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[Transposition of great vessels in Cantrell syndrome]
L Czarnecki1, U Mikołajczak-Mejer, E Zinka
1Poradni Kardiologii Dzieciecej Wojewódzkiej Przychodni Matki, Dziecka i Młodziezy, Koszalinie.
Insights
This case report details a rare instance of complete transposition of great arteries (d-TGA) within Cantrell syndrome, a complex congenital condition. It highlights the first documented occurrence of d-TGA in patients with this syndrome.
Area of Science:
- Pediatric Cardiology
- Congenital Abnormalities
- Syndromology
Background:
- Cantrell syndrome is a rare congenital condition characterized by a specific set of defects including omphalocele, ectopia cordis, sternal defects, diaphragmatic defects, and pericardial defects.
- Intracardiac defects are common in Cantrell syndrome, with ventricular septal defects being the most frequently reported.
- Complete transposition of great arteries (d-TGA) is a critical congenital heart defect where the aorta and pulmonary artery are switched.
Observation:
- A case of Cantrell syndrome is presented with a unique combination of anomalies.
- The patient exhibited complete transposition of great arteries (d-TGA), atrial septal defect, ventricular septal defect, and coarctation of the pulmonary artery.
- This represents the first reported instance of d-TGA occurring in conjunction with Cantrell syndrome.
Findings:
- The study documents a novel association between Cantrell syndrome and d-TGA.
- The findings expand the known spectrum of intracardiac defects associated with Cantrell syndrome.
- This case underscores the variability and complexity of congenital anomalies in Cantrell syndrome.
Implications:
- This case provides valuable insights for the diagnosis and management of complex congenital heart disease in Cantrell syndrome.
- It emphasizes the importance of thorough cardiac evaluation in patients diagnosed with Cantrell syndrome.
- Further research into the embryological basis of this specific combination of defects may be warranted.
Abstract:
A case is presented of complete transposition of great vessels with atrial and ventricular septum defect and coarctation of the pulmonary artery in Cantrell syndrome. The Cantrell syndrome consists of: congenital heart disease, defect of pericardium, diaphragm, sternum, and anterior abdomen wall. In all cases of Cantrell syndrome described as yet ventricular septum defect was present alone or in combination with other intracardiac defects. The presented case is the first report of congenital abnormality in the from of d-TGA in Cantrell syndrome.