The first successful double switch operation for congenitally corrected transposition of the great arteries in Slovenia

Congenitally corrected transposition of the great arteries is a rare congenital heart defect characterized by atrioventricular and ventriculoarterial discordance and can be potentially associated with several other concomitant anomalies, such as ventricular septal defect or congenital complete atrioventricular block. Different surgical options are used for treating the condition. Anatomic repair yields the best long-term outcomes; however, it is technically the most challenging. We present a case of a 3-year old female patient who was electively admitted for complete repair after pulmonary artery banding and pacemaker implantation soon after birth. Subsequently, an anatomic repair (double switch) was performed with an uneventful postoperative course and excellent mid-term outcomes.

The Natural History of Medial Meniscal Root Tears: A Biomechanical and Clinical Case Perspective

Posterior medial meniscus root tears (PMMRTs) make up a relatively notable proportion of all meniscus pathology and have been definitively linked to the progression of osteoarthritis (OA). While known risk factors for development of OA in the knee include abnormal tibial coronal alignment, obesity and female gender, PMMRTs have emerged in recent years as another significant driver of degenerative disease. These injuries lead to an increase in average contact pressure in the medial compartment, along with increases in peak contact pressure and a decrease in contact area relative to the intact state. Loss of the root attachment impairs the function of the entire meniscus and leads to meniscal extrusion, thus impairing the force-dissipating role of the meniscus. Anatomic meniscus root repairs with a transtibial pullout technique have been shown biomechanically to restore mean and peak contact pressures in the medial compartment. However, nonanatomic root repairs have been reported to be ineffective at restoring joint pressures back to normal. Meniscal extrusion is often a consequence of nonanatomic repair and is correlated with progression of OA. In this study, the authors will describe the biomechanical basis of the natural history of medial meniscal root tears and will support the biomechanical studies with a case series including patients that either underwent non-operative treatment (5 patients) or non-anatomic repair of their medial meniscal root tears (6 patients). Using measurements derived from axial MRI, the authors will detail the distance from native root attachment center of the non-anatomic tunnels and discuss the ongoing symptoms of those patients. Imaging and OA progression among patients who were treated nonoperatively before presentation to the authors will be discussed as well. The case series thus presented will illustrate the natural history of meniscal root tears, the consequences of non-anatomic repair, and the findings of symptomatic meniscal extrusion associated with a non-anatomic repair position of the meniscus.

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

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.

Congenitally corrected transposition of great arteries: a case series of five unoperated African children

Background Congenitally corrected transposition of great arteries (ccTGA) is rare. It is commonly associated with ventricular septal defect (VSD), pulmonary stenosis and heart block. Early anatomic repair is recommended between 3 and 6 months of age to prevent development of tricuspid valve regurgitation and systemic right ventricular failure. Case presentation We retrospectively identified five cases of ccTGA. Cases were between one and 13 years of age. All the cases were unoperated. Four of the five cases had associated intracardiac defects/complications. These included: VSD, pulmonary stenosis, tricuspid valve regurgitation, right ventricular systolic dysfunction and heart block. Conclusion These cases demonstrate the challenges of access to early diagnosis and surgery in a low resource setting. This delay in anatomic repair leads to complications of tricuspid valve regurgitation and systemic right ventricular failure.

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

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.