-for-surgical-treatment-outcome
#2
Oksana Kamenskaya, Irina Loginova, Alexander Chernyavskiy, Aleksander Edemskiy, Vladimir V Lomivorotov, Aleksander Karaskov
OBJECTIVE: The aim of our study was to investigate the prevalence of chronic obstructive pulmonary disease (COPD) in patients with chronic thromboembolic pulmonary hypertension (CTEPH) and examine their impact on the results of pulmonary thrombendarterectomy (PEA). METHODS: We enrolled 136 patients with CTEPH who scheduled for elective PEA. Pulmonary function tests (PFTs) including full-body plethysmography with bronchodilation test and lung diffusion capacity assessment were performed in all patients prior to surgery treatment...
April 16, 2018: Clinical Respiratory Journal
https://www.readbyqxmd.com/read/29650821/-continuation-of-axitinib-for-advanced-renal-cell-carcinoma-by-the-application-of-homecare-urology-a-case-report-and-literature-review
#3
Satoshi Yazawa, Yuji Kato, Jin Nakahara, Toshio Miyata, Mototsugu Oya
A 56-year-old man with advanced RCC and a past medical history of type 2 diabetes underwent a radical left nephrectomy following a histological diagnosis of papillary RCC, G2, INF b, pT3, V1 in 1999. In 2008, sorafenib was started to treat multiple pulmonary metastases of RCC. In 2011, sorafenib was switched to sunitinib when radiologic progression was observed. In 2014, sunitinib was switched to axitinib when further radiologic progression was observed. In 2015, the patient was referred to Yazawa clinic for homecare urology when hospital visits became difficult due to cancer pain and bilateral lower-extremity muscle weakness...
April 2018: Gan to Kagaku Ryoho. Cancer & Chemotherapy
https://www.readbyqxmd.com/read/29608809/chronic-thromboembolic-pulmonary-hypertension-from-the-perspective-of-patients-with-pulmonary-embolism
#4
REVIEW
Frederikus A Klok, Marion Delcroix, Harm Jan Bogaard
Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare but feared long-term complication of acute pulmonary embolism (PE), although CTEPH may occur in patients with no history of symptomatic venous thromboembolism. It represents the most severe presentation of the so-called 'post-PE syndrome', a phenomenon of permanent functional limitations after PE caused by deconditioning after PE or ventilatory or circulatory impairment due to unresolved pulmonary artery thrombi. Because the post-PE syndrome may occur in up to 50% of PE survivors, and CTEPH tends to have an insidious and non-specific clinical presentation, CTEPH is often not diagnosed or diagnosed after very long delay...
April 2, 2018: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/29608499/diagnosis-treatment-and-management-of-orthotopic-liver-transplant-candidates-with-portopulmonary-hypertension
#5
Anna Koulava, Abdallah Sannani, Avi Levine, Chhaya Aggarwal Gupta, Sarina Khanal, William Frishman, Roxana Bodin, David C Wolf, William Aronow, Gregg M Lanier
Portopulmonary hypertension (POPH) is seen in 5-8% of orthotopic liver transplantation (OLT) candidates and has significant implications for clinical outcomes. POPH is characterized by vasoconstriction and remodeling of the pulmonary vasculature. It is exacerbated by the hyperdynamic circulation that is common in advanced liver disease. Screening all OLT candidates with transthoracic echocardiography to assess pulmonary pressures and right ventricular function is crucial, as clinical symptoms alone are not reliable...
March 30, 2018: Cardiology in Review
https://www.readbyqxmd.com/read/29605286/pulmonary-hypertension-in-patients-with-interstitial-lung-disease
#6
REVIEW
Theodoros Karampitsakos, Argyrios Tzouvelekis, Serafeim Chrysikos, Demosthenes Bouros, Iraklis Tsangaris, Wassim H Fares
Interstitial lung diseases (ILDs) comprise a broad and heterogeneous group of more than two hundred diseases with common functional characteristics. Their diagnosis and management require a multidisciplinary approach. This multidisciplinary approach involves the assessment of comorbid conditions including pulmonary hypertension (PH) that exerts a dramatic impact on survival. The current World Health Organization (WHO) classification of PH encompasses many of the interstitial lung diseases into WHO Group 3, while sarcoidosis, Pulmonary Langerhans Cell Histiocytosis and lymphangioleiomyomatosis are placed into WHO Group 5 as diseases with unclear or multifactorial mechanisms...
March 29, 2018: Pulmonary Pharmacology & Therapeutics
https://www.readbyqxmd.com/read/29605204/pulmonary-hypertension-in-chronic-hemolytic-anemias-pathophysiology-and-treatment
#7
REVIEW
Alexandra Haw, Harold I Palevsky
Pulmonary hypertension has emerged as a major cause of morbidity and mortality in patients with hemoglobinopathies and chronic hemolytic anemias. These hematological diseases include - but are not limited to - sickle cell disease (SCD), thalassemia, paroxysmal nocturnal hematuria, and hereditary spherocytosis. Although most studies have been based on the use of echocardiography as a screening tool for pulmonary hypertension as opposed to the gold standard of right heart catheterization for definitive diagnosis, the association between chronic hemolytic anemia and pulmonary hypertension is evident...
April 2018: Respiratory Medicine
https://www.readbyqxmd.com/read/29603764/a-prospective-phase-ii-study-of-low-dose-lenalidomide-plus-dexamethasone-in-patients-with-newly-diagnosed-poems-syndrome
#8
Jian Li, Xu-Fei Huang, Qian-Qian Cai, Chen Wang, Hao Cai, Hao Zhao, Lu Zhang, Xin-Xin Cao, Robert Peter Gale, Dao-Bin Zhou
POEMS syndrome is a rare plasma dyscrasia without standard treatment. This phase II prospective trial evaluates the safety and response of 12 cycles of low dose lenalidomide (10mg) plus dexamethasone (Rdex) in patients with newly diagnosed POEMS syndrome. Forty-one patients (28 men) were enrolled and the median age at diagnosis was 49 years (range, 21-70 years). Twenty-one patients (46%) achieved complete hematologic response and the neurologic response rate was 95%. The median serum VEGF declined from 5155 pg/ml (range, 534-14328 pg/ml) to 832 pg/ml (95-6254 pg/ml) after therapy...
March 31, 2018: American Journal of Hematology
https://www.readbyqxmd.com/read/29602480/-tunnelled-central-venous-catheter-infection-during-treatment-with-epoprostenol
#9
M Koszutski, M Faure, A Guillaumot, E Gomez, M Mercy, F Chabot, A Chaouat
INTRODUCTION: Pulmonary arterial hypertension is a pulmonary vascular disease with a poor prognosis. Continuous intravenous treatment with prostacyclin analogues requires the placement of a tunnelled catheter. The occurrence of catheter-related infections in this context is unusual due to the alkaline pH of the prostacyclin analogue solutions. OBSERVATION: A 50-year-old patient with inherited pulmonary artery hypertension, treated with bosentan, sildenafil and epoprostenol, experienced generalized malaise associated with a weight loss of 9kg over a 12-month period without evidence of a source of infection or malignancy...
March 28, 2018: Revue des Maladies Respiratoires
https://www.readbyqxmd.com/read/29570688/diagnosis-evaluation-and-treatment-of-pulmonary-arterial-hypertension-in-children
#10
REVIEW
Benjamin S Frank, D Dunbar Ivy
Pulmonary Hypertension (PH), the syndrome of elevated pressure in the pulmonary arteries, is associated with significant morbidity and mortality for affected children. PH is associated with a wide variety of potential underlying causes, including cardiac, pulmonary, hematologic and rheumatologic abnormalities. Regardless of the cause, for many patients the natural history of PH involves progressive elevation in pulmonary arterial resistance and pressure, right ventricular dysfunction, and eventually heart failure...
March 23, 2018: Children
https://www.readbyqxmd.com/read/29559180/fluid-challenge-predicts-clinical-worsening-in-pulmonary-arterial-hypertension
#11
Michele D'Alto, Yoshiki Motoji, Emanuele Romeo, Paola Argiento, Giovanni Maria Di Marco, Agostino Mattera Iacono, Antonello D'Andrea, Gaetano Rea, Paolo Golino, Robert Naeije
AIM: A fluid challenge with rapid saline infusion during right heart catheterization has been shown to be useful for the differential diagnosis between pre- and post-capillary pulmonary hypertension. The aim of this study was to evaluate the prognostic relevance of fluid challenge-induced changes in pulmonary hemodynamics in patients with pulmonary arterial hypertension (PAH). METHODS: Overall, 118 PAH patients (mean age 57 ± 15 years, 80 female) underwent hemodynamic measurements before and after rapid saline infusion (7 mL/kg in 10 min) and were followed up for 19 ± 4 months...
March 7, 2018: International Journal of Cardiology
https://www.readbyqxmd.com/read/29557420/bosentan-inducing-autoimmune-hepatitis-in-a-patient-with-idiopathic-pulmonary-arterial-hypertension
#12
Alexandre de Araujo, Augusto Mantovani, Carlos Thadeu Schmidt Cerski, Antonio Barros Lopes, Luiza Cristina Bortoncello, Marcelo Basso Gazzana, Hugo Cheinquer
A 41-year-old woman diagnosed with idiopathic pulmonary hypertension presented symptoms despite the use of vasodilators, requiring treatment with bosentan. Previously, the patient had no signs of autoimmunity and had normal liver function. After three years of bosentan use, aminotransferase levels increased, without improvement after bosentan suspension, leading to complementary investigation. The diagnosis of autoimmune hepatitis was confirmed by biopsy, already in the stage of cirrhosis. In conclusion, in case of aminotransferase levels that remain persistently elevated, despite the reduction in doses and/or suspension of bosentan, autoimmune hepatitis must be investigated and treated urgently due to possibly rapid progression to cirrhosis...
March 2018: Journal of Gastrointestinal and Liver Diseases: JGLD
https://www.readbyqxmd.com/read/29546917/-the-role-of-pulmonary-hypertension-on-bronchopulmonary-dysplasia
#13
D Valenzuela, F Moya, M Luco, J L Tapia
50 years ago, Northway described Broncopulmonary Dysplasia (BPD) in preterm infants exposed to mechanical ventilation. Since then, their survival has increased, nevertheless a "new BPD" has appeared and its incidence has not diminished. One of the characteristics of this pathology is the the abnormal vascular remodeling, which in its most severe expression is known as Pulmonary Hyper tension (PH); with an incidence of 17% in patients with BPD, which is proportional to the severity of the disease (33% in severe BPD), and as mortality factor (up to 48% 2-year mortality in PH-BPD)...
December 2017: Revista Chilena de Pediatría
https://www.readbyqxmd.com/read/29540357/pulmonary-arterial-hypertension-pathogenesis-and-clinical-management
#14
REVIEW
Thenappan Thenappan, Mark L Ormiston, John J Ryan, Stephen L Archer
Pulmonary hypertension is defined as a resting mean pulmonary artery pressure of 25 mm Hg or above. This review deals with pulmonary arterial hypertension (PAH), a type of pulmonary hypertension that primarily affects the pulmonary vasculature. In PAH, the pulmonary vasculature is dynamically obstructed by vasoconstriction, structurally obstructed by adverse vascular remodeling, and pathologically non-compliant as a result of vascular fibrosis and stiffening. Many cell types are abnormal in PAH, including vascular cells (endothelial cells, smooth muscle cells, and fibroblasts) and inflammatory cells...
March 14, 2018: BMJ: British Medical Journal
https://www.readbyqxmd.com/read/29528272/angiogenic-and-inflammatory-biomarkers-for-screening-and-follow-up-in-patients-with-pulmonary-arterial-hypertension
#15
D Kylhammar, R Hesselstrand, S Nielsen, C Scheele, G Rådegran
OBJECTIVE: To identify circulating angiogenic and inflammatory biomarkers with potential in screening for pulmonary arterial hypertension (PAH) in systemic sclerosis (SSc), and in early diagnosis and determination of treatment response in PAH. METHOD: Plasma samples were taken at the time of PAH diagnosis and at treatment follow-up after a median (interquartile range) of 4 months (3-9.8 months) in idiopathic (n = 9) and SSc-associated PAH (n = 11). In patients with SSc-associated PAH, plasma samples had also been gathered a median of 2 years (0...
March 12, 2018: Scandinavian Journal of Rheumatology
https://www.readbyqxmd.com/read/29508628/drug-associated-pulmonary-arterial-hypertension
#16
Michael McGee, Nicholas Whitehead, Jennifer Martin, Nicholas Collins
INTRODUCTION: While pulmonary arterial hypertension remains an uncommon diagnosis, various therapeutic agents are recognized as important associations. These agents are typically categorized into "definite", "likely", "possible", or "unlikely" to cause pulmonary arterial hypertension, based on the strength of evidence. OBJECTIVE: This review will focus on those therapeutic agents where there is sufficient literature to adequately comment on the role of the agent in the pathogenesis of pulmonary arterial hypertension...
March 6, 2018: Clinical Toxicology
https://www.readbyqxmd.com/read/29485908/phosphodiesterase-5-inhibitor-therapy-for-pulmonary-hypertension-in-the-us-actual-vs-recommended-use
#17
Deborah Kim, Kyung Min Lee, Marc R Freiman, W Ryan Powell, Elizabeth S Klings, Seppo Rinne, Donald Miller, Adam J Rose, Renda Soylemez Wiener
RATIONALE: Care of patients with pulmonary hypertension (PH) is complex. While pulmonary vasodilators are effective for Group 1 PH, clinical guidelines and the Choosing Wisely Campaign recommend against routine use for Groups 2 and 3 PH (the most common types of PH) due to a lack of benefit, potential for harm, and high cost ($10,000-$13,000 per patient per year treated). Little is known about how these medications are used in practice. OBJECTIVE(S): To determine national patterns of phosphodiesterase-5-inhibitor (PDE5i) prescribing for PH in the Veterans Health Administration (VA)...
February 27, 2018: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/29485420/hypocalcemia-after-denosumab-in-a-pulmonary-hypertension-patient-receiving-epoprostenol
#18
Benedicte De Muynck, Mathias Leys, Joke Cuypers, Dirk Vanderschueren, Marion Delcroix, Catharina Belge
We report the case of a 50-year-old woman with anorexigen-induced pulmonary arterial hypertension treated with epoprostenol, who presented with Trousseau's sign, leading to the diagnosis of severe hypocalcemia for which substitution was started (initially orally, followed by intravenous substitution). After further analysis, we assume that epoprostenol-induced diarrhea caused malabsorption (as other reasons were excluded), leading to nutritional osteomalacia with secondary hyperparathyroidism. We discovered that even more severe hypocalcemia was induced by the treatment with the anti-osteoporotic drug denosumab, which was started after the diagnosis of osteoporosis on bone densitometry...
2018: Respiration; International Review of Thoracic Diseases
https://www.readbyqxmd.com/read/29480834/percutaneous-treatment-for-silicosis-induced-pulmonary-artery-stenosis-a-case-report-and-review-of-the-literature
#19
Ruifeng Zhang, Guofeng Ma, Xiaoling Xu, Li Liang
BACKGROUND: Exposure to crystalline silica results in silicosis with initiation and progression of pulmonary fibrosis. The impaired lung parenchyma leads to pulmonary arterial hypertension and increased pressure in the right ventricle of the heart. Usually, the silicosis may be followed by enlargement of hilar lymphnodes, but silicosis-induced pulmonary artery stenosis with severe pulmonary hypertension is rare. Percutaneous pulmonary artery stenting and balloon angioplasty were performed to relieve stenosis and pulmonary hypertension...
January 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29472044/guidelines-on-the-diagnosis-and-treatment-of-pulmonary-hypertension-summary-of-recommendations
#20
Joan Albert Barberà, Antonio Román, Miguel Ángel Gómez-Sánchez, Isabel Blanco, Remedios Otero, Raquel López-Reyes, Isabel Otero, Gregorio Pérez-Peñate, Ernest Sala, Pilar Escribano
Pulmonary hypertension is a hemodynamic disorder defined by abnormally high pulmonary artery pressure that can occur in numerous diseases and clinical situations. The causes of pulmonary hypertension are classified into 5 major groups: arterial, due to left heart disease, due to lung disease and/or hypoxemia, chronic thromboembolic, with unclear and/or multifactorial mechanisms. This is a brief summary of the Guidelines on the Diagnostic and Treatment of Pulmonary Hypertension of the Spanish Society of Pulmonology and Thoracic Surgery...
February 19, 2018: Archivos de Bronconeumología
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