Articles: heterotaxy-syndrome-diagnostic-imaging.
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Case Reports
Severe COVID-19 acute respiratory distress syndrome in an adult with single-ventricle physiology: a case report.
COVID-19 can induce acute respiratory distress syndrome (ARDS). In patients with congenital heart disease, established treatment strategies are often limited due to their unique cardiovascular anatomy and passive pulmonary perfusion. ⋯ ARDS treatment strategies including ventilatory settings, prone positioning therapy and cannulation techniques for extracorporeal oxygenation must be adopted carefully considering the passive venous return in patients with single-ventricle physiology.
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Review
HUMAN LATERALITY DISORDERS: PATHOGENESIS, CLINICAL MANIFESTATIONS, DIAGNOSIS, AND MANAGEMENT.
Human laterality disorders comprise a group of diseases characterized by abnormal location (situs) and orientation of thoraco-abdominal organs and vessels across the left-right axis. Situs inversus totalis is mirror image reversal of thoraco-abdominal organs/great vessels. Situs ambiguus, better known as heterotaxy, is abnormal arrangement of thoraco-abdominal organs across the left-right axis excluding situs inversus totalis. ⋯ Right and left atrial isomerism are associated with multiple complex congenital cardiac and vascular anomalies, many of which are lethal when untreated. Isomerism may also affect the lungs, spleen, liver, gall bladder, and intestines. Innovative surgical therapy of heterotaxy/isomerism has reduced early mortality and markedly improved long-term prognosis.
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Right atrial isomerism (RAI) is one of the most severe forms of congenital heart disease. This case of RAI was so complex that it incorporated 7 heart defects. It can be challenging to display the spatial relationship between different anatomical structures using conventional two-dimensional and color ultrasound (2D-Doppler imaging); therefore, we used spatio-temporal image correlation (STIC) and high definition live flow imaging technology to vividly display this case of RAI in a stereoscopic mode. ⋯ Accurate diagnosis of RAI is essential for clinical and parent decision making. 2D-Doppler imaging combined with STIC-HD live flow can be used to visualize the spatial morphology of blood vessels, including the cardiac chambers and great vessels of the fetal heart, and smaller peripheral vessels.
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Cardiology in the young · Apr 2016
Determining bronchial morphology for the purposes of segregating so-called heterotaxy.
Introduction Heterotaxy is a unique clinical entity in which lateralisation of the thoraco-abdominal organs is abnormal, typically with isomerism of the bronchial tree and atrial appendages. This study was carried out to determine whether routine clinical imaging such as chest radiographs, angiographic images, and CT/MRI can determine bronchial isomerism, and how sidedness of bronchial isomerism correlates with overall features anticipated in hearts with isomeric atrial appendages. Methods and results We identified 73 patients with heterotaxy, in whom imaging clearly demonstrated the bronchial tree, seen at our institution since 1998. We calculated bronchial angles and lengths using all the available imaging modalities to determine the presence and sidedness of bronchial isomerism. This was then compared with the anticipated presence of isomeric atrial appendages based on the overall clinical findings, as the appendages themselves had not specifically been imaged. The ratio of bronchial lengths revealed bronchial isomerism in all patients, with bronchial angles permitting distinction of right as opposed to left isomerism. We noted discordances between the identified bronchial isomerism and the presumed arrangement of the atrial appendages in nearly 20% of the patients in our cohort. ⋯ Routine clinical imaging with chest radiographs, angiographic imaging, and CT/MRI can determine the presence of bronchial isomerism in patients with so-called heterotaxy. Right as opposed to left isomerism can be distinguished based on bronchial angles. The finding of bronchial isomerism correlates well, but not totally, with the presumed isomerism of the atrial appendages as predicted from the identified intra-cardiac morphology.