The modern methods of X-Ray based diagnostic in cases of ectopia cordis associated with pentalogy of Cantrell

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Abstract


Pentalogy of Cantrell with ectopia cordis is an extremely rare and lethal congenital anomaly included congenital hearth disease (CHD), midline supraumbilical abdominal wall defect, defect of lower sternum part, deficiency of the anterior diaphragm, a defect in the diaphragmatic pericardium. The complexity of this syndrome is usually incompatible with life. Depending on the location of the protruding heart and on the extent of the body wall defect, ectopia cordis may be grouped into cervical, thoracic, thoracoabdominal, or abdominal types. The heart mostly uncovered, covered with a serous membrane less often, and covered with skin rare. Depending on combination defects pentalogy of Cantrell classified for 3 different classes by Toyama (classified in 1972): 1 class – complete syndrome (all five defects), 2 class – probable syndrome (included intracardial defect and ventral abdominal wall defect) 3 class – incomplete syndrome (with various combinations of defects present, including a sternal abnormality). Presented clinical case newborn pentalogy of Cantrell first Toyama class. We used X-Ray for firstly diagnostic, for visualization CHD was performed CT. For postnatal diagnostic CHD are used Echo and CT. CT allows graphically to see the type of CHD and predict a surgical treatment. The strategy of surgical treatment and further prognose are depend on combination of anomalies within the framework of pentalogy of Cantrell. By CT we determined atresia of the pulmonary artery with major aorto-pulmonary collateral arteries (MAPCA), ventricular and atrial septal defects.


Elena V. Kosovtsova

Author for correspondence.
vovchenkomri@gmail.com
St Petersburg State Pediatric Medical University, Ministry of Healthcare of the Russian Federation
Russian Federation, Saint Petersburg

radiologist, Radiology Department

Alexandr V. Pozdnyakov

pozdnyakovalex@yandex.ru
St Petersburg State Pediatric Medical University, Ministry of Healthcare of the Russian Federation
Russian Federation, Saint Petersburg

MD, PhD, Dr Med Sci, Professor, Head, Department of medical Biophysics

Nikolay G. Pilyugov

pilyugovn@gmail.com
St Petersburg State Pediatric Medical University, Ministry of Healthcare of the Russian Federation
Russian Federation, Saint Petersburg

Assistant Professor, Department of Anesthesiology, Resuscitation and Emergency Pediatrics, Faculty of Postgraduate Education

Alexey B. Naumov

naumov@gmail.com
St Petersburg State Pediatric Medical University, Ministry of Healthcare of the Russian Federation
Russian Federation, Saint Petersburg

Assistant Professor, Department of Anesthesiology, Resuscitation and Emergency Pediatrics, Faculty of Postgraduate Education

Sergey P. Marchenko

sergeimarchenkospb@gmail.com
St Petersburg State Pediatric Medical University, Ministry of Healthcare of the Russian Federation
Russian Federation, Saint Petersburg

MD, PhD, Dr Med Sci, Professor, Department of Cardiovascular Surgery

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Copyright (c) 2017 Kosovtsova E.V., Pozdnyakov A.V., Pilyugov N.G., Naumov A.B., Marchenko S.P.

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