Midterm evaluation of echocardiographic examination, cardiac catheterization findings and surgical results of patients with congenitally corrected transposition of great arteries: A single center experience (original) (raw)
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Long-term outcome in congenitally corrected transposition of the great arteries
Journal of the American College of Cardiology, 2000
The purpose of this study was to determine long-term outcome in adults with congenitally corrected transposition of the great arteries (CCTGA), with particular emphasis on systemic ventricular dysfunction and congestive heart failure (CHF). BACKGROUND Patients with CCTGA have the anatomical right ventricle as their systemic pumping chamber, with ventricular dysfunction and CHF being relatively common in older adults.
Outcome of 121 Patients with Congenitally Corrected Transpositionof the Great Arteries
Pediatric Cardiology, 2002
Congenitally corrected transposition of the great arteries (ccTGA) is a rare disorder with reduced survival that is in¯uenced by the presence of associated anomalies, tricuspid regurgitation (TR), and right ventricular (RV) function. The double switch procedure has been proposed as an aggressive surgical approach in selected patients. We sought to review our experience with conventional repair to determine if a change in surgical strategy was warranted. Clinical records of 121 patients with ccTGA and two adequate-sized ventricles were retrospectively reviewed. Median length of follow-up was 9.3 years; 5-, 10-, and 20-year survival rates were 92%, 91%, and 75%, respectively. Surgery was performed in 86 patients, including conventional biventricular repair in 47 patients. Risk factors for mortality by univariate analysis included age at biventricular repair (p = 0.04), complete atrioventricular (AV) canal defect (p = 0.02), dextrocardia (p = 0.05), moderate or severe TR (p = 0.05), and poor RV function (p = 0.001). By multivariate analysis, complete AV canal defect (p = 0.006) and poor RV function (p = 0.002) remained signi®cant as risk factors for mortality. Risk factors for the development of signi®cant TR included conventional biventricular repair (p = 0.03) and complete AV block (p = 0.04). Risk factors for progressive RV dysfunction included conventional biventricular repair (p = 0.02), complete AV block (p = 0.001), and moderate or severe TR (p < 0.001). This is the largest nonselected cohort of patients with ccTGA followed at a single center. Our results con-®rm that signi®cant TR and poor RV function are risk factors for poor outcome and provide convincing evidence that patients undergoing conventional biventricular repair are at higher risk for deterioration of tricuspid valve and right ventricular function compared to palliated or unoperated patients. We support a move toward an alternative surgical approach (double switch procedure) in carefully selected patients.
Eplasty, 2013
Congenitally corrected transposition of great arteries (CCTGA) is characterized by atrioventricular and ventriculoarterial discordance. Characterizations of these anomalies are important because they may influence surgical approach and management. We present a case of newly diagnosed CCTGA at the age of 50. He presented with sudden onset of shortness of breath for the first time and was diagnosed with CCTGA. Echocardiogram, magnetic resonance imaging, and cardiac catheterization were utilized to elucidate the pathology. Intraoperatively, patient's CCTGA and ventricularization of the right ventricle were confirmed. The severe systemic atrioventricular valve regurgitation was replaced with a bioprosthetic valve (Medtronic Mosaic No. 29) with placement of epicardial ventricular leads for possible future placement of automatic implantable cardioverter defibrillators. Pathology report confirmed a degeneration of the systemic atrioventricular valve. Significant coronary artery anomali...
The Heart Surgery Forum
Background: Congenitally corrected transposition of the great arteries (ccTGA) is a rare condition that accounts for just 1% of all congenital heart disease. Diagnosis of ccTGA often is missed in adulthood, despite imaging and cardiology consultation. Case report: We present the case of an intraoperatively diagnosed ccTGA with severe tricuspid valve regurgitation and secundum atrial septal defect in a 54-year-old woman, who preoperatively was diagnosed with mitral valve regurgitation in atrioventricular and ventriculoarterial concordance heart. Intraoperatively, options considered were anatomical repair with atrial-arterial double switch operation after retraining the left ventricle or a conventional repair that focused on the associated defects without addressing the discordant connections. Considering our patient’s age and condition, we decided to carry on with the conventional repair to prevent further systemic right ventricle dysfunction that may lead to poor outcome and decreas...
Congenitally corrected transposition of the great arteries: the systemic right ventricle
Progress in Pediatric Cardiology, 1999
L-Transposition of the great arteries (L-TGA) is characterized by atrioventricular and ventriculoarterial discordance such that the morphologic right ventricle and tricuspid valve directly provide systemic cardiac output. The ability of the right ventricle to support the systemic circulation is often complicated by associated cardiac defects which provide numerous potential mechanisms for the development of ventricular dysfunction. Theoretical risk factors for
Journal of the American College of Cardiology, 2002
The goal of this study was to determine the presentation and outcome of the unoperated adult with congenitally corrected transposition of the great arteries. The presentation of this disorder and the outcome in unoperated adults have not been well defined. All unoperated patients &amp;amp;amp;amp;amp;gt; or =18 years old were evaluated for spectrum of disease, hemodynamic severity, timeliness of diagnosis and referral, and outcome. Forty-four patients aged 20 to 79 years (mean, 44) were followed up to 144 months. In 29 (66%), the correct diagnosis was first made at age &amp;amp;amp;amp;amp;gt; or =18 years; the diagnosis was missed in seven of these patients in a prior cardiology consultation, despite cardiac imaging. Systemic atrioventricular valve (SAVV) regurgitation (grade &amp;amp;amp;amp;amp;gt; or =3/4) was noted in 26 patients (59%). Thirty (68%) had surgical intervention, including SAVV replacement in all, with no early mortality. Preoperatively, this subset had significant dysfunction of the systemic ventricle (SV) (ejection fraction [EF], 40 +/- 10%), and most had advanced symptoms (25 with ability index &amp;amp;amp;amp;amp;gt; or =2/4). In 16 (53%), SAVV regurgitation &amp;amp;amp;amp;amp;gt; or =3/4 and ventricular dysfunction had been documented for &amp;amp;amp;amp;amp;gt;6 months. The mean EF of the SV decreased significantly postoperatively (34 +/- 11%, p = 0.006). Four patients (13%) eventually required cardiac transplantation. Poor preoperative EF of the SV predicted eventual need for transplantation (p = 0.001). Patients with unoperated congenitally corrected transposition of the great arteries are often misdiagnosed in adulthood and are referred late despite symptomatic SAVV regurgitation and significant SV dysfunction. Although excellent early surgical results can be achieved, significant residual dysfunction of the SV is common.