Role of Echocardiography for Assessment of Cardiac Remodeling in Congenitally Corrected Transposition of Great Arteries.

BACKGROUND: The purpose of this study was to assess the role of echocardiography for a comprehensive assessment of cardiac remodeling, and the relationship between indices of cardiac remodeling and cardiovascular events (defined as the composite end point of heart failure hospitalization, heart transplant, or cardiovascular death) in adults with congenitally corrected transposition of great arteries (cc-TGA). METHODS: This is a retrospective study of adults with cc-TGA who underwent echocardiogram (2003-2020). Offline image analysis was performed in all patients. Chamber (atrial and ventricular) function and size were assessed by strain imaging and 2-dimensional echocardiography. RESULTS: Of 233 patients with cc-TGA (40±15 years), 123 (55%) had at least one cardiac procedure before baseline echocardiogram. Of 233 patients, 76% and 61% had left atrial dysfunction and systemic right ventricular dysfunction, respectively; while 43% and 11% had right atrial dysfunction and left ventricular dysfunction, respectively. During a median follow-up of 8.9 years, 114 (49%) underwent additional cardiac procedures, and 66 (28%) had cardiovascular events. Left atrial reservoir strain, right ventricular global longitudinal strain, right atrial reservoir strain, left ventricular systolic pressure, and left ventricular global longitudinal strain were independently associated with cardiovascular events. CONCLUSIONS: In addition to the clinical importance of right ventricular systolic dysfunction in cc-TGA that is already well described, the current study demonstrated, for the first time, that biatrial dysfunction was common and was associated with clinical outcomes. Since there are currently no effective therapies for atrial and ventricular dysfunction in patients with cc-TGA, there is a need for research to identify novel strategies to prevent atrial and ventricular dysfunction in this population.

Conventional Repair of an Intraoperatively Diagnosed Congenitally Corrected Transposition of the Great Arteries in an Adult.

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 decreased survival. She was discharged one week after surgery and resumed her normal activity at 3-month follow up. CONCLUSION: Although it rarely happens, CHD such as ccTGA in an adult must always be considered. Careful examination is essential. The treatment of ccTGA in an adult is challenging, with more limited options compared with pediatric patients. However, early management could still provide favorable outcomes.

The Fate of Congenitally Corrected Transposition of the Great Arteries Unoperated Before Adulthood.

BACKGROUND: The outcomes, therapeutic strategies, and risk factors of congenital corrected transposition of great arteries (ccTGA) unoperated before adulthood are unclear. METHODS: From October 2009 to January 2018, 117 adult ccTGA patients, classified into ccTGA with intact ventricular septum, ventricular septum defect, and pulmonary valve or subpulmonary outflow tract stenosis (PS) groups, were reviewed. Statistical analysis was performed with SPSS 19.0 (IBM, Armonk, NY). RESULTS: At the first visit, no patients suffered operation. The PS group had the least systemic atrioventricular valve regurgitation and the greatest systemic ventricular ejection fraction. All 49 patients underwent surgery. From the first visit to last follow-up, systemic ventricular ejection fraction of unoperated ccTGA decreased significantly. In the intact ventricular septum group, patients receiving systemic atrioventricular valve replacement/valvuloplasty had a significantly increased systemic ventricular ejection fraction and statistically more freedom from death and transplant than unoperated. In the ventricular septum defect group the late systemic ventricular ejection fraction of operated patients was not statistically different from their basic data at first visit. In the PS group patients receiving physiologic repair had significantly decreased systemic ventricular ejection fractions. Severe systemic atrioventricular valve regurgitation, physiologic repair, and systemic ventricular dysfunction (ejection fraction <40%) were risk factors for mortality, transplant, and congestive heart failure. CONCLUSIONS: PS protects against systemic atrioventricular valve regurgitation and ventricular dysfunction. Systemic atrioventricular valve replacement/valvuloplasty improved systemic ventricular function for ccTGA with an intact ventricular septum. Physiologic repair was not ideal for ccTGA with PS. Severe systemic atrioventricular valve regurgitation and systemic ventricular dysfunction were associated with suboptimal outcomes.

Global longitudinal strain correlates to systemic right ventricular function.

BACKGROUND: The aim of this retrospective study was to evaluate the relationship between right ventricular function derived from cardiac magnetic resonance imaging (CMR), echocardiography and exercise stress test performance, NT-proBNP (N-terminal proB-type natriuretic peptide) level and NYHA class in patients with a systemic right ventricle. METHODS: All patients with congenitally corrected transposition of the great arteries (ccTGA), or transposition of the great arteries after Mustard or Senning procedures, (TGA) followed at our centre who had undergone CMR, echocardiography, an exercise stress test and blood sampling, were included in the study. RESULTS: We examined 11 patients (six after the Senning procedure, one after the Mustard procedure, and four ccTGA) who have a median age of 32 years (22-67 years). A significant correlation was observed between the systemic ventricular function, expressed as the CMR-derived right ventricular ejection fraction and the right ventricular global longitudinal strain (r= -0.627; p=0.039). CONCLUSION: We have demonstrated that in patients with ccTGA or TGA right ventricular global longitudinal strain may be useful in the evaluation of the systemic right ventricular function.

Pulmonary Artery Banding for Congenitally Corrected Transposition of the Great Arteries With Hydrops Fetalis: A Case Report.

At 32 weeks of gestation, a male fetus with congenitally corrected transposition of the great arteries developed hydrops fetalis caused by a combination of mitral valve regurgitation and tricuspid valve regurgitation (TR). We performed a pulmonary artery banding (PAB) at 108 days old for gradually progressing TR, after confirming that a balloon dilatation test in the main pulmonary artery reduced TR. As the patient grew, the PAB became tighter and systolic blood pressure in the morphological left ventricle increased. At present, the patient is waiting for a double switch operation.

Double Outlet Right Ventricle With Right-Sided Aorta From the Left-Sided Morphologically Right Ventricle in the Setting of Discordant Atrioventricular Connections.

We describe the anatomic findings in a 2-year-old patient with double outlet right ventricle with right-sided aorta in the setting of usual atrial arrangement and discordant atrioventricular connections, making comparison with a specimen from the pathological archive of the Birmingham Children's Hospital in the United Kingdom having this rare combination of anatomic features. We discuss the challenges involved in diagnosis and management.