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Congenital heart disease (chd)
- 1. Congenital Heart Disease (CHD) Lecture for C-I students 1
- 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. 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. 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. CHD … Clues forEvaluation of an Infant with suspected CHD 1. On History and Physical Examination color) • Acyanotic • Cyanotic 2. Chest roentgenogram • Normal • Increased/Plethora pulmonary blood flow • Decreased/Oligemia 5
- 6. CHD … 3.Electrocardiogram - Right - Left hypertrophy - Biventricular Final diagnosis - Precordial examination - Echocardiography 6
- 7. I. Acyanotic CongenitalHeart Diseases 1. 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. 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. 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 Catheterization Prognosis - Well tolerated Complications – - pulm. Hypertension, Eismenger syndrome Treatment Surgery - For all symptomatic ASD - Asymptomatic patients with shunt ratio > 2:1 9
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- 16. Acyanotic CHD… 1.2 VentricularSeptal 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. 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. Acyanotic CHD… Diagnosis - Clinical - CXR - Cardiomegaly - Plethoric lung - ECG - Echocardiography Prognosis - 30-50% small defects close by 2 yr of age - Rarely moderate to large defects close 18
- 19. Acyanotic CHD… Complications - Infective endocarditis - Recurrent lung infection - Heart failure - Pulmonary HTN - Acquired pulmonary stenosis -- aortic valve regurgitation Treatment - 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. Acyanotic CHD… 1.3 PatentDuctus 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. Acyanotic CHD… Pathophysiology Ltto 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. Acyanotic CHD… Diagnosis - Clinical - Chest X-ray - ECG - Echocardiography Prognosis - Small PDA - normal life - Large PDA - CHF Complications - Infective Endocarditis/Endarteritis - CHF - Embolization - Pulmonary HTN Treatment - Medical - Surgical closure 25
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- 28. Acyanotic CHD… 2. ObstructiveLesions 2.1 Pulmonic Stenosis - 4 types - Valvular - Infundibular - Supra valvular - Peripheral Pathophysiology - Rt outlet obstruction → Pressure work ↓ Rt vent. hyperthropy 28
- 29. Acyanotic CHD… Clinical Manifestation - Mild to moderate - asymptomatic - Critical stenosis - Systolic ejection murmur - Heart failure in neonates & infants - Rarely cyanosis 29
- 30. Acyanotic CHD… Diagnosis - Clinical - CXR - Rt vent. hypertrophy - reduced pulm. blood flow - ECG - Echocardiography Prognosis - good in mild to moderate Complications - CHF in severe Ps - rarely IE Treatment - vavular PS - ballon valvoplasty - surgery 30
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- 32. Acyanotic CHD… 2.2 AorticStenosis - Valvular - the commonest - Supra valvular - Subvalvular (subaortic) 32
- 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. Acyanotic CHD… Diagnosis - Clinical - CXR - ECG - Echocardiography - Graded exercise testing Prognosis is good for mild to moderate Treatment - Balloon valvoplasty - Surgical 34
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- 38. Acyanotic CHD… 2.3 Coarctationof 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. 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. Acyanotic CHD… Diagnosis - Clinical - CXR - cardiomegaly & pulm. congestion - Notching of ribs - ECG - Echocardiography Prognosis – Untreated cases succumb by 20-40 years Complications - CVA - I/E - Aneurysms Treatment - Medical - IV PGE1 in neonatal age - Surgery 40
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- 45. Acyanotic CHD… 3. PulmonaryVascular Disease (Eismenger syndrome) - Occur in shunt lesions VSD - mainly ASD PDA - Reversal of shunt due to pulm. HTN → Cyanosis 45
- 46. Acyanotic CHD… 4. RegurgitantLessons - Pulmonary valvular insufficiency - Congenital mitral valve insufficiency - Mitral valve prolapse 46
- 47. II. Cyanotic CongenitalHeart Disease 1. Cyanotic lesions with decreased pulmonary blood flow 1.1 Tetralogy of Fallot Consists: 1. Rt ventricular outflow obst. 2. Ventricular septal defect 3. Dextroposition of the aorta 4. Right ventricular hypertrophy 47
- 48. 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. 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. Cyanotic CHD… Diagnosis CXR - Narrow base & uplifted apex - A boot or wooden shoe - decreased pulm. vascularity - Right side aortic arch in 20% ECG Echocardiography Complication - Cerebral thrombosis - in < 2 years - Brain abscess - Infective endocarditis - Polycythemia - CHF in pink TOF 50
- 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. 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. Cyanotic CHD… Clinical Manifestation - Cyanosis at birth - Respiratory distress - Single 2nd heart sound - No murmur Diagnosis - CXR - ECG - Echocardiography Treatment - PGE1 - Surgery 59
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- 61. Cyanotic CHD… 1.3 Tricuspidatresia - No outlet from Right atrium to Left vent. - Systemic venous return Rt atrium Lt atrium Left ventricule systemic Pulmonic (VSD, PDA) 61
- 62. Cyanotic CHD… Clinical Manifestation - Cyanosis at birth - Polycythemia - Easily fatiguability - Exertional dyspnea Diagnosis - EXR -Pulm. Under circulation - ECG -Lt axis deviation & Lt vent. hypertrophy - Echocardiography 62
- 63. Cyanotic CHD… Treatment - PGE1 - Surgery - Aortico - pulmonary Shunt - Bidirectional Glenn shunt - Modified Fontan operation 63
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- 65. Cyanotic CHD… 1.4 EbsteinAnomaly 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. Cyanotic CHD… Clinical Manifestations - Easly fatiguability - Cyanosis - Dysrhythmia - Rt to Lt shunt through formen ovale - Holosystolic M at tricuspid area - Heart failure Diagnosis - CXR - box shaped heart - ECG - Right BBB - Superior axis deviation Treatment - PGE1 - Surgery 66
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- 70. Cyanotic CHD… 2.Cyanotic CHDWith 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. Cyanotic CHD… *Systemic &Pulmonary Circulations Consists of two parallel circuits *Survival is with associated - patent foramen ovale or - VSD or - PDA Clinical Manifestations - Tachypnea & cyanosis at birth - Rarely congestive heart failure 71
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- 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. Cyanotic CHD… Discordantatrio-ventricular relation (ventricular inversion) Transposition of great arteries Clinical Manifestation Depends on associated malformation 75
- 76. Cyanotic CHD… Diagnosis - Clinical - CXR - Cardiomegaly - Narrow mediastinum - Increased pulmonary blood flow - ECG - Echocardiography Treatment - PGE1 - emergency - Surgery 76
- 77. Cyanotic CHD… 2.2 Truncusarteriosus - Single arterial trunk for both pulm. & systemic circ. - 4 types depending the origin of pulmonary artery Clinical Manifestation - Cyanosis - CHF at 2-3rd m - Systalic ejection m Treatment - surgery 77
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- 80. Cyanotic CHD… 2.3 TotalAnomalous Pulm. Venous return - Pulm. drainage into systemic vein 80
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- 83. Cyanotic CHD… 2.4 Single Ventricle - No interventricular septum 83
- 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. Cyanotic CHD… 2.6 Persistentfetal circulation 87
- 88. Cyanotic CHD… 2.6 Dextropositionof the heart 2.7 Dextrocardia 88
- 89. THANK YOU!! 89