Left atrial isomerism detected in fetal life

被引:50
作者
Phoon, CK
Villegas, MD
Ursell, PC
Silverman, NH
机构
[1] UNIV CALIF SAN FRANCISCO,MED CTR,DEPT PATHOL,SAN FRANCISCO,CA 94143
[2] UNIV CALIF SAN FRANCISCO,DIV PEDIAT CARDIOL,SAN FRANCISCO,CA 94143
关键词
D O I
10.1016/S0002-9149(96)00137-3
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Left and right atrial isomerism, comprising congenital heart defects with disturbances in normal left-right asymmetry, are phenotypically distinct after birth, although animal models suggest a common embryologic origin, We postulated that the prenatal phenotype may indeed be similar in both syndromes but that differential fetal loss is responsible for the distinct postnatal phenotypes, Distinctive fetal echocardiographic features of these syndromes have not been described in detail, We therefore sought markers of left atrial isomerism that could be recognized prenatally by echocardiography and compared our results with postnatal data to identify unique intrauterine features. We reviewed 10 cases at our center and 28 published cases of cardiac malformations with atrial isomerism detected by fetal echocardiography, Postnatal imaging and autopsies provided definitive diagnoses, Ninety-five percent of cases exhibited left atrial isomerism and formed the primary study population, Echocardiographic markers included a large azygos continuation of an interrupted inferior vena cava, atrioventricular block with structural heart disease, and viscerocardiac heterotaxy, At least 1 of these markers was seen in all of our center's cases. The incidences of most cardiac lesions detected prenatally were similar to those detected postnatally, However, although the incidences of atrioventricular septal defect and pulmonary outflow obstruction in live births were 50% and 45%, respectively, they were found much more frequently among stillbirths (80% each). In summary, we identified key fetal echocardiographic features highly sensitive for left atrial isomerism, Fetal loss selects against certain lesions such as atrioventricular septal defect, The spectrum of cardiac disease suggests a greater primitivity of the fetal heart than previously shown; the typical cardiac phenotypes are closer to right atrial isomerism than are their extrauterine presentations.
引用
收藏
页码:1083 / 1088
页数:6
相关论文
共 25 条
[1]  
ALLAN LD, 1984, BRIT HEART J, V52, P542
[2]   THE ACCURACY OF FETAL ECHOCARDIOGRAPHY IN THE DIAGNOSIS OF CONGENITAL HEART-DISEASE [J].
ALLAN, LD ;
CHITA, SK ;
SHARLAND, GK ;
FAGG, NLK ;
ANDERSON, RH ;
CRAWFORD, DC .
INTERNATIONAL JOURNAL OF CARDIOLOGY, 1989, 25 (03) :279-288
[3]   PROSPECTIVE DIAGNOSIS OF 1,006 CONSECUTIVE CASES OF CONGENITAL HEART-DISEASE IN THE FETUS [J].
ALLAN, LD ;
SHARLAND, GK ;
MILBURN, A ;
LOCKHART, SM ;
GROVES, AMM ;
ANDERSON, RH ;
COOK, AC ;
FAGG, NLK .
JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY, 1994, 23 (06) :1452-1458
[4]  
BROOK MM, 1993, WESTERN J MED, V159, P286
[5]   LINKAGE MAPPING OF A MOUSE GENE, IV, THAT CONTROLS LEFT RIGHT ASYMMETRY OF THE HEART AND VISCERA [J].
BRUECKNER, M ;
DEUSTACHIO, P ;
HORWICH, AL .
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 1989, 86 (13) :5035-5038
[6]   PRENATAL-DIAGNOSIS OF ASPLENIA POLYSPLENIA SYNDROME [J].
CHITAYAT, D ;
LAO, A ;
WILSON, RD ;
FAGERSTROM, C ;
HAYDEN, M .
AMERICAN JOURNAL OF OBSTETRICS AND GYNECOLOGY, 1988, 158 (05) :1085-1087
[7]  
DEARAUJO LML, 1987, J ULTRAS MED, V6, P667
[8]  
FRIEDMAN AH, 1993, SEMIN PERINATOL, V17, P76
[9]   LEFT ISOMERISM AND COMPLETE ATRIOVENTRICULAR-BLOCK - A REPORT OF 6 CASES [J].
GARCIA, OL ;
MEHTA, AV ;
PICKOFF, AS ;
TAMER, DF ;
FERRER, PL ;
WOLFF, GS ;
GELBAND, H .
AMERICAN JOURNAL OF CARDIOLOGY, 1981, 48 (06) :1103-1107
[10]  
Ho S Y, 1991, Pediatr Pathol, V11, P589