Author + information
- Masaki Nii,
- Keisuke Sato,
- Jun Yoshimoto,
- Sung-Hae Kim,
- Norie Mitsushita and
- Yasuhiko Tanaka
Background: The atrioventricular valve function is one of the most important determinants of mortality in patients with heterotaxy syndrome (HS). Though there are many reports to show the impact of atrioventricular valve regurgitation (AVVR) on the outcome of patients with HS, there are paucity of data analyzing the AVV function longitudinally from fetus to late after Fontan. The aims of this study are: 1) to elucidate the longitudinal change of AVVR from fetus to late after Fontan; 2) to assess the impact of AVVR on mortality on each stage.
Methods: The medical charts of patients with HS who visited Shizuoka Children's Hospital postnatally between 1986 and 2015, and the records of fetal echocadiography between 1998 and 2015 were reviewed. The grade of AVVR was classified into 0 to 3.
Results: There were 227 postnatal patients with HS and 45 fetuses with HS on medical records. Among them, only patients with single ventricle and common AVV were analyzed. 1): postnatal patients: 198 (right atrial isomerism (RAI): 159; left atrial isomerism (LAI): 83; age at 1st visit: 15 days (0 day – 13 years) (median (range)); and 2): 40 fetuses (RAI: 31; LAI: 9; gestational week at 1st visit: 30 (18 – 37) weeks). The grade of AVVR increased from 2nd to 3rd trimester and it increased significantly after birth (AVVR: fetus in 3rd trimester: 1 (0 – 3); after birth: 2 (0 – 3) (p<0.05)). The grade of AVVR after birth increased continuously until Glenn operation and it decreased after Glenn and decreased further after Fontan, however it increased again late after Fontan. The mortality of fetuses with more than moderate AVVR was 75%, which was significantly worse than that of fetuses with less than mild AVVR (p<0.05). The grade of AVVR during fetal and neonatal period showed significant impact on mortality (p<0.05), whereas, the grade of AVVR in later stages showed no impact on survival. Sixty two patients had AVV plasty, 21 patients had multiple AVV plasties, and 8 patients had AVV replacement. The number of AVV platy showed no impact on survival.
Conclusions: The grade of AVVR increased continuously from 2nd trimester of pregnancy to before Glenn operation. The grade of AVVR during perinatal period showed significant impact on survival.
Poster Hall, Hall C
Sunday, March 19, 2017, 9:45 a.m.-10:30 a.m.
Session Title: Congenital Heart Disease: Novel Insights in Pediatric Cardiology
Abstract Category: 10. Congenital Heart Disease: Pediatric
Presentation Number: 1271-018
- 2017 American College of Cardiology Foundation