Clinicopathological analysis of autopsy findings in 25 fetuses with double outlet of right ventricle
Wu Ying
Chen Dong
Shang Jianfeng
Fang Wei
Fu Wen
Teng Fei
Cui Yayan
Yu Wei
Zhu Qing
Abstract：Objective To analyze anatomical and pathological characteristics of 25 fetal double outlet of right ventricle(DORV).Methods Totally 391 fetal autopsies of congenital heart malformation from January 2007 to June 2017 at Beijing Anzhen Hospital,Capital Medical University were reviewed;25 cases were diagnosed of DORV.The method of cardiac segmental analysis was used to observe cardiac deformity;pathological features of different subtypes of DORV were analyzed.Results ①The rate of DORV was 6.4％ (25/391),including 11 cases of male and 14 cases of female.②There were 20 cases of SDD type,2 cases of SDL and 3 cases of ADD.③There were 16 cases(64.0％) of simple types,including 10 cases of DORV with subaortic ventricular septal defect and 6 cases of DORV with subpulmonary ventricular septal defect (Taussig-Bing).There were 9 cases (36.0％) of complex types,including 7 cases of DORV with mitral atresia/left ventricular dysplasia and 3 cases of DORV with complete atrioventricular septal defect(CAVSD).④Aortas in most of the cases were located at right side of pulmonary artery and the 2 main arteries were juxtaposed,which was commonly seen in DORV with subaortic ventricular septal defect;in DORV with subpulmonary ventricular septal defect,aortas in all cases were seen at right anterior side of pulmonary artery.There were 6 cases (24.0％) of main arteries straddling interventricular septum.⑤DORV was often accompanied by other cardiac malformations,including 11 cases(45.8％) of abnormal body-pulmonary vein connection,8 cases (33.3％) of arterial duct stenosis/absence,4 cases (16.7％) of aortic arch anomaly and 3 cases(12.0％) of CAVSD.Extracardiac malformations[11 cases(45.8％)] in DORV fetuses mainly included horseshoe kidney,omphalocele,esophageal atresia,phrenic hernia,heterotaxia syndrome,polysplenia and asplenia syndrome.Conclusions DORV has complex pathological features and it is often accompanied by cardiac and extracardic malformations.Location of ventricular septal defect and its relation with main arteries,pulmonary stenosis and pulmonary outflow tract obstruction are important in fetal autopsy to define accurate pathological classification of DORV,which can provide an important basis for precise diagnosis and molecular genetic mechanism study.
Keywords：Congenital heart malformationDouble outlet of right ventricleVentricular septal defectPulmonary stenosisLeft ventricular dysplasiaPolysplenic syndromeAnsplenic syndrome
Publication Date：2017-12-30
Online Publishing Date：2026-09-14(First online date of this platform, not the publication date of the document)
Pages：5( 1809-1813 )