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Congenital heart disease

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Congenital heart disease

  1. 1. CONGENITAL HEART DISEASE Presenter : Dr Abdulrafey Usmani Mmed. Paediatrics
  2. 2. INTRODUCTION  Congenital cardiovascular disease is defined as an abnormality in cardio-circulatory structure or function that is present at birth, even if it is discovered much later.  Congenital cardiovascular malformations usually result from altered embryonic development of a normal structure or failure of such a structure to progress beyond an early stage of embryonic or fetal development.
  3. 3. EPIDEMIOLOGY  About 0.8% of live births are complicated by a cardiovascular malformation.  PDA, Ebstein anomaly of the tricuspid valve, and atrial septal defect (ASD) are more common in females, whereas aortic valve stenosis, coarctation of the aorta, hypoplastic left heart, pulmonary and tricuspid atresia, and transposition of the great arteries (TGA) are more common in males.  Extracardiac anomalies occur in about 25% of infants with significant cardiac disease, and their presence may significantly ↑mortality.
  4. 4.  General concepts regarding the etiology of congenital malformations o Unknown in almost 90% of cases o Environmental factors: Maternal rubella, ingestion of thalidomide and isotretinoin early during gestation, and chronic maternal alcohol abuse o Genetic factors are also clearly involved o well-defined associations with certain chromosomal abnormalities (e.g., trisomies 13, 15, 18, and 21 and Turner syndrome) ETIOLOGY
  5. 5. CLASSIFICATION OF CONGENITAL HEART DISEASES  Cyanotic or non-cyanotic  Non-cyanotic: ASD, VSD, sinus venosus defect, patent ductus arteriosus, aortic stenosis, pulmonary stenosis, aortic coarctation  Cyanotic: Tetralogy of Fallot, Ebstein’s anomaly, transposition of the great arteries, Eisenmenger’s syndrome, truncus arteriosus, tricuspid atresia, total anomalous pulmonary venous return “5 Ts and 2 Es”
  6. 6. CLASSIFICATION OF CONGENITAL HEART DISEASES  Simple or complex Simple includes ASD, VSD, or singular valvular abnormalities (Ebstein’s anomaly) Complex includes those with multiple defects, AV canal defects, or “single” ventricle physiology
  8. 8. NORMAL EMBRYOLOGIC DEVELOPMENT OF THE HEART  During the first month of gestation, the primitive, straight cardiac tube is formed, comprising the : Sinuatrium (Most Cephalad),  Primitive Ventricle,  Bulbus Cordis, And Truncus Arteriosus (Most Caudad) In Series.
  9. 9. NORMAL EMBRYOLOGIC DEVELOPMENT OF THE HEART  In the second month of gestation, this tube doubles over on itself to form two parallel pumping systems, each with two chambers and a great artery.  The two atria develop from the sinuatrium.  The AV canal is divided by the endocardial cushions into tricuspid and mitral orifices, and  The right and left ventricles develop from the primitive ventricle and bulbus cordis.
  10. 10. NORMAL EMBRYOLOGIC DEVELOPMENT OF HEART  Differential growth of myocardial cells causes the straight cardiac tube to bear to the right, and the bulboventricular portion of the tube doubles over on itself, bringing the ventricles side by side.  Migration of the AV canal to the right and of the ventricular septum to the left serves to align each ventricle with its appropriate AV valve.  At the distal end of the cardiac tube, the bulbus cordis divides into a subaortic and a subpulmonary muscular conus;  The subpulmonary conus elongates and the subaortic conus resorbs,allowing the aorta to move posteriorly and connect with the left ventricle.
  12. 12. ABNORMAL DEVELOPMENT  A host of anomalies can result from defects in this basic developmental pattern.  Double-inlet left ventricle is observed if the tricuspid orifice does not align over the right ventricle.
  13. 13. ABNORMAL DEVELOPMENT  The various types of persistent truncus arteriosus result from failure of the truncus to divide into main pulmonary artery and aorta.  Double-outlet anomalies of the right ventricle are produced by failure of either the subpulmonary or subaortic conus to resorb whereas resorption of the subpulmonary instead of the subaortic conus may lead to TGA.
  14. 14. ATRIA  The primitive sinuatrium is separated into right and left atria by the down growth from its roof of the septum primum toward the AV canal, thereby creating an inferior interatrial ostium primum opening.  Numerous perforations form in the anterosuperior portion of the septum primum as the septum secundum begins to develop to the right of the septum primum, the coalescence of these perforations forms the ostium secundum.  The septum secundum completely separates the atrial chambers except for a central opening—the fossa ovalis—that is covered by tissue of the septum primum, forming the valve of the foramen
  15. 15. ATRIA  Fusion of the endocardial cushions anteriorly and posteriorly divides the AV canal into tricuspid and mitral inlets.  The inferior portion of the atrial septum, the superior portion of the ventricular septum, and portions of the septal leaflets of both the tricuspid and mitral valves are formed from the endocardial cushions.  The integrity of the atrial septum depends on growth of the septum primum and septum secundum and proper fusion of the endocardial cushions.  ASDs and various degrees of AV defect are the result of developmental deficiencies of this process.
  16. 16. VENTRICLES  Partitioning of the ventricles occurs as cephalic growth of the main ventricular septum results in its fusion with the endocardial cushions and the infundibular or conus septum.  Defects in the ventricular septum may occur because of :  a deficiency of septal substance;  malalignment of septal components in different planes preventing their fusion; or  an overly long conus, keeping the septal components apart.  These defects probably generate the VSDs in tetralogy of Fallot and transposition
  17. 17. GREAT ARTERIES  The truncus arteriosus is connected to the dorsal aorta in the embryo by six pairs of aortic arches.  Partition of the truncus arteriosus into two great arteries is a result of the fusion of tissue arising from the back wall of the vessel and the truncus septum.  Rotation of the truncus coils the aortopulmonary septum and creates the normal spiral relation between aorta and pulmonary artery.  Semilunar valves and their related sinuses are created by absorption and hollowing out of tissue at the distal side of the truncus ridges.  Aortopulmonary septal defect and persistent truncus arteriosus represent various degrees of partitioning failure
  20. 20. ACYANOTIC CHD  Left-to-Right Shunts  The three most important and common types of acyanotic congenital heart disease are:  • Atrial septal defect.  • Ventricular septal defect.  • Patent ductus arteriosus.
  21. 21. ATRIAL SEPTAL DEFECT (ASD)  Four types of ASDs or interatrial communications exist: Ostium primum, Ostium secundum,  Sinus venosus, and  Coronary sinus defects.
  22. 22. ASD
  23. 23. OSTIUM PRIMUM •15% of all ASDs •Occur if the septum primum and endocardial cushion fail to fuse and are often associated with abnormalities in other structures derived from the endocardial cushion (e.g., mitral and tricuspid valves). •Occurs in the lower part of the atrial septum, adjacent to the atrioventricular valves, deformed and regurgitant •Also associated with cleft in anterior mitral valve leaflet or septal leaflet of the tricuspid valve. • Common in Down syndrome
  25. 25. OSTIUM SECUNDUM •Defect in fossa ovalis, midseptal •Represents 75% of all ASDs •Occurs when the septum secundum does not enlarge sufficiently to cover the ostium secundum •Associated with mitral valve
  26. 26. SINUS VENOSUS DEFECT •Located in the upper atrial septum, near the entry of the superior vena cava •Represents 10% of all ASD defects •Associated with anomalous pulmonary venous connection from the right lung to the superior vena cava or right
  27. 27. PATENT FORAMEN OVALE (PFO) •The foramen ovale is a tunnel-like space between the overlying septum secundum and septum primum and typically closes in 75% of patients at birth by fusion of the septum primum and secundum. •In utero the foramen ovale is necessary for blood flow across the fetal atrial septum. •Oxygenated blood from the placenta returns to the IVC, crosses the foramen ovale, and enters the systemic circulation. •In about 25% of pts, a PFO persists into adulthood.
  28. 28. VENTRICULAR SEPTAL DEFECT (VSD)  VSD is an abnormal opening in the ventricular septum, which allows free communication between the Rt & Lt ventricles  Accounts for 25% of CHD
  29. 29. VENTRICULAR SEPTAL DEFECT  The ventricular septum can be divided into three major components—inlet, trabecular, and outlet—all abutting on a small membranous septum lying just underneath the aortic valve.  VSDs are classified into three main categories according to their location and margins .  Muscular VSDs are bordered entirely by myocardium and can be trabecular, inlet, or outlet in location.  Membranous VSDs often have inlet, outlet, or trabecular extension and are bordered in part by fibrous continuity between the leaflets of an AV valve and an arterial valve.  Doubly committed subarterial VSDs are more common in Asian patients, are situated in the outlet septum, and are bordered by fibrous continuity of the aortic and pulmonary valves.
  31. 31. VSD  A restrictive VSD does not cause significant hemodynamic derangement and may close spontaneously during childhood and sometimes in adult life.  A moderately restrictive VSD imposes a hemodynamic burden on the left ventricle, which leads to left atrial and ventricular dilation and dysfunction as well as a variable increase in pulmonary vascular resistance.  A large or nonrestrictive VSD features left ventricular volume overload early in life with a progressive rise in pulmonary artery pressure and a fall in left-to-right
  32. 32. PATENT DUCTUS ARTERIOSUS  The ductus arteriosus derives from the left sixth primitive aortic arch and connects the proximal left pulmonary artery to the descending aorta, just distal to the left subclavian artery.  The ductus is widely patent in the normal fetus, carrying deoxygenated blood from the right ventricle through the descending aorta to the placenta, where the blood is oxygenated.  Functional closure of the ductus from vasoconstriction occurs shortly after a term birth, whereas anatomical closure from intimal proliferation and fibrosis takes several weeks to complete.  patency of a ductus is a true congenital malformation
  33. 33. PDA
  35. 35. TETRALOGY OF FALLOT (TOF)  The 4 components of TOF are  An outlet VSD,  Obstruction to right ventricular outflow,  Overriding of the aorta (<50%), and  Right ventricular hypertrophy.  The fundamental abnormality contributing to each of these features is anterior and cephalad deviation of the outlet septum, which is malaligned with respect to the trabecular septum.  The dominant site of obstruction is usually at the sub-valve level.  Progressive hypoxemia in the first years of life is expected. Survival to adult life is rare without
  36. 36. TOF
  37. 37. TRANSPOSITION OF THE GREAT ARTERIES  This is a common and potentially lethal form of heart disease in newborns and infants.  The malformation consists of the origin of the aorta from the morphological RV and that of the PA from the morphological LV.  Consequently, the pulmonary and systemic circulations are connected in parallel rather than the normal in-series connection.  In one circuit, systemic venous blood passes to the right atrium, the right ventricle, and then to the aorta.  In the other, pulmonary venous blood passes through the left atrium and ventricle to the pulmonary artery.  This situation is incompatible with life unless mixing of the 2circuits occurs.
  38. 38. TRANSPOSITION OF THE GREAT ARTERIES 1=transposition of the great arteries; 2=atrial baffles; 3=pulmonary vein blood flow through tricuspid valve to RV; 4=IVC and SVC blood flow through mitral valve to LV
  39. 39. EBSTEIN ANOMALY  The common feature in all cases of Ebstein anomaly is apical displacement of the septal tricuspid leaflet in conjunction with leaflet dysplasia.  Although the anterior leaflet is never displaced apically, it may be adherent to the free wall of the right ventricle, causing right ventricular outflow tract obstruction.  The displacement of the tricuspid valve results in “Atrialization” (functioning as an atrial chamber) of the inflow tract of the right ventricle and consequently produces a variably small, functional right ventricle.  Associated anomalies include PFO or ASD in ≈ 50% of patients.