29 February 2020
: Case report
Late Presentation of a Congenitally Corrected Transposition of Great Arteries and Hemodynamically Balanced Ventricles Associated with a Large Ventricular Septal Defect and Severe Pulmonary Stenosis: A Case Report and a Multi-Imaging Approach
Challenging differential diagnosis, Congenital defects / diseases
Khaled Elenizi12ABCDEFG*, Rasha Alharthi2ABCDEFG, Anthony Matta23ABCDEFG, Abdulrahman Aljuayli24ABCDEFG, Michel Galinier2ADGDOI: 10.12659/AJCR.920822
Am J Case Rep 2020; 21:e920822
Abstract
BACKGROUND: Congenitally corrected transposition of great arteries (ccTGA) represents a distinct rare group of congenital heart diseases. Survival of unoperated ccTGA in the presence of large ventricular septal defect (VSD) is exceptional. Furthermore, late presentation of such patients in the absence of severe pulmonary hypertension or severe systemic right ventricle dysfunction is unusual.
CASE REPORT: We report a rare late presentation of ccTGA associated with large VSD in the absence of severe pulmonary hypertension or systemic ventricle dysfunction. An associated severe pulmonary valve stenosis maintained a balanced and stable condition up to the fourth decade of life. The patient has also dextrocardia, which is an unusual association. The diagnosis was reached using multimodality imaging including transthoracic echocardiogram (TTE), transesophageal echography (TEE), cardiac magnetic resonance imaging (CMR), and cardiac computed tomography (cardiac CT).
CONCLUSIONS: The presence of pulmonary stenosis can provide a physiological protection that avoids unnecessary surgical correction of large VSD in ccTGA patients. However, such a decision should be made on an individual basis and following a careful anatomical and functional evaluation.
Keywords: Cardiac Electrophysiology, Congenital Abnormalities, Transposition of Great Vessels, Congenitally Corrected Transposition of the Great Arteries, Dextrocardia, Heart Septal Defects, Ventricular, Pulmonary Valve Stenosis
In Press
Case report
Am J Case Rep In Press; DOI: 10.12659/AJCR.946956
Case report
Am J Case Rep In Press; DOI: 10.12659/AJCR.946875
Case report
Am J Case Rep In Press; DOI: 10.12659/AJCR.947289
Case report
Am J Case Rep In Press; DOI: 10.12659/AJCR.946614
Most Viewed Current Articles
21 Jun 2024 : Case report
95,940
DOI :10.12659/AJCR.944371
Am J Case Rep 2024; 25:e944371
07 Mar 2024 : Case report
52,046
DOI :10.12659/AJCR.943133
Am J Case Rep 2024; 25:e943133
20 Nov 2023 : Case report
30,832
DOI :10.12659/AJCR.941424
Am J Case Rep 2023; 24:e941424
18 Feb 2024 : Case report
23,344
DOI :10.12659/AJCR.943030
Am J Case Rep 2024; 25:e943030