Management Options for Congenitally Corrected Transposition: Which, When, and for Whom?

Management strategies for congenitally corrected transposition of the great arteries (ccTGA) historically consisted of a physiologic repair, resulting in the morphologic right ventricle (mRV) supporting systemic circulation. This strategy persisted despite the development of heart failure by middle age because of the reasonable short-term outcomes, and the natural history of some patients with favorable anatomy (felt to demonstrate the mRV's ability to function in the long-term), and due to the less-than-optimal outcomes associated with anatomical repair. As outcomes with anatomical repair improved, and the long-term risk of systemic mRV dysfunction became apparent, more have begun to realize its advantages. In addition to the decision on whether or not to pursue anatomical repair, and the optimal timing, studies demonstrating the nuance to morphologic left ventricle retraining have demonstrated its feasibility. Further considerations in ccTGA have begun to be better understood, including: the management of a poorly functioning mRV, systemic tricuspid valve regurgitation, the utility of morphologic left ventricle outflow tract obstruction (native or surgically created) and pacing strategies. While some considerations are apparent: biventricular pacing is superior to univentricular, tricuspid regurgitation must be managed early with either progression towards anatomical repair (pulmonary artery banding if needed for retraining) or tricuspid replacement (not repair) based on the patient's age; others remain to be completely elucidated. Overall, the heterogeneity of ccTGA, as well as the unique presentation with each patient regarding ventricular and valvular function and center-to-center variability in management strategies has made the interpretation of published data difficult. That said, more recent long-term outcomes favor anatomical repair in most situations.

Congenitally corrected transposition of the great arteries outcomes of different surgical techniques in a paediatric population: A single-centre report.

BACKGROUND: Congenitally corrected transposition of the great arteries is a complex pathology characterised by atrioventricular and ventriculo-arterial discordance. Optimal surgical approaches are still a matter of debate. OBJECTIVE: To evaluate the outcomes of different surgical treatments in a single centre. METHODS: Between 1998 and 2020, 89 patients were studied. The cohort was divided into three groups: physiologic, anatomic, and univentricular repair. RESULT: Physiologic correction (56.18%) was associated with significant tricuspid valve regurgitation progress (42%) and complete AV block (30%) compared to anatomic repair. Right ventricular systolic dysfunction was developed in 14%. Instead, anatomic correction (30.34%) (double switch 59% and Rastelli type 40.7%) presented moderate to severe aortic regurgitation (4%) and left ventricular systolic dysfunction (11%). Complete AV block was developed in 14.8%. Rate of reintervention was 34% for physiologic and 26% for anatomic. Univentricular palliation (13.8%) presented no complications or late mortality during the follow-up. The overall survival at 5 and 10 years, respectively, was 80% (95% CI 69, 87) and 75% (95% CI 62, 84). There was no statistically significant difference in mortality between the groups (p log-rank = 0.5752). CONCLUSION: Management of congenitally corrected transposition of the great arteries remains a challenge. In this cohort, outcomes after physiologic repair were satisfactory in spite of the progression of tricuspid regurgitation and the high incidence of AV block. Anatomic repair improved tricuspid regurgitation but increased the risk of aortic regurgitation and left ventricular systolic dysfunction. The Fontan group showed the lowest incidence of complications.

Congenitally Corrected Transposition of the Great Arteries: Anatomic, Physiologic Repair, and Palliation.

Congenitally corrected transposition of the great arteries (ccTGA) is a lesion that rarely occurs in isolation. The presenting physiology of ccTGA is predominantly secondary to the concurrent cardiac lesions; however, as the child ages, unrepaired ccTGA results in progressive failure of the morphologic right ventricle under the strain of maintaining a systemic pressure. Repair of ccTGA was initially focused on rectification of the underlying physiologic aberrations, but in recent years, the focus of repair has shifted toward anatomic correction to avoid failure of the morphologic right ventricle. This anatomic repair is commonly associated with improved long-term mortality at the cost of increased short-term mortality. Key preoperative considerations such as morphologic left ventricular pressure, tricuspid valve competency, and out flow tract obstructions can assist in determining the optimal repair for individual patients. An alternative, single ventricle, pathway has been proposed for any patient without optimal preoperative anatomy to improve long-term survival. Adjunctive repair options including pulmonary artery banding and one-and-a-half ventricle repairs have also been proposed to augment the survival curves.

A case report of late physiologic repair of congenitally corrected transposition of the great arteries and pulmonary stenosis in a severely cyanotic patient: better late than never.

BACKGROUND: Patients with congenitally corrected transposition of great arteries (ccTGA) not infrequently seek medical attention for the first time late in life. Optimal management of natural history ccTGA is debated and must be tailored. CASE SUMMARY: A 38-year-old male patient was referred to our centre because of severe cyanosis and worsening dyspnoea. Investigations disclosed situs solitus, mesocardia, double discordance, large ventricular septal defect (VSD), severe pulmonary stenosis, and no significant atrio-ventricular valves regurgitation. The patient underwent physiologic repair: VSD closure, placement of a left ventricle to pulmonary artery conduit, and epicardial atrio-biventricular pacemaker implantation. The conduit was intentionally undersized to promote tricuspid valve continence. Post-operative course was uneventful, transthoracic echocardiography showed good biventricular function without significant tricuspid regurgitation. At 1 month after discharge, the patient is in New York Heart Association Class II. DISCUSSION: Management of late presenter patients with ccTGA depends on the associated lesion and estimation of surgical risk. In selected patients markedly symptomatic physiologic repair is a rationale option, providing a normal saturation and biventricular circulation with a significantly lower surgical risk as compared with an anatomic repair.

Long-term results after physiologic repair for congenitally corrected transposition of the great arteries.

OBJECTIVE: We aimed to evaluate the long-term results of physiologic repair for associated lesions of congenitally corrected transposition of great arteries (ccTGA) and to provide a basis for comparison with anatomic repair for this entity. SUBJECTS AND METHODS: Sixteen ccTGA patients who underwent physiologic repair from 1970 to 2000 comprise this retrospective study. Conventional Rastelli procedure was performed in 12 patients with pulmonary stenosis or atresia (PS/PA). Ventricular septal defect closure was carried out in 2 patients, atrial septal closure in 1, and tricuspid valvuloplasty in 1 without PS/PA. Mean follow-up period was 19.4 years. Long-term survival rates were assessed with respect to the presence or the absence of preoperative PS/PA and specifically in relation with the magnitude of pre- and postoperative tricuspid regurgitation (TR). RESULTS: There has been no long-term mortality in the ccTGA patients without PS/PA. Twenty-year survival rate after conventional Rastelli was 71 %. Overall 20-year freedom from more than mild TR or tricuspid valve replacement was 44 %. The development of postoperative more than mild TR was significantly linked with pre-repair right ventricular enlargement (p = 0.019), but not with the magnitude of pre-repair TR (p = 0.85). CONCLUSION: Long-term outcomes of physiologic repair for ccTGA were equivalent to those of reported anatomic repair performed in several centers during the same era. Notably, significant TR was observed in more than half of physiologically repaired patients over the 20 years after repair. The degree of pre-repair TR cannot predict the long-term function of tricuspid valve after physiologic repair.