Contemporary management and outcomes in congenitally corrected transposition of the great arteries

Abstract

Congenitally corrected transposition of the great arteries (ccTGA) can occur in isolation, or in combination with other structural cardiac anomalies, most commonly ventricular septal defect, pulmonary stenosis and tricuspid valve disease. Clinical recognition can be challenging, so echocardiography is often the means by which definitive diagnosis is made. The tricuspid valve and right ventricle are on the systemic arterial side of the ccTGA circulation, and are therefore subject to progressive functional deterioration. The natural history of ccTGA is also greatly influenced by the nature and severity of accompanying lesions, some of which require surgical repair. Some management strategies leave the right ventricle as the systemic arterial pump, but carry the risk of worsening heart failure. More complex 'double switch' repairs establish the left ventricle as the systemic pump, and include an atrial baffle to redirect venous return in combination with either arterial switch or Rastelli operation (if a suitable ventricular septal defect permits). Occasionally, the anatomic peculiarities of ccTGA do not allow straightforward biventricular repair, and Fontan palliation is a reasonable option. Regardless of the approach selected, late cardiovascular complications are relatively common, so ongoing outpatient surveillance should be established in an age-appropriate facility with expertise in congenital heart disease care.

Systematic review and meta-analysis of outcomes of anatomic repair in congenitally corrected transposition of great arteries

“…We report pooled outcomes from 895 patients with a sizeable follow up of > 5000 PY. Management patterns for patients with cc-TGA are dictated by variations in anatomic substrate and often by the experience of individual centers[ 3 ]. The long-term outcomes with a physiologic repair strategy keeping the right ventricle as the systemic ventricle, although considered safe unmistakably leads to progressive congestive heart failure.…”

“…The anatomic repairs allow for the left ventricle to become the systemic ventricle. This reduces the long-term deleterious risk of systemic right ventricular (RV) failure, and the propensity for progressive systemic TV insufficiency[ 3 , 4 ].…”

Congenitally Corrected Transposition of Great Arteries with Dextrocardia, Patent Ductus Arteriosus, Atrial Septal Defects and Ventricular Septal Defects in a 15-Year-Old Marfanoid Habitus Patient: A Case Study

“…In addition to the transposed ventricles and arteries, CCTGA can also present with pulmonary atresia and pulmonary stenosis. ASD and VSD have also been seen to arise in these patients and exist independently [ 13 ]. Table 1 compares the features of TOF and CCTGA with the findings of our presented case.…”

Successful case of complex atrial flutter occurring in a patient with congenitally corrected transposition of the great arteries, aberrant left atrial appendage, and situs inversus

“…Congenitally corrected transposition of great arteries (ccTGA) is a rare form of congenital heart disease (CHD) with the prevalence being less than 0.5% [1,2]. Since many other associated anatomical defects, such as atrial septal defect, ventricular septal defect, pulmonary stenosis and abnormalities of the morphological tricuspid valve, occurred in the majority of ccTGA patients, surgical treatment was performed in quite a number of these patients [3,4].…”