Congenital Anomalies of The Heart & Blood Vessels

1. بسم الله الرحمن الرحيم Cardiovascular System CMD-312 Congenital Anomalies of The Heart & Blood Vessels Adviced by: Dr. masood ahmad By: MOHAMMED ALI ALANZ

2. List the developmental anomalies of the heart and main arteries arising from aortic arch. 1 2 Describe atrial and ventricular septa defect. Describe the developmental anomalies of the main arteries arising from the aortic arch 3 Objectives:

3. Introduction • The Heart: • It’s a conical muscular organ having apex directed downward, forwards and to the left, and base directed backward and upwards . • divided into 4 chambers : 2 atria (Right, left) 2 ventricles (Right, left). • The 2 atria separated from ventricles by atrio-ventricular sulcus or coronary sulcus . • The 2 ventricles are separated from each other by anterior & posterior interventricular groove .

5. Congenital anomalies of the heart and blood vessels: is a general term used to describe abnormalities of the heart or great vessels that are present from birth. Most such disorders arise from faulty embryogenesis during gestational weeks 3 through 8, when major cardiovascular structures develop .

6. classification of the congenital heart defect : • dependent on : • Dependent on the anatomical structure defect . • Malposition • Atrialseptal defect • Ventricalseptal defect • Truncal defect • Defect in the heart vessels 2. Dependent on the time of developmental .

7. Most major defects arise during weeks 6 and 7 of development .

8. 3. Dependent on the presence of cyanosis: Cyanotic. Acynotic. 4. Dependent on the type of shunt: • Left-to-right shunt. • Right-to-left shunt. • obstructive (no shunt).

9. * Classification of congenital heart diseases:

10. Congenital Heart Defect and Great vessels: • Dextrocradia • Ectopia cordis • Atrial septal defect • Ventricular septal defect • Truncus arteriosus • Transposition of the great arteries • Unequal division of the truncus arteriosus • Corbiloculare • Undivided truncus arteriosus • Aortic or pulmonary stenosis and atresia • Transposition of the ascending aorta and pulmonary trunk

11. Atrial septal :- • Develpent : • Roof of primitive atrium ----) septum primum • b/w septum primum &A.V cushions (foramen primum) • Septum primum grow down & deganart ---) foramen secundum • Roof of primitive atrium right side ----) septum secundum

12. The septum primum will close the foramen primum . • b/w margin of septum primum & septum secundum form a foramen ovale --)close at birth

13. * Atrial Septal Defect : • Definition: • An ASD is an abnormal opening in the atrial septum that allows communication of blood between the left and right atria (not to be confused with a patent foramen ovale, present in up to one-third of normal individuals). • Three major types of ASDs, classified according to their location in the septum.

14. * Atrial Septal Defect : • These three types are: • Ostiumsecundum defect (90%): defect located at and resulting from a deficient or fenestrated oval fossa. • Ostiumprimum defect (5%): adjacent to the AV valves and are usually associated with a cleft anterior mitral leaflet. • Sinus venosus defect (5%): are located near the entrance of the superior vena cava. They are commonly accompanied by anomalous connections of right pulmonary veins to the superior vena cava or right atrium.

15. Ostium secundum defect Ostium primum defect

16. Sinus venosus defect

17. Ventricular septal • Developent : • It have 2 part • Muscular part : floor of primitive ventricle ----)muscular septum has 2 horns ventral reaches rt. Bulbar & ventral A.V cushions b/w free edge of muscular septum & atrioventricular cushions form a interventricular foramen

18. Membranous part : - Bulbar & A.V cushions ----) membranous septum ----)close the interventricular foramen

19. Ventricular septal defect • Definition : • VSD is abnormal opening in the ventricular septum allowing shunting of blood ; abnormal communication between left and right ventricle . • Most VSDs occur in the membranous part of the interventrecular sptum • VSDs are associated with other cardiac malformations ,esp.tetraology of fallot.

20. VSDs are due to : • complete absence of the membranous part of the septum (Membranous VSD) • complete absence the muscular part of the septum (Muscular VSD) • complete absence of both membranous and muscular parts (Cortrilocolarebiatriatum) • Partial defect of the membranous part of the septum (Rogers disease )

21. Membranous VSD Muscular VSD

22. Tetralogy of the Fallot :- • This is classic group of four cardiac defect consists of the following : • Pulmonary stenosis .( obstruction to right ventricular out flow ) • Ventricular septal defect . • Overriding ( Dextroposition )of the aorta . • Right ventricular hypertrophy . The pulmonary trunk is usually small and there may be varying degrees of pulmonary artery stenosis as well.

24. Transposition of The Great Arteries ( TGA) :- • Characterized by aorta arising from right ventricle and pulmonary artery from the left ventricle and often associated with other cardiac abnormalities

25. The defect result from the aorta lies anterior & the right of the pulmonary trunk and arise interiorly from the morphologic right ventricle + the pulmonary trunk arise from the morphologic left ventricle . • TGA often associated with other cardiac anomalies ASD.VSD

26. Aortic stenosis & atresia:- • In this anomaly the edges of the valve are usually fused to form a dome with a narrow opening • The valvular stenosis cause extra work for the heart lead to hypertrophy of LV & abnormal heart sound ( murmurs) • Aortic atresia is present when obstruction of the aorta or its valve is complete .

27. Aortic Arch Anomalies: • Patent ductus arteriosus • Coarctation of the Aorta • Double Aortic Arch • Right Arch of the Aorta • Abnormal origin of the right subcluvian artery

28. Patent ductus arteriosus • Before birth the ductus arteriosus is allow the passage of the blood from pulmonary trunk to arch of aorta due to nonfunctional lungs • After birth by few hours (+)O2 tension in blood ---)bradykinin ---)contracion&clossure of ductus arteriosus • At 3mont tunica intema ---) fibrous tissue ----) fibrosis of duct ---)ligamentum arteriosum

29. The ductus arteriosus (DA) is the vascular connection between the pulmonary artery and the aortic arch • … the blood passes through the ductus arteriosus from the pulmonary artery to the arch of aorta

31. Coarctation of the aorta:- • Carctation = constriction • It is characterized by a constriction of varying length to the aorta . • the site of defect may be preductal coarctation or postductal coarctation .

32. Narrowing is just superior to ductus arteriosus. It is associated with PDA Preductal Narrowing of the arch is opposite to ductus arteriousus. There is discrete ridge like infolding of the aorta just opposite the closed DA Ductal Narrowing is just inferior to the ductus arteriosus Postductal

34. Double Aortic Arch • It is characterized by vascular ring around the trachea and esophagus . • It is result from failure of the distal part of the right dorsal aorta to disappear that lead to right and left arches form . • Usually the right arch of the aorta is the large one and passed posterior to the trachea and esophagus . • It is cause varying degree of compression of trachea and esophagus .

36. Right arch of the aorta • When the entire right dorsal aorta persists and the distal part of the left dorsal aorta involutes , a right aortic arch result . • There are two main types : • right arch of the aorta without a retroesophageal component : • the DA ( or ligamentum arteriosum )pass from the right pulmonary artery to the right arch of the aorta . • right arch of the aorta with a retroesophageal component : • A small left arch of the aorta probably involuted , leaving the right arch of the aorta posterior to the esophagus • The DA( or ligamentum arteriosum ) attaches to the distal part of the arch of the aorta and forms a ring which may constrict the esophagus and trachea .

37. Abnormal origin of the right subcluvian artery • It occurs when the right 4th aortic arch and the right dorsal aorta cranial to the seventh cervical intersegmental artery abnormally regress . • The right subclavian artery will come to lie on the left side just inferior to the left subclavian artery. • The abnormal artery must cross the midline posterior of the trachea and oesophagus to supply the right upper limb and then constrict them .

38. Thank you Any question?

39. summary • congenital heart defect : Dependent on anatomical structure defect . • Atrial septal defect • Ventrical septal defect Defect in the heart vessels: • Patent ductus arteriosus • Coarctation of the Aorta • Double Aortic Arch • Right Arch of the Aorta

40. References • Longman’s Embryology by sadler • Clinical Anatomy for medical students by Richard S. Snell • Before we are born by Moore • Dividson Medicine • Some web site for the picture