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Pediatric pulmonary arterial hypertension

Hong-Yu Kuang, Yu-Hao Wu, Qi-Jian Yi, Jie Tian, Chun Wu, We Nian Shou, Tie-Wei Lu
BACKGROUND: Oral bosentan has been widely applied in pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD). A systemic review and meta-analysis was conducted for a therapeutic evaluation of oral bosentan in both adult and pediatric patients with PAH-CHD. The acute responses and a long-term effect were respectively assessed in a comparison with baseline characteristics, and the improvement of exercise tolerance was analyzed. METHODS: PubMed, Medline, Embase, and Cochrane Central Register of clinical controlled trails or observational studies have been searched for a recording of bosentan effects on the PAH-CHD participants...
March 2018: Medicine (Baltimore)
Martin Koestenberger, Alexander Avian, Hannes Sallmon, Andreas Gamillscheg, Gernot Grangl, Stefan Kurath-Koller, Sabrina Schweintzger, Ante Burmas, Georg Hansmann
OBJECTIVE: The right ventricular outflow tract (RVOT) is pivotal for adequate RV function and known to be adversely affected by elevated pulmonary arterial pressure (PAP) in adults with pulmonary hypertension (PH). Aim of this study was to determine the effects of increased RV pressure afterload in children with PH on RVOT size, function, and flow parameters. METHODS: We conducted a transthoracic echocardiographic study in 51 children with PH (median age: 5.3 years; range 1...
March 5, 2018: Echocardiography
Lia Gore, Pamela R Kearns, Maria Lucia de Martino Lee, Carmino Antonio De Souza, Yves Bertrand, Nobuko Hijiya, Linda C Stork, Nack-Gyun Chung, Rocio Cardenas Cardos, Tapan Saikia, Franca Fagioli, Jong Jin Seo, Judith Landman-Parker, Donna Lancaster, Andrew E Place, Karen R Rabin, Mariana Sacchi, Rene Swanink, C Michel Zwaan
Purpose Safe, effective treatments are needed for pediatric patients with chronic myeloid leukemia in chronic phase (CML-CP). Dasatinib is approved for treatment of adults and children with CML-CP. A phase I study determined suitable dosing for children with Philadelphia chromosome-positive (Ph+) leukemias. Methods CA180-226/NCT00777036 is a phase II, open-label, nonrandomized prospective trial of patients < 18 years of age receiving dasatinib. There are three cohorts: (1) imatinib-resistant/intolerant CML-CP, (2) imatinib-resistant/intolerant CML in accelerated/blast phase or Ph+ acute lymphoblastic leukemia (n = 17), and (3) newly diagnosed CML-CP treated with tablets or powder for oral suspension...
March 2, 2018: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
Michal Schäfer, Kathryn K Collins, Lorna P Browne, D Dunbar Ivy, Steven Abman, Richard Friesen, Benjamin Frank, Brian Fonseca, Michael DiMaria, Kendall S Hunter, Uyen Truong, Johannes C von Alvensleben
BACKGROUND: Electrical and right ventricular (RV) mechanical dyssynchrony has been previously described in pediatric pulmonary arterial hypertension (PAH), but less is known about the relationship between electrical dyssynchrony and biventricular function. In this study we applied cardiac magnetic resonance (CMR) imaging to evaluate biventricular size and function with a focus on left ventricular (LV) strain mechanics in pediatric PAH patients with and without electrical dyssynchrony...
February 6, 2018: Journal of Heart and Lung Transplantation
Ling Luo, Huanling Wang, Hongwei Fan, Jing Xie, Zhifeng Qiu, Taisheng Li
Chronic active Epstein-Barr virus (CAEBV) infection is a rare disease with high mortality. Most of CAEBV patients have been reported from Japan and are pediatric cases.The goal was to describe the clinical characteristics and the immunophenotypic features of peripheral lymphocytes in adult onset CAEBV patients.We retrospectively reviewed and analyzed all adult onset CAEBV cases admitted to Peking Union Medical College Hospital (PUMCH) between 2012 and 2016. Demographic, clinical, laboratory data, and the immunophentyping data of peripheral lymphocytes were collected...
March 2018: Medicine (Baltimore)
Devi Manuel, Gopal Ghosh, George Joseph, Anandaroop Lahiri, Paul V George
OBJECTIVE: To study the echocardiographic features of criss-cross heart (CCH), a congenital cardiac anomaly characterized by crossed ventricular inflow streams, in Indian patients. METHODS: In this retrospective observational study, all pediatric echocardiograms performed in a single tertiary care institution in South India over a three-year period were scrutinized for a diagnosis of CCH. Demographic, clinical and echocardiographic data were collected from patients' medical records and echocardiographic database...
January 2018: Indian Heart Journal
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
Q Liu, C Zhang, Q Q Li, Y Zhu, D Zhang, W G Zhao, H Gu
Objective: To analyze the prognosis and associated risk factors of pediatric idiopathic pulmonary arterial hypertension. Methods: A total of 119 patients under 18 years of age diagnosed as idiopathic pulmonary arterial hypertension in the Pulmonary Arterial Hypertension Center in Beijing Anzhen Hospital between June 2007 and May 2017 were enrolled in this retrospective study. The clinical informations and follow-up data were collected. The endpoints of follow-up were defined as death or undergoing lung transplantation...
January 2, 2018: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
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
Fernando Palvo, Alexandre Todorovic Fabro, Maria Célia Cervi, Davi Casale Aragon, Fernando Silva Ramalho, Ana Paula de Carvalho Panzeri Carlotti
We aimed to investigate the clinicopathological features of pertussis in children admitted to a tertiary-care university hospital in Brazil.This was a retrospective cohort study of all pediatric hospital admissions with pertussis from January 1, 2008 to December 31, 2014. We also reported the autopsy findings in children who died.Fifty-five patients admitted to the hospital over the study period had laboratorial confirmation of Bordetella pertussis infection, 17 (30.9%) needed pediatric intensive care unit (PICU) admission and 6 (10...
December 2017: Medicine (Baltimore)
Marian Gaballah, Rachelle Goldfisher, John B Amodio
Takayasu Arteritis (TA) is an inflammatory disorder involving the thoracoabdominal aorta and its branches and the pulmonary arteries, with eventual vascular stenosis, occlusion, or aneurysm formation. Conventional angiography has been the reference imaging standard for diagnosis of TA. The purpose of this case report is to demonstrate the utility of MR imaging and MR angiography in the diagnosis of Takayasu Arteritis in a pediatric patient. The patient is a 15-year-old female patient presenting with anemia, hypertension, and acute kidney injury...
2017: Case Reports in Pediatrics
Amir-Reza Hosseinpour, Marie-Hélène Perez, David Longchamp, Jacques Cotting, Nicole Sekarski, Michel Hurni, René Prêtre, Stefano Di Bernardo
OBJECTIVE: Congenital cardiac malformations with high pulmonary blood flow and pressure due to left-to-right shunts are usually repaired in early infancy for both the benefits of early relief of heart failure and the fear that the concomitant pulmonary hypertension may become irreversible unless these defects are corrected at an early age. Age, however, has been a poor predictor of irreversibility of pulmonary hypertension in our experience, which is presented here. DESIGN: A retrospective observational study...
October 25, 2017: Congenital Heart Disease
Saadoun Bin-Hasan, Abdullah Khayat, Tilman Humpl, Janette T Reyes, Suhail Al-Saleh
Central sleep apnea (CSA) and periodic breathing are unusual findings described in pediatric patients with congestive heart failure. However, CSA has not been reported in children with pulmonary hypertension. We hereby report on a 10-year-old girl with idiopathic pulmonary arterial hypertension who had frequent central events in a periodic breathing fashion seen in her polysomnography, which was normalized following medical treatment leading to improvement of the pulmonary pressures.
2017: Case Reports in Pediatrics
Shilpa Shah, Jacqueline R Szmuszkovicz
The perioperative period is an extremely tenuous time for the pediatric patient with pulmonary arterial hypertension. This article will discuss a multidisciplinary approach to preoperative planning, the importance of early identification of pulmonary hypertensive crises, and practical strategies for postoperative management for this unique group of children.
October 24, 2017: Children
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
Michal Schäfer, Uyen Truong, Lorna P Browne, Gareth J Morgan, Michael Ross, Richard Ing, Kendall S Hunter, Vitaly O Kheyfets, Steven H Abman, D Dunbar Ivy, Neil Wilson
We sought to compare pulmonary flow hemodynamic indices obtained by Fick and thermodilution catheterization techniques with phase-contrast MRI (PC-MRI) in children with diverse etiologies of pulmonary arterial hypertension (PAH). Calculation of pulmonary flow ([Formula: see text]) using the Fick principle in most catheter laboratories relies on an estimate of oxygen consumption which may limit its reliability. Flow hemodynamic indices acquired from thirty patients with PAH who underwent successful same-day PC-MRI and catheterization were evaluated for absolute and percent bias...
October 17, 2017: Pediatric Cardiology
Richard M Friesen, Michal Schäfer, Dale A Burkett, Courtney J Cassidy, D Dunbar Ivy, Pei-Ni Jone
Right ventricular (RV) failure is a significant cause of morbidity and mortality in patients with pulmonary hypertension (PH). Myocardial performance index measured by tissue Doppler imaging (TDI-MPI) has been useful in assessing RV dysfunction in adults with PH. However, TDI-MPI as a marker for RV dysfunction or disease severity has not been evaluated in pediatric PH. The aim of this study was to investigate TDI-MPI and correlate with invasive hemodynamics in pediatric PH patients. Eighty pediatric PH patients undergoing cardiac catheterization and simultaneous transthoracic echocardiography were analyzed...
October 4, 2017: Pediatric Cardiology
Divyakant Parmar, Ketav Lakhia, Pankaj Garg, Kartik Patel, Ritesh Shah, Jigar Surti, Jigar Panchal, Himani Pandya
OBJECTIVE: The objective of our study was to determine the feasibility of early extubation and to identify the risk factors for delayed extubation in pediatric patients operated for ventricular septal defect closure. METHODS: A prospective, observational study was carried out at our Institute. This study involved consecutive 135 patients undergoing ventricular septal defect closure. Patients were extubated if feasible within six hours after surgery. Based on duration of extubation, patients were divided two groups: Group 1= extubation time ≤ 6 hours, Group 2= extubation time >6 hours...
July 2017: Brazilian Journal of Cardiovascular Surgery
Satoshi Ikehara, Shinichi Takatsuki, Tomotaka Nakayama, Kazuyuki Naoi, Hiroyuki Matsuura, Tsutomu Saji
BACKGROUND: Few studies have investigated the clinical impact of pulmonary artery (PA) dilatation on outcomes in pediatric pulmonary arterial hypertension (PAH).Methods and Results:This study investigated the clinical outcomes of idiopathic or heritable PAH in 66 children aged <18 years at diagnosis. Main PA/thorax (MPA/T) ratio was measured on chest radiography in PAH patients. Patients were divided into 2 groups based on MPA/T ratio, and compared with a control group of 166 age- and gender-matched healthy children...
September 13, 2017: Circulation Journal: Official Journal of the Japanese Circulation Society
Maurice Beghetti
Pulmonary hypertension (PH) is diagnosed using cardiac catheterization and is defined as an elevation of mean pulmonary artery pressure (PAP) greater than 25 mmHg. Although invasive hemodynamics remains the gold standard and is mandatory for disease confirmation, transthoracic echocardiography (TTE) is an extremely useful non-invasive and widely available tool that allows for screening and follow-up, in particular, in the acute setting. TTE may be a valuable alternative, allowing for direct measurement and/or indirect assessment of PAP...
2017: Frontiers in Pediatrics
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