Assessment of ventricular size and function in congenitally corrected transposition of the great arteries

doi:10.1016/S0002-9149(83)80043-5

The American Journal of Cardiology

Volume 51, Issue 2, 15 January 1983, Pages 244-251

https://doi.org/10.1016/S0002-9149(83)80043-5 Get rights and content

Twenty-four quantitative cineangiographic studies were performed in 19 patients with congenitally corrected transposition of the great arteries to assess right and left ventricular size and function. Ages ranged from 7 days to 44 years and associated lesions included ventricular septal defect (13 of 19), pulmonary stenosis (9 of 19), and systemic (tricuspid) valvular insufficiency (7 of 19). Systemic (anatomically right) ventricular end-diastolic volume was within normal limits in most patients and averaged 119% of predicted normal. Pulmonary (anatomically left) ventricular end-diastolic volume also was normal in most patients, averaged 112% of predicted, and was not different from systemic (right) ventricular end-diastolic volume. Systemic ventricular ejection fraction (RVEF) averaged 0.61 ± 0.02 and was not different from pulmonary ventricular ejection fraction (LVEF) (0.65 ± 0.02), but important differences were apparent when age was considered. With exclusion of 2 patients with hypoplastic systemic ventricles and 2 studies performed <6 months after open heart surgery, all 12 patients aged <10 years had a normal RVEF, whereas 2 of 5 patients aged >17 years had a definitely low RVEF and 1 of 5 had a value at the lower limit of normal. In children, systemic and pulmonary ventricular pump function is usually normal in congenitally corrected transposition of the great arteries and any deviation from normal should suggest ventricular hypoplasia or an increase in afterload. After childhood, systemic ventricular dysfunction is more common and may reflect the inability of the anatomic right ventricle to function as the systemic pumping chamber over a normal lifetime in most patients with congenitally corrected transposition of the great arteries.

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Cited by (102)

Determinants of Aerobic Capacity After Tricuspid Valve Replacement in Congenitally Corrected Transposition of Great Arteries
2022, JACC: Advances
Systemic tricuspid regurgitation and impaired aerobic capacity are common in adults with congenitally corrected transposition of great arteries (cc-TGA), but it is unknown whether systemic tricuspid valve replacement (sTVR) is associated with improvement in aerobic capacity.
The purpose of this study was to determine whether sTVR was associated with postoperative improvement in aerobic capacity as measured by peak oxygen consumption (VO₂).
This is a retrospective cohort study of adults with cc-TGA and ≥moderate tricuspid regurgitation that underwent sTVR and exercise test pre- and post-sTVR (2003-2019). We selected a propensity-matched control group of patients with cc-TGA and ≥moderate tricuspid regurgitation that did not undergo sTVR between exercise tests. Improvement in aerobic capacity was calculated as the difference between baseline and follow-up exercise tests.
The study comprised 48 patients in each group, and both groups had similar peak VO₂ at baseline. Compared to the baseline exercise test, the sTVR group had more improvement in peak VO₂ (6 ± 4% vs −8 ± 3%-predicted, P = 0.001), and the factors associated with postoperative improvement in peak VO₂ were baseline systolic blood pressure (<120 mm Hg), systemic right ventricular global longitudinal strain (<−16%), and preoperative peak VO₂ (>60%-predicted).
There was an improvement in aerobic capacity after sTVR in patients with cc-TGA and severe tricuspid regurgitation. The determinants of improvement in aerobic capacity were baseline systolic blood pressure, systemic right ventricular global longitudinal strain, and preoperative peak VO₂. Further studies are required to determine whether strict blood pressure control and optimal timing of sTVR based on these indices would result in improved clinical outcomes in this population.
Prognostic Implications of Progressive Systemic Ventricular Dysfunction in Congenitally Corrected Transposition of Great Arteries
2022, JACC: Cardiovascular Imaging
The purpose of this study was to determine the risk factors for and prognostic implications of progressive right ventricular systolic dysfunction (RVD) in adults with congenitally corrected transposition of great arteries.
There are no effective therapies for RVD; hence the need to identify and modify risk factors for progressive RVD.
RV systolic function was assessed by using RV longitudinal strain (RV-LS). The first echocardiogram (baseline echocardiogram) and all subsequent annual echocardiograms performed within 5 years from the baseline echocardiogram were analyzed. Progressive RVD (temporal decline in RV-LS) was assessed as the average annual change in RV-LS within 5 years of imaging follow-up.
Of 186 patients (mean age 40 ± 12 years), the RV-LS at baseline was –17% ± 4%, and the annual decline in RV-LS was –4% (95% CI: –6 to –2). The risk factors for progressive RVD were left ventricular (LV) systolic dysfunction, LV pacing, and systemic hypertension. Cardiovascular events (heart failure hospitalization, heart transplant, and death) occurred in 57 (27%) patients. Progressive RVD was associated with cardiovascular events, independent of RV systolic function at baseline. In subgroup analyses assessing impact of therapies (medical therapy, cardiac resynchronization therapy, and tricuspid valve replacement), only tricuspid valve replacement was associated with improvement in RV systolic function when performed before onset of RVD.
Patients with congenitally corrected transposition of great arteries were at risk for progressive RVD, and the risk factors for progressive RVD were LV pacing, systemic hypertension, and concomitant LV dysfunction. Further studies are required to determine whether strict blood pressure control and early tricuspid valve replacement will prevent progressive RVD.
Right ventricular dysfunction in congenitally corrected transposition of the great arteries and risk of ventricular tachyarrhythmia and sudden death
2018, International Journal of Cardiology
It is unknown whether systemic right ventricular (SRV) dysfunction confers increased risk for sudden death in congenitally corrected transposition of the great arteries (ccTGA). We sought to define risk of ventricular arrhythmias and sudden death attributable to systemic right ventricular dysfunction in ccTGA.
The study cohort is comprised of adult patients with a diagnosis of ccTGA. Clinical information and clinical outcomes including ICD therapy, incidence of VT/VF, and cause of death were reviewed.
129 patients with ccTGA were followed for 7.2 ± 3.4 years. Mean SRV ejection fraction (SRVEF) was 39% (n = 56 with an EF <35%). Significant documented arrhythmia (sustained VT requiring defibrillation or cardiac arrest due to VT/VF) occurred in 13/56 patients (23%) with SRV EF <35% compared with 2/73 (3%) with SRV EF >35% (p < 0.001). There was no significant difference in age at death, death from documented arrhythmia, or death from all cause between patients with SRV EF <35% and >35%. However, risk of sudden death was higher in patients with SRV EF <35% (16% vs 1%, p = 0.002). The combined clinical endpoint of ICD therapy, clinically significant VT/VF, and sudden death was significantly higher in patients with SRV EF <35% than in patients with SRV EF >35%, independent of the presence of LV EF <35% (p < 0.001).
Dysfunction of the systemic RV in ccTGA increases the risk of sudden death and clinically significant ventricular tachyarrhythmias. Further study is needed to determine if ventricular tachyarrhythmias comprise the primary cause of sudden death in these patients and if ICDs offer any significant mortality benefit.
Congenitally corrected transposition of the great arteries
2018, Diagnosis and Management of Adult Congenital Heart Disease: Third Edition
Congenitally Corrected Transposition of the Great Arteries
2017, Diagnosis and Management of Adult Congenital Heart Disease
Anatomic repair for corrected transposition with left ventricular outflow tract obstruction
2013, Annals of Thoracic Surgery
Citation Excerpt :
From the patients’ medical records obtained from our outpatient clinic, including the echocardiography and catheter examination reports, the following variables were evaluated: (1) Survival rate and risk analysis by the Kaplan-Meyer method and Cox proposal hazard model; (2) Reoperation-free rate for the postoperative systemic ventricular outflow tract, right ventricular outflow tract, and pacemaker implantation for developed arrhythmia by the Kaplan-Meyer method; (3) postoperative intervention rate for caval obstruction and risk analysis by the Kaplan-Meyer method and log-rank test; (4) analysis of correlations between preoperative right ventricular volume and postoperative cardiac index ;and right ventricular end-diastolic pressure measured 1 year after the operation; and (5) New York Heart Association (NYHA) functional classification, current medication status, echocardiography findings, and biomarkers for heart failure as late clinical conditions. Right ventricular end diastolic volume was calculated using biplane right ventriculography findings [16, 17]. The VSD size was measured by both preoperative echocardiography and intraoperative direct inspection.
We investigated the long-term outcomes of anatomic repair for congenitally corrected transposition of great arteries (ccTGA) and its variant associated with left ventricular outflow tract obstruction (LVOTO) and ventricular septal defect (VSD).
From 1987 to 2011, 47 patients (27 with pulmonary atresia and 20 with pulmonary stenosis) with ccTGA and its variant associated with LVOTO and VSD underwent anatomic repair. The mean operative age was 5.5 ± 3.7 years old (range, 1.6 to 21.3). The preoperative right ventricular end-diastolic volume was 133% ± 31% (81 to 222) of their normal size. The atrial switch procedure was Mustard in 31 patients and Senning in 16, with the latter used in all from 2002.
The mean follow-up period was 11.6 ± 7.3 years (maximum, 22.7). The VSD was concomitantly enlarged in 4 patients and Damus-Kaye-Stansel (DKS) anastomosis was added in 9 patients with pulmonary stenosis and restrictive VSD. The overall survival rate at 20 years was 70.2% and no mortality has been observed in 21 consecutive patients since 1997. No patient required reoperation for the postoperative systemic ventricular outflow tract obstruction. A surgical heart block developed in 1 patient (2.1%) who underwent concomitant VSD enlargement. None of the patients developed a moderate or greater aortic or neo-aortic regurgitation.
Recent outcomes after anatomic repair for congenitally corrected transposition of great arteries associated with LVOTO and VSD were excellent. For patients with pulmonary stenosis and restrictive VSD, additional DKS anastomosis seems to be an effective approach to avoid postoperative systemic ventricular outflow tract obstruction and surgical heart block.

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^*: This study was supported in part by Grant 1R01 HL-21985-02 RAD of the National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Maryland.

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Assessment of ventricular size and function in congenitally corrected transposition of the great arteries*

Am J Cardiol

Am J Cardiol

Am Heart J

Chest

Am J Cardiol

Am J Cardiol

Hypoplasia of the systemic ventricle in congenitally corrected transposition of the great arteries

J Thorac Cardiovasc Surg

Isolated congenitally corrected transposition of the great arteries

Congenitally corrected transposition: a review of the world literature

Dan Med Bull

Preoperative and postoperative right ventricular function in children with transposition of the great vessels

Circulation

Abnormalities of right ventricular function following Mustard operation for transposition of the great arteries

Circulation

Right and left ventricular function after the Mustard procedure and transposition of the great arteries

Am J Cardiol