2,359 results match your criteria: "Tricuspid Atresia"
Computer-generated Clinical Decision-making in the Treatment of Pulmonary Atresia with Intact Ventricular Septum.
Cardiovasc Eng Technol
December 2024
Department of Mechanical Engineering, Koc University, Rumeli Feneri Campus, Sarıyer, Istanbul, 34450, Turkey.
Purpose: Pulmonary atresia with intact ventricular septum is a multifactorial disease requiring complex surgeries. The treatment route is determined based on the right ventricle (RV) size, tricuspid annulus size and coronary circulation dependency of RV. Since multiple parameters influence the post-operative success, a personalized decision-making based on computed hemodynamics is hypothesized to improve the treatment efficacy.
View Article and Find Full Text PDFCircular Shunt: A Loop Not to Be Ignored.
J Cardiothorac Vasc Anesth
December 2024
Department of Cardiothoracic Surgery, National Heart Center, Royal Hospital, Muscat, Oman.
A circular shunt, initially described by Shone et al. in 1962, refers to abnormal blood recirculation through complete intracardiac or intra- and extracardiac communications, bypassing the capillary beds. This pathophysiological condition is most commonly associated with complex congenital heart defects, such as Ebstein's malformation, pulmonary atresia, Gerbode defect, and so on.
View Article and Find Full Text PDFThoracic Lymphatic Perfusion Patterns Assessed by Magnetic Resonance Imaging and Late Fontan Failure.
Diagnostics (Basel)
November 2024
Department of Pediatric Cardiac Surgery and Congenital Heart Disease, Instituto Nacional de Cardiología Ignacio Chávez, Mexico City 14080, Mexico.
Background: Fontan circulation maintains an elevated venous pressure; this promotes venous and lymphatic congestion and may lead to late circuit failure. Our objective was to determine the association between thoracic lymphatic perfusion patterns assessed by magnetic resonance imaging and late Fontan failure.
Methodology: A retrospective study was performed.
Association Between Location of Pulmonary Outflow Obstruction and Right Ventricular Size and Function After Repair of Tetralogy of Fallot, Pulmonary Atresia, and Major Aortopulmonary Collaterals.
Pediatr Cardiol
December 2024
Division of Pediatric Cardiology, Department of Pediatrics, Stanford University School of Medicine, 750 Welch Road, Suite 305, Palo Alto, CA, 94304, USA.
Quantification of right ventricular (RV) size and function after tetralogy of Fallot repair is critical for determining timing of reintervention and outcomes. Tetralogy of Fallot patients with pulmonary atresia and major aortopulmonary collateral arteries (TOF/PA/MAPCAs) are a unique group in which the RV is subjected to various loading conditions, allowing for direct comparison. Retrospective evaluation of RV echocardiographic indices in repaired pediatric TOF/PA/MAPCAs patients (2/2002 - 4/2018).
View Article and Find Full Text PDFEmergent surgical intervention for a neonate with premature constriction of the ductus arteriosus with ductal-dependent tricuspid atresia: a case report.
Eur Heart J Case Rep
December 2024
Department of Cardiovascular Surgery, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo 160-8582, Japan.
Background: Premature constriction of the ductus arteriosus (PCDA) makes management difficult in neonates with congenital heart defects, particularly those with ductal-dependent pulmonary circulation. This report highlights the challenges and management of a neonate diagnosed with tricuspid atresia and severe right ventricular outflow tract obstruction (RVOTO), complicated by PCDA.
Case Summary: A male neonate was diagnosed prenatally with tricuspid atresia and severe RVOTO.
Management of Tricuspid Atresia With Normally Related Great Arteries and Left-Sided Obstruction.
World J Pediatr Congenit Heart Surg
November 2024
Labatt Family Heart Centre, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.
Article Synopsis
- Tricuspid atresia (TA) is a heart condition affecting infants and is the second most common type of functionally univentricular heart, with rare cases of left ventricular outflow tract obstruction (LVOTO) in patients who have normally related great arteries.
- A study involving 445 patients with Type I TA showed that 3% of infants needed interventions for LVOTO, and most of those who underwent surgical procedures had promising outcomes.
- The long-term survival rate for these infants was estimated at 79% over 20 years, indicating that timely surgical management can lead to successful interventions and good heart function later in life.
Pulmonary valve replacement for porcelain right ventricular outflow tract following repeated surgical intervention.
Gen Thorac Cardiovasc Surg Cases
January 2024
Department of Cardiovascular Surgery, Kyushu University Hospital, 3-1-1, Maidashi, Higashi-Ku, Fukuoka, 812-8582, Japan.
Article Synopsis
- * A 43-year-old patient with a history of multiple valve replacements experienced severe heart failure and protein loss, but was successfully treated with additional valve replacements using a mechanical valve despite significant surgical risks due to adhesions and calcification.
- * The surgery provided relief from heart failure and related symptoms, demonstrating that this approach can yield better results compared to traditional methods when dealing with severe complications in the right ventricular outflow tract.
Staged Norwood operation for patients with hypoplastic left heart syndrome and cor triatriatum: a report of two cases.
Gen Thorac Cardiovasc Surg Cases
March 2024
Department of Pediatric Cardiac Surgery, Saitama Medical University International Medical Center, Hidaka, Saitama, 350-1298, Japan.
Article Synopsis
Mid-term results and late events after the Fontan operation: A single-center experience.
Turk Gogus Kalp Damar Cerrahisi Derg
July 2024
Deparment of Pediatric Cardiology, Çukurova University Faculty of Medicine, Adana, Türkiye.
Article Synopsis
Comparison of pulmonary artery growth between ductus stent and systemic-to-pulmonary shunt as single-ventricle palliation.
J Thorac Cardiovasc Surg
October 2024
Department of Congenital and Pediatric Heart Surgery, German Heart Center Munich, University Hospital of Technische Universität München, Munich, Germany; Division of Congenital and Pediatric Heart Surgery, University Hospital of Munich, Ludwig-Maximilians-Universität, Munich, Germany; Europäisches Kinderherzzentrum München, Munich, Germany. Electronic address:
Article Synopsis
- The study compared pulmonary artery growth in infants with univentricular hearts who received ductus stenting (DS) versus those who had a systemic to pulmonary shunt (SPS) for initial palliation from 2009 to 2022.
- A total of 130 infants were evaluated, revealing that while most pulmonary artery metrics were similar between groups, the DS group had a significantly lower left pulmonary artery index and a higher occurrence of veno-venous collaterals compared to the SPS group.
- The findings suggest that infants after DS are more likely to need further pulmonary artery interventions between stages II and III palliation, highlighting the need for further investigation into long-term outcomes after the Fontan procedure
A Comparison of Active and Passive Surveillance Strategies for Selected Birth Defects in New York.
Birth Defects Res
September 2024
Birth Defects Registry, New York State Department of Health, Albany, New York, USA.
Background: The New York State Birth Defects Registry (BDR) has passive and active components. As part of statewide passive ascertainment, the BDR receives reports of International Classification of Diseases, Tenth Revision (ICD-10) codes and descriptive narratives on a wide range of birth defects. The BDR conducts enhanced active surveillance for selected birth defects in 14 counties, which includes medical record abstraction and clinician review.
View Article and Find Full Text PDFEstablishment of a new human iPSC cell line (UOMi012-A) from a patient with congenital heart defect who has undergone Fontan procedure.
Stem Cell Res
October 2024
Institute of Cardiovascular Sciences, St. Boniface Hospital Albrechtsen Research Centre, Regenerative Medicine Program, Department of Physiology and Pathophysiology, Max Rady College of Medicine, Rady Faculty of Health Sciences, University of Manitoba, Canada. Electronic address:
Article Synopsis
- - Patients with complex congenital heart defects that can't be surgically fixed often undergo a procedure called Fontan Circulation (FC) for palliative care.
- - A new induced pluripotent stem cell (iPSC) line has been created from a 38-year-old patient who had tricuspid and pulmonary atresia and completed the Fontan procedure at age 13.
- - This iPSC line will help researchers investigate the underlying genetic and cellular factors contributing to cardiac issues, as well as explore potential future treatments.
Successful Thoracic Duct Embolization Following Fontan-Related Chylothorax in a Six-Year-Old Girl: A Case Report.
Cureus
June 2024
Interventional Radiology, Jawaharlal Nehru Medical College, Datta Meghe Institute of Higher Education and Research, Wardha, IND.
Article Synopsis
- - Chylothorax is a serious complication after the Fontan procedure, leading to potential health issues like nutritional depletion and fluid loss, as shown in a case study of a six-year-old girl with complex congenital heart defects.
- - The patient presented with symptoms like fever and cough, and imaging revealed bilateral pleural effusion, which was confirmed to be chylothorax through fluid analysis.
- - Successful treatment involved a procedure to embolize the leaking thoracic duct, which resolved her effusion and stabilized her condition, allowing for her discharge with necessary follow-up care.
Major aortopulmonary collateral arteries: clinical, echocardiographic, and radiologic characteristics.
Pol J Radiol
June 2024
Department of Radiology and Radiodiagnostics, Faculty of Medical Sciences in Zabrze, Medical University of Silesia, Katowice, Poland.
Article Synopsis
- The study focuses on major aortopulmonary collateral arteries (MAPCAs), rare congenital anomalies commonly seen alongside heart conditions like tetralogy of Fallot (TOF) and pulmonary atresia (PA).
- A retrospective analysis of 46 cases was conducted using advanced imaging techniques, revealing a strong link between MAPCAs and congenital heart diseases, along with distinctive echocardiographic and radiologic traits.
- The findings highlight the need for early detection and treatment of MAPCAs to improve patient outcomes and suggest the necessity for ongoing research into these complex conditions.
Increased Diastolic Energy Loss Associated with Cardiac Events in Adults with Pulmonary Atresia Suffering from Intact Ventricular Septum.
Magn Reson Med Sci
July 2024
Department of Diagnostic imaging & Nuclear Medicine, Tokyo Women's Medical University, Tokyo, Japan.
Article Synopsis
- The study aims to evaluate right heart diastolic energy loss (EL) as a measure of cardiac workload and its links to major cardiac events (MACE) in adults with pulmonary atresia and an intact ventricular septum (PAIVS).
- Researchers compared 30 adult patients, including those with PAIVS and controls with pulmonary stenosis (PS), assessing factors like energy loss ratios, tricuspid regurgitation, and other cardiac metrics through MRI and echocardiography.
- Findings show that adults with PAIVS had a higher diastolic EL/cardiac output ratio and worse tricuspid regurgitation compared to PS patients; this ratio was also linked to MACE, indicating that even with
A complex case of univentricular heart with multiple congenital malformations diagnosed in a newborn: a case report and literature review.
Ann Med Surg (Lond)
July 2024
Department of Cardiology Department, Alwatani Hospital, Hama University, Hama.
Article Synopsis
- * A case is presented of a newborn boy diagnosed with double-inlet left ventricle (DILV), who showed symptoms of cyanosis and feeding difficulties, leading to the need for palliative surgery, specifically the Glenn procedure at 6 months old.
- * Early postnatal intervention is vital for improving survival and reducing complications in children with this disease, highlighting the importance of ongoing research and development of treatment strategies.
Pulmonary Atresia with Intact Ventricular Septum: Correlation of Preoperative Computed Tomography-Derived Parameters with Echocardiographic Tricuspid Valve Z-Score and Surgical Outcomes.
Pediatr Cardiol
July 2024
Department of Epidemiology, Harvard T.H. Chan School of Public Health, Boston, MA, USA.
Article Synopsis
- The study investigates how preoperative cardiac CT can aid in treatment planning for neonates with pulmonary atresia and intact ventricular septum (PA-IVS).
- CT was used to evaluate the presence of ventriculocoronary arterial connections and to compare cardiac volumetric parameters with echocardiographic findings in 12 neonates.
- Results showed a significant correlation between CT measurements and echocardiographic data, indicating that CT can provide valuable information in determining surgical options, particularly in complex cases.
Molecular Pathways and Animal Models of Tricuspid Atresia and Univentricular Heart.
Adv Exp Med Biol
June 2024
Department of Biochemistry and Molecular Genetics, American University of Beirut, Beirut, Lebanon.
Article Synopsis
- The formation of heart valves is a complicated process that involves various molecular pathways working together at specific times.
- Key molecules identified in tricuspid atresia (TA) include TGF-ß, BMP, and NOTCH, along with downstream targets like NKX2-5 and GATA4.
- While some mouse models exhibit features of TA, none accurately represent the condition, highlighting the need for better models to understand the molecular mechanisms underlying the disease.
Human Genetics of Tricuspid Atresia and Univentricular Heart.
Adv Exp Med Biol
June 2024
Faculty of Medicine, American University of Beirut, Beirut, Lebanon.
Article Synopsis
- - Tricuspid atresia (TA) is a rare heart defect where the right atrioventricular valve is completely absent, leading to serious health issues.
- - Limited research on TA's genetic factors has identified specific chromosomal abnormalities and several potentially involved genes, indicating a complex genetic underpinnings of the condition.
- - The diversity in symptoms and genetic profiles among TA patients highlights the need for further study to better understand the intricate genetic network associated with this congenital heart defect.
Clinical Presentation and Therapy of Tricuspid Atresia and Univentricular Heart.
Adv Exp Med Biol
June 2024
Cardiovascular Genetics, Charité - Universitätsmedizin Berlin, Berlin, Germany.
Article Synopsis
- - The terms "single ventricle" and "univentricular heart" often refer to complex congenital heart defects, but most hearts actually have two ventricles.
- - In some cases, one ventricle may be too small to work properly, leading to functional issues.
- - A more accurate description of these hearts is "functional single ventricle," highlighting that they still contain two ventricles, even if one isn't fully operational.
Prenatal Cardiac Ultrasound Markers of Outcomes in Ebstein's Anomaly: An International Multicenter Case Series.
Pediatr Cardiol
June 2024
Department of Obstetrics, Paulista School of Medicine, Federal University of São Paulo (EPM-UNIFESP), São Paulo-SP, Brazil, Rua Belchior de Azevedo, 156 Apto. 111 Torre Vitória, 05089-030.
Article Synopsis
- - The study analyzed prenatal cardiac ultrasound markers in 35 fetuses diagnosed with Ebstein's anomaly (EA) across three countries, focusing on perinatal mortality and outcomes through retrospective data analysis.
- - Out of 29 fetuses eligible for the study, 53.1% were born alive, while others faced a mix of stillbirths, neonatal deaths, and fetal deaths; among the surviving patients, most underwent successful biventricular repair surgery postnatally.
- - The findings indicated that severe tricuspid regurgitation and pulmonary atresia were common, with significant cardiomegaly noted in many cases, while a few fetuses exhibited abnormal karyotypes linked to poorer outcomes.
Presentation of Complex Congenital Cardiac Anomalies in a Newborn Pediatric Patient: A Case Report.
Cureus
April 2024
Pediatrics, Willis Knighton Health System, Shreveport, USA.
Article Synopsis
- * A case study is presented showing a stepwise treatment approach for a patient with tricuspid atresia, involving imaging techniques and procedures like balloon atrial septostomy.
- * The case highlights the importance of a multidisciplinary team in managing complex congenital heart defects, aiming to improve patient outcomes and potentially reduce mortality rates.