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Pulmonary hypertension diagnosis and treatment

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https://www.readbyqxmd.com/read/29045954/-pulmonary-arterial-hypertension-as-leading-manifestation-of-methylmalonic-aciduria-clinical-characteristics-and-gene-testing-in-15-cases
#1
X Q Liu, H Yan, J X Qiu, C Y Zhang, J G Qi, X Zhang, H J Xiao, Y L Yang, Y H Chen, J B DU
OBJECTIVE: To deepen our understanding of Methylmalonic aciduria (MMA) associated pulmonary hypertension (PH) by analyzing the characteristics of clinical presentation, pulmonary high resolusion CT(HRCT), treatment response and gene mutation. METHODS: This study includes 15 cases of pediatric patients with MMA associated PH diagnosed and treated in Peking University First Hospital pediatric department between May 2012 and May 2016 with symptoms of PH as their leading presentation...
October 18, 2017: Beijing da Xue Xue Bao. Yi Xue Ban, Journal of Peking University. Health Sciences
https://www.readbyqxmd.com/read/29044995/mir-200c-regulates-endothelin-1-induced-pasmcs-abnormal-proliferation-and-apoptosis
#2
Chao Yuan, Min Xu, Rong Rong, Yong Mei, Wenyan Cai, Lin Li, Yao Xue, Baoli Zhu, Kai Sun, Lei Han
miR-200c is an antioncogene in multiple tumors. However, its function in the pathogenesis of pulmonary arterial hypertension (PAH) has not been thoroughly investigated nor understood. In this study, we discovered that miR-200c was able to substantially upregulate in pulmonary arterial smooth muscle cells (PASMCs) treated with endothelin-1 (ET-1). miR-200c also induced cell proliferation and suppressed cell apoptosis in PASMCs in vitro. However, miR-200c had no effect on G1/S/G2 transitions during the cell cycle...
October 16, 2017: IUBMB Life
https://www.readbyqxmd.com/read/29035757/pulmonary-hypertension-associated-with-bronchopulmonary-dysplasia-in-preterm-infants
#3
REVIEW
Christine B Bui, Merrin A Pang, Arvind Sehgal, Christiane Theda, Jason C Lao, Philip J Berger, Marcel F Nold, Claudia A Nold-Petry
Bronchopulmonary dysplasia (BPD) and BPD-associated pulmonary hypertension (BPD-PH) are chronic inflammatory cardiopulmonary diseases with devastating short- and long-term consequences for infants born prematurely. The immature lungs of preterm infants are ill-prepared to achieve sufficient gas exchange, thus usually necessitating immediate commencement of respiratory support and oxygen supplementation. These therapies are life-saving, but they exacerbate the tissue damage that is inevitably inflicted on a preterm lung forced to perform gas exchange...
October 2, 2017: Journal of Reproductive Immunology
https://www.readbyqxmd.com/read/29035287/cardio-pulmonary-involvement-in-systemic-sclerosis-a-study-at-a-tertiary-care-center
#4
Geetakiran Arakkal, Sudha Rani Chintagunta, Vasavilatha Chandika, Sudha Vani Damarla, Srinivas Manchala, B Udaya Kumar
BACKGROUND: Systemic sclerosis is a multisystem disorder characterized by microangiopathy, dysregulation of the immune system and massive deposition of collagen in the connective tissue of the skin, blood vessels and various internal organs. Although the mortality from renal crises has dropped significantly due to the use of angiotensin-converting enzyme inhibitors, cardiac and pulmonary involvement accounts for significant morbidity and mortality. We studied 28 patients with systemic sclerosis at Gandhi Medical College and Hospital, Hyderabad, over a period of two years for cardiopulmonary involvement...
November 2017: Indian Journal of Dermatology, Venereology and Leprology
https://www.readbyqxmd.com/read/29029950/survival-of-idiopathic-pulmonary-arterial-hypertension-patients-in-the-modern-era-in-australia-and-new-zealand
#5
Geoff Strange, Edmund M Lau, Eleni Giannoulatou, Carolyn Corrigan, Eugene Kotlyar, Fiona Kermeen, Trevor Williams, David S Celermajer, Nathan Dwyer, Helen Whitford, Jeremy P Wrobel, John Feenstra, Melanie Lavender, Kenneth Whyte, Nicholas Collins, Peter Steele, Susanna Proudman, Vivek Thakkar, Dominic Keating, Anne Keogh
BACKGROUND: Epidemiology and treatment strategies continue to evolve in pulmonary arterial hypertension (PAH). We sought to define the characteristics and survival of patients with idiopathic, heritable and drug-induced PAH in the current management era. METHODS: Consecutive cases of idiopathic, heritable and drug-induced PAH were prospectively enrolled into an Australian and New Zealand Registry. RESULTS: Between January 2012 and December 2016, a total of 220 incident cases were enrolled (mean age 57...
September 20, 2017: Heart, Lung & Circulation
https://www.readbyqxmd.com/read/29028375/health-disparities-in-patients-with-pulmonary-arterial-hypertension-a-blueprint-for-action-an-official-american-thoracic-society-statement
#6
Arunabh Talwar, Joe G N Garcia, Halley Tsai, Matthew Moreno, Tim Lahm, Roham T Zamanian, Roberto Machado, Steven M Kawut, Mona Selej, Stephen Mathai, Laura Hoyt D'Anna, Sonu Sahni, Erik J Rodriquez, Richard Channick, Karen Fagan, Michael Gray, Jessica Armstrong, Josanna Rodriguez Lopez, Vinicio de Jesus Perez
BACKGROUND: Health disparities have a major impact in the quality of life and clinical care received by minorities in the United States. Pulmonary arterial hypertension (PAH) is a rare cardiopulmonary disorder that affects children and adults and that, if untreated, results in premature death. The impact of health disparities in the diagnosis, treatment, and clinical outcome of patients with PAH has not been systematically investigated. OBJECTIVES: The specific goals of this research statement were to conduct a critical review of the literature concerning health disparities in PAH, identify major research gaps and prioritize direction for future research...
October 15, 2017: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/29027516/-chronic-thromboembolic-pulmonary-hypertension-recognition-diagnosis-and-treatment
#7
D Ruigrok, P Symersky, P W G Elbers, S Kamminga, L J Meijboom, H J Bogaard
- Chronic thromboembolic pulmonary hypertension (CTEPH), characterised by pulmonary hypertension and persistent perfusion defects despite adequate anticoagulation, causes significant morbidity and mortality.- Persistent dyspnoea after acute pulmonary embolism is frequent and an indication for additional diagnostics. Only a minority of these patients develop CTEPH.- Echocardiography and perfusion scintigraphy are the cornerstone of diagnostics when suspecting CTEPH. Right-heart catheterisation and pulmonary angiography should confirm the diagnosis...
2017: Nederlands Tijdschrift Voor Geneeskunde
https://www.readbyqxmd.com/read/28983709/late-complications-of-biliary-atresia-hepatopulmonary-syndrome-and-portopulmonary-hypertension
#8
Frederick M Karrer, Bradley J Wallace, Arturo E Estrada
Children with biliary atresia (BA) following Kasai portoenterostomy have a high risk for portal hypertension, however, while variceal and hemorrhagic complications have been more commonly studied, less frequent but no less possibly devastating complications of hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PPH) remain less well understood. HPS and PPH both occur in a setting of portal hypertension, however, paradoxically patients with HPS develop pulmonic vasculature dilation leading to shunting and hypoxia, while those with PPH develop an opposite progression of pulmonary vasoconstriction eventually leading to cor pulmonale and decompensation...
October 5, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28980901/long-term-treatment-of-scleroderma-related-digital-ulcers-with-iloprost-a-cohort-study
#9
Michele Colaci, Federica Lumetti, Dilia Giuggioli, Serena Guiducci, Silvia Bellando-Randone, Ginevra Fiori, Marco Matucci-Cerinic, Clodoveo Ferri
OBJECTIVES: Raynaud's phenomenon and chronic/recurrent digital ulcers (DU) are main features of systemic sclerosis (SSc). Their treatment includes both systemic (i.e., iloprost) and local therapies. We report the therapeutic effects of iloprost in a cohort of SSc patients during a long-lasting follow-up period. METHODS: Fifty consecutive SSc patients (M/F 7/43, age at SSc diagnosis 43.5±12.7SD years) received iloprost infusions for 10±4.2SD years. Iloprost schedule consisted in monthly infusion at 0...
September 18, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28978212/overview-of-idiopathic-pulmonary-fibrosis-ipf-and-evidence-based-guidelines
#10
Roozbeh Sharif
Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive form of interstitial lung disease (ILD), characterized by fibrosis and worsening lung function, that primarily occurs in those 50 years and older. Various causes including genetic susceptibility, environmental risk factors, and exposures have been suggested in the literature. All of these cause repetitive micro-injury to the lung tissue and vasculature, which triggers a cascade of inflammatory response and fibrosis. Symptoms are nonspecific and most patients present several years after the initial radiographic changes occur...
July 2017: American Journal of Managed Care
https://www.readbyqxmd.com/read/28976395/-pulmonary-hypertension-in-the-elderly
#11
Serdar Küçükoğlu, Ümit Yaşar Sinan
Pulmonary hypertension is defined as 25mmHg or higher mean pulmonary arterial pressure measured by right heart catheterization. Miscellaneous diseases may cause PH. Among these are pulmonary arterial hypertension (PAH), left heart disease, pulmonary disorders and hypoemia, and chronic thromboembolic disease. Pulmonary hypertension (PH) is seen frequently in the elderly. Although diseases resulting in systolic and diastolic dysfunction with accompanying pulmonary venous hypertension (PVH) account for a large percentage of PH in this population, PH associated with other respiratory diseases, such as chronic obstructive pulmonary disease (COPD), interstitial lung disease (ILD), and sleep apnea can also be seen...
September 2017: Türk Kardiyoloji Derneği Arşivi: Türk Kardiyoloji Derneğinin Yayın Organıdır
https://www.readbyqxmd.com/read/28966921/echocardiographic-evaluation-of-hemodynamics-in-neonates-and-children
#12
REVIEW
Yogen Singh
Hemodynamic instability and inadequate cardiac performance are common in critically ill children. The clinical assessment of hemodynamic status is reliant upon physical examination supported by the clinical signs such as heart rate, blood pressure, capillary refill time, and measurement of the urine output and serum lactate. Unfortunately, all of these parameters are surrogate markers of cardiovascular well-being and they provide limited direct information regarding the adequacy of blood flow and tissue perfusion...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28964391/-bilateral-proximal-pulmonary-embolism-without-associated-hypoxemia-case-report
#13
M Bahloul, K Chtara, O Turki, M M Kammoun, W Bouaziz, M Bouaziz
INTRODUCTION: Pulmonary embolism is a classic complication in intensive care. It is characterized by hypoxemia secondary to perturbed ventilation/perfusion ratios. We report a case of proximal and bilateral pulmonary embolism that occurred without associated hypoxemia. A spiral computed tomography (CT) scan was performed to explore unexplained fever in a patient with a negative infectious investigation. We discuss the mechanisms underlying the absence of hypoxemia in this patient. CLINICAL CASE: A 43-year-old patient with no significant pathological history was admitted to intensive care for the management of multiple injuries following a road accident...
October 2017: J Med Vasc
https://www.readbyqxmd.com/read/28959103/long-term-survival-of-patients-with-pulmonary-arterial-hypertension-at-a-single-center-in-taiwan
#14
Le-Yung Wang, Kuang-Tso Lee, Chia-Pin Lin, Lung-An Hsu, Chun-Li Wang, Tsu-Shiu Hsu, Wan-Jing Ho
BACKGROUND: Clinical studies have suggested predictive parameters in mortality risk assessment for pulmonary arterial hypertension (PAH) patients. However, these studies predominantly include Caucasian population; information in Asian population is relatively deficient. In this study, we investigated the long-term survival of PAH patients and the predictors of mortality in our population. METHODS: We prospectively collected 70 patients with PAH at the Chang Gung Memorial Hospital between March 2002 and February 2015...
September 2017: Acta Cardiologica Sinica
https://www.readbyqxmd.com/read/28950325/management-of-tricuspid-valve-regurgitation-position-statement-of-the-european-society-of-cardiology-working-groups-of-cardiovascular-surgery-and-valvular-heart-disease
#15
Manuel J Antunes, José Rodríguez-Palomares, Bernard Prendergast, Michele De Bonis, Raphael Rosenhek, Nawwar Al-Attar, Fabio Barili, Filip Casselman, Thierry Folliguet, Bernard Iung, Patrizio Lancellotti, Claudio Muneretto, Jean-François Obadia, Luc Pierard, Piotr Suwalski, Pepe Zamorano
Tricuspid regurgitation (TR) is a very frequent manifestation of valvular heart disease. It may be due to the primary involvement of the valve or secondary to pulmonary hypertension or to the left-sided heart valve disease (most commonly rheumatic and involving the mitral valve). The pathophysiology of secondary TR is complex and is intrinsically connected to the anatomy and function of the right ventricle. A systematic multimodality approach to diagnosis and assessment (based not only on the severity of the TR but also on the assessment of annular size, RV function and degree of pulmonary hypertension) is, therefore, essential...
August 21, 2017: European Journal of Cardio-thoracic Surgery
https://www.readbyqxmd.com/read/28948051/persistent-unexplained-dyspnea-a-case-of-hepatopulmonary-syndrome
#16
Alfonso Campanile, Alessandro Colombo, Maurizio Del Pinto, Claudio Cavallini
Regarding a patient with dyspnea, the history and physical examination often lead to the correct diagnosis. In some circumstances, when more than one underlying disease is present, the diagnostic process can be more challenging. We describe an unusual case of dyspnea and persistent hypoxemia related to a hepatopulmonary syndrome in a 53-year-old patient with known heart failure and chronic liver disease. Initially managed with intravenous diuretic therapy, due to signs of lung and peripheral congestion, our patient did not improve as expected; therefore we performed more advanced studies with a chest-abdomen CT scan and a right heart catheterization...
2017: Case Reports in Cardiology
https://www.readbyqxmd.com/read/28936439/a-case-of-pulmonary-arteriovenous-malformation-role-of-interventional-radiology-in-diagnosis-and-treatment
#17
Pranav Sharma, Puneet Kochar, Salil Sharma, Nishant Gupta, Shuo Li, Kusum Hooda, Yogesh Kumar
Pulmonary arterio-venous malformations (PAVMs) are abnormal pulmonary arteries and pulmonary veins communicating directly without interposition of a capillary bed and about 80-90% of patients with PAVMs eventually may present with hereditary hemorrhagic telangiectasia (HHT), remaining ones are sporadic cases. On the other hand, about 15-35% of HHT patients may present with PAVMs. The PAVMs have a tendency to grow and increase in size over time and various factors like puberty, pregnancy and pulmonary arterial hypertension (PAH) affect growth...
September 2017: Annals of Translational Medicine
https://www.readbyqxmd.com/read/28923474/beyond-the-inhaled-nitric-oxide-in-persistent-pulmonary-hypertension-of-the-newborn
#18
REVIEW
Mei-Yin Lai, Shih-Ming Chu, Satyan Lakshminrusimha, Hung-Chih Lin
Persistent pulmonary hypertension (PPHN) is a consequence of failed pulmonary vascular transition at birth and leads to pulmonary hypertension with shunting of deoxygenated blood across the ductus arteriosus (DA) and foramen ovale (FO) resulting in severe hypoxemia, and it may eventually lead to life-threatening circulatory failure. PPHN is a serious event affecting both term and preterm infants in the neonatal intensive care unit. It is often associated with diseases such as congenital diaphragmatic hernia, meconium aspiration, sepsis, congenital pneumonia, birth asphyxia and respiratory distress syndrome...
August 10, 2017: Pediatrics and Neonatology
https://www.readbyqxmd.com/read/28904255/predictors-of-favorable-responses-to-immunosuppressive-treatment-in-pulmonary-arterial-hypertension-associated-with-connective-tissue-disease
#19
Hidekata Yasuoka, Yuichiro Shirai, Yuichi Tamura, Tsutomu Takeuchi, Masataka Kuwana
BACKGROUND: The potential efficacy of immunosuppressive (IS) treatment has been reported in patients with pulmonary arterial hypertension (PAH) associated with connective tissue disease (CTD), but its positioning in the treatment algorithm remains uncertain. The aim of this study was to identify predictors of favorable responses to first-line IS treatment.Methods and Results:This single-center retrospective study included 30 patients with PAH accompanied by systemic lupus erythematosus (SLE), mixed CTD (MCTD), or primary Sjögren's syndrome (SS) who received first-line IS treatment alone or in combination with pulmonary vasodilators...
September 12, 2017: Circulation Journal: Official Journal of the Japanese Circulation Society
https://www.readbyqxmd.com/read/28890877/pulmonary-hypertension-diagnosis-imaging-techniques-and-novel-therapies
#20
REVIEW
Alan B Goldberg, Wojciech Mazur, Dinesh K Kalra
Pulmonary hypertension (PH), defined as the elevation of mean pulmonary arterial pressure (mPAP) above 25 mmHg, has numerous causes, which the World Health Organization (WHO) has divided into five distinct categories based upon the underlying mechanism of action. This review will explore the pathophysiology, diagnostic testing, and treatment of PH. Identification of PH depends on a strong clinical suspicion, a detailed history, and a thorough physical exam. We review the evidence supporting experimental and clinical laboratory parameters for diagnosis and monitoring of PH...
August 2017: Cardiovascular Diagnosis and Therapy
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