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Pulmonary Atresia With Intact Ventricular Septum

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Neonatal Pulmonary Atresia With Intact Ventricular Septum-8-Y Surgical Experience at One Center.

Li Guanhua, Cen Jianzheng, Xu Gang, Wen Shusheng, Zhuang Jian, Chen Jimei,

BACKGROUND:Surgical treatment of pulmonary atresia with intact ventricular septum (PA/IVS) in neonates is challenging because of the broad variations of right ventricular (RV) malformations. In this retrospective study, we summarized our 8-y experience in surgical management for neonatal PA/IVS patients. METHODS:Thirty-four neonates with PA/IVS between July 1, 2006 and June ... Read more >>

J. Surg. Res. (The Journal of surgical research)
[2020, 251:38-46]

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Fetal cardiac interventions: Where do we stand?

Kevin G Friedman, Wayne Tworetzky,

Fetal cardiac intervention (FCI) is a novel and evolving technique that allows for in utero treatment of a subset of congenital heart disease. This review describes the rationale, selection criteria, technical features, and current outcomes for the three most commonly performed FCI: fetal aortic stenosis with evolving hypoplastic left heart ... Read more >>

Arch Cardiovasc Dis (Archives of cardiovascular diseases)
[2020, 113(2):121-128]

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[Efficacy of transcatheter pulmonary valve perforation by micro-guidewire and balloon dilation in neonates with pulmonary atresia with intact ventricular septum].

L Q Zhao, S Chen, Y R Wu, J P Yang, X T Jiao, W H Jin, K Sun,

Objective: To investigate the safety and efficacy of transcatheter perforation of pulmonary valve by micro-guidewire and balloon dilation in the treatment of neonatal pulmonary atresia with intact ventricular septum (PA-IVS). Methods: The retrospective study included 21 neonates (14 males and 7 females) with PA-IVS who underwent transcatheter micro-guidewire pulmonary valve ... Read more >>

Zhonghua Er Ke Za Zhi (Zhonghua er ke za zhi = Chinese journal of pediatrics)
[2020, 58(2):96-100]

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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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Tricuspid atresia with absent pulmonary valve and intact ventricular septum: successful bidirectional cavopulmonary anastomosis with complete exclusion of the right ventricle.

Cheul Lee, Kyung Min Kim, Jae Young Lee, Jihong Yoon,

Tricuspid atresia with absent pulmonary valve and intact ventricular septum is an extremely rare cardiac malformation, historically associated with a poor prognosis. Only a few cases with successful surgical palliation have been reported in the literature. We present the case of an 8-month-old infant with this malformation who underwent successful ... Read more >>

Cardiol Young (Cardiology in the young)
[2020, 30(1):126-128]

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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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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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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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Prenatal echocardiographic classification and prognostic evaluation strategy in fetal pulmonary atresia with intact ventricular septum.

Lin Liu, Hongdan Wang, Cunying Cui, Yanan Li, Yuanyuan Liu, Ying Wang, Taibing Fan, Bangtian Peng,

Fetal pulmonary atresia with intact ventricular septum (PA/IVS) is a rare congenital heart disease. The present study aimed to classify PA/IVS and determine the relationship between prenatal echocardiographic characteristics and postnatal biventricular or univentricular repair strategies.A total of 51 fetuses with PA/IVS were examined from 2012 to 2019. Data on ... Read more >>

Medicine (Baltimore) (Medicine)
[2019, 98(42):e17492]

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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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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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Systemic ventricular strain and torsion are predictive of elevated serum NT-proBNP in Fontan patients: a magnetic resonance study.

Li-Wei Hu, Xin-Rong Liu, Qian Wang, Gregory P Barton, Rong-Zhen Ouyang, Ai-Min Sun, Chen Guo, Tong-Tong Han, Xiao-Fen Yao, Christopher J François, Yu-Min Zhong,

Background:This study aimed to investigate the associations between cardiac strain, cardiac torsion, ventricular volumes, and ventricular ejection fraction, with N-terminal pro-B-type natriuretic peptide (NT-proBNP) levels in Fontan patients who were age- and gender-matched with healthy control subjects. Methods:Cardiovascular magnetic resonance (CMR) studies performed in 22 (15 male, 7 female) patients ... Read more >>

Quant Imaging Med Surg (Quantitative imaging in medicine and surgery)
[2020, 10(2):485-495]

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An unusual association of sinus of Valsalva aneurysm with pulmonary atresia and intact ventricular septum.

Rishabh Khurana, Niraj Nirmal Pandey, S H Chandrashekhara, Arun Sharma,

J Cardiovasc Comput Tomogr (Journal of cardiovascular computed tomography)
[2019, :]

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Missing pouches in high-density mapping of atrial tachyarrhythmia in congenital heart diseases.

Sit-Yee Kwok, Tak-Cheung Yung, Ngai-Lun Ho, Jo-Jo Hai, Sabrina Tsao, Hung-Fat Tse,

Background:The use of high-density electroanatomical mapping in the Chinese population for congenital heart disease (CHD) is not well reported. Methods:Retrospective review of consecutive transcatheter ablation of atrial tachyarrhythmia using high-density mapping for CHD patients (at least moderate complexity) in the only tertiary congenital heart center in the territory from January ... Read more >>

J Arrhythm (Journal of arrhythmia)
[2019, 35(6):821-829]

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Quantitative Assessment of Left Ventricular Dysfunction in Fetal Ebstein's Anomaly and Tricuspid Valve Dysplasia.

Luke G Eckersley, Lisa W Howley, Mary E van der Velde, Nee S Khoo, Kandice Mah, Paul Brooks, Timothy Colen, Lisa K Hornberger,

BACKGROUND:Fetal Ebstein's anomaly and tricuspid valve dysplasia (EA/TVD) are associated with high perinatal mortality relative to pulmonary atresia with intact ventricular septum (PAIVS), despite both requiring redistribution of the cardiac output (CO) to the left ventricle (LV). LV dysfunction is suspected to contribute to adverse outcomes in EA/TVD. OBJECTIVE:We sought ... Read more >>

J Am Soc Echocardiogr (Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography)
[2019, 32(12):1598-1607]

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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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Severe Pulmonary Stenosis or Atresia with Intact Ventricular Septum in the Fetus: The Natural History.

Ingo Gottschalk, Brigitte Strizek, Tina Menzel, Ulrike Herberg, Johannes Breuer, Konrad Brockmeier, Annegret Geipel, Ulrich Gembruch, Christoph Berg,

PURPOSE:To assess the intrauterine course, the outcome, and to establish a new prenatal echocardiographic scoring system to predict biventricular (BV) versus univentricular (UV) outcome of fetuses with severe pulmonary stenosis or atresia with intact ventricular septum (PSAIVS). METHODS:All cases of PSAIVS diagnosed prenatally over a period of 14years were retrospectively ... Read more >>

Fetal. Diagn. Ther. (Fetal diagnosis and therapy)
[2019, :1-9]

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Modeling the congenital heart condition of pulmonary atresia with intact ventricular septum using human induced pluripotent stem cells.

Wendy Keung, Yin Yu Lam, Chun Ho Chan, Lin Geng, Yiu-Fai Cheung, Ronald A Li,

J Pharmacol Toxicol Methods (Journal of pharmacological and toxicological methods)
[2019, 99:106595]

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Percutaneous obliteration of the right ventricle to avoid coronary damage by sinusoids in patients with pulmonary atresia intact ventricular septum during staged single ventricle palliation.

Jelena Hubrechts, Bjorn Cools, Stephen C Brown, Benedicte Eyskens, Ruth Heying, Derize Boshoff, Marc Gewillig,

BACKGROUND AND AIMS:Suprasystemic pressure waves can damage the coronary arteries resulting in myocardial ischemia and excess early mortality. We aimed to reduce the coronary pressure wave through the sinusoids by abolishing RV volume with percutaneous devices. METHODS AND RESULTS:Four patients with PA-IVS and coronary sinusoids from the hypertensive rudimentary RV ... Read more >>

Catheter Cardiovasc Interv (Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography & Interventions)
[2019, 94(5):722-726]

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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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Prediction of biventricular repair by echocardiography in borderline ventricle.

Xiao-Jing Ma, Guo-Ying Huang,

OBJECTIVE:In recent years, attempting the biventricular pathway or biventricular conversions in patients with borderline ventricle has become a hot topic. However, inappropriate pursuit of biventricular repair in borderline candidates will lead to adverse clinical outcomes. Therefore, it is important to accurately assess the degree of ventricular development before operation and ... Read more >>

Chin. Med. J. (Chinese medical journal)
[2019, 132(17):2105-2108]

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Isolated persistence of the fifth aortic arch in an infant presenting with congestive heart failure.

Giovanni Meliota, Maristella Lombardi, Pierluigi Zaza, Maria Rosaria Tagliente, Paolo Versacci, Gabriele Scalzo, Ugo Vairo,

The persistence of the fifth aortic arch (PFAA) in postnatal life is an extremely rare and controversial cardiovascular malformation. PFAA is defined as an extra-pericardial vessel arising from the ascending aorta proximal to the origin of the brachiocephalic arteries, terminating either in the dorsal aorta or in the pulmonary arteries ... Read more >>

Ann Pediatr Cardiol (Annals of pediatric cardiology)
[2020, 13(1):91-94]

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The science and art of aortic and/or pulmonary root translocation.

Supreet P Marathe, Sachin Talwar,

This review aims to present and compare different surgical techniques of root translocation of the great arteries except the Ross procedure. The historical aspects, technical considerations, and results are briefly elucidated. ... Read more >>

Ann Pediatr Cardiol (Annals of pediatric cardiology)
[2020, 13(1):56-66]

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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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[Spontaneous Closure of Atrial Septal Defect Caused Severe Heart Failure in a Child with Pulmonary Atresia and Intact Ventricular Septum Having an Extracardiac Total Cavopulmonary Connection;Report of a Case].

Tetsuri Takei, Yukihiro Kaneko, Ikuya Achiwa, Ryouichi Kondo, Yasushi Misaki, Hiroshi Ono,

Unobstructed atrial communication is a prerequisite for favorable pulmonary circulation in patients with single-ventricle physiology and atrial-communication-dependent pulmonary venous drainage. It might also be a prerequisite for favorable coronary circulation in patients with single-ventricle physiology and atrial-communication-dependent coronary venous drainage. Obstruction of atrial communication, on which coronary venous drainage depends, ... Read more >>

Kyobu Geka (Kyobu geka. The Japanese journal of thoracic surgery)
[2019, 72(7):560-563]

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