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https://www.readbyqxmd.com/read/28077181/at-what-age-should-tetralogy-of-fallot-be-corrected
#1
Julien I E Hoffman
Tetralogy of Fallot can be corrected with very low mortality at any age, even in neonates, but this does not necessarily mean that it should be corrected in the neonatal period. Although there are many advantages to early correction, a high proportion of these neonates have residual stenosis or pulmonary regurgitation that impairs ventricular function and may require further surgery or implantation of a pulmonary valve. Before we had the ability to correct this anomaly with low mortality in small children, a variety of palliative procedures had to be performed...
January 12, 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/28072626/acquired-coronary-artery-disease-in-adult-patients-with-congenital-heart-disease-a-true-or-a-false-problem
#2
Alessandro Giamberti, Mauro Lo Rito, Erika Conforti, Alessandro Varrica, Mario Carminati, Alessandro Frigiola, Lorenzo Menicanti, Massimo Chessa
BACKGROUND: The population of adults with congenital heart disease (ACHD) is increasing and aging, and a large percentage of this population is now over 65 years of age. For this reason, it is probable that acquired coronary artery disease (CAD) will become an important issue that needs to be addressed also in these patients. We retrospectively analyzed all ACHD patients who underwent surgery in our Institution with the aim to investigate the incidence of associated CAD and the results of surgical treatment...
January 7, 2017: Journal of Cardiovascular Medicine
https://www.readbyqxmd.com/read/28070915/mitral-valve-resistance-determines-hemodynamic-consequences-of-severe-rheumatic-mitral-stenosis-and-immediate-outcomes-of-percutaneous-valvuloplasty
#3
Hamidreza Sanati, Reza Zolfaghari, Niloufar Samiei, Yousef Rezaei, Mitra Chitsazan, Ali Zahedmehr, Farshad Shakerian, Reza Kiani, Ata Firouzi, Reza Rezaei Tabrizi
INTRODUCTION: The mitral valve area (MVA) poorly reflects the hemodynamic status of (MS). In this study, we compared the MVA with mitral valve resistance (MVR) with regard to the determination of hemodynamic consequences of MS and the immediate outcomes of percutaneous balloon mitral valvuloplasty (PBMV). METHODS: In a prospective study, 36 patients with severe rheumatic MS with left ventricular ejection fraction (LVEF) >50% were evaluated. They underwent transthoracic echocardiography (TTE) and catheterization...
January 10, 2017: Echocardiography
https://www.readbyqxmd.com/read/28056476/-advanced-systemic-sclerosis-endovascular-reconstruction-of-the-aortic-bifurcation-for-limb-salvage
#4
Florian Stein, Lars Kamper, Konstantinos Meletiadis, Ulrich Kusenack, Patrick Haage
History and admission findings A 69-year-old patient was initially hospitalized because of a 1.5 cm ulceration at the back of the right foot which had existed for the last year and become increasingly swollen and painful. Medical history revealed PmScl-positive systemic sclerosis presenting with a massive calcinosis cutis, advanced pulmonary fibrosis and peripheral artery disease. Examinations Inflammatory markers were normal. MR-Angiography of the lower extremities revealed a multi-segmental high-grade stenosis of the aortic bifurcation due to extensively calcified plaques...
January 2017: Deutsche Medizinische Wochenschrift
https://www.readbyqxmd.com/read/28050459/cardiovascular-abnormalities-in-egyptian-children-with-mucopolysaccharidoses
#5
Laila Selim, Nehal Abdelhamid, Emad Salama, Amera Elbadawy, Iman Gamaleldin, Mohamed Abdelmoneim, Abeer Selim
INTRODUCTION: The Mucopolysaccharidoses (MPS) are rare inherited metabolic disorders. They are characterized by the progressive systemic deposition of Glycosaminoglycans (GAGs). GAGs accumulate in the myocardium and the cardiac valves. Enzyme Replacement Therapy (ERT) is available for MPS I, II, and VI. However, ERT does not appear to improve cardiac valve disease in patients with valve disease present at the start of ERT. AIM: To evaluate the cardiac involvement in Egyptian children with MPS...
November 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28050205/tracheostomy-in-the-intensive-care-unit-a-university-hospital-in-a-developing-country-study
#6
Mohammad Waheed El-Anwar, Ahmad Abdel-Fattah Nofal, Mohammad A El Shawadfy, Ahmed Maaty, Alaa Omar Khazbak
Introduction Tracheostomy is the commonest surgical procedure in intensive care units (ICUs). It not only provides stable airway and facilitates pulmonary toilet and ventilator weaning, but also decreases the direct laryngeal injury of endotracheal intubation, and improves patient comfort and daily living activity. Objective The objective of this study is to assess the incidence, indications, timing, complications (early and late), and the outcome of tracheostomy on patients in the intensive care units (ICU) at a university hospital in a developing country...
January 2017: International Archives of Otorhinolaryngology
https://www.readbyqxmd.com/read/28049534/case-report-maternal-mosaicism-resulting-in-inheritance-of-a-novel-gata6-mutation-causing-pancreatic-agenesis-and-neonatal-diabetes-mellitus
#7
Daphne Yau, Elisa De Franco, Sarah E Flanagan, Sian Ellard, Miriam Blumenkrantz, John J Mitchell
BACKGROUND: Haploinsufficiency of the GATA6 transcription factor gene was recently found to be the most common cause of pancreatic agenesis, a rare cause of neonatal diabetes mellitus. Although most cases are de novo, we describe three siblings with inherited GATA6 haploinsufficiency and the rare finding of parental mosaicism. CASE PRESENTATION: The proband was born at term with severe intrauterine growth restriction, the first child of non-consanguineous parents...
January 3, 2017: Diagnostic Pathology
https://www.readbyqxmd.com/read/28043783/pulmonary-homograft-stenosis-in-the-ross-procedure-incidence-clinical-impact-and-predictors-in-long-term-follow-up
#8
Laura Pardo González, Martin Ruiz Ortiz, Mónica Delgado, Dolores Mesa, Rafael Villalba, Sara Rodriguez, Francisco J Hidalgo, Pedro Alados, Jaime Casares, Jose Suarez de Lezo
BACKGROUND: The Ross procedure is used in the treatment of selected patients with aortic valve disease. Pulmonary graft stenosis can appear in the long-term follow-up after the Ross intervention, but the factors involved and its clinical implications are not fully known. AIM: To describe the incidence, clinical impact and predictors of homograft stenosis and reintervention after the Ross procedure in a prospective series in a tertiary referral hospital. METHODS: From 1997 to 2009, 107 patients underwent the Ross procedure (mean age: 30±11 years; 69% men; 21 aged<18 years), and were followed for echocardiographic homograft stenosis (peak gradient>36mmHg) and surgical or percutaneous homograft reintervention...
December 30, 2016: Archives of Cardiovascular Diseases
https://www.readbyqxmd.com/read/28043758/-ebstein-s-like-anomaly-ventricular-double-inlet-a-rare-association
#9
Luis Muñoz Castellanos, Magdalena Kuri Nivon
INTRODUCTION AND OBJECTIVES: The association of univentricular heart with double inlet and Ebstein's "like" anomaly of the common atrioventricular valve is extremely rare. METHODS: Two hearts with this association are described with the segmental sequential system which determine the atrial situs, the types of atrioventricular and ventriculoarterial connections and associated anomalies. RESULTS: Both hearts had atrial situs solitus, and a univentricular heart with common atrioventricular valve, a foramen primum and double outlet ventricle with normal crossed great arteries...
December 30, 2016: Archivos de Cardiología de México
https://www.readbyqxmd.com/read/28043612/post-tuberculosis-pulmonary-function-and-noninfectious-pulmonary-disorders
#10
Muhammad Irfan
Tuberculosis (TB) is among the top infectious causes of morbidity and mortality worldwide and is associated with frequent pulmonary damage despite microbiological cure. Patients with treated TB may remain lifelong sufferers of disabling structural and functional sequelae of the disease, which subsequently impair quality of life. Long-term follow-up studies have revealed that many patients with treated pulmonary TB show signs of permanent impairment of their lung function. Impairment is variable in pattern and severity, ranging from none to severe, and shows restrictive, obstructive, or mixed patterns...
December 2016: International Journal of Mycobacteriology
https://www.readbyqxmd.com/read/28043470/single-center-experience-with-the-senning-procedure-in-the-current-era
#11
Giuseppe Ferro, Raghav Murthy, Vinod A Sebastian, Kristine J Guleserian, Joseph M Forbess
The Senning procedure is an operative technique for atrial inversion in congenital heart anomalies. We sought to evaluate our contemporary outcomes employing this technique. A retrospective analysis of all patients who underwent the Senning procedure at our institution was performed. Hospital records were reviewed, and follow-up data were obtained to evaluate outcomes. Overall, a total of 19 patients underwent a Senning procedure between August 2005 and July 2014. Median age at repair was 594 days (range: 5 days to 15 years)...
July 2016: Seminars in Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28043465/the-ongoing-evolution-of-sutureless-repairs-for-pulmonary-vein-anomalies
#12
EDITORIAL
Rachel D Vanderlaan, Christopher A Caldarone
The sutureless technique has evolved from an effective treatment modality for postrepair pulmonary venous stenosis to a prophylactic strategy for primary repair of total anomalous pulmonary venous connection. Although conclusive data demonstrating efficacy as a universal prophylactic strategy is lacking, Zhang et al provide evidence that supports the sutureless technique as a safe and an effective prophylactic strategy in certain subsets of high-risk patients.
July 2016: Seminars in Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28043462/right-ventricular-outflow-tract-reconstruction-with-a-polytetrafluoroethylene-monocusp-valve-a-20-year-experience
#13
Mohineesh Kumar, Mark W Turrentine, Mark D Rodefeld, Teresa Bell, John W Brown
In patients with tetralogy of Fallot (TOF), pulmonary atresia (PA), and other congenital right ventricular outflow tract (RVOT) malformations, polytetrafluoroethylene (PTFE) monocusp outflow tract patches (MOTP) relieve obstruction and provide pulmonary valve competence. The purpose of this study was to determine whether our PTFE-MOTP was an acceptable short- and mid-term remedy for patients with TOF or PA as assessed by freedom from severe pulmonary regurgitation and freedom from reoperation. From 1994-2014, 171 patients (mean age 1...
July 2016: Seminars in Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28043457/porcine-intestinal-submucosa-cormatrix-for-semilunar-valve-repair-in-children-a-word-of-caution-after-midterm-results
#14
Massimo A Padalino, Biagio Castaldi, Marny Fedrigo, Michele Gallo, Fabio Zucchetta, Vladimiro L Vida, Ornella Milanesi, Annalisa Angelini, Giovanni Stellin
Surgery for congenital valve anomalies in children is a challenging topic. We aim to assess early and late functional outcomes of CorMatrix scaffold after repair of aortic and pulmonary valves (PV) in congenital heart disease in a prospective nonrandomized clinical study on children with congenital aortic (Group 1) or PV (Group 2) disease. Primary endpoints were reoperation or reintervention on semilunar valves and echocardiographic evidence of regurgitation or stenosis greater than mild. Results of PV repair in tetralogy of Fallot were compared with a control group of patients who underwent PV repair with polytetrafluoroethylene...
July 2016: Seminars in Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28043454/surgical-techniques-for-repair-of-peripheral-pulmonary-artery-stenosis
#15
Richard D Mainwaring, Frank L Hanley
Peripheral pulmonary artery stenosis (PPAS) is a rare form of congenital heart disease that is most frequently associated with Williams and Alagille syndromes. These patients typically have systemic level right ventricular pressures secondary to obstruction at the lobar, segmental, and subsegmental branches. The current management of patients with PPAS remains somewhat controversial. We have pioneered an entirely surgical approach for the reconstruction of PPAS. This approach initially entailed a surgical patch augmentation of all major lobar branches and effectively reduced the right ventricular pressures by more than half...
July 2016: Seminars in Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28041807/recurrent-pulmonary-vein-stenosis-after-pulmonary-vein-isolation
#16
Arash Khamooshian, Ting Hai, Yannis Amador, Jelliffe Jeganathan, Robina Matyal
No abstract text is available yet for this article.
October 19, 2016: Journal of Cardiothoracic and Vascular Anesthesia
https://www.readbyqxmd.com/read/28041804/endovascular-treatment-of-the-common-femoral-artery-in-the-vascular-quality-initiative
#17
Jeffrey J Siracuse, Kathryn Van Orden, Jeffrey A Kalish, Mohammad H Eslami, Marc L Schermerhorn, Virendra I Patel, Denis Rybin, Alik Farber
OBJECTIVE: Endovascular interventions of the common femoral (CFA) and deep (DFA) femoral arteries are becoming more common. However, there is very little published data for guidance. Our objective was to analyze practice patterns and outcomes from these interventions. METHODS: The Vascular Quality Initiative (2010-2015) was queried for all endovascular interventions of the CFA and DFA. Cases that were emergent or for acute limb ischemia were excluded. Those with isolated CFA with or without DFA treatment were analyzed...
December 29, 2016: Journal of Vascular Surgery
https://www.readbyqxmd.com/read/28040141/prenatal-diagnosis-of-smith-magenis-syndrome-in-two-fetuses-with-increased-nuchal-translucency-mild-lateral-ventriculomegaly-and-congenital-heart-defects
#18
Ting-Ying Lei, Ru Li, Fang Fu, Jun-Hui Wan, Yong-Ling Zhang, Xiang-Yi Jing, Can Liao
OBJECTIVE: Smith-Magenis syndrome (SMS) is a multiple congenital anomalies/mental retardation disorder characterized by an interstitial deletion involving chromosome 17p11.2 containing the retinoic acid-induced 1 (RAI1) gene or due to mutation of RAI1. Few cases have been reported in the medical literature regarding prenatal diagnosis of SMS. We report on the prenatal diagnosis of SMS in two fetuses with increased nuchal translucency (NT), mild lateral ventriculomegaly, and congenital heart defects by whole-genome and high-resolution chromosome microarray analysis (CMA)...
December 2016: Taiwanese Journal of Obstetrics & Gynecology
https://www.readbyqxmd.com/read/28038838/-interventional-cardiac-catheterization-in-congenital-heart-disease
#19
François Godart, Ali Houeijeh
Interventional cardiac catheterization has a major place in the management of congenital heart disease. Since the Rashkind atrioseptostomy in mid-1960s, many techniques have been developed. For some, it is necessary to close a cardiac or extracardiac shunt using occluder (double disc system, plug, coil…): closure of atrial septal defect, ventricular septal defect or patent arterial duct. For others, it is necessary to treat a valvular or vascular stenosis using a balloon catheter: dilatation of the pulmonary or the aortic valve, dilatation of aortic coarctation...
December 27, 2016: La Presse Médicale
https://www.readbyqxmd.com/read/28033087/unexpected-internalization-of-a-pulmonary-artery-band-in-a-porcine-model-of-tetralogy-of-fallot
#20
Zakaria Jalal, François Roubertie, Emmanuelle Fournier, Virginie Dubes, David Benoist, Jerome Naulin, Samantha Delmond, Marlène Durand, Michel Haissaguerre, Olivier Bernus, Jean-Benoit Thambo
BACKGROUND: We report our experience of an unexpected complication of internalization of a pulmonary artery (PA) band in the vascular lumen, which occurred in a chronic porcine model of repaired tetralogy of Fallot (TOF). METHODS: Twelve piglets were divided into 3 groups: (1) TOF model animals (PA band plus pulmonary valvotomy, n = 4), (2) pulmonary insufficiency (PI) animals (pulmonary valvotomy, n = 4), and (3) control animals (n = 4). A nonabsorbable, coated braided polyester tape was used to perform the main pulmonary artery banding...
January 2017: World Journal for Pediatric & Congenital Heart Surgery
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