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Prevalence of immunological aberrations and 22q11.2 deletion in children with conotruncal anomalies: A cross-sectional study.
Ann Pediatr Cardiol
December 2024
Department of Pediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Introduction: 22q11.2 deletion is associated with conotruncal anomalies and immunological aberrations. Given the common embryonic origin of conotruncus and thymus, conotruncal anomalies may be associated with immunological aberrations irrespective of 22q11.
View Article and Find Full Text PDFSpectrum of Heterotopic and Ectopic Splenic Conditions.
Radiographics
November 2024
From the Department of Radiology, University of Wisconsin School of Medicine and Public Health, E3/311 Clinical Science Center, 600 Highland Ave, Madison, WI 53792-3252 (L.W.N., M.G.L., P.J.P.); Mallinckrodt Institute of Radiology, Washington University School of Medicine, St Louis, Mo (S.M.B., S.B.); and American College of Radiology (ACR) Institute for Radiologic Pathology (AIRP), Silver Spring, Md (M.G.L., P.J.P.).
A spectrum of heterotopic and ectopic splenic conditions may be encountered in clinical practice as incidental asymptomatic detection or symptomatic diagnosis. The radiologist needs to be aware of these conditions and their imaging characteristics to provide a prompt correct diagnosis and avoid misdiagnosis as neoplasm or lymphadenopathy. Having a strong knowledge base of the embryologic development of the spleen improves understanding of the pathophysiologic basis of these conditions.
View Article and Find Full Text PDFAsplenia in left isomerism.
Ann Pediatr Cardiol
July 2024
Department of Cardiology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram, Kerala, India.
Article Synopsis
- Heterotaxy refers to abnormal arrangements of heart and other visceral structures that do not follow the typical layout.
- Isomerism in congenital heart defects features paired structures that are symmetrical mirror images across the body's left-right axis, with distinct types: right (linked with asplenia) and left (linked with polysplenia).
- The report discusses a unique case of left isomerism accompanied by asplenia in a 4-year-old girl.
Thoracic duct drainage patterns in heterotaxy.
J Cardiovasc Magn Reson
December 2024
Department of Cardiology, Boston Children's Hospital, Boston, MA, United States; Department of Pediatrics, Harvard Medical School, Boston, MA, United States.
Article Synopsis
- Disordered lymphatic drainage is common in congenital heart diseases, and this study focuses on the drainage patterns of the thoracic duct (TD) in patients with heterotaxy, a condition involving abnormal arrangement of internal organs.
- Researchers analyzed 115 patients with heterotaxy who had cardiovascular imaging, noting that the TD was visualized in nearly half, with varying sidedness: left (25), right (29), and bilateral (2).
- The study found that while various anatomical factors were associated with TD sidedness, only the sidedness of the superior vena cava and aortic arch were significant predictors, highlighting the complexity of lymphatic anatomy in heterotaxy for future medical interventions.
Clinical implications of dextrocardia based on four visceroatrial situs studies.
Pediatr Neonatol
November 2024
Department of Pediatrics, Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taoyuan, Taiwan. Electronic address:
Background: Previous studies on congenital heart diseases (CHD) associated with dextrocardia were based on selective patient databases and did not reflect the full spectrum of dextrocardia in the general population. Additionally, these studies had complex classification and presentation. Nor did these studies elaborate on the distribution of the associated CHD's complexity, the various segmental connections, and associated CHD among the four visceroatrial situs.
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