Congenitally corrected transposition

Abstract

Congenitally corrected transposition is a rare cardiac malformation characterized by the combination of discordant atrioventricular and ventriculo-arterial connections, usually accompanied by other cardiovascular malformations. Incidence has been reported to be around 1/33,000 live births, accounting for approximately 0.05% of congenital heart malformations. Associated malformations may include interventricular communications, obstructions of the outlet from the morphologically left ventricle, and anomalies of the tricuspid valve. The clinical picture and age of onset depend on the associated malformations, with bradycardia, a single loud second heart sound and a heart murmur being the most common manifestations. In the rare cases where there are no associated malformations, congenitally corrected transposition can lead to progressive atrioventricular valvar regurgitation and failure of the systemic ventricle. The diagnosis can also be made late in life when the patient presents with complete heart block or cardiac failure. The etiology of congenitally corrected transposition is currently unknown, and with an increase in incidence among families with previous cases of congenitally corrected transposition reported. Diagnosis can be made by fetal echocardiography, but is more commonly made postnatally with a combination of clinical signs and echocardiography. The anatomical delineation can be further assessed by magnetic resonance imaging and catheterization. The differential diagnosis is centred on the assessing if the patient is presenting with isolated malformations, or as part of a spectrum. Surgical management consists of repair of the associated malformations, or redirection of the systemic and pulmonary venous return associated with an arterial switch procedure, the so-called double switch approach. Prognosis is defined by the associated malformations, and on the timing and approach to palliative surgical care.

Congenital Corrected Transposition of Great Arteries (TGA)

“…A previous multicenter study demonstrated that a quarter of uncomplicated ccTGA patients developed systemic RV dysfunction by age 45. 6) This is thought to be induced by concomitant severe tricuspid regurgitation, progressive conduction tissue disturbances, a single coronary circulation dependent on morphological right coronary artery, and essential characteristics of the right ventricular muscle itself. In the present issue of International Heart Journal, Shiba and colleagues report on an 82-year-old man who had been diagnosed as having uncomplicated ccTGA and situs inversus and recently developed systemic RV failure, which was neither associated with severe TR nor advanced conduction tissue abnormalities.…”

Criss-cross heart: report of two cases, anatomic and surgical description and literature review

“…Patients with this type of anomaly, which represents 0.05% of congenital heart diseases [44] may be symptomatic not because of the criss-cross heart, but by the presence of other associated anomalies such as VSD, pulmonary outflow obstruction, tricuspid valve abnormalities [45].…”

“…The diagnosis becomes more difficult due to the similarity of the clinical presentation of the different connections abnormalities [51,52].…”

“…The subcostal window will determine the location of the heart apex and assess mainly the ventricles characteristics. The trabeculae morphological features, will determine the morphologic ventricle characteristics [51]. The great arteries connections are better visualized in the paraesternal window [54,55].…”

Cardiac Conduction System in Congenitally Corrected Transposition of the Great Arteries and Its Clinical Relevance

“…The incidence has been reported to be around 1/33 000 live births, accounting for ≈0.05% of congenital heart malformations 2. Although a familial recurrence of heart defects in subjects with ccTGA has been reported,3 the etiology of this malformation is not currently known 1, 4…”