Congenitally Corrected Transposition of the Great Arteries in the Adult

Abstract

Introduction Congenitally corrected transposition of the great arteries (CCTGA) is a rare anomaly. Current data available regarding adult cases is derived from small series, information simultaneously presented in pediatric publications, and one classical multicenter study. This review, not aimed to exhaust the subject, has the purpose to examine the literature addressing presentation, diagnostic methodology, and management of afflicted adult patients. Methods A comprehensive search was undertaken in three major databases (PubMed, Cochrane, SciELO), using the keywords “congenitally corrected transposition of the great arteries” and “adults”. Relevant articles in English, Spanish, and Portuguese were extracted and critically appraised in this review. Steps for study selection were: (1) identification of titles of records through databases searching, (2) removal of duplicates, (3) screening and selection of abstracts, (4) final inclusion in the study. Results Four hundred sixty-five publications on CCTGA in adult patients were retrieved, and 166 were excluded; 299 studies were used for this review including 76 full-text articles, 70 studies related to general aspects of the subject, and, due to the small number of publications, 153 case reports. Sixty-one articles referring to combined experiences in pediatric and adult patients and judged to be relevant, but retrieved from another sources, were also included. Conclusion Albeit clinical presentation and diagnostic criteria have been well stablished, there seems to be room for discussion related to clinical and surgical management of CCTGA in adults. Considering the rarity of the disease, well designed multicenter studies may provide answers.

Percutaneous closure of an atrial septal defect in adult patients with congenitally corrected transposition of the great arteries

“…Even in some cases, patients experience a full life without any problem and are diagnosed after the autopsy. 9,12,13 On physical examination, bradycardia caused by high-degree atrioventricular block and a single loud second heart sound which is palpable on the left sternal border are the most common findings. Associated ventricular septal defect, pulmonary stenosis, or atrioventricular valve regurgitation can cause murmur.…”

“…Varying degrees of atrioventricular block can also be present. 8,13 Another inexpensive and available diagnostic tool is echocardiography, which can provide us with valuable findings such as confirming the diagnosis and determining the presence of any associated anomalies. The anatomical approach should be detailed and sequential, starting from the visceral atrial situs and continuing to the great arteries.…”

“…Radionuclide angiography is another mentioned 16 modality for this application. 8,13 Nowadays, with the advancement of non-invasive methods such as echocardiography and cardiac MRI, the indications for cardiac catheterisation have been limited, but the measurement of left and right heart pressures and pulmonary resistance, evaluation of the coronary arteries or the aortic arch, complex pulmonary atresia, and pulmonary venous abnormalities are still potential indications. It is also an essential part of any pre-operative assessment in older patients.…”

“…9,12,17 The presence of an advanced second-or third-degree AV block, having symptoms or ventricular dysfunction are the indications of pacemaker implantation. 13 In a study conducted by Connelly et al 18 on 52 patients with corrected transposition of the great arteries, six patients (~11%) developed endocarditis; this implies the importance of antibiotic prophylaxis according to the American Heart Association guideline. 19 Surgical management: Diagnosis of corrected transposition of the great arteries does not always imply the need for surgery.…”

“…This approach is straightforward, but the fact that morphologically right ventricle will likely fail over time results in poor prognosis. 9,13 Also, if complete heart block happens during the procedure, a pacemaker must be planted. 9 The most common physiologic surgery is tricuspid valve replacement.…”