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Published on: February 5, 2021
Dynamic outflow tract obstruction in congenitally corrected transposition of the great arteries
1Heart & Vascular Institute, Department of Cardiovascular Medicine, Cleveland Clinic, Desk J1-5, Cleveland, OH 44195, USA. Zuricka@ccf.org
Insights
Congenitally corrected transposition of the great arteries (CCTGA) is a rare heart defect. This case highlights severe complications and challenges in surgical treatment for adult CCTGA patients.
Area of Science:
- Cardiology
- Congenital Heart Disease
- Cardiac Surgery
Background:
- Congenitally corrected transposition of the great arteries (CCTGA) is a rare congenital heart defect, affecting 0.5% of cases.
- Left ventricle outflow tract obstruction is a common complication in CCTGA, occurring in 44-57% of patients.
Observation:
- A 45-year-old woman with CCTGA presented with worsening dyspnea and severe tricuspid regurgitation.
- Imaging revealed severe right ventricle dysfunction and dynamic left ventricle outflow tract obstruction due to mitral valve systolic anterior motion and a ventricular septal aneurysm.
Findings:
- The patient exhibited severe systemic ventricle (morphologic right ventricle) contractile dysfunction.
- Dynamic non-systemic ventricle (morphologic left ventricle) outflow tract obstruction was identified, caused by systolic anterior motion of the mitral valve and a ventricular septal aneurysm.
Implications:
- Surgical correction was deemed too high-risk for this patient.
- Medical therapy was pursued as the primary treatment course for this complex adult CCTGA case.
Abstract:
Congenitally corrected transposition of the great arteries (CCTGA) is a rare form of congenital heart disease, constituting 0.5% of all congenital heart defects. The incidence of left ventricle (non-systemic ventricle) outflow tract obstruction ranges between 44 and 57%. Herein, we present the case of a 45 year old woman with CCTGA with progressively worsening dyspnea who had been referred for surgical correction of severe systemic ventricle (morphologic right ventricle) atrio-ventricular valve (tricuspid valve) regurgitation. Cardiac magnetic resonance imaging (CMR) and transesophageal imaging (TEE) demonstrated severe systemic ventricle (morphologic right ventricle) contractile dysfunction, as well as dynamic non-systemic ventricle (morphologic left ventricle) outflow tract obstruction due to systolic anterior motion (SAM) of the non-systemic ventricle (morphologic left ventricle) atrio-ventricular valve (mitral valve) with a large membranous ventricular septal aneurysm that protrudes into the outflow tract of the non-systemic ventricle (morphologic left ventricle). Ultimately, our patient was felt to be too high-risk for surgical correction and a course of medical therapy has been pursued.
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