Relation Between Right Ventricular Structural Alterations and Markers of Adverse Clinical Outcome in Adults With Systemic Right Ventricle and Either Congenital Complete (After Senning Operation) or Congenitally Corrected Transposition of the Great Arteries

2007 ◽  
Vol 2007 ◽  
pp. 113
Author(s):  
T.P. Graham
Keyword(s):  
Clinical Outcome ◽  
Right Ventricle ◽  
Right Ventricular ◽  
Adverse Clinical Outcome ◽  
Structural Alterations ◽  
Systemic Right Ventricle ◽  
Senning Operation ◽  
Congenitally Corrected Transposition ◽  
Corrected Transposition

The systemic right ventricle in congenitally corrected transposition of the great arteries is different from the right ventricle in dextro-transposition after atrial switch: a cardiac magnetic resonance study

2012 ◽  
Vol 23 (2) ◽  
pp. 239-247 ◽  
Author(s):  
Matthias Grothoff ◽  
Antje Fleischer ◽  
Hashim Abdul-Khaliq ◽  
Janine Hoffmann ◽  
Lukas Lehmkuhl ◽  
...  
Keyword(s):  
Cardiac Magnetic Resonance ◽  
Magnetic Resonance ◽  
Ejection Fraction ◽  
Right Ventricle ◽  
Right Ventricular ◽  
Systemic Right Ventricle ◽  
Atrial Switch ◽  
The Right ◽  
Congenitally Corrected Transposition ◽  
Corrected Transposition

AbstractBackgroundPatients with a congenitally corrected transposition of the great arteries show an increasing incidence of cardiac failure with age. In other systemic right ventricles, such as in dextro-transposition after atrial switch, excessive hypertrophy is a potential risk factor for impaired systolic function. In this trial, we sought to compare systemic function and volumes between patients with congenitally corrected transposition and those with dextro-transposition after atrial switch by using cardiac magnetic resonance imaging.Methods and ResultsA total of 19 patients (nine male) with congenitally corrected transposition and 31 patients (21 male) with dextro-transposition after atrial switch were studied using a 1.5-Tesla scanner. Cine steady-state free-precession sequences in standard orientations were acquired for volumetric and functional imaging. Patient parameters were compared with those of a group of 25 healthy volunteers. Although patients with congenitally corrected transposition were older, they presented with higher right ventricular ejection fractions (p = 0.04) compared with patients with dextro-transposition. Patients with congenitally corrected transposition showed a weak negative correlation between age at examination and systemic ejection fraction (r = −0.18, p = 0.04) but no correlation between right ventricular myocardial mass index and ejection fraction. There was no significant difference in the right ventricular end-diastolic volumes between both patient groups.ConclusionAlthough patients with congenitally corrected transposition had a longer pressure load of the systemic right ventricle, ventricular function was better compared with that in patients with dextro-transposition after atrial switch. The results suggest that the systemic ventricles might have partly different physiologies. One difference could be the post-operative situation after atrial switch, which results in impaired atrial contribution to ventricular filling.

scholarly journals Congenitally Corrected Transposition Presenting in the First Year of Life: Survival and Fate of the Systemic Right Ventricle

2019 ◽  
Vol 10 (1) ◽  
pp. 42-49 ◽  
Author(s):  
Benjamin Smood ◽  
James K. Kirklin ◽  
Jozef Pavnica ◽  
Margaret Tresler ◽  
Walter H. Johnson ◽  
...  
Keyword(s):  
Right Ventricle ◽  
First Year ◽  
Study Cohort ◽  
Surgical Mortality ◽  
Term Survival ◽  
Systemic Right Ventricle ◽  
First Year Of Life ◽  
Congenitally Corrected Transposition ◽  
Corrected Transposition

Introduction: Knowledge gaps exist in the life expectancy and functional outcome of patients with congenitally corrected transposition (ccTGA) presenting early in life, which is relevant in the evaluation of early anatomic repair. Methods: In a single-center analysis, 91 patients with ccTGA were identified over 25 years, of which 31 presented with biventricular anatomy in the first year of life and formed the study cohort. End points for analysis included survival, moderate or worse tricuspid valve regurgitation, and systemic right ventricle (RV) dysfunction. Median follow-up was 4.9 years (range: 7 days to 20 years). Results: Among 31 patients presenting in the first year of life, 9 (29%) never received cardiac surgery, while 22 (71%) underwent 36 cardiac operations. Overall freedom from moderate or severe systemic RV dysfunction was 75% at 10 years. Overall survival was 82% at 10 years. Surgical mortality was 5.6% (2/36). Among survivors with a systemic RV, 23 (100%) of 23 were Ross or NYHA class I or II at last follow-up. Conclusions: Congenitally corrected transposition presenting in the first year of life and maintaining a systemic RV can expect (1) long-term survival of more than 80% at 10 years, (2) low expected surgical mortality (overall 6%), and (3) 75% late freedom from major RV dysfunction at 10 years. Pending multi-institutional analyses, this experience with a systemic RV in ccTGA provides an initial benchmark for comparison when considering early elective anatomic correction.

Late outcome, therapy and systemic ventricular function in patients with a systemic right ventricle: data of the German National Register for Congenital Heart Defects

2021 ◽  
pp. 1-11
Author(s):  
Corinna Lebherz ◽  
Martin Gerhardus ◽  
Astrid Elisabeth Lammers ◽  
Paul Helm ◽  
Oktay Tutarel ◽  
...  
Keyword(s):  
Right Ventricle ◽  
Congenital Heart ◽  
Ventricular Dysfunction ◽  
Atrioventricular Valve ◽  
Systemic Right Ventricle ◽  
Switch Operation ◽  
Atrial Switch ◽  
Atrial Switch Operation ◽  
Congenitally Corrected Transposition ◽  
Corrected Transposition

Abstract Background: Adults with systemic right ventricle have a significant risk for long-term complications such as arrhythmias or heart failure. Methods: A nationwide retrospective study based on the German National Register for Congenital Heart Disease was performed. Patients with transposition of the great arteries after atrial switch operation or congenitally corrected TGA were included. Results: Two hundred and eight-five patients with transposition of the great arteries after atrial switch operation and 95 patients with congenitally corrected transposition of the great arteries were included (mean age 33 years). Systolic function of the systemic ventricle was moderately or severely reduced in 25.5 % after atrial switch operation and in 35.1% in patients with congenitally corrected transposition. Regurgitation of the systemic atrioventricular valve was present in 39.5% and 43.2% of the cases, respectively. A significant percentage of patients also had a history for supraventricular or ventricular arrhythmias. However, polypharmacy of cardiovascular drugs was rare (4.5%) and 38.5 % of the patients did not take any cardiovascular medication. The amount of cardiovascular drugs taken was associated with NYHA class as well as systemic right ventricular dysfunction. Patients with congenitally corrected transposition were more likely to receive pharmacological treatment than patients after atrial switch operation. Conclusion: A significant portion of patients with systemic right ventricle suffer from a relevant systemic ventricular dysfunction, systemic atrioventricular valve regurgitation, and arrhythmias. Despite this, medication for heart failure treatment is not universally used in this cohort. This emphasises the need for randomised trials in patient with systemic right ventricle.

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