Anomalies of the coronary sinus are rare in the general population but are more frequent in patients with congenital heart defects. Whatever the cardiac anatomy, the coronary sinus is invariably located in the left atrioventricular sulcus, inferior to the wall of the morphologically left atrium.

A complete morphological examination of a fetal cardiac specimen of the M3C collection, according to segmental analysis, was performed by two observers.

We report here for the first time a cardiac specimen with a venous channel receiving the majority of coronary veins, located in the right atrioventricular sulcus and therefore inferior to the wall of the morphologically right atrium, in a fetal cardiac specimen with congenitally corrected transposition in situs solitus. In addition, the anatomy of the venous drainage of the heart was mirror-imaged to that observed in a normal heart and different compared to that usually observed in congenitally corrected transposition.

This very particular anatomy occurring in association with congenitally corrected transposition might be related with an additional disturbance in the laterality pathway.

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.

To evaluate the outcomes of different surgical treatments in a single centre.

Between 1998 and 2020, 89 patients were studied. The cohort was divided into three groups: physiologic, anatomic, and univentricular repair.

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).

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.

Adults with systemic right ventricle have a significant risk for long-term complications such as arrhythmias or heart failure.

A nationwide retrospective study based on the German National Register for Congenital Heart Disease was performed. Patients with transposition of the great arteries after atrial switch operation or congenitally corrected TGA were included.

Two hundred and eight-five patients with transposition of the great arteries after atrial switch operation and 95 patients with congenitally corrected transposition of the great arteries were included (mean age 33 years). Systolic function of the systemic ventricle was moderately or severely reduced in 25.5 % after atrial switch operation and in 35.1% in patients with congenitally corrected transposition. Regurgitation of the systemic atrioventricular valve was present in 39.5% and 43.2% of the cases, respectively. A significant percentage of patients also had a history for supraventricular or ventricular arrhythmias. However, polypharmacy of cardiovascular drugs was rare (4.5%) and 38.5 % of the patients did not take any cardiovascular medication. The amount of cardiovascular drugs taken was associated with NYHA class as well as systemic right ventricular dysfunction. Patients with congenitally corrected transposition were more likely to receive pharmacological treatment than patients after atrial switch operation.

A significant portion of patients with systemic right ventricle suffer from a relevant systemic ventricular dysfunction, systemic atrioventricular valve regurgitation, and arrhythmias. Despite this, medication for heart failure treatment is not universally used in this cohort. This emphasises the need for randomised trials in patient with systemic right ventricle.

Patients with a congenitally corrected transposition of the great arteries show an increasing incidence of cardiac failure with age. In other systemic right ventricles, such as in dextro-transposition after atrial switch, excessive hypertrophy is a potential risk factor for impaired systolic function. In this trial, we sought to compare systemic function and volumes between patients with congenitally corrected transposition and those with dextro-transposition after atrial switch by using cardiac magnetic resonance imaging.

A total of 19 patients (nine male) with congenitally corrected transposition and 31 patients (21 male) with dextro-transposition after atrial switch were studied using a 1.5-Tesla scanner. Cine steady-state free-precession sequences in standard orientations were acquired for volumetric and functional imaging. Patient parameters were compared with those of a group of 25 healthy volunteers. Although patients with congenitally corrected transposition were older, they presented with higher right ventricular ejection fractions (p = 0.04) compared with patients with dextro-transposition. Patients with congenitally corrected transposition showed a weak negative correlation between age at examination and systemic ejection fraction (r = −0.18, p = 0.04) but no correlation between right ventricular myocardial mass index and ejection fraction. There was no significant difference in the right ventricular end-diastolic volumes between both patient groups.

Although patients with congenitally corrected transposition had a longer pressure load of the systemic right ventricle, ventricular function was better compared with that in patients with dextro-transposition after atrial switch. The results suggest that the systemic ventricles might have partly different physiologies. One difference could be the post-operative situation after atrial switch, which results in impaired atrial contribution to ventricular filling.