Background: Restrictive interatrial communication (IAC) causes morbidity and mortality in infants with hypoplastic left heart syndrome awaiting cardiac transplantation. We sought to create a scoring system, based on echocardiographic and clinical findings, to serve as a guide for determining the need for balloon atrial septostomy (BAS).
Methods: We retrospectively reviewed echocardiograms of 44 infants with hypoplastic left heart syndrome. Infants were studied from the time of admission to the final end-point of transplantation, Norwood procedure, or death. Seventeen infants underwent BAS for clinical indications of oxygen saturation <80% in room air. Data collected included age at BAS, maximum velocity (V(max)), and IAC diameter throughout the clinical course. We assigned higher IAC scores to smaller IAC diameter, greater V(max) through the IAC, and lower oxygen saturation value. The minimum score was 3, and the maximum score was 9.
Results: Only 10% of infants with a score <6 at presentation required BAS, whereas 67% of those with scores > or =6 required BAS. Higher IAC scores at presentation were associated with earlier need for BAS (p = 0.04).
Conclusions: The IAC scoring system can serve as a reliable clinical guide for identifying infants with hypoplastic left heart syndrome who are likely to require BAS for relief of critically restrictive IAC while awaiting cardiac transplantation.
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http://dx.doi.org/10.1016/s1053-2498(02)00820-3 | DOI Listing |
Cureus
November 2024
Pediatrics, Kagoshima University Hospital, Kagoshima, JPN.
Background Children with Down syndrome (DS) often have hypoplastic kidneys and urinary tract malformations that increase their renal dysfunction risk. They also have a higher congenital heart disease (CHD) rate, requiring cardiac surgery during infancy. Renal dysfunction in such patients may be associated with the development of cardiac surgery-associated acute kidney injury (CS-AKI), but this remains unclear.
View Article and Find Full Text PDFCase Rep Endocrinol
December 2024
Henry Ford St. John Hospital, Detroit, Michigan, USA.
Pediatr Dev Pathol
December 2024
Departments of Pathology (JB) and Pediatrics, Children's Hospital and Medical Center, Omaha, NE, USA.
An infant with intrauterine growth restriction, suspected of having MIRAGE syndrome based on prenatal ultrasound, presented with genital ambiguity, adrenal insufficiency, intractable diarrhea from birth, and a pathogenic mutation (). Endoscopic biopsies of the duodenum revealed complex light and electron microscopic abnormalities. Hypoplastic villi without signs of enteritis suggests a disorder of mucosal growth with reduced absorptive surface area contributes to intractable diarrhea.
View Article and Find Full Text PDFPeerJ Comput Sci
November 2024
Department of Computer Science, School of Engineering and Digital Sciences, Nazarbayev University, Astana, Kazakhstan.
Congenital heart disease (CHD) remains a significant global health challenge, particularly contributing to newborn mortality, with the highest rates observed in middle- and low-income countries due to limited healthcare resources. Machine learning (ML) presents a promising solution by developing predictive models that more accurately assess the risk of mortality associated with CHD. These ML-based models can help healthcare professionals identify high-risk infants and ensure timely and appropriate care.
View Article and Find Full Text PDFEarly Hum Dev
January 2025
The Zickler Family Prenatal Pediatrics Institute, Children's National Hospital, Washington, DC, USA; The Developing Brain Institute, Children's National Hospital, Washington, DC, USA; Department of Pediatrics, The George Washington University School of Medicine, Washington, DC, USA. Electronic address:
Background: Neonates with critical congenital heart disease (cCHD) undergo a complicated transition to ex-utero life. However, continuous monitoring of autonomic tone using heart rate variability is currently lacking.
Materials And Methods: We retrieved continuous electrocardiograms from the time of admission or from 10 days prior to surgery for neonates with dextro-transposition of the great arteries (d-TGA) and hypoplastic left heart syndrome (HLHS).
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