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Pulmonary hypertension diagnosis and treatment

Y Liu, Y P Liu, Y Zhang, J Q Song, H Zheng, H Dong, Y Y Ma, T F Wu, Q Wang, X Y Li, Y Ding, D X Li, Y Jin, M Q Li, Z X Wang, Y Yuan, H X Li, J Qin, Y L Yang
Objective: To analyz the current situation of the diagnosis, treatment and prevention of methylmalonic acidemia, the phenotypes, biochemical features and genotypes of the patients in the mainland of China, were investigated. Methods: Tottally 1 003 patients of methylmalonic acidemia from 26 provinces and municipalities of the mainland of China were enrolled. The clinical data, biochemical features and gene mutations were studied. Blood aminoacids and acylcarnitines, urine organic acids, and plasma total homocysteine were determined for the biochemical diagnosis...
June 2, 2018: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
Xianliang Xiong, Tengteng Zhu, Jiang Li
Pulmonary veno-occlusive disease (PVOD)/pulmonary capillary hemangiomatosis (PCH) is a rare form of pulmonary vascular disease that causes pulmonary arterial hypertension. The diagnosis of PVOD/PCH can be established by the combination of clinical features, physical examination, radiological findings, lung function, bronchoscopy and other resources. There is no established medical therapy for PVOD/PCH, and the only curative therapy for PVOD/PCH is lung transplantation. A girl with PVOD/PCH was diagnosed in the Second Xiangya Hospital...
May 28, 2018: Zhong Nan da Xue Xue Bao. Yi Xue Ban, Journal of Central South University. Medical Sciences
Michael P DeWane, Wassim H Fares, Cassius Iyad Ochoa Chaar
Aneurysms associated with arteriovenous fistulas (AVF) are rare but can cause significant morbidity. They are often diagnosed after rupture, pain, or embolization. We describe a unique case of a 35-year-old woman with a large iliac vein aneurysm related to an acquired AVF incidentally discovered on ultrasound during pregnancy. She had a diagnosis of "idiopathic" pulmonary hypertension for several years. This report details the diagnosis and treatment with an arterial stent graft that was effective in decreasing the size of the venous aneurysm and reversing the systemic changes associated with the disease...
June 7, 2018: Annals of Vascular Surgery
Anna R Hemnes
Pulmonary arterial hypertension (PAH) is a devastating disease for which there is no cure. Presently this condition is differentiated from other diseases of the pulmonary vasculature by a practitioner's history, physical examination, and clinical studies with clinical markers of disease severity primarily guiding therapeutic choices. New technologies such as next generation DNA sequencing, high throughput RNA sequencing, metabolomics and proteomics have greatly enhanced the amount of data that can be studied efficiently in patients with PAH and other rare diseases...
2018: Frontiers in Medicine
C A Quezada-Loaiza, A de Pablo Gafas, V Pérez, R Alonso, L Juarros, M I Real, E López, M Cortes, J C Meneses, I H González, V Díaz-Hellín Gude, P E Subías, P Gámez
BACKGROUND: Prognosis assessment of pulmonary hypertension (PH) is multifactorial and placement of patients on the lung transplantation (LT) waiting list requires the weighing of a complex set of criteria. The aim of this retrospective cohort study was to analyze a series of patients treated in our unit at the moment of their inclusion on the LT waiting list and long-term survival after LT. MATERIAL AND METHODS: Baseline characteristics, LT outcomes, and survival were evaluated in all patients diagnosed with pulmonary arterial hypertension (PAH) and pulmonary veno-occlusive disease (PVOD) who were included on the LT waiting list in 2011-2016...
June 2018: Transplantation Proceedings
Shan-Shan Zhou, Jing-Peng Jin, Ji-Qun Wang, Zhi-Guo Zhang, Jonathan H Freedman, Yang Zheng, Lu Cai
Cardiovascular diseases (CVD) are the leading cause of morbidity and mortality in the world. Although considerable progress has been made in the diagnosis, treatment and prognosis of CVD, there is still a critical need for novel diagnostic biomarkers and new therapeutic interventions to decrease the incidence of this disease. Recently, there is increasing evidence that circulating miRNAs (miRNAs), i.e. endogenous, stable, single-stranded, short, non-coding RNAs, can be used as diagnostic biomarkers for CVD...
June 7, 2018: Acta Pharmacologica Sinica
Yoichi Oshima, Tatsuya Suwabe, Yuji Marui, Noriko Hayami, Eiko Hasegawa, Masayuki Yamanouchi, Rikako Hiramatsu, Keiichi Sumida, Masahiro Kawada, Akinari Sekine, Hiroki Mizuno, Masahiko Oguro, Junichi Hoshino, Naoki Sawa, Yasuo Ishii, Takeshi Fujii, Kenichi Ohashi, Kenmei Takaichi, Yoshifumi Ubara
A 29-year-old woman with past medical history of hypertension was referred to our hospital for the evaluation of kidney dysfunction (serum creatinine 1.0 mg/dL), proteinuria (0.54 g/gCre), and microscopic hematuria. Renal biopsy before the first pregnancy was supportive for benign nephrosclerosis with no evidence of vasculitis. After her second pregnancy and delivery when she was 32 years old, she developed proteinuria of 3.2 g/gCre, hematuria, and elevated serum creatinine level of 2.6 mg/dL. Second renal biopsy revealed necrotizing glomerulonephritis and her serum MPO-ANCA was positive, leading to the diagnosis of MPA/renal-limited vasculitis (RLV)...
June 5, 2018: CEN Case Reports
Zahava Farkas, Abbas Haidry, Srikanth Yandrapalli, Marc Lim, Diwakar Jain, Wilbert S Aronow
With the growing use of imaging to aid in both diagnosis and treatment of patients presenting with a myriad of clinical presentations, incidental findings on imaging have become commonplace. Partial anomalous pulmonary venous connection (PAPVC) is a rare congenital cardiovascular condition that often goes undiagnosed due to lack of symptoms early in life. In adulthood, it can manifest clinically as right-sided heart failure or pulmonary hypertension. We present a case of PAPVC, which, like most cases, was discovered incidentally on chest X-ray...
April 2018: Annals of Translational Medicine
Tatsuro Ibe, Hiroshi Wada, Kenichi Sakakura, Yusuke Ugata, Miyuki Ito, Tomio Umemoto, Shin-Ichi Momomura, Hideo Fujita
Hypereosinophilic syndrome (HES) is characterized by multi-organ damage that is associated with tissue hypereosinophilia. A persistently elevated eosinophilic count is also required for the diagnosis of HES. Although HES affects various organs, damage to pulmonary artery is rarely reported. We present a case of a 39-year-old man who was diagnosed with pulmonary hypertension (PH) associated with idiopathic HES. Although the pulmonary arterial hypertension specific drugs including intravenous epoprostenol could not control his PH, corticosteroid was effective for both hypereosinophilia and PH...
May 23, 2018: International Heart Journal
Kathleen D Kolstad, Shufeng Li, Virginia Steen, Lorinda Chung
BACKGROUND: Pulmonary arterial hypertension (PAH) is a leading cause of death in patients with systemic sclerosis (SSc). The purpose of this study was to assess long-term outcomes in patients with SSc-PAH. METHODS: Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma is a prospective registry of SSc patients at high risk for or with incident pulmonary hypertension based on right heart catheterization (RHC). Incident World Health Organization Group I PAH patients were analyzed...
May 16, 2018: Chest
E M J Verstappen, A J G Maaskant-Braat, M R Scheltinga
INTRODUCTION: Calciphylaxis is a rare condition including patchy dermal necrosis that mostly affects chronic hemodialysis patients. The syndrome usually heralds impending death although patients may survive following a set of measures including an adapted dialysis regimen. The present case is a unique patient who recovered from an earlier episode of upper leg calciphylaxis 5 years previously but developed fatal bilateral breast necrosis. PRESENTATION OF CASE: A 69 year old Caucasian woman with a history of atrial fibrillation, hypertension, CVA, hyperparathyroidectomy for secondary hyperparathyroidism and end stage renal disease with hemodialysis recovered in 2012 from extensive symptomatic left upper leg necrosis due to calciphylaxis...
May 7, 2018: International Journal of Surgery Case Reports
Hui Deng, Sheng Zhao, Yunlong Yue, Yong Liu, Yali Xu, Jin Qian, Xiaorong Ma, Peiliang Gao, Xiaoyan Yao, Xin Jiang, Xiqi Xu, Zhicheng Jing, Yong Wang, Lei Pan, Xinying Xue
IgG4-related disease (IgG4-RD) is recognized as an immune-mediated condition with pathology features of lymphoplasmacytic infiltrate, storiform fibrosis, and obliterative phlebitis, accompanied with or without elevated serum IgG4 concentrations. However, few of pulmonary artery IgG4-RD causing pulmonary hypertension (PH) was reported.The medical records of 3 patients with pulmonary artery IgG4-RD inducing PH were analyzed retrospectively.Imaging findings demonstrated that the lesions of 3 patients located in pulmonary artery, which were initially diagnosed as pulmonary thrombus or malignant tumor...
May 2018: Medicine (Baltimore)
Carolina Stepffer, Evelyn Gaynor, Mariela López, Norma E González, Micaela Arri, Ana Ms De Dios
Pulmonary hypertension associated with human immunodeficiency virus infection is an extremely rare disease in pediatrics; it requires a high clinical suspicion to reach a diagnosis. Its appearance poses an unfavorable prognostic, but early diagnosis and specific treatment can improve outcomes. We report the clinical case of a fifteen-year-old patient diagnosed with human immunodeficiency virus infection of vertical transmission, without antiretroviral treatment, with cough and progressive exertional dyspnea, associated with signs of right heart failure in which severe pulmonary hypertension was diagnosed...
June 1, 2018: Archivos Argentinos de Pediatría
Maria Christiane Valeria Braga Braile-Sternieri, Eliana Migliorini Mustafa, Victor Rodrigues Ribeiro Ferreira, Sofia Braile Sabino, Giovanni Braile Sternieri, Lucia Angelica Buffulin de Faria, Bethina Canaroli Sbardellini, Cibele Olegario Vianna Queiroz, Domingo Marcolino Braile, Idiberto Jose Zotarelli Filho
The mortality rate of post-infarction cardiogenic shock (CS) was 80.0-90.0%. Recent studies show a significant reduction of hospital mortality to approximately 50.0%. CS is defined as systemic tissue hypoperfusion resulting from systolic and/or diastolic heart dysfunction, the main cause of which is acute myocardial infarction (AMI). The main predictors are biological markers such as troponin, CKMB and lactate. A systematic literature review and meta-analysis is performed in order to present and correlate the main literary findings on CS and its evolution with possible changes in biomarkers such as troponin, lactate and CKMB...
April 2018: Cardiology Research
Humna Abid Memon, Myung H Park
Pulmonary arterial hypertension (PAH) is characterized by pathological hemodynamic elevation in pulmonary artery pressure. Development of international registries over the last decade has raised awareness about the disease, leading to the development of new and improved therapies. Paradigm shifts such as these warrant review of existing literature regarding PAH, especially in females, as the disease continues to affect women more than males. The aim of this review is to provide an update on the classification, pathophysiology, diagnosis, and treatment of PAH while focusing specifically on its impact on women...
October 2017: Methodist DeBakey Cardiovascular Journal
Hang Yang, Qixian Zeng, Yanyun Ma, Bingyang Liu, Qianlong Chen, Wenke Li, Changming Xiong, Zhou Zhou
BACKGROUND: Pulmonary arterial hypertension (PAH) is a progressive and fatal disorder associated with high pulmonary artery pressure. Genetic testing enables early diagnosis and offers an opportunity for family screening. To identify genetic mutations and help make a precise diagnosis, we performed genetic testing in 191 probands with PAH and tried to analyze the genotype-phenotype correlation. METHODS: Initially, PAH samples (n = 119) were submitted to BMPR2 screening using Sanger sequencing...
May 9, 2018: Respiratory Research
Takaaki Nakamoto
Sleep-disordered breathing (SDB) is a risk factor for cardiovascular disease including acute coronary syndrome and acute myocardial infarction, and treating SDB prevents their development and recurrence and improves a patient's prognosis. Therefore, SDB is considered a therapeutic target for cardiovascular disease. In 2010, the Japanese Circulation Society published guidelines for the diagnosis and treatment of SDB in patients with cardiovascular disease. However, therapeutic intervention for patients with SDB was recently reported not to suppress the development or deterioration of cardiovascular disease in three middle- to large-sized randomized clinical trials: the SERVE-HF trial, which examined the effects of adaptive servo-ventilation (ASV) on patients with chronic heart failure (CHF) and central sleep apnea; the CAT-HF trial, which examined the effects of ASV therapy on patients with CHF after acute deterioration; and the SAVE study, which examined the secondary preventive effect of continuous positive airway pressure (CPAP) on patients with ischemic heart disease who had mild to moderate obstructive sleep apnea...
2018: Journal of Nippon Medical School, Nippon Ika Daigaku Zasshi
Stéphanie Franchi-Abella, Emmanuel Gonzales, Oanez Ackermann, Sophie Branchereau, Danièle Pariente, Florent Guérin
Congenital portosystemic shunts (CPSS) are rare vascular malformations that create an abnormal connection between portal and systemic veins resulting in complete or partial diversion of the portal flow away from the liver to the systemic venous system. Different anatomic types exist and several classifications have been proposed. They can be associated with other malformations especially cardiac and heterotaxia. The main complications include hepatic encephalopathy, liver tumors, portopulmonary hypertension, and pulmonary arteriovenous shunts...
May 5, 2018: Abdominal Radiology
Tullio Valente, Ahmad Abu-Omar, Giacomo Sica, Alfredo Clemente, Massimo Muto, Giorgio Bocchini, Salvatore Cappabianca, Gaetano Rea
BACKGROUND: Acquired peripheral or intraparenchymal pulmonary artery aneurysms (PPAA) are rare entities but are important to recognize because of the associated morbidity. Hemoptysis is their principal complication and is a potentially fatal condition. PURPOSE: To illustrate the causes, multidetector CT angiography (MDCTA) findings and differential diagnosis of acquired PPAA. MATERIALS AND METHODS: The institutional review boards approved this study...
May 2, 2018: La Radiologia Medica
Wen-Hui Wu, Ping Yuan, Si-Jin Zhang, Xin Jiang, Cheng Wu, Yuan Li, Shao-Fei Liu, Qian-Qian Liu, Jing-Hui Li, Bigyan Pudasaini, Qing-Hua Hu, Jocelyn Dupuis, Zhi-Cheng Jing
The association of sex hormone (estradiol, testosterone, and progesterone) with cardiopulmonary disease has already attracted great attention, especially in pulmonary arterial hypertension (PAH). However, the impact of sex hormones and their pituitary stimulators (follicle-stimulating hormone and luteinizing hormone) on PAH in men remains unclear. We conducted a prospective cohort study recruiting 95 patients with idiopathic PAH from 2008 to 2014 and following up for a median of 65 months for death. Compared with control, abnormal plasma levels of sex hormones were more common in patients with PAH...
April 30, 2018: Hypertension
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