Congenital Diseases of the Heart in Children.pptx

Congenital Diseases of the Heart in children

1. Congenital Diseases of the Heart in Children

2. Introduction Congenital heart disease is the structural malformations of the heart or great vessels, present at birth. It is the most common congenital malformations

3. FETAL CIRCULATION

4. Etiology Heredity Consanguineous marriage Genetic disorder Chromosomal aberrations Fetal & maternal teratogenic infections Alcoholic intake by mother Maternal IDDM Birth asphyxia

5. Classification Of CHD

6. Acyanotic Absence of cyanosis Tachypnea Frequent chest pain Precordial bulge due to cardiomegaly Tricuspid or mitral delayed diastolic murmur

7. Cyanotic Polycythemia Clubbing Normal pulmonary arterial pressure Diminished arterial pressure Increased pulmonary arterial pressure

8. Ventricular septal defect It is an abnormal opening in the septum b/w Rt & Lt ventricles It is about 25% of all CHD

9. Classification of VSD Anatomical classification Perimembraneous -75% Muscular - 5-20% Inlet – 5-8% Outlet

10. Geodynamic classification Group1 – small VSD, normal PVR, small L to R shunt Group2 – moderate VSD, variable PVR, significant L to R shunt Group3 – large VSD, moderately high PVR, significant L to R shunt Group4 – large VSD , very high PVR, significant L to R / R to L shunt

11. ECHO based classification Large defect = diameter of aorta Moderate = 1/3 – 2/3 of diameter of aorta Small = <1/3 of diameter of aorta Pinhole = <2mm

12. Pathophysiology Flow of O2 blood from high pressure Lt ven (VSD) low pressure Rt ven Increased Rt ven pressure & pulmonary arterial pressure Increased pulmonary circulation (increased PVR)

13. Clinical Features Recurrent chest infection Congestive cardiac failure Failure to thrive Exertional dyspnea Feeding difficulties Tachypnea

14. Poor wt. gain Hepatomegaly Biventricular hypertrophy Loud pansystolic murmur heard in the Lt sternal border Functional diastolic murmur

15. Diagnosis H/O P/E Auscultation of harsh systolic murmur Pulmonary second sound(P 2 ) is heard

16. Chest X-ray Enlargement of the heart Increased pulmonary vascular marking ECG Biventricular hypertrophy

17. Two dimensional ecocardiogram with Doppler study To identify size, number, site of defect Cardiac catheterization Site of the shunt

18. Complications FTT Recurrent pneumonia Infective endocarditis Repeated episodes of CCF Pulmonary hypertension Pulmonary stenosis Aortic regurgitation

19. Eisenmenger syndrome when the ratio of pulmonary to systemic vascular resistance approaches 1:1, the shunt becomes bidirectional & the child becomes cyanotic with disappearance of CCF signs

20. Atrial septal defect It is an abnormal opening between Rt & Lt atria resulting Lt to Rt shunting of blood It accounts for 9%

21. Types of ASD’s Ostium secundum ASD Ostium Primum ASD Sinus Venosus ASD

22. Ostium secundum ASD An abnormal opening in the middle of the atrial septum presents due to abnormal development of the septum secundum

23. Ostium Primum ASD An abnormal opening at the bottom of the atrial septum due to improper development of the septum Primum

24. Sinus Venosus ASD An abnormal opening at the top of the atrial septum

25. Pathophysiology Flow of O2 blood from high pressure Lt atrium (ASD) low pressure Rt atrium Overload to Rt vent & Rt vent dilation Increased blood flow to lungs Elevated pulmonary artery pressure

26. Enlargement of Rt ventricle Typical murmur of systolic ejection is heard over upper sternal border Congestive cardiac failure

27. Diagnosis H/O P/E * Ostium Secundum ASD Auscultation of soft systolic murmur heard at upper sternal border P 2 is widely split & fixed

28. * Ostium Primum ASD Auscultation of soft systolic murmur heard at Lower sternal border Chest X-ray Rt atrial & Vent dilation Increased pulmonary vascular marking ECG Rt vent hypertrophy & Rt axis deviation

29. Two dimensional ecocardiogram with Doppler study To identify size, number, site of defect Cardiac catheterization Site of the shunt

30. Management CCF & recurrent chest infections are treated Arrhythmias should be medically Antibiotic prophylaxis for endocarditis Supportive nursing care

31. A catheter is inserted into a blood vessel in the groin and guided to the heart. Through the catheter, a mesh patch or plug is put into place to close the hole. The heart tissue grows around the mesh, permanently sealing the hole.

32. Surgical Management Good nutrition with treatment of iron deficiency anemia CCF & recurrent chest infections are treated Antibiotic prophylaxis for endocarditis

33. Complications Infective endocarditis Repeated episodes of CCF Pulmonary Atrial hypertension Post operative complications after surgical repair

34. Patent Ductus Arteriosus It is the persistent vascular connection b/w pulmonary artery & the aorta Due to higher pressure in the aorta the blood flows in the ductus from the aorta

35. It is more common in female baby

36. Pathophysiology Blood flows from aorta to pulmonary artery Overload of pulmonary O 2 blood of systemic circulation flows back to pulmonary circulation Increased vascular pressure in the pulmonary tree & volume load on Lt heart Pulmonary Hypertension

37. Clinical Features Tachypnea Bounding pulse Corrigan pulsation in the neck Increased systolic pressure & low diastolic pressure with wide pulse pressure

38. Exertional dyspnea Feeding difficulties Precordial pain Hoarseness of voice Slow wt. gain CCF

39. Diagnosis H/O P/E Auscultation of continuous murmur heard at second Lf intercostal space (or) below Lt clavicle (or) at Lt sternal border P 2 is widely split

40. Chest X-ray Cardiomegaly Increased pulmonary vascular marking ECG Lt atrial dilation & Lt ventricular Hypertrophy

41. Two dimensional ecocardiogram with Doppler study To identify size, number, site of defect Cardiac catheterization Extent of problems

42. Indomethacin 0.1 – 0.25 Anti prostaglandin agent Aspirin Mefanemic acid Medical Management

43. Complications Infective endocarditis Repeated episodes of CCF Pulmonary hypertension Pulmonary vascular occlusive disease Thromboembolism Rheumatic heart disease Eisenmenger syndrome

44. Tetralogy of Fallot it is characterized by the combination of four defects Pulmonary stenosis Ventricular septal defect Overriding of Aorta Rt ventricular hypertrophy

45. Pathophysiology

46. Condition Description A: Pulmonary stenosis A narrowing of the right ventricular outflow tract and can occur at the pulmonary valve . Infundibular pulmonic stenosis is mostly caused by overgrowth of the heart muscle wall . The degree of stenosis varies between individuals with TOF, and is the primary determinant of symptoms and severity. B: Overriding of aorta An aortic valve with biventricular connection, that is, it is situated above the ventricular septal defect and connected to both the right and the left ventricle. The degree to which the aorta is attached to the right ventricle is referred to as its degree of " override”

47. C: ventricular septal defect (VSD) A hole between the two bottom chambers (ventricles) of the heart. The defect is centred around the most superior aspect of the ventricular septum.. In some cases thickening of the septum can narrow the margins of the defect . D: Right ventricular hypertrophy The right ventricle is more muscular than normal, causing a characteristic boot-shaped appearance as seen by chest X-ray. Due to the disarrangement of the external ventricular septum, the right ventricular wall increases in size to deal with the increased obstruction to the right outflow tract .

48. Clinical Features Cyanosis of lips & nail beds Dyspnea with crying Hypoxic- anoxic or blue spells Tet - spell Mental slowness

49. Diagnosis H/O P/E Auscultation of harsh systolic ejection murmur heard at upper Lt sternal border P 2 is single

50. Chest X-ray poorly vascularized lung fields A small boat shaped heart Concavity of the pulmonary artery segment ECG Rt axis deviation & Rt ventricular Hypertrophy

51. Two dimensional ecocardiogram with Doppler study To identify size, number, site of defect Cardiac catheterization Degree of extension

52. Management Cyanosis should be managed O2 therapy Correction of dehydration & anemia Antibiotic therapy Knee chest position Sedatives

53. Complications Hypoxic spells Tet spells Polycythemia Cardiac arrhythmias Complete heart block

54. Oral propranolol Vasopressors I/V fluid therapy Prostaglandin E1

55. Coarctation of Aorta It is a distinct narrowing or a long segment hypoplasia of the aortic arch, usually distal to the subclavian artery.

56. 1.Preductal Coarctation 2.Ductal Coarctation 3.Postductal Coarctation Types of COA

57. Preductal coarctation results when an intracardiac anomaly during fetal life decreases blood flow through the left side of the heart, leading to hypoplastic development of the aorta. Preductal Coarctation

58. Ductal Coarctation The narrowing occurs at the insertion of the ductus arteriosus . This kind usually appears when the ductus arteriosus closes.

59. The narrowing is distal to the insertion of the ductus arteriosus . Even with an open ductus arteriosus blood flow to the lower body can be impaired. This type is most common in adults Postductal Coarctation

60. Pathophysiology High load on Lt ventricles Elevation of both systolic & diastolic pressures

61. Clinical Features CCF Dyspnea Feeding difficulties Pitting edema Gallop rhythm Failure to thrive Systolic murmur is usually found in interscapular area

62. Fatigue Cramps Head ache Blood pressure in the arms is much higher than in the legs Dilated & tortuous collaterals may be seen over the interscapular area. This is called as Suzman sign

63. Diagnosis Chest X-ray Lt ventricular enlargement ECG Rt ventricular Hypertrophy