Congenital heart disease (chd)

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Congenital heart disease (chd)

  1. 1. Congenital Heart Disease (CHD) Lecture for C-I students 1
  2. 2. Congenital Heart Disease (CHD)Epidemiology of CHD Incidence - 8/1000 live births - 3-4/100 still born - 2/100 premature infants excluding PDA -10-25/100 abortuses• Most congenital defects are well tolerated during fetal life.Etiology - Unknown in most cases - Genetic factors - single gene defect - Chromosomal abnormality. - Environmental factors - Gender differences in type of CHD 2
  3. 3. CHD… Relative Frequency of Congenital Heart Lesions Lesions % of all Lesions- Ventricular septal defect 25-30- A trial septal defect (Secundum) 6-8- Patent ductus arteriosus 6-8- Coarctation of aorta 5-7- Tetralogy of Fallot 5-7- Pulomnary Valve Sterosis 5-7- Aortic Valve Stenosis 4-7- d-Transposition of great arteries 3-5- Hypoplastic left ventricle 1-3 3
  4. 4. CHD… Relative Frequency … Lesions % of all Lesions- Hypoplastic right ventricle 1-3- Truncus arteriosus 1-2- Total anomalous PVR 1-2- Tricuspid atresia 1-2- Single ventricle 1-2- Double-outlet right ventricle 1-2- Others 5-10 4
  5. 5. CHD …Clues for Evaluation of an Infant with suspected CHD1. On History and Physical Examination color) • Acyanotic • Cyanotic2. Chest roentgenogram • Normal • Increased/Plethora pulmonary blood flow • Decreased/Oligemia 5
  6. 6. CHD …3.Electrocardiogram - Right - Left hypertrophy - Biventricular Final diagnosis - Precordial examination - Echocardiography 6
  7. 7. I. Acyanotic Congenital Heart Diseases1. Left to Right Shunt Lesions 1.1 Atrial Septal Defect  Defect occur in any portion of the atrium - Ostium secundum (at fossa ovalis) - Ostium primum (ECD) (lower atrial septum) - Sinus venosus (upper atrial septum) Pathophysiology  Left to right shunt - Transatrial in OS & SV - Transatrial & transventricular in OP 7
  8. 8. Acyanotic CHD…Clinical Manifestations  Most are asymptomatic  Right ventricular lift  Wide & fixed split of 2nd heart sound  Systolic ejection murmur  Mid-diastolic murmur at tricuspid area  Holosystolic murmur at mitral area in OP (ECD) 8
  9. 9. Acyanotic CHD…Diagnosis  Clinical  CXR - Right. V & A enlargement - Large pulm. artery - ↑ed pulm. vascularity  ECG - volume overload, - right axis deviation - minor right ventricular conduction delay  Echocardiography  CatheterizationPrognosis - Well toleratedComplications – - pulm. Hypertension, Eismenger syndromeTreatment  Surgery - For all symptomatic ASD - Asymptomatic patients with shunt ratio > 2:1 9
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  16. 16. Acyanotic CHD…1.2 Ventricular Septal Defect  The most common cardiac malformation  Defect occur in any portion of the septum - Majority membranous - Muscular – Single or Swiss-cheese defect Pathophysiology  Lt to Rt shunt  Restrictive if defect is small (0.5cm2)  Non-restrictive - large defect (> 1cm2) - Right and left vent. Pressure equalized 16
  17. 17. Acyanotic CHD…Clinical Manifestation  Small defects with trivial Lt to Rt Shunt - Most common - Asymptomatic - Loud, harsh holosystolic M at LLSB  Large defects - Excessive pulmonary blood flow - Pulmonary hypertension - Dyspnea, feeding difficulties, poor growth, perspiration, recurrent plum. infection, heart failure - Less harsh but more blowing holosystolic murmur - Accentuated 2nd heart sound - Mid-diastolic apical M when shunt ratio > 2:1 17
  18. 18. Acyanotic CHD…Diagnosis - Clinical - CXR - Cardiomegaly - Plethoric lung - ECG - EchocardiographyPrognosis - 30-50% small defects close by 2 yr of age - Rarely moderate to large defects close 18
  19. 19. Acyanotic CHD…Complications - Infective endocarditis - Recurrent lung infection - Heart failure - Pulmonary HTN - Acquired pulmonary stenosis -- aortic valve regurgitationTreatment - Small defects - reassurance - Prophylaxis against IE - Large defects - medical treatment (control of CHF, promoting normal growth prevent IE, prevent development of p. HTN) - Surgical repair between 6-12m 19
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  23. 23. Acyanotic CHD…1.3 Patent Ductus Arteriosus  Functional closure soon after birth  Aortic end of the ductus distal to the origin of left subclavian artery and the other end at bifurcation of pulmonary artery.  Male to female ratio 1:2 Pathology – Deficiency of mucoid endothelial layer & muscular media in term infant. 23
  24. 24. Acyanotic CHD…Pathophysiology Lt to Rt shunt - size - ratio of pulm. to systemic resistance Reversal of shunt Clinical Manifestation Asymptomatic in small ductus Wide pulse pressure Bounding pulse large Continuous or machinery M at 2nd Left ICS 24
  25. 25. Acyanotic CHD…Diagnosis - Clinical - Chest X-ray - ECG - EchocardiographyPrognosis - Small PDA - normal life - Large PDA - CHFComplications - Infective Endocarditis/Endarteritis - CHF - Embolization - Pulmonary HTNTreatment - Medical - Surgical closure 25
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  28. 28. Acyanotic CHD…2. Obstructive Lesions 2.1 Pulmonic Stenosis - 4 types - Valvular - Infundibular - Supra valvular - Peripheral Pathophysiology - Rt outlet obstruction → Pressure work ↓ Rt vent. hyperthropy 28
  29. 29. Acyanotic CHD…Clinical Manifestation - Mild to moderate - asymptomatic - Critical stenosis - Systolic ejection murmur - Heart failure in neonates & infants - Rarely cyanosis 29
  30. 30. Acyanotic CHD…Diagnosis - Clinical - CXR - Rt vent. hypertrophy - reduced pulm. blood flow - ECG - EchocardiographyPrognosis - good in mild to moderateComplications - CHF in severe Ps - rarely IETreatment - vavular PS - ballon valvoplasty - surgery 30
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  32. 32. Acyanotic CHD…2.2 Aortic Stenosis - Valvular - the commonest - Supra valvular - Subvalvular (subaortic) 32
  33. 33. Acyanotic CHD…Clinical Manifestation  Mild stenosis - Normal pulse & apical impulse - Systolic ejection M - Normal to enlarged heart size  Critical stenosis - Left ventricular failure - pulm. edema, cardiomegaly - Weak peripheral pulses - Weak systolic M - Paradoxical split 2nd heart sound 33
  34. 34. Acyanotic CHD…Diagnosis - Clinical - CXR - ECG - Echocardiography - Graded exercise testingPrognosis is good for mild to moderateTreatment - Balloon valvoplasty - Surgical 34
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  38. 38. Acyanotic CHD…2.3 Coarctation of the Aorta • Occur at any site from the arch of aorta to iliac bifurcation • 98% juxta ductal Pathogenesis • In utero arch hypoplasia Rt heart output passes through the ductus 38
  39. 39. Acyanotic CHD…Clinical Manifestation  Hypertension → mechanical obstruction → renal  Differential cyanosis → pink upper extr. → cyanosed lower extr.  Classic signs - Disparity in pulse & BP - Radio-femoral delay - Systolic M at LMSB & inter-scapular area 39
  40. 40. Acyanotic CHD…Diagnosis - Clinical - CXR - cardiomegaly & pulm. congestion - Notching of ribs - ECG - EchocardiographyPrognosis – Untreated cases succumb by 20-40 yearsComplications - CVA - I/E - AneurysmsTreatment - Medical - IV PGE1 in neonatal age - Surgery 40
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  45. 45. Acyanotic CHD…3. Pulmonary Vascular Disease (Eismenger syndrome) - Occur in shunt lesions VSD - mainly ASD PDA - Reversal of shunt due to pulm. HTN → Cyanosis 45
  46. 46. Acyanotic CHD…4. Regurgitant Lessons - Pulmonary valvular insufficiency - Congenital mitral valve insufficiency - Mitral valve prolapse 46
  47. 47. II. Cyanotic Congenital Heart Disease1. Cyanotic lesions with decreased pulmonary blood flow1.1 Tetralogy of Fallot Consists: 1. Rt ventricular outflow obst. 2. Ventricular septal defect 3. Dextroposition of the aorta 4. Right ventricular hypertrophy 47
  48. 48. Cyanotic CHD…Pathophysiology - Outflow obstruction - Hypertrophy of subpulmonic muslce - Normal or small pulmonary valve annulus - Rarely pulmonary atresia - VSD - Non-restrictive, located just below aortic valve - Aortic arch is right side in 20% - Right ventricular output shunts to the aorta 48
  49. 49. Cyanotic CHD…Clinical Manifestation - Rarely pink TOF - in the absence of obstruction - Cyanosis - Clubbing - Squatting position in walking children - Paroxysmal hypercyanotic attacks  occur during 1st 2 years - Systolic ejection M - Delayed growth & development - Single 2nd heart sound 49
  50. 50. Cyanotic CHD…Diagnosis CXR - Narrow base & uplifted apex - A boot or wooden shoe - decreased pulm. vascularity - Right side aortic arch in 20% ECG EchocardiographyComplication - Cerebral thrombosis - in < 2 years - Brain abscess - Infective endocarditis - Polycythemia - CHF in pink TOF 50
  51. 51. Cyanotic CHD…Treatment Severe outflow obstruction - Medical Px - PGE1 infusion - Prevent dehydration - Partial exchange transfusion - Oral propranolol for tet spells - Surgery - Blalock Taussig - Total correction 51
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  58. 58. Cyanotic CHD…1.2 Pulmonary Atresia - With VSD - Extreme form of TOF - Without VSD - No egress of blood from Rt vent. - Shunt through foramen ovale to Lt atrium Left ventricle systemic circulation Aorta pulmonic circulation - Hypoplastic right ventricle (PDA) 58
  59. 59. Cyanotic CHD…Clinical Manifestation - Cyanosis at birth - Respiratory distress - Single 2nd heart sound - No murmurDiagnosis - CXR - ECG - EchocardiographyTreatment - PGE1 - Surgery 59
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  61. 61. Cyanotic CHD…1.3 Tricuspid atresia - No outlet from Right atrium to Left vent. - Systemic venous return Rt atrium Lt atrium Left ventricule systemic Pulmonic (VSD, PDA) 61
  62. 62. Cyanotic CHD…Clinical Manifestation - Cyanosis at birth - Polycythemia - Easily fatiguability - Exertional dyspneaDiagnosis - EXR -Pulm. Under circulation - ECG -Lt axis deviation & Lt vent. hypertrophy - Echocardiography 62
  63. 63. Cyanotic CHD…Treatment - PGE1 - Surgery - Aortico - pulmonary Shunt - Bidirectional Glenn shunt - Modified Fontan operation 63
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  65. 65. Cyanotic CHD…1.4 Ebstein Anomaly of the tricuspid valve - Down ward displacement of the tricuspid valve - Right ventricle with two parts - atrialized - normal ventricular myocardium - Abnormal tricuspid valve - Huge Rt atrium - Tricuspid regurgitation - Compromised Rt ventricular function 65
  66. 66. Cyanotic CHD…Clinical Manifestations - Easly fatiguability - Cyanosis - Dysrhythmia - Rt to Lt shunt through formen ovale - Holosystolic M at tricuspid area - Heart failureDiagnosis - CXR - box shaped heart - ECG - Right BBB - Superior axis deviationTreatment - PGE1 - Surgery 66
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  70. 70. Cyanotic CHD…2.Cyanotic CHD With increased pulmonary blood flow 2.1 Transposition of GA a. D -TGA (uncorrected) - Systemic venous return to Rt atrium Normal - Pulmonary venous return to Lt atrium - Aorta arises from Right ventricle - Pulm. artery arises from Lt vent. Pathology 70
  71. 71. Cyanotic CHD…*Systemic & Pulmonary Circulations Consists of two parallel circuits*Survival is with associated - patent foramen ovale or - VSD or - PDAClinical Manifestations - Tachypnea & cyanosis at birth - Rarely congestive heart failure 71
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  74. 74. Cyanotic CHD…b. L. TGA (corrected transposition)  Systemic VR to normally positioned Rt atrium  Through bicuspid (Mitral) valve  Right sided left ventricle  Pulmo. artery  pulm. venous return  Normally positioned Lt atrium  Through tricuspid valve  Left sided Right ventricle  Aorta 74
  75. 75. Cyanotic CHD… Discordant atrio-ventricular relation (ventricular inversion) Transposition of great arteries Clinical ManifestationDepends on associated malformation 75
  76. 76. Cyanotic CHD…Diagnosis - Clinical - CXR - Cardiomegaly - Narrow mediastinum - Increased pulmonary blood flow - ECG - EchocardiographyTreatment - PGE1 - emergency - Surgery 76
  77. 77. Cyanotic CHD…2.2 Truncus arteriosus - Single arterial trunk for both pulm. & systemic circ. - 4 types depending the origin of pulmonary arteryClinical Manifestation - Cyanosis - CHF at 2-3rd m - Systalic ejection mTreatment - surgery 77
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  80. 80. Cyanotic CHD…2.3 Total Anomalous Pulm. Venous return - Pulm. drainage into systemic vein 80
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  83. 83. Cyanotic CHD…2.4 Single Ventricle - No interventricular septum 83
  84. 84. Cyanotic CHD…2.5 Hypoplastic Left Heart Syndrome - Under development of Lt Side of the heart - Atretic aortic or mitral orifice - Non functional Lt ventricle - Hypoplasia of ascending aorta  Right ventricle supplies both pulm. & systemic circulation 84
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  87. 87. Cyanotic CHD…2.6 Persistent fetal circulation 87
  88. 88. Cyanotic CHD…2.6 Dextroposition of the heart2.7 Dextrocardia 88
  89. 89. THANK YOU!! 89

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