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Cvs chd-csbrp

For Undergraduate students of Medicine (MBBS)

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Cvs chd-csbrp

  1. 1. Congenital Heart Disease CSBR.Prasad, MD., JAN-2015-CSBRP
  2. 2. Congenital Heart Disease - CHD • CHD is a general term designating abnormalities of the heart or great vessels that are present at birth • CHD arises from faulty embryogenesis during 3-8 weeks of gestation • Incidence - 5% JAN-2015-CSBRP
  3. 3. Twelve disorders account for about 85% of cases JAN-2015-CSBRP
  4. 4. JAN-2015-CSBRP
  5. 5. Congenital Heart Disease – Cardiac Development • The fetal heart consists of a single chamber until the fifth week of gestation • Then, it is divided by the development of interatrial and interventricular septa and by the formation of atrioventricular valves from endocardial cushions • A muscular interventricular septum grows upward from the apex toward the base of the heart. It is joined by the down-growing membranous septum, thereby separating right and left ventricles JAN-2015-CSBRP
  6. 6. CHD - Cardiac Development Day 15: Multipotent progenitor cells originate in lateral mesoderm and migrate to the midline Day 20: beating tube Day 28: Migration of neural crest cells, out flow tract & aortic arch formation Endocardial cushion formation Day 50: Formation of four chambered heart JAN-2015-CSBRP
  7. 7. CHD - Cardiac Development • This well orchestrated event involves many genes, transcription factors, signaling pathways • Each heart field is differentially marked by the expression of distinct set of genes • 1st heart field: Hand1 • (most of the left ventricle derived from this field) • 2nd heart field: Hand2 and FGF-10 • (Out flow tract, right ventricle and atria are derived from this field) • Pathways involved: Wnt, Hedghog, Notch-Delta • Growth factors involved: VEGF, TGF-beta, FGF • Specific micro-RNAs • Others: Hemodynamic forces in the developing heartJAN-2015-CSBRP
  8. 8. CHD - Cardiac Development JAN-2015-CSBRP
  9. 9. CATCH-22 Down’s syndrome Trisomies (13,18, 21) Most common genetic cause of congenital heart disease is Trisomy 21 (Down’s Syndrome) JAN-2015-CSBRP
  10. 10. Congenital Heart Disease – Environmental factors • Mutations in Csx/Mkx2-5 • Down syndrome (trisomy 21) and other trisomies • Turner syndrome and • Di George syndrome • Intrauterine influences • Rubella • Alcohol • Phenytoin • Amphetamines • Lithium • Estrogenic steroids and, • Thalidomide (historical) • Maternal diabetes JAN-2015-CSBRP
  11. 11. Congenital Heart Disease - gist • CHD is a consequence of faulty embryonic development • Either as misplaced structures OR • Eg: transposition of the great vessels) or • As an arrest in the progression of a normal structure (from an early stage to one that is more mature) • Eg: ASD JAN-2015-CSBRP
  12. 12. Congenital Heart Disease – Clinical features CHDs can be organized into three major categories: 1. Malformations causing a left-to-right shunt 2. Malformations causing a right-to-left shunt 3. Malformations causing an obstruction Definition: SHUNT: is an abnormal communication between chambers or blood vessels. JAN-2015-CSBRP
  13. 13. Congenital Heart Disease – Clinical features CHDs can be organized into three major categories: 1-Malformations causing a left-to-right shunt • Not initially associated with cyanosis • However, over the course of years, the patient may develop right to left shunt – EISENMENGER syndrome Left to right shunt results in pulmonary hypertension and associated changes in the pulmonary circulation. After the development of PHT the structural defects in CHDs are considered irreparable. JAN-2015-CSBRP
  14. 14. Common congenital left-to-right shunts JAN-2015-CSBRP
  15. 15. Congenital Heart Disease – Clinical features CHDs can be organized into three major categories: 2-Malformations causing a right-to-left shunt • Associated with cyanosis • Paradoxical embolism • Hypertrophic osteoarthropathy • Polycythemia JAN-2015-CSBRP
  16. 16. Congenital Heart Disease – Clinical features -Hypertrophic osteoarthropathy JAN-2015-CSBRP
  17. 17. JAN-2015-CSBRP 1. Tetralogy of Fallot 2. Transposition of the great arteries 3. Persistent truncus arteriosus 4. Tricuspid atresia, and 5. Total anomalous pulmonary venous connection Common congenital right-to-left shunts
  18. 18. Congenital Heart Disease – Clinical features CHDs can be organized into three major categories: 3-Malformations causing an obstruction • Abnormal narrowing of chambers, valves or blood vessels • Complete obstruction is called Atresia JAN-2015-CSBRP
  19. 19. Aortic coarctation with and without PDA JAN-2015-CSBRP
  20. 20. Atrial Septal Defect - ASD • Abnormal fixed opening in the atrial septum caused by incomplete tissue formation • Atria communicate with each other • Cf: Patent foramen ovale – PFO • Failure to close foramen that is a part of normal development • Both ASD & PFO result from defects in the formation of interatrial septum • Usually asymptomatic until adulthood • Common genetic variation near a gene MSX1 is strongly associated with the risk of an ASD JAN-2015-CSBRP
  21. 21. Atrial Septal Defect Development of interatrial septum • Septum primum - originates posteriorly • Ostium primum • During fetal development, this opening allows blood to be shunted from the right atrium to the left • Ostium secundum • The ostium secundum allows continued shunting of blood from the right atrium to the left • Septum secundum - originates anteriorly • Foramen ovale • The foramen ovale is continuous with the ostium secundum, again providing for continued shunting of blood JAN-2015-CSBRP
  22. 22. Atrial Septal Defect Development of interatrial septum JAN-2015-CSBRP
  23. 23. Atrial Septal Defect JAN-2015-CSBRP
  24. 24. Atrial Septal Defect Classification According to their location: • Secundum ASD: • 90% of all ASDs • Deficient septum secundum • No other associated anomalies • May be of any size, may be fenestrated • Primum anomalies • 5% of ASDs • AV valve abnormalities • Sinus venosus defects • 5% of ASDs • Located near the entrance of SVC • Associated with anomalous venous return to RAJAN-2015-CSBRP
  25. 25. Atrial Septal Defect • The most common type of ASD: The ostium secundum type, comprises 6–10% of all congenital heart diseases • Mechanisms: • Enlarged foramen ovale • Inadequate growth of the septum secundum, or • Excessive absorption of the septum primum • 10-20% of individuals may have MVP • Lutembacher's syndrome: Ostium secundum ASD + acquired MS JAN-2015-CSBRP
  26. 26. Patent Foramen Ovale • FO closes permanently in 80% of people by 2 years of age • In the remaining 20%, the unsealed flap can open if right-sided pressures become elevated • Pulmonary hypertension or even transient increases in right-sided pressures can produce brief periods of right-to-left shunting, with the possibility of paradoxical embolism • During a bowel movement • coughing or • Sneezing JAN-2015-CSBRP
  27. 27. Patent Ductus Arteriosus - PDA Ductus Arteriosus • The ductus arteriosus (DA) arises from the PA and joins the aorta • During intrauterine life, DA shunts blood from the PA to the aorta • Shortly after birth DA constricts and is closed after 1 to 2 days • This occurs in response to: • Increased arterial oxygenation • Decreased pulmonary vascular resistance and • Reduced levels of PGE2 locally • After a few months - ligamentum arteriosum JAN-2015-CSBRP
  28. 28. Patent Ductus Arteriosus - PDA Ductus Arteriosus JAN-2015-CSBRP
  29. 29. Ductus Arteriosus Ligamentum arteriosum JAN-2015-CSBRP
  30. 30. Patent Ductus Arteriosus - PDA • Constitute 7% of cases of CHDs (and 90% of them are isolated) • Maternal Rubella infection • PDA produces a characteristic continuous harsh “machinery-like” murmur • Complications: PHT, IE • Isolated PDA should be closed as early in life as is feasible • Preservation of ductal patency may be necessary in some CHDs • Eg: Aortic valve atresia JAN-2015-CSBRP
  31. 31. JAN-2015-CSBRP
  32. 32. Ventricular Septal Defect - VSD Ventricular septal defects occur as: 1. A small hole in the membranous septum 2. A large defect involving more than the membranous region (perimembranous defects) 3. Multiple defects in the muscular portion, which are more common anteriorly but can occur anywhere in the muscular septum or 4. Complete absence of the muscular septum (leaving a single ventricle) NOTE: A small septal defect may have little functional significance and may actually close spontaneously as the child matures JAN-2015-CSBRP
  33. 33. VSD JAN-2015-CSBRP
  34. 34. VSD • VSDs are classified according to their size and location 1. Membranous VSD 2. Infundibular VSD • Membranous VSD: Single • Infundubular VSD: Multiple, small • 70-80% are associated with other CHD • 20-30% occur in isolation JAN-2015-CSBRP
  35. 35. VSD • Large VSDs • cause PHT / RVH • Shunt reversal, cyanosis and death • Small VSDs • Present much later in life • 50% may close spontaneously JAN-2015-CSBRP
  36. 36. VSD (membranous type) JAN-2015-CSBRP
  37. 37. VSD (Infundibular type) JAN-2015-CSBRP
  38. 38. TETRALOGY OF FALLOT: • DOMINANT RIGHT-TO-LEFT SHUNT • IT IS THE MOST COMMON CYANOTIC CHD • Four cardinal features are: 1. VSD 2. Obstruction to right ventricular out flow i.e. PS 3. Over riding of aorta 4. Right ventricular hypertrophy (RVH) JAN-2015-CSBRP
  39. 39. JAN-2015-CSBRP
  40. 40. JAN-2015-CSBRP
  41. 41. TETRALOGY OF FALLOT: • Heart is usually enlarged – boot shaped • Due to marked right ventricular hypertrophy JAN-2015-CSBRP
  42. 42. Persistent Truncus Arteriosus • A common trunk for the origin of the aorta, pulmonary arteries • Results from absent / incomplete partitioning of the truncus arteriosus by the spiral septum • Truncus overrides the VSD and receives blood from both the ventricles • Clinical Features: • Recurrent pulmonary infections • Cyanosis • PCV / clubbing of fingers • PHT / heart failure / early death JAN-2015-CSBRP
  43. 43. JAN-2015-CSBRP
  44. 44. Truncus Arteriosus JAN-2015-CSBRP
  45. 45. Transposition of the Great Arteries - TGA • Ventriculoarterial discordance: The aorta arises from the right ventricle, while the pulmonary artery emanates from the left ventricle • Concordant AV connections: RA joining the RV and the LA emptying into the LV • Embryology: TGA results from abnormal formation of the truncal and aortopulmonary septa • Result: Separation of pulmonary and systemic circulation • A condition incompatible with postnatal life unless a shunt exists for adequate mixing of blood JAN-2015-CSBRP
  46. 46. Transposition of the Great Arteries - TGA JAN-2015-CSBRP
  47. 47. Transposition of the Great Arteries - TGA • The outlook for infants with TGA depends on the degree of blood “mixing” • Patients with TGA and a VSD often have a stable shunt • Patients with TGA and ASD / PDA have unstable shunt as they many close early in life • RVH • Left ventricle may become thin walled JAN-2015-CSBRP
  48. 48. Coarctation of the Aorta • F:M = 1:2 • Females with Turner syndrome are also frequently affected • There are two classic forms: • Infantile form (symptomatic in early childhood) • Adult form (with a ridge like infolding of the aorta) • 50% of cases accompanied by a bicuspid aortic valve • Patients may have berry aneurysms JAN-2015-CSBRP
  49. 49. Coarctation of the Aorta Pathogenesis JAN-2015-CSBRP
  50. 50. Coarctation of the Aorta • Clinical manifestations depend on: • Severity of the narrowing and • Patency of the ductus arteriosus • Coarctation of the aorta with a PDA: Cyanosis localized to the lower half of the body • Coarctation of the aorta without PDA: Unless the aortic constriction is severe most children are asymptomatic • Typically there is hypertension in the upper extremities with weak pulses and hypotension in the lower extremities JAN-2015-CSBRP
  51. 51. Coarctation of the Aorta • Development of collateral circulation: • Intercostal and • Internal mammary arteries • X-ray - “notching” of the undersurfaces of the ribs • Pan systolic murmurs / thrill • Concentric LVH • Treatment: • Surgical resection and end-to-end anastomosis or • Replacement of the affected aortic segment by a prosthetic graft JAN-2015-CSBRP
  52. 52. Coarctation of the Aorta Collaterals, Notched ribs JAN-2015-CSBRP
  53. 53. Coarctation of the Aorta JAN-2015-CSBRP
  54. 54. CHDs - Gist • Congenital heart disease represents defects of cardiac chambers or the great vessels • These either result in shunting of blood between the right and left circulation or cause out flow obstructions • Lesions range from relatively asymptomatic to rapidly fatal • Both Environmental (toxic or infectious) and genetic factors contribute • Left-to-right shunts are most common and are typically associated with ASDs, VSDs, or a PDA • Right-to-left shunts are most commonly caused by TOF or TGA • Obstructive lesions include aortic coarctation JAN-2015-CSBRP
  55. 55. E N D JAN-2015-CSBRP
  56. 56. JAN-2015-CSBRP
  57. 57. JAN-2015-CSBRP
  58. 58. JAN-2015-CSBRP

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