Dynamic outflow tract obstruction in congenitally corrected transposition of the great arteries.
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.
Zurick
Andrew O
AO
Heart & Vascular Institute, Department of Cardiovascular Medicine, Cleveland Clinic, Desk J1-5, Cleveland, OH 44195, USA. Zuricka@ccf.org
Menon
Venu
V
D004417:Dyspnea / Q000209:etiology; D015150:Echocardiography, Doppler; D017548:Echocardiography, Transesophageal; D005260:Female; D006322:Heart Aneurysm / Q000209:etiology; D006349:Heart Valve Diseases / Q000209:etiology; D006801:Humans; D008279:Magnetic Resonance Imaging; D008875:Middle Aged; D014188:Transposition of Great Vessels / Q000150:complications*; D014694:Ventricular Outflow Obstruction / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy