Left ventricular rotational abnormalities in adult patients with congenital heart disease late after Fontan procedure: detailed analysis from the CSONGRAD Registry and MAGYAR-Path Study.

BACKGROUND

The Fontan procedure (FP) is a surgical palliation diverting blood flow from the caval veins to the pulmonary artery. The FP is used in several congenital heart diseases (CHDs), for instance in the absence of a heart valve and/or in the presence of an abnormality of a heart chamber. Since little information is available on the cardiac mechanics of FP-operated patients, the present study aimed to determine three-dimensional speckle tracking echocardiography (3DSTE)-derived basal and apical left ventricular (LV) rotations in adult patients with CHD mainly affecting the right heart late after FP.

METHODS

The present study comprised 15 CHD patients late after FP with a mean age of 32.6±8.0 years (5 males). Their results were compared to a group of 25 age- and gender-matched healthy individuals (mean age: 33.4±11.9 years, 8 males).

RESULTS

Regarding the type of the procedure, bidirectional Glenn procedure (BDGP), modified Kreutzer procedure (MKP) and total cavopulmonary connection were performed as final palliative procedures in 2, 3 and 8 patients, respectively. Regarding the ventricular anatomy, hypoplastic right heart syndrome (HRHS), univentricular heart (UH) and tricuspid atresia were present in 3, 5 and 5 patients, respectively. With 3DSTE, regarding the procedure, only MKP patients showed normal LV ejection fraction (EF) due to increased LV end-diastolic volume (EDV), other groups showed mildly reduced LV-EF. With 3DSTE, regarding the ventricular anatomy, UH patients had the largest LV-EDV, but no differences could be detected in mildly reduced LV-EF values between the subgroups. Regarding the procedure, BDGP patients had larger basal and apical LV rotations compared to the other groups resulting in increased LV twist. Regarding the ventricular anatomy, HRHS patients had larger basal and apical LV rotations compared to the other groups resulting in increased LV twist.

CONCLUSIONS

In FP patients with CHD mainly affecting the right heart, on a group level, impaired LV-EF is associated with tendentiously lower LV twist due to reduced apical LV rotation, but characteristics of LV functional and rotational mechanics show substantial differences depending on the ventricular anatomy in CHD and the procedure performed.