Fetal Cardiac Anomaly

Transcription

1 89 Symposium: OB/GY US (Room B) 12 : : 30 Fetal Cardiac Anomaly 1. One third of all congenital anomalies /1,000 live births 3. Related with more than 50% of childhood deaths and 20-30% of perinatal losses 4. One quarter of affected fetuses have other anomalies, and vice ve r s a 5. One third of affected fetuses are associated with chromosomal abnormality, and 50-80% of fetuses with abnormal chromosome have cardiac anomaly Right Atrium L a r g e, pyramidal atrial appendage Wide orifice of atrial appendage Crista and sulcus terminalis Sinus venarum receiving SVC and IVC Limbus surrounding fossa ova l i s Left Atrium Small, finger-like appendage N a r r ow orifice of atrial appendage No crista and sulcus terminalis Smooth posterior wall receiving pulmonary ve i n s Free flap of fossa ova l i s

2 90 Right Ve n t r i c l e Tricuspid va l ve with more apical septal A t t a c h m e n t H e avier trabeculations Septoparietal trabeculation for exa m p l e, moderator band Discontinuity between atriove n t r i c u l a r and semilunar va l ves with a we l l - d eveloped muscular infundibulum Left Ve n t r i c l e Mitral (bicuspid) va l ve with more cranial septal attachment Finer and more regular trabeculations No septoparietal trabeculation Fibrous continuity betwe e n a t r i oventricular and semilunar va l ves with less prominent infundibulum Step 1 Step 2 Step 3 Step 4 Step 5 Step 6 Step 7 Step 8 D E T E R M I N ATE ATRIAL ARRANGEMENT D E T E R M I N ATE STRUCTURE OF VENTRICULAR MASS A N A LYSIS OF AT R I OVENTRICULAR JUNCTIONS A N A LYSIS OF ARTERIAL SEGMENT A N A LYSIS OF VENTRICULO-ARTERIAL JUNCTIONS A N A LYSIS OF CA R D I AC POSITION A N A LYSIS OF ORGANS IN REST OF BODY CATALOGUE OF ASSOCIATED MALFO R M AT I O N S 1. Blood from the placenta -> umbilical vein -> ductus venosus -> inferior vena cava -> right atrium -> foramen ovale -> left atrium -> left ventricle -> aorta -> brachiocephalic vessels -> head and upper limbs -> superior vena cavae -> right atrium 2. Inferior vena cava flow itself divided into two components; Oxygenated blood coming from the left hepatic vein enters the foramen ova l e, and the desaturated blood from the IVC goes throughout the tricuspid va l ve 3. Blood from the SVC -> tricuspid va l ve -> right ventricle -> pulmonary artery -> ductus arteriosus -> descending aorta

3 91 1. Tr a n s verse view of upper abdomen 2. Four-chamber view 3. Left ventricular outflow tract view 4. Right ventricular outflow tract view 5. Three-vessel view 6. Aortic arch view 7. Ductal arch view 8. Short axis view 1. Does the arrangement of the live r, stomach, and spleen conform the normal situs? 2. Is the heart located normally in the thorax with its apex directed to the left anterior c h e s t? 3. Is the heart size normal? 4. Are the four chambers of the heart identified? Are their sizes approximately equal? Does the ventricular septum appear intact? 5. Are superior and inferior venae cavae and pulmonary veins connected to the appropriate atria? 6. Are there two separate atrioventricular va l ves? Are there any abnormal va l ve attachm e n t s? 7. Are the ventricles normally related? Is the atrioventricular connection concordant? 8. Do the great arteries normally arise from the appropriate ventricles? Are the outflow tracts of the ventricles from their apices down to the great arteries cross each other? 9. Are the main pulmonary artery, aorta, and superior vena cava normally arranged from the left anterior to the right posterior aspect of the fetal thorax and sized in a decreasing o r d e r? 10. Is there any narrowing or discontinuity in the aortic arch? 11. Is there any accelerated, turbulent or regurgitant flow through the cardiac va l ve s? 12. Is the heart beat regular and of normal frequency?

4 92 1. Are there additional extracardiac malformations? 2. Is the fetal ka r yotype normal? 3. Are there signs of cardiac failure (non-immune hyd r o p s )? V i ew Tr a n s verse view of upper abdomen Four-chamber view T h r e e - vessel view Left and right ventricular outflow tract view Aortic arch view Clues to Abnormality Abnormal position of liver and stomach Asplenia or polysplenia Aorta and IVC on the same side of spine Interruption of IVC D extrocardia or mesocardia C a r d i o m e g a l y Asymmetric chamber size Atrial, ventricular or atrioventricular septal defect Apical displacement of septal leaflet of tricuspid va l ve Abnormal pulmonary venous connection Dilatation of aorta, pulmonary artery or SVC One of the two great arteries being small and the other being large Abnormal vessel alignment Abnormal vessel arrangement Only two ve s s e l s Additional ve s s e l Right descending aorta Ventricular septal defect O verriding aorta or pulmonary artery Abnormal ventriculoarterial connection; TGA, DORV or DOLV, and single arterial trunk Abnormal dimension of outflow tracts and/or semilunar va l ve s Ascending aorta being smaller than descending aorta Focal or diffuse narrowing of aortic arch Interruption of aortic arch Double aortic arch

5 93 1. Venous-atrial junction TA PV R 2. Atrioventricular junction Mitral atresia Tricuspid atresia AVS D 3. Ventriculoarterial junction Aortic atresia E b s t e i n s anomaly / Tricuspid dysplasia Pulmonary atresia with VS D Critical AS, Critical PS C OA 4. Others VS D C a r d i o myo p a t hy 1. Transposition of the great arteries 2. Tetralogy of Fa l l o t 3. Double Outlet Right Ve n t r i c l e 4. Mild/moderate coarctation 5. Mild/moderate aortic and pulmonary stenosis 6. small VS D 1. Abnormal vessel size Small a-ao and large MPA Small MPA and large a-ao Dilated MPA Dilated a-ao Dilated SVC 2. Abnormal alignment Ant. Ao with small MPA C OA, IAA, Aortic atresia, HLHS TO F, PA / VSD, PA / I VS, E b s t e i n s anomaly PS, PR AS, AR Interruption of IVC with azygos continuation, Right side heart failure TO F, DORV with subps

6 94 S i d e - by-side GA s (same size) S i d e - by-side GA s (small Ao ) 3. Abnormal arrangement Right anterior Ao Left anterior Ao 4. Abnormal vessel number One artery Two SVC D O RV, some complete TG A D O RV or some TGA with subas Complete TGA, some DORV Corrected TGA, DILV, some DORV Truncus arteriosus Pulmonary atresia with VSD (no MPA ) Bilateral SVC 1. Membranous part of the ventricular septum is so thin that it may appear to be a defect. 2. Small VSD is often impossible to demonstrate. 3. VSD may close spontaneously in fetal life. 4. Mild va l ve stenosis is difficult to detect. the severity of stenosis may progress in fetal life. 5. Most of the atrial septal defect cannot be differentiated from normal patent foramen ova l e. 6. Ductus arteriosus is widely open in fetal life. 7. Mild isthmic narrowing is a normal finding in fetus, and differentiation from mild COA is often difficult. 1. Termination of pregnancy when an unacceptable lesion is found. ; Rates of termination of pregnancy varies from %. 2. P s ychosocial and economical preparation of the parents. 3. Possibility of in utero i n t e r ve n t i o n. 4. Planned delivery in a cardiac center. 5. Early initiation of postnatal treatment. 6. Reduction of perinatal mortality and morbidity. 7. Reduction of socioeconomic burden.

7 95 1. Sharland G: Changing impact of fetal diagnosis of congenital heart disease. Arch Dis Child 1997; 7 7 : F 1 - F 3 2. McGurdy CM Jr, Reed KL: Basic technique of fetal echocardiography. Seminars in US, CT, and MRI ; 1 4 ( 4 ) : Maitre Azcarate MJ, Jimenez MQ: The technique of fetal echocardiography, with its indications and results in a selected population. Cardiol Young 1991;I: Yoo S-J, Lee YH, Kim ES, Ryu HM, Kim MY, Choi H-K, Cho KS, Kim A: Three-vessel view of the fetal upper mediastinum: an easy means of detecting abnormalities of the ventricular outflow tracts and great arteries during obstetric scanning. Ultrasound Obstet Gynecol 1997;9: Friedman AH, Copel JA, Kleimann CS: Fetal echocardiography and fetal cardiology: Indications, diagnosis and management. Seminars in Perinatology 1993;17(2): Friedmann AH, Fahey JT: The transition from fetal to neonatal circulation: normal response and implications for infants with heart disease. Seminars in Perinatology 1993;17(2): Allan LD: Fetal cardiac anomalies. Progress Ped Cardiol 1996;5: Allan L, Sharland G, Milburn A, Locjhart SM, Groves AMM, Anderson RH, Cook AC, Fagg NLK: Prospective diagnosis of 1006 consecutive cases of congenital heart disease in the fetus. JACC ; 2 3 ( 6 ) : Montana E, Khoury MJ, Cragan JD, Sharma S, Dhar P, Fyfe D: Trends and outcomes after prenatal diagnosis of congenital cardiac malformations by fetal echocardiography in a well defined birth population, Atlanta, Georgia, JACC 1996;28(7): Buskens E, grobbee DE, Frofn-Mulder IME, Stewart PA, Juttmann RE, Wladimiroff JW: Efficacy of routine fetal ultrasound screening for congenital heart disease in normal pregnancy. Circulation ; 9 4 : Bromley B, Estroff JA, Sanders SP, Parad R, Roberts D, Frigoletto FD Jr, Benacerraf BR: Fetal echocardiography: Accuracy and limitations in a population at high and low risk for heart defects. Am J Obstet Gynecol 1992;166: Rasanen J, Wood DC, Weiner S, Ludomirski A, Huhta JC: Role of the pulmonary circulation in the distribution of human fetal cardiac output during the second half of pregnancy. Circulation ; 9 4 : Yoo S-J, Lee YH: Screening fetal echocardiography, Made easy ; 16 : Yoo S-J, Lee YH, Cho KS: Abnormal three-vessel view on sonography: A clue to the diagnosis of congenital heart disease in the fetus. AJR 1999;172: Anderson RH, Ho SY: Sequential segmental analysis - description and categorization for the millennium. Cardiol Young 1997;7:98-116