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Atresia pulmonary

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https://www.readbyqxmd.com/read/28208970/isolated-hypoplasia-of-left-pulmonary-artery-with-agenesis-of-left-lobe-of-thyroid-a-case-report
#1
Mohammed Abdul Khadir, Ganesh Narayana, Ganavi Ramagopal, Pradeep G Nayar
Isolated Unilateral hypoplasia or agenesis of a branch of pulmonary artery is very rare. It is usually seen associated with congenital heart diseases such as tetralogy of Fallot, atrial septal defect, coarctation of the aorta, right aortic arch, truncus arteriosus, patent ductus arteriosus and pulmonary atresia. It occurs as a result of lack of embryological development of either the left or right sixth aortic arch and has been found to present itself with various clinical manifestations as during childhood it presents as contralateral pulmonary hypertension and in adults as haemoptysis...
December 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28205731/p-19-impaired-pulmonary-function-and-ventilatory-limitation-in-children-with-successfully-repaired-oesophageal-atresia
#2
J McBride, P Field, C Clarkson, J Menzies, J Hughes, M Doumit, C Wu, S Adams, M Soma, U Krishnan, Y Belessis
No abstract text is available yet for this article.
April 1, 2016: Diseases of the Esophagus: Official Journal of the International Society for Diseases of the Esophagus
https://www.readbyqxmd.com/read/28190605/the-importance-of-age-and-weight-on-cavopulmonary-shunt-stage-ii-outcomes-after-the-norwood-procedure-planned-versus-unplanned-surgery
#3
David J Barron, Intisar Ul Haq, Adrian Crucean, John Stickley, Phil Botha, Natasha Khan, Timothy J Jones, William J Brawn
OBJECTIVE: The study objective was to evaluate the outcomes of the cavopulmonary shunt after the Norwood procedure with a particular focus on age, weight, and whether surgery was planned or expedited by clinical findings. METHODS: We studied 297 consecutive patients with hypoplastic left heart syndrome undergoing the cavopulmonary shunt operation between 2002 and 2014. All patients underwent the Norwood procedure with a right ventricle to pulmonary artery conduit...
January 17, 2017: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28163422/transcatheter-pulmonary-valve-perforation-using-chronic-total-occlusion-wire-in-pulmonary-atresia-with-intact-ventricular-septum
#4
Shweta Bakhru, Shilpa Marathe, Manish Saxena, Sudeep Verma, Rajan Saileela, Tapan K Dash, Nageswara Rao Koneti
BACKGROUND: Perforation of pulmonary valve using radiofrequency ablation in pulmonary atresia with intact ventricular septum (PA IVS) is a treatment of choice. However, significant cost of the equipment limits its utility, especially in the developing economies. OBJECTIVE: To assess the feasibility, safety, and efficacy of perforation of pulmonary valve using chronic total occlusion (CTO) wires in patients with PA IVS as an alternative to radiofrequency ablation...
January 2017: Annals of Pediatric Cardiology
https://www.readbyqxmd.com/read/28154913/right-ventricle-to-pulmonary-artery-shunt-in-pulmonary-atresia-with-a-ventricular-septal-defect-a-word-of-caution
#5
Kwang Ho Choi, Si Chan Sung, Hyungtae Kim, Hyoung Doo Lee, Gil Ho Ban, Geena Kim, Hoon Ko
The management of pulmonary atresia with a ventricular septal defect (PA/VSD) depends on the anatomy of the pulmonary artery or on the surgical strategy used at individual institutions. In our institution, we adopted a right ventricle-to-pulmonary artery (RV-PA) shunt in 2011 as a palliative procedure for PA/VSD to overcome the disadvantages of a Blalock-Taussig shunt. We evaluated the results of the RV-PA shunt as initial palliative surgery for PA/VSD. Thirteen patients with ductus-dependent PA/VSD from August 2011 to August 2015 were enrolled...
February 3, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28148318/utility-of-4d-flow-mapping-in-eisenmenger-syndrome-with-pulmonary-atresia
#6
Soha Romeih, Heba Aguib, Magdi Yacoub
Management of patients with Eisenmenger syndrome with pulmonary atresia is challenging because of the complexity of the structure-function relationship of the components of the syndrome. Multi-modality imaging including cardiac magnetic resonance (CMR) 4D Flow offers unprecedented opportunities to unravel, at least in part, some of these components, and thus help in the management of these patients. In this study, we describe the use of these integrated methods with particular reference to CMR 4D Flow in a patient with Eisenmenger syndrome and pulmonary atresia and outline both the utility and the limitations...
December 2016: Cardiology in the Young
https://www.readbyqxmd.com/read/28132091/a-case-of-unusual-configuration-of-the-right-bronchial-arteries-combined-with-cryptogenic-severe-bilateral-hypertrophy
#7
Santiago Rojas, Eduard Quintana, Marisa Ortega, Alfonso Rodríguez-Baeza
Bronchial arteries commonly originate from thoracic aorta between T5 and T6. Ectopic origins from aortic arch, supraortic trunks and their branches, coronary arteries, and even abdominal aorta have been described in the literature. In some circumstances, such as pulmonary artery malformations, chronic embolism, or inflammatory diseases of the lung, the bronchial arteries become hypertrophied and eventually could be the only supply of pulmonary circulation. Here, we describe a case of an elderly man who presented an unusual pattern of bronchial arteries of the right lung combined with severe bilateral hypertrophy of bronchial vessels...
January 28, 2017: Surgical and Radiologic Anatomy: SRA
https://www.readbyqxmd.com/read/28122228/temporally-distinct-six2-positive-second-heart-field-progenitors-regulate-mammalian-heart-development-and-disease
#8
Zhengfang Zhou, Jingying Wang, Chaoshe Guo, Weiting Chang, Jian Zhuang, Ping Zhu, Xue Li
The embryonic process of forming a complex structure such as the heart remains poorly understood. Here, we show that Six2 marks a dynamic subset of second heart field progenitors. Six2-positive (Six2(+)) progenitors are rapidly recruited and assigned, and their descendants are allocated successively to regions of the heart from the right ventricle (RV) to the pulmonary trunk. Global ablation of Six2(+) progenitors resulted in RV hypoplasia and pulmonary atresia. An early stage-specific ablation of a small subset of Six2(+) progenitors did not cause any apparent structural defect at birth but rather resulted in adult-onset cardiac hypertrophy and dysfunction...
January 24, 2017: Cell Reports
https://www.readbyqxmd.com/read/28120278/long-term-management-challenges-in-esophageal-atresia
#9
REVIEW
Abby White, Raphael Bueno
Esophageal atresia is a rare congenital anomaly, but improved surgical and critical care has resulted in survival rates exceeding 90%. Long-term survival is associated with numerous management challenges including chronic motility disorders, dysphagia, strictures, reflux, esophagitis and attendant complications, tracheomalacia and chronic restrictive lung disease, and recurrent pulmonary infections. No guidelines for adolescents and younger or older adults exist for the treatment and monitoring of this specialized patient population...
January 24, 2017: Current Treatment Options in Gastroenterology
https://www.readbyqxmd.com/read/28116756/branch-pulmonary-artery-peel-operation-in-a-patient-without-a-native-intrapericardial-pulmonary-artery
#10
Hyungtae Kim, Si Chan Sung, Kwang Ho Choi, Hyoung Doo Lee, Gil Ho Ban, Geena Kim, Hee Young Kim
A patient with pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries without an intrapericardial pulmonary artery (PA) underwent a one-stage total correction, including both branch PA reconstructions, with a bovine pericardial roll at the age of 42 months. She was readmitted 54 months after the operation because of extracardiac conduit bacterial endocarditis and pulmonary infarction. The bovine pericardial roll between the right and left PAs was enucleated, and the surrounding fibrotic tissue (peel) was used as a new branch PA vascular conduit...
January 23, 2017: Journal of Cardiac Surgery
https://www.readbyqxmd.com/read/28111407/determinants-of-aortic-size-and-stiffness-and-the-impact-on-exercise-physiology-in-patients-after-the-fontan-operation
#11
Hideo Ohuchi, Yosuke Hayama, Jun Negishi, Kanae Noritake, Aya Miyazaki, Osamu Yamada, Isao Shiraishi
The pathophysiology of congenital heart disease includes aortic dilation and increased stiffness. However, the clinical determinants and significance remain unclear in patients after the Fontan operation.Size and stiffness index (SI) of the ascending and descending aorta (aAO and dAO, respectively) were assessed using angiography in 130 consecutive Fontan patients and 30 age-matched controls. Compared with controls, Fontan patients showed a dilated aAO and smaller dAO (P < 0.0001) with greater SI (3.2 ± 0...
February 7, 2017: International Heart Journal
https://www.readbyqxmd.com/read/28100981/isolated-left-subclavian-artery-complete-atrioventricular-block-and-tricuspid-atresia-in-a-neonate
#12
Kanupriya Chaturvedi, Deepa Prasad, Ravi Ashwath, James P Strainic, Christopher S Snyder
Isolated left subclavian artery is one of the rarer aortic arch anomalies. It has been associated with other congenital heart diseases, typically tetralogy of Fallot, double-outlet right ventricle, and atrial and ventricular septal defects. Its significant clinical implications include a left-to-right shunt from the vertebrobasilar system, which causes pulmonary overcirculation and subclavian steal. We present an unusual case of a premature infant who was diagnosed prenatally with congenital complete atrioventricular block and tricuspid atresia and was found to have an isolated left subclavian artery postnatally...
December 2016: Texas Heart Institute Journal
https://www.readbyqxmd.com/read/28094013/pulmonary-outcome-of-esophageal-atresia-patients-and-its-potential-causes-in-early-childhood
#13
René Dittrich, Philippe Stock, Karin Rothe, Petra Degenhardt
INTRODUCTION: The aim of this study was to illustrate the pulmonary long term outcome of patients with repaired esophageal atresia and to further examine causes and correlations that might have led to this outcome. METHODS: Twenty-seven of 62 possible patients (43%) aged 5-20years, with repaired esophageal atresia were recruited. Body plethysmography and spirometry were performed to evaluate lung function, and the Bruce protocol treadmill exercise test to assess physical fitness...
January 3, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28077176/main-pulmonary-artery-cross-section-ratio-is-low-in-fetuses-with-tetralogy-of-fallot-and-ductus-arteriosus-dependent-pulmonary-circulation
#14
Hironori Ebishima, Kenichi Kurosaki, Jun Yoshimatsu, Isao Shiraishi
OBJECTIVES: This study aimed to determine fetal echocardiographic features of tetralogy of Fallot in association with postnatal outcomes. METHODS: The Z-scores of the main and bilateral pulmonary arteries and the aorta were measured, and the following variables were calculated in 13 fetuses with tetralogy of Fallot: pulmonary artery-to-aorta ratio and main pulmonary artery cross-section ratio - the main pulmonary artery diameter squared divided by the sum of the diameter squared of the left and right pulmonary arteries...
January 12, 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/28043470/single-center-experience-with-the-senning-procedure-in-the-current-era
#15
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/28043462/right-ventricular-outflow-tract-reconstruction-with-a-polytetrafluoroethylene-monocusp-valve-a-20-year-experience
#16
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/28033085/echocardiographic-diagnosis-of-left-main-coronary-artery-atresia
#17
Justin D Weigand, Srinath Gowda, Richard Lorber, Nitin Madan
This case describes an infantile presentation of left main coronary artery atresia (LMCAA), which involves complete absence of the left coronary ostium and left main coronary artery. The echocardiographic features of LMCAA that assist in making this diagnosis are detailed. Important imaging features that distinguish LMCAA from anomalous left coronary artery from the pulmonary artery are highlighted.
January 2017: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/28010875/achievements-and-limitations-of-a-strategy-of-rehabilitation-of-native-pulmonary-vessels-in-pulmonary-atresia-ventricular-septal-defect-and-major-aortopulmonary-collateral-arteries
#18
Jerome Soquet, Matthew Liava'a, Lucas Eastaugh, Igor E Konstantinov, Johann Brink, Christian P Brizard, Yves d'Udekem
BACKGROUND: A strategy of rehabilitation for pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries (PA/VSD/MAPCAs) comprises repetitive shunting and patching procedures of the central pulmonary arteries. We wanted to determine the feasibility and limitations of a strategy of rehabilitation. METHODS: The outcomes of 37 consecutive patients operated from June 2003 to December 2014 for PA/VSD/MAPCAs were reviewed. The patients were directed to a rehabilitation strategy, except when they presented in heart failure with very large collaterals...
December 20, 2016: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/27965298/right-ventricular-outflow-tract-stenting-in-tetralogy-of-fallot-infants-with-risk-factors-for-early-primary-repair
#19
Juan Pablo Sandoval, Rajiv R Chaturvedi, Lee Benson, Gareth Morgan, Glen Van Arsdell, Osami Honjo, Christopher Caldarone, Kyong-Jin Lee
BACKGROUND: Tetralogy of Fallot with cyanosis requiring surgical repair in early infancy reflects poor anatomy and is associated with more clinical instability and longer hospitalization than those who can be electively repaired later. We bridged symptomatic infants with risk factors for early primary repair by right ventricular outflow tract stenting (stent). METHODS AND RESULTS: Four groups of tetralogy of Fallot with confluent central pulmonary arteries were studied: stent group (n=42), primary repair (aged <3 months) with pulmonary stenosis (early-PS group; n=44), primary repair (aged <3 months) with pulmonary atresia (early-PA group; n=49), and primary repair between 3 and 11 months of age (surg>3mo group; n=45)...
December 2016: Circulation. Cardiovascular Interventions
https://www.readbyqxmd.com/read/27942762/tricuspid-atresia-with-non-compaction-an-early-experience-with-implications-for-surgical-palliation
#20
Hoang H Nguyen, Rabia Khan, Norman H Silverman, Gautam K Singh
Left ventricle non-compaction (LVNC) has worse outcomes when associated with congenital heart defects (CHD). The co-occurrence and outcomes of LVNC with tricuspid atresia (TA) are not well described. Our study aims to determine the prevalence of LVNC with functionally single ventricle due to TA, and to describe the early outcomes of surgical palliation. A retrospective database search for patients (n = 167,566) and echocardiograms (n = 44,053) was performed in order to collect clinical, echocardiographic, and hemodynamic data of pediatric patients with TA and LVNC at St...
December 10, 2016: Pediatric Cardiology
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