Comparison of Outcomes in Adults With Congenitally Corrected Transposition With Situs Inversus Versus Situs Solitus

doi:10.1016/j.amjcard.2012.07.039

The American Journal of Cardiology

Volume 110, Issue 11, 1 December 2012, Pages 1687-1691

https://doi.org/10.1016/j.amjcard.2012.07.039 Get rights and content

The long-term outcome of patients with congenitally corrected transposition of the great arteries is mainly determined by progressive morphologically tricuspid valve regurgitation, heart block, atrial arrhythmias, and/or systemic ventricular dysfunction. Situs abnormalities have been reported in ≤34% of cases, but whether clinical differences exist between a situs inversus and situs solitus arrangement has not yet been studied. The clinical records of 38 adults with congenitally corrected transposition of the great arteries (mean age 40 ± 15 years) followed for a mean period of 7.4 years were reviewed. Of these 38 patients, 8 presented with situs inversus and 30 with situs solitus. No significant differences were found between the 2 groups in age, gender, ventricular septal defect, pulmonary tract stenosis, previous surgical repair, or duration of follow-up. However, none of the patients with situs inversus presented with an Ebstein-like anomaly of the morphologically tricuspid valve and none developed nonoperative-related complete atrioventricular block compared to 15 (50%; p = 0.013) and 11 (42%; p = 0.032) of the patients with situs solitus, respectively. At follow-up, 2 patients with situs inversus (25%) presented with sustained atrial arrhythmia, severe tricuspid regurgitation, or severe systemic right ventricular systolic dysfunction compared to 22 (73%) of 30 those with situs solitus (p = 0.034). No patient with situs inversus presented with cardiac death or severe heart failure compared to 12 (40%) of 30 with situs solitus (p = 0.038). In conclusion, Ebstein-like anomaly or spontaneous complete atrioventricular block are rare in patients with congenitally corrected transposition of the great arteries with situs inversus, and late complications are uncommon. The long-term outcome of patients with situs inversus was significantly better than that for patients with situs solitus.

Section snippets

Methods

The clinical records of all adult patients aged >18 years, diagnosed with ccTGA, and referred to the Adult Congenital Heart Disease unit at La Paz University Hospital (Madrid, Spain) from January 1990 to January 2012 were reviewed. The diagnosis of ccTGA, regardless of the situs visceroatrial or ventricular looping, was determined by demonstrating discordance of both the AV and the ventriculoarterial connections using 2-dimensional echocardiography (2DE) or cardiac magnetic resonance imaging

Results

A total of 38 patients diagnosed with ccTGA and with potentially suitable anatomy for biventricular repair in the presence of balanced ventricles constituted the study population (Table 1). The mean age at the last examination was 40 ± 15 years and 21 were men (55%). Associated anomalies, including VSD, pulmonary stenosis or atresia, complete AV block, or Ebstein-like anomaly, were present in 33 patients (87%). Surgical repair of associated intracardiac lesions had been performed in 13 patients.

Discussion

To the best of our knowledge, this is the first study to evaluate the long-term outcome of adults with ccTGA with situs inversus. Our results have shown that this anomaly should not be regarded as a mirror image of ccTGA with situs solitus because of the important anatomic and physiologic dissimilitude between both types of atrial arrangements. In contrast to what happens in situs solitus, spontaneous AV block or Ebstein-like anomaly of the morphologically tricuspid valve is very uncommon in

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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
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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.
Protective Factors That Maintain Asymptomatic Longevity in Untreated Congenitally Corrected Transposition of Great Arteries
2022, CJC Open
Management Options for Congenitally Corrected Transposition: Which, When, and for Whom?
2022, Seminars in Thoracic and Cardiovascular Surgery: Pediatric Cardiac Surgery Annual
Citation Excerpt :
These patients may have a sling-like conduction system around the outflow tract and even two AV nodes.68 Interestingly, in the setting of situs inversus (IDD) the atrial septum is more aligned with the ventricular septum, thus, the AV node is located in its normal position and these patients are spared from spontaneous CHB.4,68,69 Of note, patients with IDD have superior long-term transplant-free survival compared to SLL, once again highlighting the impact of maintaining normal conduction in these patients.69
Management strategies for congenitally corrected transposition of the great arteries (ccTGA) historically consisted of a physiologic repair, resulting in the morphologic right ventricle (mRV) supporting systemic circulation. This strategy persisted despite the development of heart failure by middle age because of the reasonable short-term outcomes, and the natural history of some patients with favorable anatomy (felt to demonstrate the mRV's ability to function in the long-term), and due to the less-than-optimal outcomes associated with anatomical repair. As outcomes with anatomical repair improved, and the long-term risk of systemic mRV dysfunction became apparent, more have begun to realize its advantages. In addition to the decision on whether or not to pursue anatomical repair, and the optimal timing, studies demonstrating the nuance to morphologic left ventricle retraining have demonstrated its feasibility. Further considerations in ccTGA have begun to be better understood, including: the management of a poorly functioning mRV, systemic tricuspid valve regurgitation, the utility of morphologic left ventricle outflow tract obstruction (native or surgically created) and pacing strategies. While some considerations are apparent: biventricular pacing is superior to univentricular, tricuspid regurgitation must be managed early with either progression towards anatomical repair (pulmonary artery banding if needed for retraining) or tricuspid replacement (not repair) based on the patient's age; others remain to be completely elucidated. Overall, the heterogeneity of ccTGA, as well as the unique presentation with each patient regarding ventricular and valvular function and center-to-center variability in management strategies has made the interpretation of published data difficult. That said, more recent long-term outcomes favor anatomical repair in most situations.
Long-term outcomes of arrhythmia and distinct electrophysiological features in congenitally corrected transposition of the great arteries in an Asian cohort: Arrhythmia in ccTGA
2021, American Heart Journal
Citation Excerpt :
The incidence of cAVB has been reported in the range of 22% to 48% in other studies, which were mostly in the Western countries (Table III).4,7,9,18-20 Additionally, these studies have reported that the major risk factor for the development of cAVB is an intact ventricular septum and that patients with mirror-image atrial arrangement might have lower cAVB incidence.18,20 Simmons et al reported that ccTGA with single- and dual-ventricle physiology had similar incidence rates as cAVB after excluding surgical history.4
Congenitally corrected transposition of the great arteries (ccTGA) is associated with various types of arrhythmia, including supraventricular tachycardia (SVT) and complete atrioventricular block (cAVB). Our study aims to characterize the arrhythmia burden, associated risk factors, arrhythmia mechanisms, and the long-term follow-up results in patients with ccTGA in a large Asian cohort.
We enrolled 104 patients (43 women and 61 men) diagnosed with ccTGA at our institution. The mean age at last follow-up was 20.8 years.
For 40 patients (38%) with tachyarrhythmia, paroxysmal SVT (PSVT) and atrial arrhythmia were observed in 17 (16%) and 27 (26%) patients, respectively, with 4 patients (4%) having both types of SVT. The 20-year and 30-year SVT-free survival rates were 68% and 54%, respectively. Seven patients (7%) developed cAVB: 2 (2%) developed spontaneously, and the other 5 (5%) was surgically complicated (surgical risk of cAVB: 7%, all associated with ventricular septal defect repair surgery). PSVT was mostly associated with accessory pathways (5/9) but also related to twin atrioventricular nodal reentry tachycardia (3/9) and atrioventricular nodal reentry tachycardia (1/9). Most of the accessory pathways were located at tricuspid valve (9/10). Catheter ablation successfully eliminated all PSVT substrates (10/10) and most of the atrial arrhythmia substrates (3/5), with low recurrence rate.
The arrhythmia burden in patients with ccTGA is high and increases over time. However, cAVB incidence was relatively low and kept stationary in this Asian cohort. The mechanisms of SVT are complicated and can be controlled through catheter ablation.
Congenitally Corrected Transposition of the Great Arteries at Age 88 Years
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Most patients with congenital heart disease are diagnosed shortly after birth; however, some patients are overlooked and diagnosed in adulthood. We present the case of a man in whom congenitally corrected transposition of the great arteries (CCTGA) was first diagnosed at the age of 88 years. CCTGA is a rare disease; patients without frequently associated cardiac anomalies are considered to have long life expectancies; however, their prognosis remains unclear. This case is the oldest patient at the time of CCTGA diagnosis in the literature, which may provide new insights for CCTGA without frequently associated cardiac anomalies.
La plupart des cardiopathies congénitales sont diagnostiquées peu après la naissance; certains cas passent toutefois inaperçus et sont diagnostiqués une fois le patient arrivé à l’âge adulte. Nous présentons le cas d’un homme chez qui une transposition congénitalement corrigée des gros vaisseaux (TCCGV) a été diagnostiquée alors qu’il avait 88 ans. La TCCGV est une maladie rare; les patients qui ne présentent pas aussi l’une ou l’autre des anomalies cardiaques qui y sont fréquemment associées ont généralement une bonne espérance de vie, bien que le pronostic demeure obscur. De tous les cas rapportés dans la littérature médicale, le patient qui est présenté ici est le plus âgé qui ait jamais reçu un diagnostic de TCCGV, ce qui pourrait éclairer sous un jour nouveau la TCCGV non accompagnée des autres anomalies cardiaques qui y sont fréquemment associées.
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2020, Revista Portuguesa de Cardiologia

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Congenital heart diseaseComparison of Outcomes in Adults With Congenitally Corrected Transposition With Situs Inversus Versus Situs Solitus

Section snippets

Methods

Results

Discussion

Am J Cardiol

J Am Coll Cardiol

Chest

J Am Coll Cardiol

Am J Cardiol

J Am Coll Cardiol

J Thorac Cardiovasc Surg

Lancet

Ann Thorac Surg

Ann Thorac Surg

Am J Cardiol

Am J Cardiol

J Am Coll Cardiol

Am J Cardiol

Die Defekte der Scheidewande des Herzens:Pathologisch-anatomische Abhandlung

Congenital heart disease
Comparison of Outcomes in Adults With Congenitally Corrected Transposition With Situs Inversus Versus Situs Solitus