Anatomic correction of congenitally corrected transposition and its close cousins

2006 ◽  
Vol 16 (S3) ◽  
pp. 85-90 ◽  
Author(s):  
Edward L. Bove ◽  
Richard G. Ohye ◽  
Eric J. Devaney ◽  
Hiromi Kurosawa ◽  
Toshiharu Shin'oka ◽  
...  
Keyword(s):  
Surgical Approaches ◽  
Long Term Effects ◽  
The Past ◽  
Congenital Cardiac Malformation ◽  
Associated Malformations ◽  
Atrioventricular Discordance ◽  
Congenitally Corrected Transposition ◽  
Corrected Transposition

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.

Anatomic correction of congenitally corrected transposition and its close cousins

2006 ◽  
Vol 16 (S3) ◽  
pp. 85-90
Author(s):  
Edward L. Bove ◽  
Richard G. Ohye ◽  
Eric J. Devaney ◽  
Hiromi Kurosawa ◽  
Toshiharu Shin'oka ◽  
...  
Keyword(s):  
Surgical Approaches ◽  
Long Term Effects ◽  
The Past ◽  
Congenital Cardiac Malformation ◽  
Associated Malformations ◽  
Atrioventricular Discordance ◽  
Congenitally Corrected Transposition ◽  
Corrected Transposition

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.

Abstract 14198: Anatomic Repair of Congenitally Corrected Transposition of the Great Arteries is Associated With Significant Mid- and Long-term Electrophysiologic Morbidity

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Rebecca R Hartog ◽  
Kimberly J Watkins ◽  
Megan Wilde ◽  
Tiffany R Lim ◽  
Andrew Rodenbarger ◽  
...  
Keyword(s):  
Proportional Hazards ◽  
Pulmonary Stenosis ◽  
Cox Proportional Hazards ◽  
Anatomic Repair ◽  
Atrial Switch ◽  
Increased Risk ◽  
Congenitally Corrected Transposition ◽  
Corrected Transposition

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.

Congenitally corrected transposition of the great arteries

ESC CardioMed ◽  
2018 ◽  
pp. 837-840
Author(s):  
Robert Yates ◽  
Marietta Charakida
Keyword(s):  
Heart Block ◽  
Right Ventricular Failure ◽  
Adverse Impact ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Surgical Strategies ◽  
Congenitally Corrected Transposition ◽  
Corrected Transposition

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.

Abstract 15704: Morbidity and Mortality After Anatomic Repair of Congenitally Corrected Transposition of the Great Arteries: Understanding Risks for Patient and Family Counseling

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Kimberly J Watkins ◽  
Rebecca R Hartog ◽  
Megan Wilde ◽  
Tiffany R Lim ◽  
Andrew Rodenbarger ◽  
...  
Keyword(s):  
Systolic Dysfunction ◽  
Family Counseling ◽  
Secondary Outcome ◽  
Term Survival ◽  
Anatomic Repair ◽  
Functional Benefit ◽  
Congenitally Corrected Transposition ◽  
Corrected Transposition

Introduction: Anatomic repair of congenitally corrected transposition of the great arteries (ccTGA) generally poses short-term risk for presumed long-term functional benefit by restoring the left ventricle to the systemic circulation. Understanding early and late surgical outcomes is crucial for counseling patients and families. Methods: All patients with anatomic repair at a single institution from 1993-2018 were included. Follow-up data was obtained by contact with patients’ primary cardiologists. The primary outcome was long-term survival and the secondary outcome was freedom from reintervention. Univariate Cox proportional hazard model was used to identify risk factors for mortality. In-hospital mortality and morbidity were compared between the most recent (2010-2018) and past surgical eras. Results: Anatomic repair was performed in 118 patients: 64 Senning/arterial switch operations (ASO) and 54 Senning/Rastelli operations. Survival was 85%, 82%, and 70% at 1, 10, and 20 years after repair, respectively, and was significantly lower in the Senning/Rastelli group compared to Senning/ASO (Hazard ratio 3.0, p=0.01; Figure 1). Preoperative factors associated with mortality were heterotaxy syndrome (p=0.03) and right ventricular systolic dysfunction (p=0.01). During a median follow up of 9.4 years, 47 of 98 patients (48%) required surgical or catheter-based reintervention; median freedom from reintervention was 11.7 years. For the most recent era, 30 of 32 patients (94%) with Senning/ASO and 19 of 22 patients (86%) with Senning/Rastelli operations survived to hospital discharge. In-hospital morbidity was similar between eras (30% in 1993-2009 and 28% in 2010-2018, p=0.82). Conclusions: Short- and long-term outcomes are favorable following Senning/ASO. Mortality following Senning/Rastelli operations remains high, highlighting the need for careful patient selection and family counseling.

Congenitally corrected transposition: complex anatomic repair or Fontan pathway?

2017 ◽  
Vol 25 (6) ◽  
pp. 432-439 ◽  
Author(s):  
Supreet P Marathe ◽  
Matthew I Jones ◽  
Julian Ayer ◽  
Jessica Sun ◽  
Yishay Orr ◽  
...  
Keyword(s):  
Medium Term ◽  
Term Survival ◽  
Anatomic Repair ◽  
Group 2 ◽  
Fontan Palliation ◽  
Congenitally Corrected Transposition ◽  
Corrected Transposition ◽  
Group 1

Background Successful anatomic repair of congenitally corrected transposition of the great arteries achieves excellent outcomes. Several centers report excellent long-term survival with the Fontan pathway as well. We have selectively applied both approaches depending on individual patient morphology, with anatomic repair preferred but utilizing the Fontan pathway when high technical complexity or operative risk is anticipated. Methods Hospital records over an 18-year period (1998–2016) were reviewed to identify patients with congenitally corrected transposition of the great arteries who underwent surgical management. Physiological repairs and hypoplastic ventricles were excluded. Patient- and procedure-related variables were reviewed. Results We identified 19 patients. Group 1 consisted of 12 anatomic repairs, of which 10 (83.3%) required prior interim staging procedures. Mean age at anatomic repair was 2.6 ± 1.3 years, mean follow-up was 8.7 ± 5.3 years. Nine (75%) patients experienced important complications and 4 (33.3%) required reintervention during follow-up. There were no deaths; one patient required heart transplantation. Group 2 (7 patients) underwent Fontan palliation. Mean age at Fontan completion was 7.2 ± 3.8 years, mean follow-up was 6.3 ± 4 years. There was no reintervention, death, or transplant. Conclusion Patients with congenitally corrected transposition of the great arteries and two adequate-sized ventricles do well with both anatomic repair and the Fontan pathway in the medium term. Excellent outcomes with reduced early complication and reintervention rates can be achieved for this cohort of patients when a strategy of avoiding complex anatomic repair in favor of the Fontan pathway is used.

scholarly journals Prenatal diagnosis, associated findings and postnatal outcome in fetuses with congenitally corrected transposition of the great arteries

2020 ◽  
Author(s):  
Andrea Krummholz ◽  
I. Gottschalk ◽  
A. Geipel ◽  
U. Herberg ◽  
C. Berg ◽  
...  
Keyword(s):  
Prenatal Diagnosis ◽  
Fetal Echocardiography ◽  
Survival Rates ◽  
Cardiac Anomalies ◽  
Prenatal Counselling ◽  
Associated Malformations ◽  
Postnatal Outcome ◽  
Congenitally Corrected Transposition ◽  
Corrected Transposition

Abstract Purpose To analyze anatomic features and associated malformations in 37 prenatally detected cases of congenitally corrected transposition of the great arteries (ccTGA) and to evaluate the prenatal course, neonatal outcome and mid-term follow-up. Methods Retrospective analysis of prenatal ultrasound of 37 patients with ccTGA in two tertiary centers between 1999 and 2019. All fetuses received fetal echocardiography and a detailed anomaly scan. Postnatal outcome and follow-up data were retrieved from pediatric reports. Results Isolated ccTGA without associated cardiac anomalies was found in 13.5% (5/37), in all other fetuses additional defects such as VSD (73.0%), pulmonary obstruction (35.1%), tricuspid valve anomalies (18.9%), aortic arch anomalies (13.5%), ventricular hypoplasia (5.4%) or atrioventricular block (5.4%) were present. The rate of extracardiac malformations or chromosomal aberrations was low. There were 91.9% (34/37) live births and postnatal survival rates reached 91.2% in a mean follow-up time of 4.98 years. The prenatal diagnosis of ccTGA was confirmed postnatally in all but one documented live birth and the prenatal counselling regarding the expected treatment after birth (uni- versus biventricular repair) was reassured in the majority of cases. The postnatal intervention rate was high, 64.7% (22/34) received surgery, the intervention-free survival was 36.7%, 35.0% and 25.0% at 1 month, 1 year and 10 years, respectively. Conclusions ccTGA is a rare heart defect often associated with additional heterogeneous cardiac anomalies that can be diagnosed prenatally. The presented study demonstrates a favorable outcome in most cases but the majority of patients require surgical treatment early in life.

Organizational Reforms as Routines

2017 ◽  
Author(s):  
Nils Brunsson
Keyword(s):  
Long Term Effects ◽  
The Past ◽  
Limited Experience

This chapter argues that organizational reforms are driven by problems to be addressed, by solutions to be applied, and by forgetfulness. The greater the supply of any of these factors, the more likely it is that reforms will occur. Without problems, reforms are difficult to justify; without solutions they cannot be formulated; and without forgetfulness there is a risk that people will be discouraged by the fact that similar reforms have been tried and have failed in the past. In contemporary large organizations, problems tend to be easily found. Those interested in selling solutions often try to supply problems as well — problems that can be solved by their solutions. Forgetfulness can be promoted by the use of consultants with limited experience of the implementation and long-term effects of reforms. Reforms are also self-referential; they tend to cause new reforms. Thus, reforms can be considered as routines: they are likely to be repeated over and over again.

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