Tetralogy of Fallot with absent pulmonic valve is a rare but life-threatening syndrome. Children with this complex may have aneurysmally dilated pulmonary arteries which compress the tracheobronchial tree and cause airway compromise and death. This paper reviews the course of 10 children with this syndrome who were seen at our institution from 1962 until the present. Two infants with respiratory distress secondary to tetralogy plus absent pulmonary valve were treated with early aggressive medical intervention followed by operation. After closure of the ventricular septal defect and reconstruction of the right ventricular outflow tract, the pulmonary artery aneurysms were resected. Both children are growing and are well more than 1 year after the operations. Our experience with these two patients suggests that such infants may benefit from this form of therapy.
Download full-text PDF |
Source |
|---|
Publication Analysis
Top Keywords
Similar Publications
Defining variances in practice for use of electrophysiology studies in risk stratification of patients with repaired tetralogy of Fallot: a PACES survey.
Cardiol Young
January 2025
Department of Medical Education and Clinical Sciences, Elson S Floyd College of Medicine, Washington State University, Spokane, WA, USA.
Sudden cardiac death poses a significant risk in patients with surgically repaired tetralogy of Fallot. Despite extensive research, risk stratification practices vary. This study surveyed the Pediatric and Adult Congenital Electrophysiology Society to identify these differences.
View Article and Find Full Text PDFAssociation of MTHFD1 G1958A (rs2236225) gene polymorphism with the risk of congenital heart disease: a systematic review and meta-analysis.
BMC Med Genomics
January 2025
Department of Cardiovascular Surgery, Gansu Provincial Hospital, No. 204, Donggang West Road, Lanzhou City, Gansu Province, 730000, China.
Background: We did this study to better clarify the correlations of methylenetetrahydrofolate dehydrogenase 1 (MTHFD1)-G1958A (rs2236225) gene polymorphism with the risk of congenital heart diseases (CHD) and its subgroups.
Methods: Relevant articles were searched in PubMed, Web of Science, Cochrane Library, Embase, CNKI, VIP database and Wanfang DATA until October 2023. We will use odds ratios (ORs) and 95% confidence intervals (CIs) to examine the potential associations of MTHFD1- G1958A gene polymorphism with CHD and its subgroups.
RNA-sequencing unveils FLT4 splice site variants in variable congenital heart disease.
Eur J Hum Genet
January 2025
Center for Medical Genetics, Ghent University Hospital, Ghent, Belgium.
The etiology of congenital heart disease (CHD) is complex, comprising both genetic and environmental factors. Despite documented familial occurrences, the genetic etiology remains largely elusive. Trio exome sequencing identified a heterozygous FLT4 splice site variant in two families with respectively tetralogy of Fallot (TOF), and variable CHD comprising both the TOF spectrum and aortic coarctation.
View Article and Find Full Text PDFLong term outcome after surgical tetralogy of Fallot repair at young age: Longitudinal follow-up up to 50 years after surgery.
Int J Cardiol
January 2025
Department of Cardiology, Cardiovascular Institute, Thorax Center, Erasmus MC, Rotterdam, the Netherlands; European Reference Network for Rare, Low Prevalence and Complex Diseases of the Heart (ERN GUARD-Heart), Amsterdam, the Netherlands.
Background: Little is known about the very long-term outcome in Tetralogy of Fallot (ToF) patients.
Objectives: To prospectively evaluate clinical outcome and quality-of-life after surgical repair of ToF.
Methods: Single-centre, longitudinal cohort-study evaluating every decade 144 ToF patients who underwent surgical repair <15 years of age between 1968 and 1980.
Development of right ventricular electromechanical dyssynchrony following surgical repair of tetralogy of Fallot in infants.
Front Pediatr
January 2025
Division of Pediatric Cardiology, Division of Pediatrics, Children's Mercy Hospital, University of Missouri-Kansas City, Kansas City, MO, United States.
Background: In adolescents and adults with tetralogy of Fallot (TOF), right ventricle (RV) electromechanical dyssynchrony (EMD) due to right bundle branch block (RBBB) is associated with reduced exercise capacity and RV dysfunction. While the development of RBBB following surgical repair of tetralogy of Fallot (rTOF) is a frequent sequela, it is not known whether EMD is present in every patient immediately following rTOF. The specific timing of the onset of RBBB following rTOF therefore provides an opportunity to assess whether acute RBBB is associated with the simultaneous acquisition of EMD.
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!