Zoe Brown, Susanna Proudman, Kathleen Morrisroe, Wendy Stevens, Dylan Hansen, Mandana Nikpour
BACKGROUND: Systemic sclerosis (scleroderma, SSc) is a chronic multisystem autoimmune disease characterised by fibrosis of the skin and internal organs and vasculopathy. One of the major contributors to mortality in patients with SSc is pulmonary arterial hypertension (PAH). International recommendations advise annual screening for the early detection of PAH in asymptomatic patients with SSc. OBJECTIVES: To evaluate by systematic review current measures employed for screening for PAH...
April 4, 2021: Seminars in Arthritis and Rheumatism
Zhiliang Zuo, Jirong Yue, Bi Rong Dong, Taixiang Wu, Guan J Liu, Qiukui Hao
BACKGROUND: Thrombolytic therapy is usually reserved for people with clinically serious or massive pulmonary embolism (PE). Evidence suggests that thrombolytic agents may dissolve blood clots more rapidly than heparin and may reduce the death rate associated with PE. However, there are still concerns about the possible risk of adverse effects of thrombolytic therapy, such as major or minor haemorrhage. This is the fourth update of the Cochrane review first published in 2006. OBJECTIVES: To assess the effects of thrombolytic therapy for acute pulmonary embolism...
April 15, 2021: Cochrane Database of Systematic Reviews
Haizhao Zhao, Aili Guo, Minmin Wang, Zhifeng Cai, Xiaoyue Liu, Qingyu Kong, Cuifen Zhao
Pulmonary artery hypertension (PAH) is a common and serious disease which is characterized by pulmonary vascular remodeling. Bosentan (BST) is the first approved oral targeted drug of endothelin-1 (ET-1) receptor antagonists for the treatment of PAH. MicroRNA-27a (miR-27a) and peroxisome proliferator-activated receptor γ (PPARγ) were found to be related to the pathogenesis of PAH. To further explore the signal transduction mechanism of BST in the treatment of PAH, we examined the effects of BST on endothelin receptors, miR-27a, and PPARγ...
April 15, 2021: Pediatric Cardiology
Simon Malenfant, Marius Lebret, Émilie Breton-Gagnon, François Potus, Roxane Paulin, Sébastien Bonnet, Steeve Provencher
Exercise intolerance is a cardinal symptom of pulmonary arterial hypertension (PAH) and strongly impacts patients' quality of life (QoL). Although central cardiopulmonary impairments limit peak oxygen consumption ( V' O2peak ) in patients with PAH, several peripheral abnormalities have been described over the recent decade as key determinants in exercise intolerance, including impaired skeletal muscle (SKM) morphology, convective O2 transport, capillarity and metabolism indicating that peripheral abnormalities play a greater role in limiting exercise capacity than previously thought...
June 30, 2021: European Respiratory Review: An Official Journal of the European Respiratory Society
Anand Kumar Mishra, Sanjeev Hanumantacharya Naganur, Vidur Bansal, Pratyaksha Rana
Aortopulmonary window is a rare congenital heart defect. Left main coronary artery extrinsic compression by an enlarged pulmonary artery is a rare complication and a potential cause for chest pain and sudden cardiac death in patients with pulmonary hypertension. Here, we present the case of a 14-year-old boy with a large aortopulmonary window who was planned for a device closure, but during the procedure, he developed ST-T segment changes while the device was being deployed, and hence the procedure was abandoned...
April 15, 2021: Cardiology in the Young
Megan S Joseph, Francis Tinney, Abhijit Naik, Raviprasenna Parasuraman, Milagros Samaniego-Picota, Nicole M Bhave
INTRODUCTION: Pulmonary hypertension is common among patients with end-stage renal disease, although data regarding the impact of right ventricular (RV) failure on postoperative outcomes remain limited. We hypothesized that echocardiographic findings of RV dilation and dysfunction are associated with adverse clinical outcomes after renal transplant. METHODS: A retrospective review of adult renal transplant recipients at a single institution from January 2008 to June 2010 was conducted...
April 14, 2021: Cardiorenal Medicine
Giovanni Meliota, Ugo Vairo, Romina Ficarella, Leonardo Milella, Maria Felicia Faienza, Gabriele D'Amato
BACKGROUND: Filamin A (FLNA) is an intracellular actin-binding protein, encoded by the FLNA gene, with a wide tissue expression. It is involved in several cellular functions, and extracellular matrix structuring. FLNA gene alterations lead to diseases with a wide phenotypic spectrum, such as brain periventricular nodular heterotopia (PVNH), cardiovascular abnormalities, skeletal dysplasia, and lung involvement. CLINICAL FINDINGS: We present the case of a female infant who showed at birth aortic valve stenosis and PVNH, and subsequently developed interstitial lung disease with severe pulmonary hypertension...
April 12, 2021: Advances in Neonatal Care: Official Journal of the National Association of Neonatal Nurses
Ays Lee, K A Patterson, D J Tan, M E Wilson, S M Proudman, W Stevens, M Nikpour, J Sahhar, G-S Ngian, J Roddy, P J Roberts-Thomson, J G Walker
Objective : We undertook a comprehensive cross-sectional analysis of a multicentred Australian cohort of systemic sclerosis (SSc) patients to evaluate the associations of anti-Ro52/TRIM21 with SSc pulmonary involvement. Method : The study included 596 patients from the Australian Scleroderma Cohort Study database whose anti-Ro52/TRIM21 status was known. Anti-Ro52/TRIM21 was measured via line immunoassay. Data on demographic variables, autoantibody profiles, presence of interstitial lung disease (ILD), presence of pulmonary arterial hypertension (PAH), oxygen saturation, Six-Minute Walk Test distance, Borg dyspnoea score, and lung function tests were extracted...
April 14, 2021: Scandinavian Journal of Rheumatology
Richard Coulie, Dmitriy M Niyazov, Michael J Gambello, Elodie Fastré, Pascal Brouillard, Miikka Vikkula
Hypotrichosis-lymphedema-telangiectasia syndrome (HLTS) is a rare condition caused by pathogenic variants in the SOX18 gene. SOX18 plays a key role in angio- and lymphangiogenesis due to its expression in venous endothelial cells from which the lymphatic system develops. It is also expressed in embryonic hair follicles, heart, and vascular smooth muscle cells. The main clinical symptoms of HLTS include sparse hair, alopecia totalis, lymphedema, most often affecting lower limbs, and telangiectatic lesions. Only 10 patients with a SOX18 pathogenic variant have been described that presented with additional features such as hydrocele, renal failure, arterial or pulmonary hypertension, aortic dilatation, and facial dysmorphism...
April 14, 2021: American Journal of Medical Genetics. Part A
Arun Jose, Christine Zhou, Rachel Baker, Jackson Walker, Nicholas Kurek, Robert E O'Donnell, Jean M Elwing, Myron Gerson
BACKGROUND: The clinical significance of incidentally found RV abnormalities on low-risk SPECT studies is not well-defined. The objective of this study was to determine the predictive value of incidental right ventricular (RV) abnormalities identified on single photon emission computed tomography (SPECT) scans for mortality and pulmonary hypertension (PH). METHODS: We retrospectively analyzed all low-risk SPECT studies in patients without known coronary artery or pulmonary vascular disease, performed at our institution, from 2007-2020...
April 13, 2021: Journal of Nuclear Cardiology
Le Pavec Jérôme, Feuillet Séverine, Mercier Olaf, Pradère Pauline, Dauriat Gaëlle, Crutu Adrian, Florea Valentina, Savale Laurent, Levy Marilyne, Laverdure Florent, Stephan François, Fabre Dominique, Mitilian Delphine, Boulate David, Mussot Sacha, Hascoët Sébastien, Bonnet Damien, Humbert Marc, Fadel Elie
PURPOSE: Pulmonary arterial hypertension (PAH) is rare but remains fatal in infants and children despite the advance of targeted therapies. Lung transplantation (LTx), first performed in pediatric patients in the 1980s, is, with the Potts shunt, the only potentially life-extending option in patients with end-stage PAH but is possible only in tightly selected patients. Size-matching challenges severely restrict the donor organ pool, resulting-together with peculiarities of PAH in infants-in high waitlist mortality...
March 26, 2021: Journal of Heart and Lung Transplantation
Wenlei Li, Li Ma, Shuliang Xia, Minghui Zou, Weidan Chen, Xinxin Chen
BACKGROUND: This research aims to summarize the findings of the early single-stage revascularization of remnant pulmonary artery in unilateral absent intrapericardial pulmonary artery. METHODS: We retrospectively analyzed the medical records of 10 patients with unilateral absent pulmonary artery, in which 7 were right and 3 were left, the median age and mean weight at surgery was 4 months and 5.6 kg, respectively. The patients received operation from January 2009 to June 2020...
April 13, 2021: Journal of Cardiothoracic Surgery
Dalya Munves Ferguson, Vikas S Gupta, Pamela A Lally, Matias Luco, KuoJen Tsao, Kevin P Lally, Neil Patel, Matthew T Harting
INTRODUCTION: Pulmonary hypertension (PH) is the major pathophysiologic consequence of congenital diaphragmatic hernia (CDH). We aimed to evaluate the association between early CDH-associated PH (CDH-PH) and inpatient outcomes. METHODS: The CDH Study Group registry was queried for infants born 2015-2019 with echocardiograms before 48h of life. PH was categorized using echocardiographic findings: none, mild (right ventricular systolic pressure <2/3 systemic), moderate (between 2/3 systemic and systemic), or severe (supra-systemic)...
April 13, 2021: Neonatology
Kim M Kerr, C Greg Elliott, Raymond L Benza, Richard N Channick, Kelly Chin, R Duane Davis, Sonia Jain, Andrea LaCroix, Michael M Madani, Vallerie V McLaughlin, Myung Park, Victor F Tapson, William R Auger
BACKGROUND: Background: Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare sequela of acute pulmonary embolism that is treatable when recognized. Awareness of this disease has increased with recent advancements in therapeutic options, but delays in diagnosis remain common and diagnostic and treatment guidelines are often not followed. Data gathered from international registries has improved our understanding of CTEPH, but this data may not be applicable to the U.S. population due to differences in demographics and medical practice patterns...
April 13, 2021: JMIR Research Protocols
Miharu Ito, Keita Terui, Kouji Nagata, Masaya Yamoto, Masayuki Shiraishi, Hiroomi Okuyama, Hideo Yoshida, Naoto Urushihara, Katsuaki Toyoshima, Masahiro Hayakawa, Tomoaki Taguchi, Noriaki Usui
Congenital diaphragmatic hernia (CDH) is a birth defect of the diaphragm in which abdominal organs herniate through the defect into the thoracic cavity. The main pathophysiology is respiratory distress and persistent pulmonary hypertension because of pulmonary hypoplasia caused by compression of the elevated organs. Recent progress in prenatal diagnosis and postnatal care has led to an increase in the survival rate of patients with CDH. However, some survivors experience mid- and long-term disabilities and complications requiring treatment and follow-up...
April 13, 2021: Pediatrics International: Official Journal of the Japan Pediatric Society
Aya Banno, Tomoyuki Kanazawa, Kazuyoshi Shimizu, Tatsuo Iwasaki, Kenji Baba, Shinichi Otsuki, Hiroshi Morimatsu
PURPOSE: The relationship between regional cerebral oxygen saturation (rSO2 ) and the amount of left-to-right shunt in ventricular septal defect (VSD) patients has not yet been investigated. The purpose of this study was to identify the association of preoperative pulmonary to systemic blood flow (Qp/Qs) ratio and preoperative rSO2 in patients with VSD. METHODS: We retrospectively evaluated 49 VSD surgical closure candidates at a single institution. Preoperative Qp/Qs ratio was compared with rSO2 measurements at the time of VSD closure surgery...
April 13, 2021: Journal of Anesthesia
Warren B Gefter, Kyung Soo Lee, Mark L Schiebler, Grace Parraga, Joon Beom Seo, Yoshiharu Ohno, Hiroto Hatabu
Pulmonary functional imaging may be defined as the regional quantification of lung function by using primarily CT, MRI, and nuclear medicine techniques. The distribution of pulmonary physiologic parameters, including ventilation, perfusion, gas exchange, and biomechanics, can be noninvasively mapped and measured throughout the lungs. This information is not accessible by using conventional pulmonary function tests, which measure total lung function without viewing the regional distribution. The latter is important because of the heterogeneous distribution of virtually all lung disorders...
April 13, 2021: Radiology
William H Marshall, Stephen Gee, Woobeen Lim, Saurabh Rajpal
INTRODUCTION: The use of echocardiography to evaluate the probability for pulmonary hypertension (PH) in pregnant women has not been reported or correlated with outcomes. We hypothesized that in women with elevated right ventricular systolic pressure (RVSP) on echocardiography first identified during pregnancy, those with low probability for PH would have fewer major adverse cardiac events (MACE). METHODS: We performed a retrospective cohort study of pregnant women with RVSP >35 mm Hg on echocardiogram first identified during pregnancy...
April 13, 2021: Echocardiography
Daniele Guarino, Francesco Saia, Nevio Taglieri, Fabio Dardi, Mariangela Rotunno, Alessandra Manes, Fabio Niro, Sofia Martin Suarez, Davide Pacini, Nazzareno Galiè, Massimiliano Palazzini
Chronic thromboembolic pulmonary hypertension (CTEPH) is a complex disease where organized pulmonary thrombi and progressive vascular remodeling of the pulmonary arterial tree act synergistically to increase pulmonary vascular resistance and cause pulmonary hypertension. Balloon pulmonary angioplasty (BPA) has gained a renewed interest for the treatment of patients with CTEPH who are not undergoing surgery with pulmonary endarterectomy (PEA) or with persistent/recurrent pulmonary hypertension after PEA and has shown promising results in several observational studies conducted to date...
March 2021: Giornale Italiano di Cardiologia
Francesco Saia, Fabio Dardi, Nevio Taglieri, Mariangela Rotunno, Alessandra Manes, Daniele Guarino, Elisa Zuffa, Alessandro De Lorenzis, Ilenia Magnani, Alberto Ballerini, Fabio Niro, Sofia Martin Suarez, Davide Pacini, Enrico Gotti, Nazzareno Galiè, Massimiliano Palazzini
BACKGROUND: Balloon pulmonary angioplasty (BPA) represents a therapeutic option for the treatment of chronic thromboembolic pulmonary hypertension (CTEPH) in patients who are not eligible for surgical pulmonary endarterectomy (PEA) or with persistent/recurrent symptomatic pulmonary arterial hypertension after PEA. This study evaluated the safety of BPA during 5 years of experience of the only Italian center systematically performing this procedure. METHODS: The BPA program was activated at the S...
March 2021: Giornale Italiano di Cardiologia
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