Objectives: Aortic valve reconstruction (AVRec) with neocuspidization or the Ozaki procedure with complete cusp replacement for aortic valve disease has excellent mid-term results in adults. Limited results of AVRec in pediatric patients have been reported. We report our early outcomes of the Ozaki procedure for congenital aortic and truncal valve disease.
Methods: A retrospective analysis was performed on all 57 patients with congenital aortic and truncal valve disease who had a 3-leaflet Ozaki procedure at a single institution from August 2015 to February 2019. Outcome measures included mortality, surgical or catheter-based reinterventions, and echocardiographic measurements.
Results: Twenty-four patients had aortic regurgitation (AR), 6 had aortic stenosis (AS), and 27 patients had AS/AR. Two patients had quadricuspid valves, 26 had tricuspid, 20 had bicuspid, and 9 had unicusp aortic valves. Four patients had truncus arteriosus. Thirty-four patients had previous aortic valve repairs and 5 had replacements. Preoperative echocardiography mean annular diameter was 20.90 ± 4.98 cm and peak gradient for patients with AS/AR was 53.62 ± 22.20 mm Hg. Autologous, Photofix, and CardioCel bovine pericardia were used in 20, 35, and 2 patients. Eight patients required aortic root enlargement and 20 had sinus enlargement. Fifty-one patients had concomitant procedures. Median intensive care unit and hospital length of stay were 1.87 and 6.38 days. There were no hospital mortalities or early conversions to valve replacement. At discharge, 98% of patients had mild or less regurgitation and peak aortic gradient was 16.9 ± 9.5 mm Hg. Two patients underwent aortic valve replacement. At median follow-up of 8.1 months, 96% and 91% of patients had less than moderate regurgitation and stenosis, respectively.
Conclusions: The AVRec procedure has acceptable short-term results and should be considered for valve reconstruction in pediatric patients with congenital aortic and truncal valve disease. Longer-term follow-up is necessary to determine the optimal patch material and late valve function and continued annular growth.
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http://dx.doi.org/10.1016/j.jtcvs.2020.01.087 | DOI Listing |
J Cardiothorac Vasc Anesth
January 2025
Division of Congenital Cardiac Anesthesiology, University of Alabama at Birmingham School of Medicine, Birmingham, AL; Bruno Pediatric Heart Center, Children's of Alabama, Birmingham, AL. Electronic address:
Cureus
December 2024
University of Florida Health Congenital Heart Center, University of Florida College of Medicine, Gainesville, USA.
Anomalous aortic origin of a coronary artery (AAOCA) comprises a set of rare congenital abnormalities in the origin or path of the coronary arteries with highly variable clinical implications. This is a pilot feasibility study where we investigated the influence of the anomalous coronary artery inlet architecture on coronary perfusion using coronary blood flow computational simulations to help predict the risk for coronary ischemia in patients with anomalous aortic origin of the right coronary artery (AAORCA) with these types of anomalous coronary artery inlet architectures. We developed a protocol for generating 3D models of patient coronary artery anatomies from an IRB-approved dataset of cardiac CT images of patients with AAORCA at our institution.
View Article and Find Full Text PDFMalawi Med J
January 2025
Department of Paediatrics, College of Medicine, University of Nigeria Ituku/Ozalla and University of Nigeria Teaching Hospital Ituku/Ozalla, Enugu, Enugu State, Nigeria.
Background: Children with Tetralogy of Fallot (TOF) usually present with right heart abnormalities, however much is not known if these children presents with left ventricular dysfunction.
Objectives: This article is aimed to ascertain the left ventricular function of children with TOF compared with those without any congenital heart disease. It also elicits the correlation if any, between left ventricular mass and descending aorta blood flow.
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 PDFRadiol Case Rep
March 2025
Division of Maternal Fetal Medicine, Department of Obstetrics and Gynecology, State University of New York (SUNY), Downstate Health Sciences University, Brooklyn, NY, USA.
Extensive congenital pulmonary airway malformation (CPAM) of the left fetal lung and associated marked dextroposition of the fetal heart were noted at 21 weeks' gestation. The right fetal lung appeared compressed with the cardiomediastinal shift angle measuring approximately 20 degrees. Potential subsequent right pulmonary hypoplasia was considered.
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