embryology of the heart and the great vessels：的心脏和大血管的胚胎学

Embryology of Embryology of the heart and the heart and the great vesselsthe great vessels Dr Gerrit Engelbrecht Dept of Radiology UFS ESTABLISHMENT OF THE CARDIOGENIC FIELD FORMATION AND POSITION OF THE HEART TUBE FORMATION OF THE CARDIAC LOOP MOLECULAR REGULATION OF CARDIAC DEVELOPMENT DEVELOPMENT OF THE SINUS VENOSUS FORMATION OF THE CARDIAC SEPTAE FORMATION OF THE CONDUCTING SYSTEM OF THE HEART VASCULAR DEVELOPMENT Steps in the embryology Steps in the embryology of the vascular systemof the vascular system ESTABLISHMENT OF ESTABLISHMENT OF THE CARDIOGENIC THE CARDIOGENIC FIELDFIELD Dorsal view of a late presomite embryo (approximately 18 days) after removal of the amnion. Prospective myoblasts and hemangioblasts reside in the splanchnic mesoderm in front of the neural plate and on each side of the embryo after migrating up from the primitive streak Transverse section through a similar-staged embryo to show the position of the blood islands in the splanchnic mesoderm layer. With time, the islands unite and form a horseshoe-shaped endothelial-lined tube surrounded by myoblasts. This region is known as the cardiogenic field In addition to the cardiogenic region, other blood islands appear bilaterally, parallel and close to the midline of the embryonic shield. These islands form a pair of longitudinal vessels, the dorsal aortae. 223 Cephalocaudal section through a similar staged embryo showing the position of the pericardial cavity and cardiogenic field. FORMATION AND POSITION OF FORMATION AND POSITION OF THE HEART THE HEART TUBETUBE 2 processes responsible for positioning of the heart 1. Folding of the embryo in a cephalocaudal direction 2. Simultanous folding laterally Cephalocaudal folding Figures showing effects of the rapid growth of the brain on positioning of the heart. Initially the cardiogenic area and the pericardial cavity are in front of the buccopharyngeal membrane. A. 18 days. B. 20 days. C. 21 days. D. 22 days Lateral folding of the embryo Formation of the cardiac Formation of the cardiac looploop Bulbus cordisBulbus cordis Primitive ventriclePrimitive ventricle The primitive atrium and The primitive atrium and sinus venosussinus venosus Abnormalities of cardiac Abnormalities of cardiac loopinglooping Dextrocardia, in which the heart lies on the right side of the thorax instead of the left, is caused because the heart loops to the left instead of the right. Dextrocardia may coincide with situs inversus Molecular regulation of Molecular regulation of cardiac developmentcardiac development Dependent on the activation of two transcription factors NKX2.5 Specifies cardiogenic field Septation Conduction system TBX5 Septation Development of the sinus Development of the sinus venosusvenosus 4th week receives blood from right and left sinus horns 5th week Obliteration R umbilical vein left vitelline vein Development of the sinus Development of the sinus venosus(2)venosus(2) 10th week left common cardinal vein obliterates Development of the Development of the venous valvesvenous valves Formation of the cardiac Formation of the cardiac septaeseptae The major septae are formed between the 27 and 37th days of development It is a simultanuous process if the following areas Septum formation in the common atrium Septum formation in the atrioventricular canal Septum formation in the truncus arteriosus and conus cordis Septum formation in the ventricles Septum formation in the Septum formation in the common atriacommon atria At the end of the fourth week, a sickle-shaped crest grows from the roof of the common atrium into the lumen. This crest is the first portion of the septum primum A. 30 days (6 mm). B. Same stage as A, viewed from the right. Septum formation of the Septum formation of the common atria (2)common atria (2) C. 33 days (9 mm). D. Same stage as C, viewed from the right When the lumen of the right atrium expands as a result of incorporation of the sinus horn, a new crescent-shaped fold appears. This new fold, the septum secundum never forms a complete partion in the atrial cavity Septum formation of the Septum formation of the common atria(3)common atria(3) E. 37 days (14 mm) F. Newborn. G. The atrial septum from the right; same stage as F. When the upper part of the septum primum gradually disappears, the remaining part becomes the valve of the oval foramen. Further differentiation of Further differentiation of the atriathe atria Coronal sections through the heart to show development of the smoothwalled portions of the right and left atrium. Both the wall of the right sinus horn (blue) and the pulmonary veins (red) are incorporated into the heart to form the smooth-walled parts of the atria. Septum formation of the Septum formation of the atrioventricular canalatrioventricular canal At the end of the fourth week, two mesenchymal cushions, the atrioventricular endocardial cushions, appear at the superior and inferior borders of the atrioventricular canal, two additional lateral cushions appear at the left and right borders. At the end of the fifth week there is complete fusion of the superior and inferior cushions with complete division of the canal into left and right orifices. Atrioventricular valvesAtrioventricular valves Formation of the atrioventricular valves and chordae tendineae. The valves are hollowed out from the ventricular side but remain attached to the ventricular wall by the chordae tendineae. Clinical correlatesClinical correlates Heart defectsHeart defects Largest category of birth defects Multifactorial ( viruses, medicines, alchohol, diabetes, hypertension. Genetic syndromes: DiGeorge and Downs TBX5 gene defect: Holt Oram syndrome( ASD + pre axial abnormalities. ASD Premature closure of the oval foramen Endocardial cushion defects AV canal( persistent AV canal) Tricuspid atresia ASDASD Persistent Persistent common common AV canalAV canal Tricuspid atresiaTricuspid atresia Septum formation of the truncus arteriosus Septum formation of the truncus arteriosus and conus cordisand conus cordis Fifth week, pairs of opposing ridges appear in the truncus and conus cordis Septum formation of the truncus arteriosus Septum formation of the truncus arteriosus and conus cordisand conus cordis Proliferations of the right and left conus cushions, combined with proliferation of the inferior endocardial cushion, close the interventricular foramen and form the membranous portion of the interventricular septum. A. 6 weeks(12 mm). B. Beginning of the seventh week (14.5 mm). C. End of the seventh week (20 mm). Septum formation of the Septum formation of the ventriclesventricles End of the fourth week the two primitive ventricles start to expand. The medial walls of the expanding ventricles become apposed and gradually merge, forming the muscular interventricular septum Semilunar valvesSemilunar valves When partitioning of the truncus is almost complete, primordia of the semilunar valves become visible as small tubercles found on the main truncus swellings. One of each pair is assigned to the pulmonary and aortic channels, respectively A third tubercle appears in both channels opposite the fused truncus swellings. Gradually the tubercles hollow out at their upper surface, forming the semilunar valves. Recent evidence shows that neural crest cells contribute to formation of these valves. Longitudinal section through the semilunar valvesLongitudinal section through the semilunar valves 6 weeks seven weeks 9 weeks Clinical correlatesClinical correlates VSD related defectsVSD related defects Isolated lesion Conotruncal lesions : Tetralogy of Fallot Persistent truncus arteriosus Transposition of the great vessels Pulmonar valvular atresia Aorta valvular stenosis and artresia Isolated VSDIsolated VSD Tetralogy of FallotTetralogy of Fallot Persistent truncus Persistent truncus arteriosusarteriosus Transportation of the great vesselsTransportation of the great vessels Pulmonar valvular atresiaPulmonar valvular atresia Aortic valvular stenosis Aortic valvular stenosis and atresiaand atresia Formation of the conducting Formation of the conducting system of the heartsystem of the heart Sinu atrial node Initially the pacemaker for the heart lies in the caudal part of the left cardiac tube. Later the sinus venosus assumes this function, and as the sinus is incorporated into the right atrium, pacemaker tissue lies near the opening of the superior vena cava. Thus, the sinuatrial node is formed. Atrioventricular node The atrioventricular node and bundle (bundle of His) are derived from two sources: (a) cells in the left wall of the sinus venosus (b) cells from the atrioventricular canal. Once the sinus venosus is incorporated into the right atrium, these cells lie in their final position at the base of the interatrial septum. Vascular developmentVascular development Arterial system Venous system Arterial systemArterial system Aortic arches Vitelline and umbilical arteries Aortic archesAortic arches Key factsKey facts Arise from the aortic sac(truncus arteriosus ) to pharyngeal arches ( 4-5 weeks) Terminate in the left and right dorsal aorta These arches and vessels appear in a cranial to caudal sequence and not all simultanously The aortic sac also forms left and right horns which give rise to the brachiocephalic artery and proximal arch respectively Aortic archesAortic arches Aortic Aortic archesarches Ductus Ductus arteriosusarteriosus Vitelline and umbilical Vitelline and umbilical arteriesarteries Vitelline arteries fuse and form the Coeliac, SMA and IMA Umbilical arteries Initially paired ventral branches of the aorta Fourth week acquire a secondary connection with the aorta the common iliac artery After birth the proximal part persist as the internal iliac and internal iliac and superior vesical and the distal part obliterate to form the medial umbilical ligaments. Clinical correlatesClinical correlates Arterial system defectsArterial system defects Patent ductus arteriosus Coarctation of the aorta ( preductal and post ductal) Abnormal origin of the right subclavian artery Right aortic arch Interrupted aortic arch Coarctation of the aortaCoarctation of the aorta Abnormal origin of the Abnormal origin of the right subclavian arteryright subclavian artery Double aortic archDouble aortic arch Interrupted aortic archInterrupted aortic arch Venous systemVenous system Vitelline veins Umbilical veins Cardinal veins End of fourth week Vitelline veinsVitelline veins Forms a plexus around the duodenum, forms the portal vein. Pass through the septum transversum Hepatic cords grow into the septum and form the hepatic sinusoids Connects to the sinus venosum Vitelline veins (2)Vitelline veins (2) Enlargement of the right vitelline vein. Forms the hepatic cardiac part of the IVC The SMV derives from the right vitelline vein The left vitelline vein disappears. Umbilical veinsUmbilical veins Proximal and distal right umbilical vein disappears Proximal left umbilical vein disappears The left distal umbilical vein remains and carry blood from the placenta to the liver The ductus venosus form between the left umbilical vein and right hepatic cardiac channel, it bypasses the sinusoidal plexus Both are obliterated after birth to form the ligamentum teres and venosum respectively Anterior cardinal veins, which drain the cephalic part of the embryo Posterior cardinal veins, which drain the rest of the embryo. Common cardinal veins. During the fourth week this forms a symmetrical system Cardinal veins From the fifth to the seventh week additional veins are formed: a) Subcardinal veins, which mainly drain the kidneys; b) Sacrocardinal veins, which drain the lower extremities c) Supracardinal veins, which drain the body wall by way of the intercostal veins, taking over the functions of the posterior cardinal veins Formation of the vena cava system is characterized by the appearance of anastomoses between left and right in such a manner that the blood from the left is channeled to the right side. Anterior cardinal veins - left brachocephalic vein Left posterior cardinal vein terminal portion - left superior intercostal vein Right common cardinal vein + proximal right anterior cardinal vein- SVC Subcardinal veins - left renal vein Left subcardinal vein distal portion - left gonadal vein Right subcardinal vein - renal segment of the IVC Sacrocardinal veins - left common iliac vein Right sacro cardinal vein - sacro cardinal segment When the renal segment of the inferior vena cava connects with the hepatic segment, which is derived from the right vitelline vein, the inferior vena cava, consisting of hepatic, renal, and sacrocardinal segments, is complete. Right 4th to 11th intercostal veins - right supracardinal vein + post cardinal vein - azygos vena Left 4th to 7th intercostal veins - left supracardinal vein ( hemiazygos vein ) - Azygos vein Clinical correlatesClinical correlates Left superior vena cava: Persistence of the left anterior cardinal vein Obliteration of the common cardinal and anterior cardinal veins on the right Double superior vena cava: