scholarly journals Long term follow up after surgery in congenitally corrected transposition of the great arteries with a right ventricle in the systemic circulation

2010 ◽  
Vol 5 (1) ◽  
Author(s):  
Ad J J C Bogers ◽  
Stuart J Head ◽  
Peter L de Jong ◽  
Maarten Witsenburg ◽  
Arie Pieter Kappetein
Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Rebecca R Hartog ◽  
Kimberly J Watkins ◽  
Megan Wilde ◽  
Tiffany R Lim ◽  
Andrew Rodenbarger ◽  
...  

Introduction: Limited data exist on the electrophysiologic outcomes of patients undergoing anatomic repair (AR) for congenitally corrected transposition of the great arteries (ccTGA). AR was defined as an atrial switch procedure plus either arterial switch (ASO) or Rastelli operation. Aims: To report mid and late electrophysiologic outcomes after AR and identify risk factors for those outcomes. Methods: Single center retrospective cohort study of patients undergoing AR between 1993-2017. Data were collected from available records. Transplant-free survival to 1 year post repair was required for inclusion. Standard descriptive statistical analysis and Cox proportional hazards were used. Results: Of 85 patients included, 95% had lesions in addition to ccTGA: most commonly VSD (84%) and pulmonary stenosis or atresia (58%). Median age at AR was 1.5y (IQR 0.9-2.8) with Senning/ASO in 56%, Senning/Rastelli in 38%, and hemi-Senning/Glenn/Rastelli in 6%. During a median follow-up of 10.6y, 45 (53%) patients developed an arrhythmia requiring intervention. Atrial tachycardia (AT) in 27 (32%) or ventricular tachycardia (VT) in 11 (13%) patients required intervention at a median of 7.4y (IQR 1.6-15.3y) and 15.9y (IQR 4.5-17.9) post-AR, respectively. Treatments included chronic medications in 29 (64%), cardioversion in 15 (33%) and catheter ablation in 10 (22%). Median freedom from AT and VT was 17.3y and 25y post-AR, respectively. D-looped ventricles (p=0.03) and multiple operations prior to AR (p=0.02) were associated with increased AT risk; and native pulmonary stenosis with increased VT risk (p=0.01). Those needing heart failure/transplant referral had increased risk of both AT and VT (both p=0.04). Pacemaker was implanted for heart block and/or SND prior to or during AR in 14 (16%), immediately post-op in 9 (11%), and late (median 6y post-AR) in 24 (28%). ICDs were implanted in 5 (6% of cohort), 4 for primary prevention. No patient had an appropriate shock. Conclusions: Anatomic ccTGA repair is associated with significant electrophysiologic morbidity. AT, VT, and SND develop at a similar incidence to that reported for d-TGA patients after atrial switch. The incidence of AV block follows a similar trajectory to that of physiologically palliated ccTGA.


2020 ◽  
Vol 30 (3) ◽  
pp. 409-412
Author(s):  
Murat Surucu ◽  
İlkay Erdoğan ◽  
Birgül Varan ◽  
Murat Özkan ◽  
N. Kürşad Tokel ◽  
...  

AbstractObjective:Double-chambered right ventricle is characterised by division of the outlet portion of the right ventricle by hypertrophy of the septoparietal trabeculations into two parts. We aim to report our experiences regarding the presenting symptoms of double-chambered right ventricle, long-term prognosis, including the recurrence rate and incidence of arrhythmias after surgery.Methods:We retrospectively investigated 89 consecutive patients who were diagnosed to have double-chambered right ventricle and underwent a surgical intervention from 1995 to 2016. The data obtained by echocardiography, cardiac catheterisation, and surgical findings as well as post-operative follow-up, surgical approaches, post-operative morbidity, mortality, and cardiac events were evaluated.Results:Median age at the time of diagnosis was 2 months and mean age at the time of operation was 5.3 years. Concomitant cardiac anomalies were as follows: perimembranous ventricular septal defect (78 patients), atrial septal defect (9 patients), discrete subaortic membrane (32 patients), right aortic arch (3 patients), aortic valve prolapse and/or mild aortic regurgitation (14 patients), and left superior caval vein (2 patients). The mean follow-up period was 4.86 ± 4.6 years. In these patients, mean systolic pressure gradient in the right ventricle by echocardiography before, immediately, and long-term after surgical intervention was 66.3, 11.8, and 10.4 mmHg, respectively. There were no deaths during the long-term follow-up period. Surgical reinterventions were performed for residual ventricular septal defect (2), residual pulmonary stenosis (1), and severe tricuspid insufficiency (1).Conclusion:The surgical outcomes and prognosis of double-chambered right ventricle are favourable, recurrence and fatal arrhythmias are unlikely in long-term follow-up.


2006 ◽  
Vol 16 (S3) ◽  
pp. 85-90 ◽  
Author(s):  
Edward L. Bove ◽  
Richard G. Ohye ◽  
Eric J. Devaney ◽  
Hiromi Kurosawa ◽  
Toshiharu Shin'oka ◽  
...  

The congenital cardiac malformation characterized by discordant connections between the atriums and ventricles, as well as those between the ventricles and the arterial trunks, has been given many names. The terms atrioventricular discordance, l-transposition of the great arteries, ventricular inversion, and congenitally corrected transposition have all been used. Regardless of terminology, this complex congenital anomaly has only recently been studied to analyze the long-term effects of its natural history and outcomes following traditional surgical repair of the associated malformations which serve to uncorrect the circulatory pathways. As more patients survive into adulthood, the effects of this condition are now better understood, and the surgical approaches used in the past are being re-examined in light of longer-term follow up.


2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
U Annone ◽  
P Omede' ◽  
F D'Ascenzo ◽  
A Montefusco ◽  
A Milan ◽  
...  

Abstract Introduction Prognosis in pulmonary hypertension (PH) is strictly linked to right ventricle (RV) failure, which results from uncoupling between RV and the superimposed pressure load; in first phases, coupling between these two actors still be preserved, at the price of augmented right ventricle wall tension (RVWT). Purpose We sought to describe how to estimate RVWT with echocardiography, how it correlates with RV hemodynamics and if it may predict prognosis. Methods A total of 190 patients without overt RV failure, with suspected pulmonary hypertension (PH) to a previous echocardiography, underwent to right heart catheterization (RHC) and nearly-simultaneous echocardiography. We estimated RVWT according to Laplace law (RV length × tricuspid regurgitation peak gradient [TRPG]), in order to predict initial RV stress, and was correlated with RV hemodynamic profile; its potential prognostic impact was tested along with canonical RV function parameters. Results In patients enrolled in our study, RVWT correlated significantly with invasive estimation of right ventricle end diastolic pressure (R 0.343, p<0.001); a significant relationship between RVWT and several hemodynamic variables was observed (mean pulmonary artery pressure, pulmonary artery compliance, transpulmonary gradient, pulmonary vascular resistance, RV telediastolic pressure, right atrial pressure, RV stroke work index; all p<0.001). At a mean follow up of five years and three months, only RVWT predicted all-cause mortality (p 0.036), while TAPSE, TAPSE/TRPG, RV fractional area change and RV S' wave did not. Correlation: RWVT and RV hemodynamic Hemodynamic variable R R2 p value Mean pulmonary artery pressure 0.742 0.550 <0.001 RV differential pressure 0.794 0.630 <0.001 Pulmonary artery pulsatory pressure 0.740 0.547 <0.001 Mean right atrium pressure 0.326 0.106 <0.001 Cardiac index/right atrial pressure 0.209 0.044 0.012 RV stroke work index 0.588 0.346 <0.001 Pulmonary artery compliance 0.449 0.202 <0.001 Pulmonary vascular resistance 0.531 0.282 <0.001 Prognosis: different RV variables Discussion We identified a novel bedside echocardiographic predictor of altered RV hemodynamic, which results precociously altered in patients without overt RV failure, and able to predict all cause mortality at a long term follow up. Further studies are needed to confirm its role in PH patients.


2001 ◽  
Vol 72 (5) ◽  
pp. 1520-1522 ◽  
Author(s):  
Yoshikazu Hachiro ◽  
Nobuyuki Takagi ◽  
Tetsuya Koyanagi ◽  
Masayuki Morikawa ◽  
Tomio Abe

1988 ◽  
Vol 19 (2) ◽  
pp. 167-179 ◽  
Author(s):  
Guillermo O. Kreutzer ◽  
Adrian E. Allaria ◽  
Andres J. Schlichter ◽  
Maria I. Roman ◽  
Horacio Capelli ◽  
...  

ESC CardioMed ◽  
2018 ◽  
pp. 837-840
Author(s):  
Robert Yates ◽  
Marietta Charakida

Isolated congenitally corrected transposition of the great arteries may remain undiagnosed or cause few problems for decades. Late complete heart block and right ventricular failure with tricuspid regurgitation have an adverse impact on long-term outcome. It is much more common for congenitally corrected transposition of the great arteries to occur with major associated cardiac abnormalities, and these will determine the clinical presentation, natural history, and treatment. A number of different surgical strategies can be considered for such patients, and the best approach is not yet clear. Specialist follow-up is therefore required.


1995 ◽  
Vol 5 (1) ◽  
pp. 9-14 ◽  
Author(s):  
Denise Kitchiner ◽  
Narayanswami Sreeram ◽  
Nilima Malaiya ◽  
Mark Jackson ◽  
Kevin Walsh ◽  
...  

SummaryTo determine the long-term results in patients with critical aortic stenosis who survive initial intervention, and to identify factors which predict prognosis, we studied patients who underwent intervention between 1979 and 1992 for critical aortic stenosis treated within the first three months of life. Patients with a hypoplastic left ventricle or mitral stenosis who were not considered for a biventricular repair were excluded. Follow-up examination included cross-sectional and Doppler echocardiography. All initial and subsequent patient data were reviewed. Of the 64 patients with critical aortic stenosis, 41 (64%) survived more than one month after initial intervention (surgical valvotomy in 39, balloon valvoplasty in two). These survivors constitute the study group. Mild or moderate residual aortic stenosis or regurgitation without further intervention was found in 28 patients at a median duration of 3.1 years (range 0.2–15.0 years). A poor result with re-intervention (n=6) or death (n=7) occurred in 13 patients. The diameter of the aortic valve at presentation was smaller (p<0.02) in patients with a poor result (median 5.5; range 5–15 mm), than in those with a satisfactory result (median 8.0; range 5–10 mm). Significant residual aortic stenosis was present from the time of initial intervention in nine of the 13 patients (69%) with a poor result. No difference was found in the incidence of a duct-dependent systemic circulation, associated cardiac lesions, mechanical ventilation, acidosis or the use of inotropes preoperatively between patients with a satisfactory or a poor late outcome. Of patients with critical aortic stenosis, 64% survived for more than a month after initial intervention. A small aortic valvar diameter at presentation ( 6 mm) and residual stenosis after initial intervention were important determinants of long-term prognosis.


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