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Pulmonary Atresia With Ventricular Septal Defect

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Current era outcomes of pulmonary atresia with ventricular septal defect: A single center cohort in Thailand.

Kanthalas Lertsakulpiriya, Chodchanok Vijarnsorn, Prakul Chanthong, Paweena Chungsomprasong, Supaluck Kanjanauthai, Kritvikrom Durongpisitkul, Jarupim Soongswang, Thaworn Subtaweesin, Somchai Sriyoschati,

Pulmonary atresia with ventricular septal defect (PA/VSD) is a complex cyanotic congenital heart disease with a wide-range of presentations and treatment strategies, depending on the source of pulmonary circulation, anatomy of pulmonary arteries (PAs), and major aortopulmonary collateral arteries (MAPCAs). Data about the outcomes in developing countries is scarce. We ... Read more >>

Sci Rep (Scientific reports)
[2020, 10(1):5165]

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22q11.2 deletion syndrome is associated with increased mortality in adults with tetralogy of Fallot and pulmonary atresia with ventricular septal defect.

Dirkjan Kauw, Odilia I Woudstra, Klaartje van Engelen, Folkert J Meijboom, Barbara J M Mulder, Mark J Schuuring, Berto J Bouma,

BACKGROUND:22q11.2 Deletion syndrome (22q11.2DS) is common in patients with tetralogy of Fallot (TOF) or pulmonary atresia with ventricular septal defect (PA/VSD) and is associated with worse outcomes in children. Whether this impaired prognosis is also translated into adulthood is unknown, as data in adult patients are limited. We aimed to ... Read more >>

Int. J. Cardiol. (International journal of cardiology)
[2020, :]

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Gastrostomy and Tracheostomy After Complete Repair of Tetralogy of Fallot in Children With 22q11.2 Deletion Syndrome.

Danielle M Herington, Xinyu Tang, Beverly J Spray, Brian L Reemtsen, Parthak Prodhan, Gresham T Richter, William B Kyle, Elijah H Bolin,

OBJECTIVES:Caring for a child with gastrostomy and/or tracheostomy can cause measurable parental stress. It is generally known that children with 22q11.2 deletion syndrome are at greater risk of requiring gastrostomy or tracheostomy after heart surgery, although the magnitude of that risk after complete repair of tetralogy of Fallot has not ... Read more >>

Pediatr Crit Care Med (Pediatric critical care medicine : a journal of the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies)
[2020, :]

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An Unusual Cause of Hypoxia: Ventricular Septal Defect, Pulmonary Artery Atresia, and Major Aortopulmonary Collaterals Diagnosed in the Adult Cardiac Catheterization Lab.

Katia Bravo-Jaimes, Brian Walton, Poyee Tung, Richard W Smalling,

The association of pulmonary atresia, ventricular septal defect (VSD) and major aortopulmonary collaterals (MAPCA) is an extreme form of tetralogy of Fallot (TOF). It carries a high mortality risk if not intervened on during infancy with only 20% of unoperated patients surviving into adulthood. We present the case of a ... Read more >>

Case Rep Cardiol (Case reports in cardiology)
[2020, 2020:4726529]

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Iron-deficiency anaemia in children with congenital heart diseases at a teaching hospital in Ghana.

Inna Ossei, Kwame Ohene Buabeng, Paul Poku Sampene Ossei, Samuel Blay Nguah, William Gilbert Ayibor, Berko Panyin Anto, Agyemang-Duah Eric, Mahama Duwiejua,

Background:Uncorrected congenital heart lesions in children keep them in a state of constant hypoxia with compromised quality of life and reduced life expectancy. This requires early diagnosis and interventions including prevention and treatment of the resultant anaemia. Unfortunately, congenital heart disease (CHD) often goes unrecognized and thus untreated. Objectives:We determined ... Read more >>

Heliyon (Heliyon)
[2020, 6(2):e03408]

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Identification of rare variants in novel candidate genes in pulmonary atresia patients by next generation sequencing.

Xin Shi, Li Zhang, Kai Bai, Huilin Xie, Tieliu Shi, Ruilin Zhang, Qihua Fu, Sun Chen, Yanan Lu, Yu Yu, Kun Sun,

Pulmonary atresia (PA) is a rare congenital heart defect (CHD) with complex manifestations and a high mortality rate. Since the genetic determinants in the pathogenesis of PA remain elusive, a thorough identification of the genetic factors through whole exome sequencing (WES) will provide novel insights into underlying mechanisms of PA. ... Read more >>

Comput Struct Biotechnol J (Computational and structural biotechnology journal)
[2020, 18:381-392]

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The Value of Thoracic Lavage in the Treatment of Anastomotic Leakage After Surgery for Type III Esophageal Atresia.

Xu Cui, Yuanbin He, Liu Chen, Yu Lin, Jianqin Zhang, Chaoming Zhou,

BACKGROUND The aim of this study was to define whether the addition of thoracic lavage to chest drainage was more efficient than the use of chest drainage alone in the treatment for anastomotic leaks in type III esophageal atresia. MATERIAL AND METHODS The clinical data of 42 patients with anastomotic ... Read more >>

Med. Sci. Monit. (Medical science monitor : international medical journal of experimental and clinical research)
[2020, 26:e919962]

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Quantification of Initial Right Ventricular Dimensions by Computed Tomography in Infants with Congenital Heart Disease and a Hypoplastic Right Ventricle.

Hyun Woo Goo,

OBJECTIVE:To demonstrate the feasibility of using cardiothoracic CT for quantification of the initial right ventricle (RV) dimensions in infants with congenital heart disease (CHD) and a hypoplastic RV and to compare these measurements with those obtained in a control group with CHD without a hypoplastic RV. MATERIALS AND METHODS:Initial RV ... Read more >>

Korean J Radiol (Korean journal of radiology)
[2020, 21(2):203-209]

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Rare Association of Absent Pulmonary Valve Syndrome with Double Outlet Right Ventricle and Hypoplastic Left Heart Complex.

Erik L Frandsen, Arpan R Doshi, Sujatha Buddhe, Bhawna Arya, Sathish M Chikkabyrappa,

Kans J Med (Kansas journal of medicine)
[2020, 13:21-22]

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Long-term follow-up after transatrial-transpulmonary repair of tetralogy of Fallot: influence of timing on outcome.

Eva van den Bosch, Ad J J C Bogers, Jolien W Roos-Hesselink, Arie P J van Dijk, Marie H E J van Wijngaarden, Eric Boersma, Aagje Nijveld, Linda W G Luijten, Ronald Tanke, Laurens P Koopman, Willem A Helbing,

OBJECTIVES:Our goal was to report the long-term serial follow-up after transatrial-transpulmonary repair of tetralogy of Fallot (TOF) and to describe the influence of the timing of the repair on outcome. METHODS:We included all patients with TOF who had undergone transatrial-transpulmonary repair between 1970 and 2012. Records were reviewed for patient ... Read more >>

Eur J Cardiothorac Surg (European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery)
[2020, 57(4):635-643]

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Counseling for Prenatal Congenital Heart Disease-Recommendations Based on Empirical Assessment of Counseling Success.

Alexander Kovacevic, Andreas Simmelbauer, Sebastian Starystach, Michael Elsässer, Andreas Müller, Stefan Bär, Matthias Gorenflo,

Objectives: Empirical assessment of parental needs and affecting factors for counseling success after prenatal diagnosis of congenital heart disease (CHD). Methods:Counseling success after fetal diagnosis of CHD was assessed by a validated standardized questionnaire. The dependent variable "Effective Counseling" was measured in five created analytical dimensions (1. "Transfer of Medical ... Read more >>

Front Pediatr (Frontiers in Pediatrics)
[2020, 8:26]

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Can ductus arteriosus morphology influence technique/outcome of stent treatment?

Mieke Roggen, Bjorn Cools, Stephen Brown, Derize Boshoff, Ruth Heying, Benedicte Eyskens, Marc Gewillig,

INTRODUCTION:Results and outcomes of ductus arteriosus stenting vary widely. The aim of this study was to determine whether ductus morphology is associated with different procedural outcome. METHODS:Over an 18-year period, 123 patients presented with ductal dependent pulmonary blood flow. Results were retrospectively assessed based on radiographic anatomic features of the ... Read more >>

Catheter Cardiovasc Interv (Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography & Interventions)
[2020, :]

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Uncommon clinical presentation of cystic fibrosis in a patient homozygous for a rare CFTR mutation: a case report.

Joanna Jaworska, Aleksandra Marach-Mocarska, Dorota Sands,

BACKGROUND:Cystic fibrosis (CF) is the most common, life-threatening, autosomal-recessive disorder among Caucasians. To date, approximately 2000 mutations in the CFTR gene have been reported. Some of these mutations are very rare, and some represent individual sequence changes in the gene. The introduction of newborn screening (NBS) in high prevalence countries ... Read more >>

BMC Pediatr (BMC pediatrics)
[2020, 20(1):90]

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The Brazilian Contributions to Congenital Cardiac Surgery.

Elizabeth H. Stephens, Joseph A. Dearani,

Braz J Cardiovasc Surg (Brazilian journal of cardiovascular surgery)
[2020, 35(1):I-III]

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Pregnancy in non-palliated functionally single ventricle: challenges of management in resource-poor settings.

Desrie Delsol-Gyan, Ernest Aniteye, Samuel Oppong, Ernest Ofosu-Appiah, Frank Edwin,

Women with complex functionally univentricular hearts rarely survive into adulthood without corrective or palliative surgery. Reports of pregnancy outcome in this group of patients in resource-poor settings are sparse. We report a case of unrepaired pulmonary atresia ventricular septal defect (VSD) with major aorto-pulmonary collateral arteries (MAPCA) who survived into ... Read more >>

Pan Afr Med J (The Pan African medical journal)
[2020, 35:6]

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Effectiveness of Bidirectional Glenn Shunt Placement for Palliation in Complex Congenitally Corrected Transposed Great Arteries.

Kai Ma, Lei Qi, Zhongdong Hua, Keming Yang, Hao Zhang, Shoujun Li, Sen Zhang, Fengpu He, Guanxi Wang,

Surgery for complex congenitally corrected transposed great arteries is one of the greatest challenges in cardiovascular surgery. We report our experience with bidirectional Glenn shunt placement as a palliative procedure for complex congenitally corrected transposition. We retrospectively identified 50 consecutive patients who had been diagnosed with congenitally corrected transposition accompanied ... Read more >>

Tex Heart Inst J (Texas Heart Institute journal)
[2020, 47(1):15-22]

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Feasibility, reproducibility and accuracy of electrical velocimetry for cardiac output assessment in congenital heart disease.

Alexander C Egbe, Muhammad Wajih Ullah, Arslan Afzal, Keerthana Banala, Rahul Vojjini, Maria Najam, Karim Osman, Sahith Thotamgari, Donald J Hagler,

Background:Noninvasive cardiac output assessment is important for prognostication in patients with heart failure. Electrical velocimetry (EV), an impedance cardiography technique, can be used for noninvasive cardiac output assessment. The purpose of this study was to determine the feasibility, reproducibility and accuracy of cardiac output assessment by EV in adults with ... Read more >>

Int J Cardiol Heart Vasc (International journal of cardiology. Heart & vasculature)
[2020, 26:100464]

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Epidural anesthesia for emergency cesarean section in a woman with Fontan circulation: A case report.

Pin Wu, Sheng-Mei Zhu, Yong-Xing Yao,

RATIONALE:Anesthetic management of pregnant women with Fontan circulation remains challenging. There are few reports that describe the anesthetic management of cesarean section after Fontan surgery. Here, we present a case of successful epidural anesthesia in a woman with Fontan circulation who required emergency cesarean section. PATIENT CONCERNS:A 29-year-old woman at ... Read more >>

Medicine (Baltimore) (Medicine)
[2020, 99(4):e18986]

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Prevalence of Sensorineural Hearing Loss in Children with Palliated or Repaired Congenital Heart Disease.

Lalitha Gopineti, Mane Paulpillai, Andrea Rosenquist, Andrew H Van Bergen,

Background Children with congenital heart disease (CHD) are at increased risk of neurodevelopmental deficits, and the presence of sensorineural hearing loss (SNHL) may further lead to poor language skills acquisition and speech delays. Prevalence of SNHL in the general pediatric population is estimated to be 0.2% at birth to 0.35% ... Read more >>

Cureus (Cureus)
[2020, 12(1):e6566]

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Multi-detector computed tomography angiographic evaluation of right ventricular outflow tract obstruction and other associated cardiovascular anomalies in tetralogy of Fallot patients.

Rani Kunti R Singh, Neeraj Jain, Sunil Kumar, Naveen Garg,

Purpose:To evaluate various types of right ventricular outflow tract obstruction associated with tetralogy of Fallot (TOF) with emphasis on the abnormality of pulmonary arterial system and other associated cardiovascular anomalies using computed tomography (CT) angiography. Material and methods:We retrospectively evaluated 184 consecutive previously diagnosed TOF patients who underwent CT angiography ... Read more >>

Pol J Radiol (Polish journal of radiology)
[2019, 84:e511-e516]

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A novel index equivalent to the myocardial performance index for right ventricular functional assessment in children and adolescent patients.

Yasunobu Hayabuchi, Yukako Homma, Shoji Kagami,

The aims of the present study were to develop and check the utility and feasibility of a novel right ventricular (RV) functional index (RV angular velocity; RVω, s-1) derived from the angular velocity in harmonic oscillator kinematics obtained from the RV pressure waveform. We hypothesized that RVω reflects the myocardial ... Read more >>

Sci Rep (Scientific reports)
[2019, 9(1):19975]

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Systemic-to-pulmonary shunt vs right ventricle to pulmonary artery connection in the treatment of pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries.

Fan Fan, Bo Peng, Zhimin Liu, Yinglong Liu, Qiang Wang,

OBJECTIVE:The systemic-to-pulmonary shunt (SPS) and right ventricle to pulmonary artery (RV-PA) connection were evaluated to pursue the goal of rehabilitating dysplastic native PAs via establishment of antegrade blood flow. However, the application of these two palliative operations was still confusing. We compared the two operations to determine their different effects ... Read more >>

J Card Surg (Journal of cardiac surgery)
[2020, 35(2):345-351]

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Evidence for uteroplacental malperfusion in fetuses with major congenital heart defects.

Julia Binder, Silvia Carta, Julene S Carvalho, Erkan Kalafat, Asma Khalil, Basky Thilaganathan,

AIMS:Fetuses affected by congenital heart defects (CHD) are considered to be at increased risk of fetal growth restriction and intrauterine demise. Whether these risks are a direct consequence of fetal CHD or a result of associated uteroplacental dysfunction is not evident from the data of recent studies. The aim of ... Read more >>

PLoS ONE (PloS one)
[2020, 15(2):e0226741]

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Near-infrared spectroscopy: An important tool during the blalock-taussig shunt.

Aditya Lamba, Reena Khantwal Joshi, Raja Joshi, Neeraj Agarwal,

Near infra red spectroscopy (NIRS) is a noninvasive diagnostic tool for measuring regional oxygen saturation (rSO2). Cerebral oxygenation measured with NIRS is used to corroborate mixed venous oxygenation and hence considered an indicator of tissue perfusion. We describe NIRS guiding an anatomical variation leading to inadequate cerebral circulation or any ... Read more >>

Ann Card Anaesth (Annals of cardiac anaesthesia)
[2020, 23(1):92-94]

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Stenting of modified Blalock-Taussig shunt in adult with palliated pulmonary atresia and ventricular septal defect: a case report.

Julia Illner, Holger Reinecke, Helmut Baumgartner, Gerrit Kaleschke,

Background:Adults with complex congenital heart disease palliated with systemic-to-pulmonary artery shunts have become rare and represent a particularly challenging patient group for the cardiologist. One of the complications and causes of severe clinical deterioration during long-term follow-up are progressive obstruction or total occlusion of the shunt. The risk for surgical ... Read more >>

Eur Heart J Case Rep (European heart journal. Case reports)
[2019, 3(4):1-4]

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