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A Rare Case of Transposition of Great Arteries Uncovered With Progressive Rheumatic Aortic Stenosis.

ABSTRACT: The authors describe a case of transposition of great arteries with a large atrial septal defect with fusion of tricuspid valve leaflets and severe aortic stenosis. The latter two were likely rheumatic in etiology. The patient's condition improved after atrial switch and aortic valve replacement surgery. (Level of Difficulty: Intermediate.).

SUBMITTER: Yadav S

PROVIDER: S-EPMC8387841 | biostudies-literature | 2021 Aug

REPOSITORIES: biostudies-literature

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A Rare Case of Transposition of Great Arteries Uncovered With Progressive Rheumatic Aortic Stenosis.

Yadav Siddhant S Phadke Milind M Mahajan Ajay A Nathani Pratap P

JACC. Case reports 20210819 10

The authors describe a case of transposition of great arteries with a large atrial septal defect with fusion of tricuspid valve leaflets and severe aortic stenosis. The latter two were likely rheumatic in etiology. The patient's condition improved after atrial switch and aortic valve replacement surgery. (<b>Level of Difficulty: Intermediate.</b>). ...[more]

PMID: 34471880

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Project description:BackgroundAdults with unoperated congenitally corrected transposition of the great arteries are rare but form a distinct group among adults with congenital heart disease. Patients with congenitally corrected transposition of the great arteries often have one or more associated cardiac anomalies that dictate the need for, and timing of, surgical intervention in childhood. However, in a proportion of patients, the hemodynamics does not require surgical attention during childhood, and, in some patients, a correct diagnosis is not established until adulthood. Here we report an adult case of unoperated congenitally corrected transposition of the great arteries with a large ventricular septal defect and probable pulmonary arterial hypertension.Case presentationOur patient was a 46-year-old Korean man. Transthoracic echocardiography and cardiac catheterization demonstrated hemodynamically balanced ventricles with a non-regurgitant systemic atrioventricular valve, normal pulmonary arterial pressure, and a reasonable difference between the oxygen saturation values of the aorta and pulmonary trunk, even with the presence of a large ventricular septal defect. Further morphological assessments using cardiac computed tomography and three-dimensional modeling/printing of his heart revealed that the mitral valve was straddling over the posteriorly positioned ventricular septal defect, which could explain the functional and anatomical subvalvular pulmonary stenosis and a small amount of shunt flow through the large ventricular septal defect. We interpreted this combination of cardiac defects as able to sustain his stable cardiac function. Thus, we decided to maintain his unoperated status.ConclusionA detailed anatomical understanding based on transthoracic echocardiography, cardiac computed tomography, and three-dimensional printing can justify a decision to not operate in cases of congenitally corrected transposition of the great arteries with hemodynamically balanced pulmonary stenosis and a ventricular septal defect, as observed in the present case.

Dataset Information

A Rare Case of Transposition of Great Arteries Uncovered With Progressive Rheumatic Aortic Stenosis.

Publications

A Rare Case of Transposition of Great Arteries Uncovered With Progressive Rheumatic Aortic Stenosis.

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