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.

Melody® pulmonary valve implantation in two teenage patients with congenitally corrected transposition of the great arteries status after Senning atrial switch operation

2016 ◽  
Vol 27 (3) ◽  
pp. 600-604
Author(s):  
Rodrigo Rios ◽  
Susan R. Foerster ◽  
Todd M. Gudausky
Keyword(s):  
Pulmonary Artery ◽  
Right Ventricle ◽  
Switch Operation ◽  
Atrial Switch ◽  
Atrial Switch Operation ◽  
The Right ◽  
Valve Implantation ◽  
Congenitally Corrected Transposition ◽  
Artery Conduit ◽  
Corrected Transposition

AbstractThe Melody® transcatheter pulmonary valve system was developed for placement within right ventricle-to-pulmonary artery conduits in patients with CHD for treatment of stenosis or regurgitation, providing an alternative to open-heart surgery. Abnormal systemic venous connections altering the catheter course to the right ventricle-to-pulmonary artery conduit may present a challenge to Melody® valve implantation. We present two such cases, in which the Melody® valve was successfully implanted in teenage patients with congenitally corrected transposition of the great arteries after Senning atrial switch operation. Despite the abnormal catheter course, the right ventricle-to-pulmonary artery was approachable via the right femoral vein allowing for deployment of the Melody® valve in the appropriate position. This suggests that systemic vein-to-left atrium baffles are not prohibitive of Melody® valve implantation. This is an important implication considering the substantial population of ageing patients with CHD who have undergone atrial switch. Melody® valve implantation can be considered as a viable option for treatment of these patients if they develop right ventricle-to-pulmonary artery conduit failure.

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.

Management of Congenitally Corrected Transposition of the Great Arteries With Intact Ventricular Septum: Anatomic Repair or Palliative Treatment?

2021 ◽  
Author(s):  
Hujun Cui ◽  
Ali Hage ◽  
Breanna L. Piekarski ◽  
Gerald R. Marx ◽  
Christopher W. Baird ◽  
...  
Keyword(s):  
Left Ventricle ◽  
Right Ventricle ◽  
Ventricular Dysfunction ◽  
Ventricular Septum ◽  
Composite Outcome ◽  
Intact Ventricular Septum ◽  
Single Institution ◽  
Anatomic Repair ◽  
Congenitally Corrected Transposition ◽  
Corrected Transposition

Background: Management of the congenitally corrected transposition of the great arteries (ccTGA) with intact ventricular septum (IVS) is controversial and includes either anatomic repair with systemic left ventricle (morphological left ventricle) or maintenance of a systemic right ventricle (morphological right ventricle). The purpose of this study was to describe outcomes of ccTGA-IVS with either strategy. Methods: A single-institution, retrospective review of all patients with a diagnosis of ccTGA-IVS who either underwent double switch operation (DSO) or managed with systemic morphological right ventricle (no-DSO). The composite end points included death, heart transplantation, or development of significant systemic ventricular dysfunction or atrioventricular valve regurgitation. Results: Between January 1980 and October 2019, 128 patients with ccTGA-IVS underwent primary treatment at a single institution (91 no-DSO and 37 DSO). Mean age at initial presentation was younger in the DSO compared with the no-DSO cohort (2.1 versus 18.2 years; P <0.001). Fifty patients underwent pulmonary artery banding (35 DSO and 15 no-DSO). In the no-DSO cohort, the overall mortality was 5.5% (n=5), 17.6% (n=16) developed morphological right ventricular dysfunction, and 40.7% (n=37) required pacemaker. In the DSO cohort, mortality was 2.7% (n=1), 8.1% (n=3) developed morphological left ventricular dysfunction, and 43.6% (n=16) required pacemaker. Freedom from composite end point at 5 and 10 years was 88% and 87% in the no-DSO compared with 94% and 84% in the DSO cohort. The DSO cohort had 0.29× the hazards of developing the composite outcome compared with the no-DSO cohort (hazard ratio, 0.29 [95% CI, 0.089–0.94]; P =0.039). Among patients with initial age at presentation ≤5 years, DSO was associated with 0.16× the hazard compared with no-DSO (hazard ratio, 0.16 [95% CI, 0.04–0.62]; P =0.008). Pacemaker implantation was not associated with increased hazard of the composite outcome. Conclusions: Systemic ventricular dysfunction is a late complication of ccTGA-IVS regardless of the strategy pursued. Compared with those undergoing anatomic repair, patients with systemic morphological right ventricle may have lower long-term freedom from cardiovascular complications.

Diffuse fibrosis is common in the left, but not in the right ventricle in patients with transposition of the great arteries late after atrial switch operation

2018 ◽  
Vol 34 (8) ◽  
pp. 1241-1248 ◽  
Author(s):  
Nerejda Shehu ◽  
Christian Meierhofer ◽  
Daniel Messroghli ◽  
Naira Mkrtchyan ◽  
Stefan Martinoff ◽  
...  
Keyword(s):  
Right Ventricle ◽  
Diffuse Fibrosis ◽  
Switch Operation ◽  
Atrial Switch ◽  
Atrial Switch Operation ◽  
The Right
Sign in / Sign up

Export Citation Format

Share Document