Clinical Features in Patients With Asplenia Syndrome Combining Complex Congenital Heart Disease
10.3969/j.issn.1000-3614.2017.07.012
- VernacularTitle:无脾综合征患者合并复杂性先天性心脏病类型特点
- Author:
Yan SUN
;
Jianpeng WANG
;
Hui LI
;
Xin QUAN
;
Minghui ZHANG
;
Li ZHANG
;
Ning XU
;
Hao WANG
- Keywords:
Echocardiography;
Heart defect;
congenital;
Asplenia syndrome
- From:
Chinese Circulation Journal
2017;32(7):672-675
- CountryChina
- Language:Chinese
-
Abstract:
To statistically study the patients with asplenia syndrome combining complex congenital heart disease (CHD) for their common cardiac malformation, frequency of occurrence and to explore the anatomical features with possible mechanism. Methods: A total of 47 patients with asplenia syndrome were analyzed including 27 male and 20 female at the age from 23 days to 32 years. The common cardiac malformation and frequency of occurrence were statistically studied. Results: ① The cardiac malformations were mainly with the following types: abnormal position of heart in 16 (34.0%) cases, a wide range of septal tissue defect in 47 (100%) cases, abnormal atrio-ventricular valve in 42 (89.4%) cases, abnormal aortic origin in 47 (100%) cases, abnormal position of 2 grate arteries in 46 (97.8%) cases, right ventricular outflow obstruction/pulmonary arterydysplasiain 45 (95.7%) cases, anomalous systemic venous drainage in 44 (91.5%) cases and anomalous pulmonary venous drainage in 28 (59.6%) cases. ② The cardiac malformations were usually involved in several positions as 1 (2.1%) patient with 4 kinds of abnormal structures, 5 (10.6%) patients with 5 kinds of abnormal structures, 13 (27.7%) patients with 6 kinds of abnormal structures, 23 (48.9%) patients with 7 kinds of abnormal structures and 7 (14.9%) patients with 8 kinds of abnormal structures. Conclusion: Asplenia syndrome combining cardiac malformation has been complex while with specificity. Laterality dysfunction might be the primarycause for series malformations.
