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Congenital Heart Diseases

10/9/14. Congenital Heart Diseases. Dr. Ksheera Cariappa Assistant Professor. The Heart. Embrology. Lateral mesoderm moves to midline Crescent of cells ---- first and second heart fields by day 15. First heart field – TBX5, Hand1 transcription factor expression

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Congenital Heart Diseases

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  1. 10/9/14 Congenital Heart Diseases Dr. Ksheera Cariappa Assistant Professor

  2. The Heart

  3. Embrology • Lateral mesoderm moves to midline • Crescent of cells ---- first and second heart fields by day 15. • First heart field – TBX5, Hand1 transcription factor expression • Second heart field--- Hand 2, FGF10 • Endocardium, myocardium, smooth muscle cells

  4. Embrology • First heart field -- LV • Second heart field – RV, RA,LA, Outflow tract • Day 20 beating tube • Heart chambers by 28days • Cells from neural crest migrate to form septae, endocardial cushions, aortic arches

  5. Embrology • Day 50– septation of ventricles, atria, atroventricular valves – four chambered heart • Signaling pathways – Wnt, VEGF, TGF- b, FGF, Notch pathways, bone morphogenetic factor. • Coordinated by micro RNA.

  6. Fetal circulation

  7. Post-Natal Circulation

  8. Etiopathogenesis • Sporadic genetic abnormalities • Single gene mutations • Small chromosomal deletions • Trisomy/monosomy • Single gene mutations encoding transcription factors for normal heart development

  9. Genetic causes of CHD

  10. Genetic causes : syndromic

  11. CHD • Acyanotic • Cyanotic • Obstructive

  12. Acyanotic CHD • Left to right shunts • VSD • ASD • Patent foramen ovale • PDA

  13. Cyanotic CHD • Right to left shunts • TOF • TGV • TA • TAPVC • PTA • Ebstein’s Anomaly

  14. Obstructive Congenital Heart Lesions • Impede the forward flow of blood and increase ventricular afterloads. • Pulmonary Stenosis and atresia • Aortic Stenosis and atresia • Coarctation of the Aorta

  15. Coarctation of the Aorta • Absent or weak femoral pulses. • Systolic pressure higher in upper extremities than in lower extremities; diastolic pressures are similar. • Harsh systolic murmur heard in the back.

  16. Coarctation of the Aorta • Males twice as frequently as females. • 98% of all coarctations at segment of aorta adjacent to ductus arteriosus. • Produced by both an external narrowing and an intraluminal membrane. • Blood flow to the lower body maintained through collateral vessels.

  17. Coarctation of the Aorta

  18. COA • Turners syndrome • Infantile form with PDA • Do not survive neonatal period • Adult form without PDA • LVH, Cardiomegaly

  19. CHD • that INCREASE Pulmonary Arterial Blood Flow • that DECREASE Pulmonary Arterial Blood Flow

  20. Congenital Heart Lesions that INCREASE Pulmonary Arterial Blood Flow • Atrial Septal Defect • Complete Atrioventricular Canal • Ventricular Septal Defect • Patent Ductus Arteriosis • CYANOTIC: • Total Anomalous Pulmonary Venous Connection • Truncus Arteriosus

  21. 1. Atrial Septal Defect • Acyanotic; asymptomatic, or dyspnea on exertion. • Right ventricular lift. • Fixed, widely split second heart sound. • Depending upon the location of the defect, there are 3 types of ASD

  22. Atrial Septal Defect • i) Fossa ovalis type or ostium secundum type is the most common form (90%). The defect is in the region of the fossa ovalis • ii) Ostium primum type about (5%)cases. The defect lies low in the interatrial septum adjacent to atrioventricular valves. There may be cleft in the aortic leaflet of the mitral valve producing mitral insufficiency.

  23. Atrial Septal Defect • iii) Sinus venosus type accounts for about 5% cases of ASD.The defect is located high in the interatrial septum near the entry of the superior vena cava.

  24. Morphologic Features The effects of ASD are produced due to left-to-right shunt at the atrial level with increased pulmonary flow. These effects are • Volume hypertrophy of the right atrium and right ventricle. • Enlargement and haemodynamic changes of tricuspid and pulmonary valves. • Focal or diffuse endocardial hypertrophy of the right atrium and right ventricle. • Volume atrophy of the left atrium and left ventricle. • Small-sized mitral and aortic orifices.

  25. Atrial Septal Defect

  26. Atrial septal defect fossa ovalis type, a schematic representation (LA = Left atrium; LV = Left ventricle; PV = Pulmonary vein; AO = Aorta; PT = Pulmonary trunk; RA = Right atrium; RV = Right ventricle; SVC = Superior vena cava; IVC = Inferior vena cava).

  27. Atrial Septal Defect

  28. Atrial Septal Defect

  29. 2. Ventricular Septal Defect • M/C of all CHDs (30%) • Asymptomatic if defect is small. • Heart failure with dyspnea, frequent respiratory infections, and poor growth if defect is large. • Membranous VSD (90%) • Infundibular VSD (Subpulmonic/subaortic) • Multiple : Swiss cheese septum

  30. Ventricular Septal Defect • Often one component of another more complex congenital heart lesion. • Heart is enlarged and lung fields are overcirculated. • Many of the defects will close spontaneously by age 7-8 years.

  31. Morphologic Features • The effects of VSD are produced due to ltrt shunt at the ventricular level, increased pulmonary flow and increased volume in the left side of the heart. These effects are: • Volume hypertrophy of the right ventricle. • Enlargement and haemodynamic changes in the tricuspid and pulmonary valves. • Endocardial hypertrophy of the right ventricle. • Pressure hypertrophy of the right atrium. • Volume hypertrophy of the left atrium & ventricle. • Enlargement and haemodynamic changes in the mitral and aortic valves.

  32. Ventricular Septal Defect

  33. 3. Patent Ductus Arteriosis • Accounts for 10% of CHDs • Murmur usually systolic, sometimes continuous, “machinery” • Poor feeding, respiratory distress, and frequent respiratory infections in infants with heart failure. • Physical exam and echocardiography.

  34. Cause • is not known but possibly it is due to continued synthesis of PGE2 after birth which keeps it patent as evidenced by association of PDA with respiratory distress syndrome in infants and pharmacologic closure of PDA with administration of indomethacin to suppress PGE2 synthesis

  35. In about 90% of cases, it occurs as an isolated defect, while in the remaining cases it may be associated with other anomalies like VSD, coarctation of aorta and pulmonary or aortic stenosis. • A patent ductus may be upto 2 cm in length and upto 1 cm in diameter

  36. Morphologic Features The effects of PDA on heart occur due to left-to-right shunt at the level of ductus resulting in increased pulmonary flow and increased volume in the left heart. These effects are: i) Volume hypertrophy of the left atrium and left ventricle. ii) Enlargement and haemodynamic changes of the mitral and pulmonary valves. iii) Enlargement of the ascending aorta.

  37. Patent Ductus Arteriosus

  38. Patent Ductus Arteriosis

  39. Patent Ductus Arteriosis

  40. Congenital Heart Lesions that DECREASE Pulmonary Arterial Blood Flow • Tetralogy of Fallot • Transposition of the Great Arteries • Tricuspid Atresia • Ebstein’s Anomaly

  41. 4.Tetralogy of Fallot • M/C cyanotic CHD (10% of all) • Its components are: i) Ventricular septal defect (VSD) (‘shunt’). ii) Displacement of the aorta to right so that it overrides the VSD. iii) Pulmonary stenosis(‘obstruction’). iv) Right ventricular hypertrophy.

  42. Tetralogy of Fallot • Addition of an atrial septal defect falls in the category of Pentalogy of Fallot. • Absence of PS  Triology • Hypoxic spells and squatting. • Cyanosis and clubbing.

  43. Clinically • The severity of the clinical manifestations is related to two factors: extent of pulmonary stenosis and the size of VSD. • Boot shaped heart • Accordingly, there are two forms of tetralogy: cyanotic and acyanotic:

  44. Cyanotic Tetralogy • PS is > VSD so that there is more resistance to the outflow of blood from rt ventricle resulting in rtlt shunt at the ventricular level and cyanosis. The effects on heart are : i) Pressure hypertrophy of the right atrium and right ventricle. ii) Smaller and abnormal tricuspid valve. iii) Smaller left atrium and left ventricle. iv) Enlarged aortic orifice.

  45. Acyanotic Tetralogy • VSD is larger and pulmonary stenosis is mild so that there is mainly left-to-right shunt with increased pulmonary flow and increased volume in the left heart but no cyanosis. The effects on heart are : i) Pressure hypertrophy of the right ventricle and right atrium. ii) Volume hypertrophy of the left atrium and left ventricle. iii) Enlargement of mitral and aortic orifices.

  46. Tetralogy of Fallot

  47. Tetralogy of Fallot

  48. THANK YOU

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