Long-term function of the morphologic right ventricle in adult patients with corrected transposition of the great arteries

doi:10.1016/0002-8703(89)90268-8

American Heart Journal

Volume 118, Issue 3, September 1989, Pages 526-530

https://doi.org/10.1016/0002-8703(89)90268-8 Get rights and content

Abstract

Because of the concern about the ability of the morphologic right ventricle (MRV) to function over a long term as a systemic ventricle, adult patients with congenitally corrected transposition of the great arteries (CCTGA) were evaluated to determine the long-term function of the MRV. Morphologic right ventricular function was assessed by functional clinical classification and angiographic ejection fraction in 18 adult patients with congenitally corrected transposition of the great arteries. These patients had a mean age of 30.2 ± 14.5 years (range 10 to 67 years). All but one had hemodynamically significant lesions, the most common being left atrioventricular valvo regurgitation (11 patients), ventricular septal defect (seven patients), atrial septal defect (four patients), and pulmonic stenosis (three patients). The mean MRV ejection fraction at presentation was 55% ± 11.5% (range 24% to 74%). Twelve of the 18 patients (67%) were followed clinically, with a mean follow-up time of 9.9 ± 7.1 years (range 1 to 22 years). Eight were reassessed angiographically, with a mean MRV ejection fraction of 51.3% ± 10.7% (range 30% to 67%). The other four were followed up clinically and evaluated by two-dimensional echocardiography, with normal MRV function in two patients. Eight of 12 patients (67%) were in functional class I at follow-up, one was in functional class II, one was in functional class III, and two had died. Our data suggest that the morphologic right ventricle can function appropriately over a long term in adult patients with congenitally corrected transposition of the great arteries. This raises the question of whether other physiologic factors or management issues may be the causes of poor right ventricular function in some patients with dextrotransposition of the great arteries.

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Long-term outcome of patients with transposition of the great arteries and a systemic right ventricle: A systematic review and meta-analysis
2023, International Journal of Cardiology
Patients with a transposition of the great arteries (TGA) and a systemic right ventricle are at risk of heart failure (HF) development, arrhythmia and early mortality. Prognostic evaluations in clinical studies are hampered by small sample sizes and single-centred approaches. We aimed to investigate yearly rate of outcome and factors affecting it.
A systematic literature search of four electronic databases (PubMed, EMBASE, Web of Science and Scopus) was conducted from inception to June 2022. Studies reporting the association of a systemic right ventricle with mortality with a minimal follow-up of 2 years during adulthood were selected. Incidence of HF hospitalization and/or arrhythmia were captured as additional endpoints. For each outcome, a summary effect estimate was calculated.
From a total of 3891 identified records, 56 studies met the selection criteria. These studies described the follow-up (on average 7.27 years) of 5358 systemic right ventricle patients. The mortality incidence was 1.3 (1–1.7) per 100 patients/year. The incidence of HF hospitalization was 2.6 (1.9–3.7) per 100 patients/year. Predictors of poor outcome were a lower left ventricular (LV) and right ventricular ejection fraction (RVEF) (standardized mean differences (SMD) of −0.43 (−0.77 to −0.09) and − 0.85 (−1.35 to −0.35), respectively), higher plasma concentrations of NT-proBNP (SMD of 1.24 (0.49–1.99)), and NYHA class ≥2 (risk ratio of 2.17 (1.40–3.35)).
TGA patients with a systemic right ventricle have increased incidence of mortality and HF hospitalization. A lower LVEF and RVEF, higher levels of NT-proBNP and NYHA class ≥2 are associated with poor outcome.
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
Subaortic right ventricular characteristics and relationship to exercise capacity in congenitally corrected transposition of the great arteries
2012, Journal of the American Society of Echocardiography
In congenitally corrected transposition of the great arteries (cc-TGA), the morphologic right ventricle acts as the subaortic (systemic) ventricle, and deterioration of the ventricle over time is a well-known complication. The objective of this study was to characterize the right ventricle and explore factors that may be contributing to ventricular dilation and dysfunction and the relationship with exercise capacity.
This was a prospective study of adults with cc-TGA. All patients underwent cardiopulmonary stress testing, ventricular volume and fibrosis assessment by cardiac magnetic resonance imaging, and Velocity Vector Imaging strain echocardiography.
Twenty-six patients were included (mean age, 38 ± 16 years; 54% women). Exercise capacity was significantly reduced in patients with cc-TGA compared with normal subjects (20.9 ± 6.0 vs 30.8 ± 9.2 mL/kg/min, P = .001). The majority of patients (61%) had right ventricular (RV) ejection fractions ≤ 40%. There was no evidence of fibrosis on cardiac magnetic resonance imaging. There was a significant difference in diastolic volumes among those with RV ejection fractions > 40% versus ≤ 40% (173 ± 29mL vs 233 ± 65 mL, P = .02) and moderate or severe versus no or mild tricuspid regurgitation (240 ± 80mL vs 190 ± 38mL, P = .04). RV apical longitudinal and mid free wall circumferential strain was decreased compared with these values in controls.
In this relatively “well” cc-TGA population, subaortic RV dilation, dysfunction, and exercise intolerance are a common problem. Significant systemic tricuspid atrioventricular valvular regurgitation is an important contributor to the problem. In this study, subaortic RV myocardial deformation parameters were found to be abnormal, suggesting that there is a failure of the ventricle to adapt to systemic pressures, and therapies to reduce afterload should be explored. Recurrent ischemia resulting in fibrosis likely does not contribute to RV dilation or dysfunction as demonstrated by the magnetic resonance imaging findings in this study.
Congenitally corrected transposition of the great arteries: Ventricular function at the time of systemic atrioventricular valve replacement predicts long-term ventricular function
2011, Journal of the American College of Cardiology
Citation Excerpt :
A visual estimate of SVEF relies heavily on experience and may in fact reflect a qualitative appreciation. Currently used normal values for SVEF are derived from small studies using radionuclide ventriculography (12,21). Angiography has also been used (18).
The objective was to evaluate the systemic ventricular ejection fraction (SVEF) at the time of systemic atrioventricular valve (SAVV) replacement as a predictor of SVEF ≥1 year after surgery in patients with congenitally corrected transposition of the great arteries (CCTGA).
Progressive SAVV regurgitation causes systemic ventricular failure in CCTGA patients, who are commonly referred late for intervention. Survival after surgery is poor when the pre-operative SVEF is <44%.
We retrospectively reviewed 46 patients (pre-operative SVEF ≥40% in 27 patients and <40% in 19 patients) with 2 good-sized ventricles, a morphologically right systemic ventricle, and SAVV regurgitation requiring surgery. Median follow-up was not different in patients with a pre-operative SVEF ≥40% (8.8 years) or <40% (7.7 years, p = 0.36).
Pre-operative SVEF was the only independent predictor of ≥1-year post-operative SVEF (p < 0.0001). The late SVEF was preserved (defined as ≥40%) in 63% of patients who underwent surgery with an SVEF ≥40% compared with 10.5% of patients who underwent surgery with an SVEF <40%. Pre-operative variables associated with late mortality were an SVEF ≤40%, a subpulmonary ventricular systolic pressure ≥50 mm Hg, atrial fibrillation, and New York Heart Association functional class III to IV.
Post-operative systemic ventricular function after SAVV replacement can be predicted from the pre-operative SVEF. For best results, operation should be considered at an earlier stage, before the SVEF falls below 40% and the subpulmonary ventricular systolic pressure rises above 50 mm Hg.
Is the Morphologic Mitral Valve in Discordant Atrioventricular Connections Always Normal?
2008, Annals of Thoracic Surgery
Citation Excerpt :
Enthusiasm for anatomic biventricular repair would better be reconsidered, and the surgeon should carefully choose the optimal candidates for such an extensive procedure. Systemic right ventricular dysfunction [17] and tricuspid valve regurgitation can progress after functional biventricular repair, whereas channel obstructions and abnormal mitral valve can pose potential problems, together with late atrial arrhythmia related to the surgical procedure for intraatrial redirection of blood, after the anatomic repair. The associated structural abnormalities in the coronary arterial circulation [18] are another factor to be taken into account.
The purpose of this study was to determine structural features and variations in the mitral valve guarding inflow of the morphologic left ventricle in hearts with discordant atrioventricular connections.
Morphologic investigation was carried out on the inlet valve to the morphologic left ventricle in 48 autopsied specimens with this particular entity of malformation.
The tension apparatus was straddling to the morphologic right ventricle in 2. Another 34 hearts had a bifoliate valve and the papillary muscles abnormally oriented; a solitary papillary muscle in 3, two papillary muscles but deviated in 5, one of two papillary muscles being dysmorphic in 9, and three or more papillary muscles in 17. The origin of these papillary muscles was frequently deviated. In 5 hearts with a trifoliate or quadrifoliate valve, multiple papillary muscles were also the case. One of these had thick leaflets, a part of which was adherent to a membranous flap around the ventricular septal defect as well as to subpulmonary fibrous tissue tags. Eventually, the valve was comparable with the mitral valve seen in the normally structured heart in only 7 hearts (15%). There was no obvious correlation noted between the presence of abnormality in the mitral valve and that in the tricuspid valve.
Architectural abnormalities are not rare in the morphologic mitral valve in this setting. The valvar structure needs precise recognition.

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Long-term function of the morphologic right ventricle in adult patients with corrected transposition of the great arteries

Abstract

Am J Cardiol

Am J Cardiol

Am J Cardiol

Am J Cardiol

J Thorac Cardiovasc Surg

Die Defecte der Scheidewande des Herzens