Congenital Heart Diseases in Children.pptx

Description of common congenerous heart diseases

1. Congenital Heart D iseases in Children Presented b y - Dr. Writtika Majumdar (DCH student) Dr. MD. Ashik Kamal Alvee (MD Student) D e pt. o f Paediatrics Dhaka Medical College Hospital Seminar on

2. Objectives : Epidemiology of CHD Developmental changes of Heart Risk factors Classification Approach to a child with CHD Discussion about common CHD including important clinical findings, investigations, treatment and natural history.

3. What Is Congenital Heart D isease Congenital heart d iseases are problems with the heart's structure that are present at birth. These defects can involve: -The interior walls of the heart -The valves inside the heart -The arteries and veins that carry blood to the heart or out to the body

4. Epidemiology CHD occurs in approx. 0.8% of live births. Incidence is higher in stillborns (3-4%), Spontaneous abortuses (10-25%) and premature infants. A pprox. 2-3/1000 newborn will be symptomatic with heart disease in 1 st year of life. WHO reports the incidence of CHD in Bangladesh is 6% (2015)

5. Development Of Heart

6. The Fetal Circulation

7. Closure of umbilical arteries Closure of umbilical vein Closure of ductus venosus Closure of ductus arteriosus Closure of foramen ovale Enlargement of pulmonary veins Changes in fetal circulation after birth

8. Maternal drug use Chromosomal abnormality Maternal diseases Risk Factors For CHDs Rubella infection: PDA, PS SLE: Complete Heart Block DM, HTN Maternal CHD Epilepsy Anemia Down syndrome: ASD, VSD Turner syndrome: CoA, Aortic stenosis Marfan’s syndrome Aortic aneurysm Warferin therapy: PDA,PS Fetal alcohol syndrome: ASD, VSD, TOF Thalidomide Sulfonamide Anti epileptic drug

9. Classification Of CHD Acyanotic Cyanotic Left to right shunt Outflow obstruction Ventricular septal defect Atrial septal defect Persistent ductus arteriosus Pulmonary Stenosis Aortic Stenosis Coarctation of aorta Te t ralogy of Fallot (TOF) Transposition of the great arteries Tricuspid Atresia Truncus arteriosus Total anomalous pulmonary venous return Common Ventricle

10. Relative Frequency of Common Congenital Heart Defects Ventricular septal defect Atrial septal defect (Secundum) Patent ductus arteriosus Coarctation of aorta Tetralogy of Fallot Pulmonary Valve Stenosis Aortic Valve Stenosis Transposition of great arteries Hypoplastic left ventricle 30-35% 6-8% 6-8% 5-7% 5-7% 5-7% 4 -7% 3-5 % 1-3%

11. How To Approach a Patient With Congenital Heart Disease

12. Approach to a patient with CHD History Taking Physical Examination Investigations

13. HISTORY TAKING

14. Acyanotic Heart Disease Cyanotic Heart Disease Feeding difficulty Dyspnea or shortness of breath Excessive sweating during exertion Recurrent respiratory infections Growth impairment Exercise intolerance Easy fatigability Bluish or blackish discoloration From Birth: TGA, TAPVR Later onset: TOF Adoption of squatting position (after 18 month ususally ) Convulsion/Unconsciousness Sudden weakness of one side of body History of present Illness

15. Birth History Maternal history Perinatal history Exposure to drugs Alcohol intake TORCH infection Any chronic Disease Exposure to radiation or chemical Bad obstetrical history Cyanosis at birth Gestational age Birth weight Features of heart failure Any birth defects (heart-related or not)

16. Family History When 2 first degree relatives have CHD , there is risk of 20-30 % for subsequent child . If 1 st born has CHD, risk of a 2 nd child is 2-6% History of chromosomal abnormality( Down, Marfan , Turner ) History of still birth/ spontaneous abortion

17. Physical Examination

18. General Examination Appearance: -Ill looking -Features of Down syndrome, Turner syndrome, Marfan syndrome may be present Cyanosis: Central or Peripheral Pallor (due to malnutrition, shock) Plethora (TOF) Clubbing Edema Engorged neck vein (in older children)

19. Anemia Clubbing Plethora Cyanosis

20. Respiratory rate: Tachypnea may be present Pulse: General Examination Bradycardia : complete heart block in TGA High volume pulse with wide pulse pressure : AR in TOF Collapsing pulse : PDA Radiofemoral delay : CoA

21. Blood Pressure Upper extr e mities HTN: CoA (CoA is suspected when systolic pressure <20mm hg in legs than arms.) A narrow pulse pressure: severe AS. Spo2 Low in cyanotic CHD or CoA, PDA with pulmonary HTN. General Examination

22. Anthropometry Growth impairment Low birth weight, premature infant have more chance of CHD.

23. Precordi um Examination Inspection Bulged precordium- VSD , ASD, TOF Hyperdynamic precordium- VSD ,PDA Palpation Abnormal apex beat -Tapping: Mitral stenosis -Thrusting : MR, AR -Heaving : AS, Systemic HTN -Double apical impulse: Hypertrophic cardiomyopathy

24. Thrill : -Left sternal border- VSD -Rt upper sternal border- Severe AS Left parasternal heave : -Indicates RVH Precordi um Examination

25. Auscultation Heart sounds Added sounds -Murmurs -Pericardial rub Precordi um Examination

26. Liver: Palpable in CCF Spleen: Palpable in IE Ascites: Heart failure Focal neurological sign: Cerebral abcess (TOF, VSD) Systemic examination

27. Investigations

28. Chest X-ray ECG Echocardiography Cardiac Catheterization Hematological investigations -Complete blood count -ESR, HCT -CRP -ASO Titre Investigations

29. Brief Discussion About Common Congenital Heart Defects

30. Acyanotic Heart Disease Left to Right shunt

31. The commonest congenital cardiac disease ( 30-3 5%) Can occur as Isolated defect or component of other complex defect such as TOF May occur in any portion of ventricular septum but most are membranous type Ventricular septal defect

32. Types of VSD According To Location: Membranous VSD : 70-80 % Muscular VSD : 5-20 % Supracristal :5-30 % Endocardial cushion: 5-8% According to size: Small : < 5mm Moderate: 5-10mm Large: > 10mm

33. Hemodynamics Of VSD

34. V SD: Symptoms Small defect Asymptomatic Failure to thrive Recurrent URTI Profuse perspiration Moderate to Large defect

35. V SD: Physical findings

36. VSD: Investigations Chest X-ray: Cardiomegaly , increase vascular marking. ECG: Large VSD: LVH, Biventricular hypertrophy Echocardiogram: Position, size of VSD, shunt size, pressure gradient Cardiac catheterization: When laboratory data not fits or suspected pulmonary vascular disease

37. Treatment Of VSD Small VSD: Reassurance No restriction on physical activity

38. Moderate to large VSD: 1.Medical treatment Diuretic: Frusemide (1-3mg/kg/day) Afterload reducing agent: - Enalapril (0.1-0.5mg/kg/day-12 hourly) -Captopril( 0.05-0.1mg/kg/dose-8hourly) -Digoxin: if f/o heart failure present 2.Transcatheter device closure 3.Surgical treartment Treatment Of VSD

39. Treatment Of VSD contd. Indications of s urgical treatment: At any age : Large VSD where clinical symptoms & FTT can not be controlled medically At 6-12 months : Large VSD with pulmonary HTN (even if symptoms controlled by medication) At age >24month : Qp:Qs ratio>2:1 Contraindication of surgery: Severe pulmonary vascular disease, non responsive to pulmonary vasodilator

40. Prognosis of VSD Small VSD : closed spontaneously 30-50% during 1 st 2 year of life ( muscular upto 80%, membranous 35%) Moderate to large VSD closes upto 8% Supracristal variety : never closes spontaneously

41. Atrial Septal Defect ASD is an opening permitting shunting of blood between two atria Female: male -3:1 Types of ASD: Ostium Secondum (commonest) Ostium Primum Sinus Venosus type

42. Hemodynamics of ASD

43. ASD: Symptoms Small defect Asymptomatic Failure to thrive Recurrent URTI Moderate to Large defect

44. ASD: Physical findings

45. ASD: investigation Chest x-ray: Cardiomegaly with increased pulmonary vasculature marking.

46. ECG: Large defect: Right axis deviation due to RVH Confirmatory diagnosis: Echocardiography ASD investigation

47. Treatment o f ASD Medical treatment: -Diuretics ( Frusemide or Thiazide or combination) -Digoxin Surgical treatment: Small secondum ASD with minimal shunt without right ventricular enlargement: No need of closure Device or surgical closure

48. Transcatheter or surgical closure: -For all symptomatic patient -Asymptomatic with Qp:Qs ratio at least 2:1 -Patient with right ventricular enlargement; Timing of closure: preferably after 1 st year and before entry into school. Treatment o f ASD

49. Devices Used For ASD Closure

50. Prognosis of ASD Spontaneous closure of osmium secundum defect is rare beyond 1st year of life. Secundum ASD is well tolerated during childhood ,usually go asymptomatic detected 3rd decade or later Congestive cardiac failure is the most common cause of death in secundum ASD

51. Patent Ductus Arteriosus (PDA) PDA results from persistence of ductus arteriosus after birth Aortic blood shunted left to right into pulmonary artery. Female :male - 3: 1 Common problem in premature infant. Maternal rubella infection associated with PDA

52. Haemodynamics of PDA

53. PDA : Symptoms Small defect Asymptomatic Failure to thrive Exercise intolerance Easy fatiguability Repeated RTI Moderate to Large defect

54. PDA : Physical findings

55. PDA: Investigations Chest X-ray: Cardiomegaly Increased Pulmonary vascular markings ECG: l eft ventricular hypertrophy , biventricular hypertrophy Confirmatory Diagnosis: Echocardiogram Cardiac catheterization

56. PDA: Diagnosis

57. PDA: Management 1. Medical Treatment : ( effective if given within 72 hours ) Indomethacin :3 doses, 0.2mg/kg 12-24 hours apart I/V - slowly over 30 minutes Ibuprofen : Oral, For 3 days . 1 st day 10mg /kg, 2 nd and 3 rd dose 5mg/kg 2. Transcatheter device 3. Surgical closure 4. Prophylaxis for infective endocarditis

58. PDA: Management Surgical Treatment: By Transcatheter device or by lateral thoracotomy Preferably before 1 year, as Heart failure develops earlier in PDA. Small PDA: By intravascular coils Moderate to large PDA: By umbrella like device.

59. PDA: Management

60. Complication of PDA Blood Ductus H eart lungs Pulmonary HTN Pulmonary artery embolism Infective endocarditis Congestive Cardiac failure Systemic embolism Calcification Of ductus

61. Eisenmenger syndrome Shunt reversal May occur in VSD PDA AV canal defect

62. Cyanotic Heart Disease Right to left shunt

63. Tetralogy OF Fallot (TOF) First described by Eti e nne L A Fallot Accounts for 5-7 % of all congenital heart diseases. F our components : Obstruction of Right ventricular outflow or Pulmonary stenosis VSD Overriding of the aorta (over the VSD) RVH

64. Hemodynamics Of TOF

65. TOF: Pathological Effects L ess blood in pulmonary circulation (Oligemic lung field) Low oxygen saturation in systemic c irculation (Cyanosis) Polycythemia Growth failure

66. Symptoms of TOF Cyanosis Paroxysmal hyper cyanotic attack Easy fatigability and dyspnea on exertion Growth failure or developmental delay

67. TOF: Hypercyanotic Spell Hall mark of severe TOF Usually occurs during first 2 year of life, most commonly at 4-6 month of life. Most frequently occurs in the morning on awakening or vigorous cry

68. TOF: Hypercyanotic Spell Hypoxic spells are characterized by: Sudden onset of hyperapnea , sometimes gasping respiration and syncope Sudden deepening of cyanosis Alteration of consciousness, sometimes convulsion and hemiparesis Temporary disappearance or decrease in the intensity of the systolic murmur. Metabolic acidosis

69. TOF: Signs General examination: Clubbing Cyanosis Polycythemia Stunting Examination of Precordium: Bulged chest. Tapping apex beat Left parasternal heave Systolic thrill at left upper ICS S1 is normal, S2 is loud & single A loud ejection systolic murmur at upper sternal border

70. TOF: Diagnosis CBC: Hb: Elevated Haematocrit : Elevated PBF: Microcytic hypochromic anemia Chest X-ray : Boot shaped heart Oligemic lung field

71. TOF: Diagnosis ECG: Right axis deviation RVH Confirmatory: Echocardiography

72. Management of TOF Non-surgical management Iron , vitamin and mineral supplementation Adequate fluid intake High calorie diet Oral propranolol 0. 2 5 -1 mg/kg/day prophylaxis

73. Management of TOF Neonates with severe cyanosis is treated with I/V infusion of prostaglandin E1 Treatment of cyanotic spell Place the infant in knee-chest position. Older children squat spontaneously Oxygen inhalation Inj Morphine

74. Management of TOF I/V normal saline 10ml/kg bolus followed by maintenance fluid I/V NaHCO3 I/V Propranolol If not controlled: Phenylephrine 10-20 microgram/kg bolus IM or SC, then 0.1-0.5 mircrogram / kg/min infusion IV

75. Management of TOF Surgical management Total correction: Palliative surgery : Blalock Taussig Shunt

76. Complication s of TOF Blood Growth & Development H eart Brain Cerebral abcess Thrombo- embolism Stroke Infective endocarditis Cardiac failure Severe polycythemia Failure of growth and development

77. Transposition Of Great Arteries (TGA) 2 nd most common cyanotic heart defect. The Aorta arises from right ventricle The pulmonary trunk arises from left ventricle

78. TGA: Clinical features Symptoms Cyanosis Tachypnea Signs Left parasternal heave may present 2 nd heart sound single, loud and may split. Murmur absent or a soft ejection systolic murmur may present

79. Chest X ray: E gg on string appearance of heart Normal to increased pulmonary vascular marking Mild cardiomegaly Investigations of TGA

80. Echocardiogrm Transposed ventricular-arterial connection (Confirmation of diagnosis) Investigations of TGA

81. Management Non-invasive management I V Prostaglandin Invasive management Balloon atrial septostomy Permanent correction by Atrial switch operation Balloon atrial septostomy

82. Treatment availability Of CHDs in Bangladesh Device closure available at: NICVD, NHF&RI, BSMMU, BICH, BIRDEM, CMH, APOLLO hospital Surgical treatments are available at: Inside Dhaka: NICVD, NHF&RI, BSMMU, BICH, BIRDEM, Lab Aid Outside Dhaka: Fortis Escort Hospital ( Chittagong & Khulna) Metropolitan Hospital ( Chittagong )

83. 7 th -14 th F ebruary

84. Thank You…