Pulmonary atresia with intact ventricular septum and major systemic-pulmonary collateral arteries is a very rare congenital heart lesion with dismal natural history. Herein we report on a case of pulmonary atresia with intact ventricular septum with hypoplastic right ventricle, very small confluent pulmonary arteries, absent arterial duct, and pulmonary blood flow exclusively provided by bronchial-type systemic-pulmonary collateral arteries that was successfully treated at our institution.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1016/s0003-4975(01)03277-5 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Impact of cardiac position, morphology and operative technique on long-term fontan outcomes in heterotaxy.
Eur J Cardiothorac Surg
January 2025
Division of Cardiovascular Surgery, The Labatt Family Heart Centre, The Hospital for Sick Children, Toronto, ON, Canada.
Objectives: This study aimed to assess the outcomes of heterotaxy patients undergone the Fontan operation, focusing on morphological features and surgical techniques.
Methods: Eighty-two consecutive heterotaxy patients who underwent the Fontan operation from 1985 to 2021 were compared to 150 patients with tricuspid atresia (TA) and 144 patients with hypoplastic left heart syndrome (HLHS). The Kaplan-Meier method and Cox proportional hazard model were used to analyze transplant-free survival and predictor of outcomes.
Single-Stage Midline Unifocalization Is Associated With Excellent Outcomes in Infants of All Ages.
World J Pediatr Congenit Heart Surg
January 2025
Department of Cardiothoracic Surgery, Lucile Packard Children's Hospital, Stanford University, Palo Alto, CA, USA.
Objective: Tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collateral arteries are a complex congenital heart defect. For years, our program has recommended early single-stage midline unifocalization at three to six months of age. However, many patients are referred beyond six months.
View Article and Find Full Text PDFPrevalence, predictors, and mortality of Super-Fontan patients in a single-centre nationwide cohort.
Cardiol Young
January 2025
Children's Heart Centre, Motol University Hospital, Prague, Czech Republic.
Aims: To evaluate the prevalence, long-term mortality, and clinical characteristics in total cavopulmonary connection patients with excellent functional outcomes.
Methods And Results: A retrospective study of cardiopulmonary exercise test results in 288 patients after total cavopulmonary connection from a single-centre nationwide database. A subgroup of 88 (30.
Assessment of right ventricular endocardial fibroelastosis in fetuses with critical pulmonary stenosis and pulmonary atresia with intact ventricular septum.
Front Pediatr
January 2025
Heart Center, Women and Children's Hospital, Qingdao University, Qingdao, China.
Background: This study aimed to assess right ventricular (RV) endocardial fibroelastosis (EFE) in fetuses with critical pulmonary stenosis (CPS) and pulmonary atresia with intact ventricular septum (PA-IVS) and to investigate the implications of RV EFE for circulatory outcomes.
Methods: Fetal echocardiographic data from July 2018 to January 2021 were collected. Three reviewers independently graded EFE based on the presence and extent of endocardial echogenicity.
Pulmonary Atresia with Intact Ventricular Septum, an Evolving Strategy in the Era of PDA Stenting: Single Center Experience.
Pediatr Cardiol
January 2025
Department of Pediatrics, Section of Cardiology, Rady Children's Hospital, University of California-San Diego, San Diego, CA, USA.
Repair or palliation of pulmonary atresia with intact ventricular septum (PA/IVS) often falls into one of 4 categories: cardiac transplant, 2-ventricular circulation, 1.5 ventricle circulation, or single ventricle circulation. The optimal management strategy has been an area of much debate.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!