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Congenitally corrected transposition in adults with left atrioventricular valve incompetence
Insights
Congenitally corrected transposition of the great vessels (CCT) can be challenging to diagnose in adults. New radiological signs, including a concave ascending aorta, double mediastinal stripe, and straight left lower cardiac border, aid in identifying this condition.
Area of Science:
- Cardiology
- Radiology
- Medical Imaging
Background:
- Congenitally corrected transposition of the great vessels (CCT) is a rare congenital heart defect.
- Diagnosis is often delayed until adulthood, particularly in asymptomatic individuals without associated cardiac malformations.
- Adult presentation commonly involves left atrioventricular valve incompetence and conduction disturbances.
Purpose of the Study:
- To investigate the diagnostic challenges of congenitally corrected transposition of the great vessels (CCT) in adult patients.
- To identify and describe specific radiological findings indicative of CCT in adults.
- To enhance the early detection of CCT through improved interpretation of chest radiographs.
Main Methods:
- Retrospective analysis of seven adult cases with congenitally corrected transposition of the great vessels (CCT).
- Review of plain chest radiographs to assess cardiovascular silhouette and aortic course.
- Correlation of radiological findings with clinical presentation and echocardiographic data.
Main Results:
- In four out of seven adult CCT cases, the diagnosis was not apparent on initial plain radiographs.
- Atypical presentation included a concave, rather than the typical convex, course of the ascending aorta.
- Two novel radiological signs were identified: the double mediastinal stripe and the straight left lower cardiac border.
Conclusions:
- Congenitally corrected transposition of the great vessels (CCT) can present with atypical radiological features in adults.
- The described radiological signs (double mediastinal stripe, straight left lower cardiac border) are valuable for diagnosing CCT.
- Increased radiologist awareness of these signs can improve the detection rate of CCT in adult populations.
Abstract:
Congenitally corrected transposition of the great vessels (CCT) is usually detected in childhood owing to the presence of associated cardiac malformations. If such malformations are absent, the patient may remain asymptomatic until adulthood; such persons usually present with left atrioventricular valve incompetence and conduction disturbances. The chest radiograph may be the first clue to the diagnosis, demonstrating the typical cardiovascular silhouette produced by the convex left ascending aorta. The authors examined seven cases of CCT manifested in adulthood and found that in four of them the diagnosis was not apparent from the plain image because the ascending aorta instead followed a concave course. Hallmarks of this phenomenon are discussed, and two radiological signs are presented: the double mediastinal stripe and the straight left lower cardiac border.