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Background: The approach to coarctation of the aorta with hypoplastic aortic arch is controversial. We evaluated the outcomes in patients with coarctation of the aorta with or without hypoplastic aortic arch operated through a posterior left lateral thoracotomy.
Methods: A retrospective cohort of patients with aortic coarctation, who underwent repair between January 2009 and October 2017, was analyzed. Preoperative, postoperative, and echocardiographic characteristics were reviewed. Statistical analysis examined survival, freedom from reintervention, and freedom from recoarctation.
Results: In nine years, 389 patients who underwent surgical treatment for coarctation of the aorta were identified; after exclusion criteria and complete echocardiographic reports, 143 patients were analyzed, of which 29 patients had hypoplastic aortic arch. The modification in the extended end-to-end anastomosis technique was a wide dissection and mobilization of the descending aorta that was achieved due to the ligation and division of 3 to 5 intercostal vessels. In both groups, patients were close to one month of age and had a median weight of 3.6 and 3.4 kg for hypoplastic and nonhypoplastic arch, respectively. In postoperative events, there was no statistically significant difference between the groups ( P = .57 for renal failure, P = .057 for transient, nonpermanent neurologic events, P = .496 for sepsis), as for intensive care unit ( P = .502) and total in-hospital stay ( P = .929). There was one case of postoperative mortality in each group and both were associated with noncardiac comorbidities. Regarding survival (log-rank = 0.060), freedom from reintervention (log-rank = 0.073), and freedom from recoarctation (log-rank = 0.568), there was no statistically significant difference between the groups.
Conclusion: We believe that it is the modified technique that allowed greater mobilization of the aorta and successful repair of hypoplastic arch through thoracotomy, without an increase in paraplegia or other adverse outcomes.
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http://dx.doi.org/10.1177/2150135118799631 | DOI Listing |
G3 (Bethesda)
August 2025
Department of Biochemistry, University of Otago, Dunedin 9016, New Zealand.
RECQL4 encodes a RecQ helicase, one of a family of DNA unwinding enzymes with roles in DNA replication, double strand break repair and genomic stability. Pathogenic variants in RECQL4 are clinically associated with three rare autosomal recessive conditions: Rothmund-Thomson Syndrome type II, Baller-Gerold Syndrome and RAPADILINO syndrome. These three syndromes show overlapping growth retardation, low bone density and skeletal defects affecting the arms and hands.
View Article and Find Full Text PDFBMC Pediatr
August 2025
Department of Cardiology, Beijing Children's Hospital, Capital Medical University, National Centre for Children's Health, Nanlishi Road 56, Xicheng District, Beijing, 100045, China.
Background: MYRF gene mutations can lead to the development of Cardio-Urogenital Syndrome (CUGS), characterized by congenital heart disease, abnormalities in the internal and external reproductive organs, and ocular anomalies. CUGS can manifest with various types of congenital heart diseases, such as Tetralogy of Fallot, Scimitar syndrome, Hypoplastic Left Heart Syndrome, Atrial Septal Defect, Ventricular Septal Defect, Dextrocardia, Aortic Arch Anomalies, and Pulmonary Vein Anomalies et al. Male patients (46,XY) may present with unilateral cryptorchidism, ambiguous genitalia, or even typical female genitalia.
View Article and Find Full Text PDFJACC Case Rep
August 2025
Ward Family Heart Center, Children's Mercy Hospital, Kansas City, Missouri, USA; Department of Pediatrics, University of Missouri-Kansas City School of Medicine, Kansas City, Missouri, USA.
Background: Mitral valve dysplasia syndrome (MVDS) is a rare cardiac defect requiring accurate prenatal identification and appropriate counseling, delivery planning, and postnatal management.
Case Summary: A 39-year-old woman underwent fetal echocardiography at 34 weeks' gestation owing to cardiomegaly and hydrops. Biatrial enlargement, mild to moderate atrioventricular valve insufficiency, dysplastic mitral valve apparatus, biventricular dilation with systolic dysfunction, endocardial fibroelastosis, hypoplastic aortic outflow, and severely restrictive atrial septum were attributed to MVDS.
Neonatal Netw
August 2025
Department of Pediatrics (Cardiology), University of Arizona, Tucson, AZ, USA
Studies suggest that in utero opioid exposure may be associated with congenital heart disease (CHD). We sought to assess the incidence of CHD in infants with neonatal abstinence syndrome (NAS). A review of a national, administrative database from January 2019 to December 2022 was conducted for neonates with an code for NAS and moderate or severe CHD.
View Article and Find Full Text PDFEur Heart J Qual Care Clin Outcomes
August 2025
Department of Paediatric and Adult Congenital Cardiology, M3C National Reference Centre, Bordeaux University Hospital, Bordeaux, France.
Objectives: Coarctation of the aorta (CoA) is the most common undiagnosed congenital heart disease during prenatal screening and its overall prognosis relies on the quality of the surgical repair. This study aimed to identify the prenatal and postnatal factors associated with the type of surgical technique repair in infant CoA.
Methods: Multicentre, retrospective, observational study in 680 infants (68% males) aged <1 year who underwent CoA surgical repair in 4 CHD surgical centres in France over 11 years.