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Pulmonary hypertension diagnosis and treatment

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https://www.readbyqxmd.com/read/28819824/-update-pulmonary-arterial-hypertension-definitions-diagnosis-therapy
#1
N Sommer, M J Richter, K Tello, F Grimminger, W Seeger, H A Ghofrani, H Gall
The term pulmonary arterial hypertension comprises a group of pulmonary vascular diseases of different etiologies that are characterized by similar precapillary vascular remodeling processes and result in exertional dyspnea and right heart insufficiency. The specific pharmacological treatment approach considers the risk of mortality and phenotypical properties and includes treatment with phosphodiesterase type 5 inhibitors, endothelin receptor antagonists and prostanoids, as well as with more novel substances, such as a soluble guanylyl cyclase stimulator and an oral prostacyclin receptor agonist...
August 17, 2017: Der Internist
https://www.readbyqxmd.com/read/28818881/selexipag-for-the-treatment-of-connective-tissue-disease-associated-pulmonary-arterial-hypertension
#2
Sean Gaine, Kelly Chin, Gerry Coghlan, Richard Channick, Lilla Di Scala, Nazzareno Galiè, Hossein-Ardeschir Ghofrani, Irene M Lang, Vallerie McLaughlin, Ralph Preiss, Lewis J Rubin, Gérald Simonneau, Olivier Sitbon, Victor F Tapson, Marius M Hoeper
Patients with connective tissue disease-associated pulmonary arterial hypertension (PAH-CTD) have a poor prognosis compared with other aetiologies. The underlying CTD can influence treatment response and outcomes. We characterised the GRIPHON study PAH-CTD subgroup and evaluated response to selexipag.Of 334 patients with PAH-CTD, PAH was associated with systemic sclerosis (PAH-SSc) in 170, systemic lupus erythematosus (PAH-SLE) in 82 and mixed CTD/CTD-other in 82. For the primary composite endpoint of morbidity/mortality, hazard ratios (HR) and 95% CI were calculated using Cox proportional hazard models...
August 2017: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/28817465/new-insights-into-the-recognition-classification-and-management-of-systemic-sclerosis-associated-pulmonary-hypertension
#3
Christopher J Mullin, Stephen C Mathai
PURPOSE OF REVIEW: Pulmonary hypertension is a common complication of systemic sclerosis (SSc), and remains a leading cause of morbidity and mortality. We will review recent developments in the recognition, classification and treatment of pulmonary hypertension in SSc. RECENT FINDINGS: Advances in screening for pulmonary arterial hypertension (PAH) and use of exercise haemodynamics may help to identify pulmonary vascular disease earlier in SSc. Recent studies have led to changes in recommendations for adjunct therapy and selection of pulmonary vasodilators for the treatment of SSc-associated PAH...
August 16, 2017: Current Opinion in Rheumatology
https://www.readbyqxmd.com/read/28813774/pulmonary-embolism-caused-by-popliteal-vein-aneurysm-a-case-report
#4
Kevin Marquez, Kalyan Chakravarthy Potu, Chad Laurich, Randall Lamfers
In this case report, we describe an unusual episode of bilateral submassive pulmonary embolism (PE) caused by a popliteal vein aneurysm (PVA). The development of PE stems from many risk factors including obesity (BMI³ 30 kg/m2), hypertension, cigarette smoking (greater than 25 cigarettes per day), increasing age, surgery, immobility, malignancy, and inherited thrombophilia. A PVA is a rare but significant cause of PE. A 28-year-old male presented to the emergency department with progressive shortness of breath...
March 2017: South Dakota Medicine: the Journal of the South Dakota State Medical Association
https://www.readbyqxmd.com/read/28794135/pulmonary-vasodilator-therapy-is-associated-with-greater-survival-in-eisenmenger-syndrome
#5
Clare Arnott, Geoff Strange, Andrew Bullock, Adrienne C Kirby, Clare O'Donnell, Dorothy J Radford, Leeanne E Grigg, David S Celermajer
OBJECTIVE: Eisenmenger syndrome (ES) is a severe form of pulmonary hypertension in adults with congenital heart disease (CHD) and has a poor prognosis. We aimed to understand factors associated with survival in ES and particularly to assess the potential benefits of advanced pulmonary vasodilator therapy (AT). METHODS: From January 2004, when AT became generally available for patients with ES, we followed 253 ES adults from 12 adult congenital heart disease centres across Australia and New Zealand...
August 9, 2017: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/28794092/optic-disc-swelling-in-a-patient-with-tuberculous-meningitis-a-diagnostic-challenge
#6
Hanis Zuhaimy, Sue Ngein Leow, Suresh K Vasudevan
We discuss the diagnostic challenge in an adult patient presented with purely ocular symptoms diagnosed with tuberculous meningitis (TBM). A 38-year-old woman presented with bilateral painless blurring of vision. There were bilateral sixth cranial nerve palsy and bilateral optic disc swelling. Optic nerve function tests were normal. Patient was lucid with no signs of meningism. Brain imaging were normal. She had a positive Mantoux test, high erythrocyte sedimentation rate but no clinical evidence of active pulmonary tuberculosis infection...
August 9, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28791832/prognostic-factors-in-pulmonary-arterial-hypertension-literature-review
#7
REVIEW
Emilia Stępnowska, Ewa Lewicka, Alicja Dąbrowska-Kugacka, Paweł Miękus, Grzegorz Raczak
Pulmonary arterial hypertension is a disease that has a bad influence on the patient's prognosis. Recently, the possibility of therapy has dramatically changed. Nowadays, the treatment of this disease is concerned mainly with the pathophysiological target. In clinical practice, it is important to start therapy at the appropriate time, when the patient is qualified because of an unsatisfactory examination result or improve therapy when the patient is getting worse. The understanding of prognosis factors in pulmonary arterial hypertension is necessary, because it is used to determine the length of patients' life expectancy...
May 2017: Advances in Clinical and Experimental Medicine: Official Organ Wroclaw Medical University
https://www.readbyqxmd.com/read/28790444/anti-smd1-antibodies-are-associated-with-renal-disorder-seizures-and-pulmonary-arterial-hypertension-in-chinese-patients-with-active-sle
#8
Chaojun Hu, Mengtao Li, Jian Liu, Junyan Qian, Dong Xu, Shulan Zhang, Ping Li, Jiulang Zhao, Xinping Tian, Xiaofeng Zeng
Detection of autoantibodies in systemic lupus erythematosus (SLE) plays an important role in timely diagnosis and earlier treatment of SLE. In this study, we used a SmD1 polypeptide-based ELISA to determine anti-SmD1 antibody in 269 SLE, including100 naïve (had not been treated with steroids or immunosuppressants at study inception) SLE patients and 169 non-naive SLE patients; 233 controls with other rheumatic diseases (RDC) (70 RA, 40 AS, 73SSc, and 50 SS), and 110 healthy controls (HC) group. The positive rate of anti-SmD1 among all SLE patients was 60...
August 8, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28790235/the-challenge-in-diagnosis-and-current-treatment-of-chronic-thromboembolic-pulmonary-hypertension
#9
Anggoro Budi Hartopo, Lucida Kris Dinarti, Abdul S Wahab
Chronic thromboembolic pulmonary hypertension (CTEPH) is currently underdiagnosis and consequently undertreatment in the clinical practice. A deficient in diagnostic modality and treatment availability especially in developing countries makes the CTEPH diagnosis unlikely to confirm. However, high index of clinical suspicion of CTEPH will lead to proper diagnosis and correct treatment  with significant reduction in morbidity and mortality. Left untreated, the mean survival time is 6.8 years and the three year mortality rate may be as high as 90 %...
April 2017: Acta Medica Indonesiana
https://www.readbyqxmd.com/read/28768975/sudden-death-in-a-patient-with-pulmonary-veno-occlusive-disease-pvod-and-severe-pulmonary-hypertension
#10
Minako Saito, Nobuharu Ohshima, Hirotoshi Matsui, Akira Hebisawa, Ken Ohta
A 58-year-old woman was referred to our hospital with a chief complaint of exertional dyspnea. Bronchoscopy failed to establish a diagnosis, and the patient subsequently died suddenly due to respiratory insufficiency because of advanced pulmonary hypertension (PH). The pathological diagnosis at autopsy was pulmonary veno-occlusive disease (PVOD). PVOD is difficult to diagnose antemortem and has a poor prognosis. Lung transplantation is the only curative treatment for PVOD.
2017: Internal Medicine
https://www.readbyqxmd.com/read/28762624/chronic-complications-of-venous-thromboembolism
#11
M-P Winter, G H Schernthaner, I M Lang
Venous thromboembolism (VTE) is a common disease (~700 per 100 000) that is associated with significant risk of recurrence, chronic complications, and substantial mortality, with reported death rates of up to 40% at 10 years. The development of novel anticoagulants has revolutionized the treatment of acute VTE, while strategies for prevention and treatment of chronic complications still seek for such a landmark change. Impaired thrombus resolution is the common denominator behind VTE complications, which are postthrombotic syndrome (PTS) and chronic thromboembolic pulmonary hypertension (CTEPH)...
August 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28759737/differential-diagnosis-of-hepatopulmonary-syndrome-hps-portopulmonary-hypertension-pph-and-hereditary-hemorrhagic-telangiectasia-hht
#12
REVIEW
Inna Krynytska, Mariya Marushchak, Anna Mikolenko, Anzhela Bob, Iryna Smachylo, Ludmyla Radetska, Olga Sopel
Hepatopulmonary syndrome (HPS) is a severe complication of advanced liver disease associated with an extremely poor prognosis. HPS is diagnosed in 4-47% of patients with cirrhosis and in 15-20% of candidates for liver transplantation. In addition, severe hypoxia is associated with a high risk of complications of liver transplantation (a 30% chance during the first 90 days) and increases the gap between transplantation and improving arterial oxygenation. The pathogenesis of HPS is not fully understood, and no effective pharmacological treatment has been developed yet...
July 31, 2017: Bosnian Journal of Basic Medical Sciences
https://www.readbyqxmd.com/read/28758715/serial-sonographic-assessment-of-pulmonary-edema-in-patients-with-hypertensive-acute-heart-failure
#13
Jennifer L Martindale, Michael Secko, John F Kilpatrick, Ian S deSouza, Lorenzo Paladino, Andrew Aherne, Ninfa Mehta, Alyssa Conigiliaro, Richard Sinert
OBJECTIVES: Objective measures of clinical improvement in patients with acute heart failure (AHF) are lacking. The aim of this study was to determine whether repeated lung sonography could semiquantitatively capture changes in pulmonary edema (B-lines) in patients with hypertensive AHF early in the course of treatment. METHODS: We conducted a feasibility study in a cohort of adults with acute onset of dyspnea, severe hypertension in the field or at triage (systolic blood pressure ≥ 180 mm Hg), and a presumptive diagnosis of AHF...
July 31, 2017: Journal of Ultrasound in Medicine: Official Journal of the American Institute of Ultrasound in Medicine
https://www.readbyqxmd.com/read/28742187/pulmonell-hypertension-vanligt-vid-kronisk-lungsjukdom-europeiska-riktlinjer-kring-utredning-och-behandling
#14
David Kylhammar, Göran Rådegran
Pulmonary hypertension due to lung diseases In 2015 the European Society of Cardiology and European Respiratory Society published new guidelines on the diagnosis and treatment of pulmonary hypertension (PH). PH due to lung diseases and/or hypoxia was classified as a separate entity. PH is common in lung diseases, but seldom severe. Nevertheless, the presence of PH in a patient with lung disease is associated with worse outcome. If there is clinical suspicion of PH in a patient with lung disease, echocardiography is recommended, and if there are signs of severe PH and/or severe right ventricular dysfunction the patient should be referred to a PH expert centre...
July 21, 2017: Läkartidningen
https://www.readbyqxmd.com/read/28734808/-congenital-cytomegalovirus-infection-manifesting-as-neonatal-respiratory-distress-in-an-hiv-exposed-uninfected-newborn
#15
A Pham, H El Mjati, N Nathan, F Kieffer, D Mitanchez
BACKGROUND: Cytomegalovirus (CMV) is one of the most common intrauterine infections, affecting approximately 1% of all live births. There are few reports on congenital CMV infections manifesting as isolated pneumonitis. CASE REPORT: We report a case of congenital CMV with neonatal respiratory distress affecting an HIV-exposed uninfected infant. This infant required noninvasive ventilation beginning within the first 15min of life. The initial chest X-ray showed diffuse bilateral ground-glass opacifications...
July 19, 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/28725444/post-tuberculous-fibrosing-mediastinitis-a-review-of-the-literature
#16
REVIEW
Zhe Wu, Hannah Jarvis, Luke S Howard, Corrina Wright, Onn Min Kon
Fibrosing mediastinitis (FM) is a rare disease where there is thickening of the fibrous tissue in the mediastinum. While histoplasmosis is the the most common recognised cause, the link with tuberculosis (TB) has been rarely documented. We review the link between TB and FM, and describe a case of probable TB-related FM.Our case is of a 74-year-old man who presented with breathlessness 3 years after fully treated TB. Scans revealed a calcified soft tissue mass within the mediastinum, and a diagnosis of fibrosing mediastinitis resulting in pulmonary hypertension was made...
2017: BMJ Open Respiratory Research
https://www.readbyqxmd.com/read/28722077/invasive-haemodynamic-evaluation-of-the-pulmonary-circulation-in-pulmonary-hypertension
#17
Alberto Pagnamenta, Andrea Azzola, Maurice Beghetti, Frédéric Lador, On Behalf Of The Swiss Society Of Pulmonary Hypertension
The term pulmonary hypertension refers to a serious condition characterised by high pulmonary vascular pressure, mainly as a consequence of various cardiac and respiratory diseases. Current clinical classification of pulmonary hypertension considers five distinct groups. Transthoracic echocardiography represents the first and most important noninvasive screening tool for estimating the probability of pulmonary hypertension. The diagnostic approach to pulmonary hypertension is supported by a proposed algorithm, which identifies the underlying cause...
July 19, 2017: Swiss Medical Weekly
https://www.readbyqxmd.com/read/28715904/considerations-for-optimal-management-of-patients-with-pulmonary-arterial-hypertension-a-multi-stakeholder-roundtable-discussion
#18
Sean M Studer, Martha Kingman, Luis Calo, H Eric Cannon, Jeffrey D Dunn, Thomas James, Sonya J Lewis, Robert J Gilkin, Janis A Pruett
A roundtable panel of national and regional managed care decision makers and providers met to discuss pulmonary arterial hypertension (PAH) and strategies for management. As a rare, complex disease with high economic costs and potentially devastating outcomes, PAH necessitates that managed care providers balance optimal care with efficient use of healthcare resources. PAH specialists are recognized by health plans as knowledgeable experts and integral partners in managing patients and resources. The diagnosis of PAH must be confirmed by a right heart catheterization...
May 2017: American Journal of Managed Care
https://www.readbyqxmd.com/read/28702341/severe-dyspnea-in-a-patient-with-neurofibromatosis-type-1
#19
P B Poble, J C Dalphin, B Degano
Neurofibromatosis type 1 (NF1) is a genetic disease in which pulmonary complications are rare, but severe, especially pulmonary hypertension (PH). The mechanisms underlying the onset of PH in patients with NF1 are unclear and might be multifactorial. In particular, the frequent presence of pulmonary parenchymal lesions makes etiological diagnosis of PH difficult. We describe here the case of a patient with NF1 admitted to our clinic with dyspnea and right heart failure revealing severe pre-capillary PH. Parenchymal lesions were mild and PH was attributed to pulmonary vascular involvement...
2017: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/28702260/renin-angiotensin-system-modifying-therapies-are-associated-with-improved-pulmonary-health
#20
Maira Soto, Soo I Bang, Jeff McCombs, Kathleen E Rodgers
BACKGROUND: Pulmonary diseases are often complicated and have diverse etiologies. One common factor is the lack of therapeutics available for these diseases. The goal of this study was to investigate the impact of Renin-Angiotensin System (RAS)-modifying medications on incidence and time to pulmonary complications. METHODS: A retrospective analysis was conducted using claims data from a US commercial insurance company (2007-2013). The study consisted of patients with an emerging hypertension (HTN) diagnosis...
2017: Clinical Diabetes and Endocrinology
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