1.CHD part 1_2.ppt have important document
- 1. Congenital Heart Disease (CHD) Dr Abdulkadir assistant professor of pediatrics & child health
- 2. Epidemiology Incidence - 8/1000 live births -2/100 premature infants excluding PDA - 3-4/100 still born - 10-25/100 abortuses – This incidence increases to 2–6% for a 2nd pregnancy after the birth of a child with congenital heart disease or if a parent is affected. – This recurrence risk is highly dependent on the type of lesion in the 1st child. • Most congenital defects are well tolerated during fetal life.
- 3. Etiology - Unknown in most cases - Genetic factors - single gene defect - Chromosomal abnormality. - Environmental factors
- 4. Cardiac defects associated with syndromes Cardiovascular disease Common cardiac defect Trisomy 21(down syndrome) AVSD Noonan syndrome PS, ASD,VSD, PDA, hypertrophic cardiomyopathy Marfan syndrome Aortic aneurysm, mitral valve disease Turner Coarctation of aorta, aortic stenosis Trisomy 13 (patau syndrome) ASD,VSD,PDA,valve abnormalitis Trisomy 18 (Edward syndrome) ASD,VSD,PDA,valve abnormalitis
- 5. Cardiac defects associated with syndromes Cardiovascular disease Common cardiac defect CHARGE syndrome ASD,VSD, TOF Williams-beuren syndrome Supravalvular AS, peripheral PS DIGeorge syndrome TOF,IAA,TA,VSD HOLT-Oram syndrome ASD,VSD,PDA Alagille syndrome Peripheral pulmonary hypoplasia, PS, TOF Cat eye syndrome TAPVR,TOF
- 6. Clues for Evaluation of an Infant with suspected CHD 1. History and Physical Examination • Acyanotic • Cyanotic – Hyperoxia test: – Get arterial blood gas analysis (ABG) on room air – If p02 <100mmhg, administer 100% Fio2 – Repeat ABG after 10 minutes – If p02: • >150mmhg…..non-cardiac • 100- 150mmhg….equivocal (Do echocardioghy) • <100mmhg…..cyanotic heart disease
- 7. 2. Chest X-ray • Normal • Increased/Plethora pulmonary blood flow • Decreased/Oligemia 3. Electrocardiogram - Right, left or biventricular hypertrophy 4. Final diagnosis - Precordial examination - Echocardiography
- 8. Relative frequency of congenital heart disease Lesions % of all Lesions - Ventricular septal defect 25-30 - Atrial septal defect (Secundum) 6-8 - Patent ductus arteriosus 6-8 - Coarctation of aorta 5-7 - Tetralogy of Fallot 5-7 - Pulmonary Valve Stenosis 5-7 - Aortic Valve Stenosis 4-7 - d-Transposition of great arteries 3-5 - Hypoplastic left ventricle 1-3
- 9. 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
- 10. I- Acyanotic Congenital Heart Disease(CHD) Volume overload 1. Left to right shunt ASD, VSD,PDA AVSD, PAPVR Aorticopulmonary window defect 2. Regurgitant lesion MR,TR,PR,AR MVP 3. Some of the cardiomyopathies Pressure load (obstructive lesions) 1. Ventricular outflow obstruction Pulmonic valve stenosis Aortic valve stenosis 2. Narrowing of the great vessels Coarctation of the aorta
- 11. I. Acyanotic Congenital Heart Diseases 1. Left to Right Shunt Lesions 1.1 Atrial Septal Defect Three major types exist : primum, secundum and sinus venosus • Secundum ASD arises either : – from an enlarged foramen ovale or – inadequate growth of the septum secundum or – excessive absorption of the septum primum.
- 12. • Pathophysiology • Lt:rt shunt pul blood flow overload of pulmonary vasculature pulmonary hypertension CHF lt=Rt atial pressure Rt>lt pressure lt:Rt shunt cease shunt reversal
- 13. Clinical Manifestations Most are asymptomatic Hyperactive precordium, Right ventricular heave Wide & fixed split of 2nd heart sound Systolic ejection murmur Mid-diastolic murmur at tricuspid area
- 14. Investigation CXR - Right. V & A enlargement - Large pulm. artery - Increased pulm. vascularity ECG – RAD of QRS in secundum ASD - LAD of QRS in primum ASD Echocardiography Catheterization
- 15. Complications - Pulmonary Hypertension - Eisenmenger syndrome - Arrhythmia Treatment Surgery-for all symptomatic Asymptomatic patients with shunt ratio > 2:1
- 18. Ventricular septal defect • VSD is the most common cardiac malformation. • VSD is a condition whereby there is a hole between the two pumping chambers of the heart. • VSDs occur in isolation or in combination with other congenital heart defects, as in an atrioventricular canal (AVC), TOF and TGA.
- 19. • Defects may occur in any portion of the ventricular septum. • 3 types – membranous - commonest – Supracristal- less common – Muscular • single or • multiple (Swiss cheese septum).
- 20. Con’d • PATHOPHYSIOLOGY – Left-to-right shunt increased pul. blood flow. • Restrictive VSDs(usually <0.5 cm2) – right ventricular pressure is normal – the size of the defect limits the magnitude of the shunt • Nonrestrictive VSDs (usually >1.0 cm2) – right and left ventricular pressure is equalized – the direction of shunting and shunt magnitude are determined by the ratio of pulmonary to systemic vascular resistance
- 21. 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 Murmur when shunt ratio > 2:1
- 22. Acyanotic CHD… Diagnosis - Clinical - CXR - Cardiomegaly - Plethoric lung - ECG - Echocardiography
- 23. Con’d • TREATMENT – medical management • has two aims: – to control heart failure and –prevent the development of pulmonary vascular disease.
- 24. – Surgical management • Indications for surgical closure : – patients at any age with large defects in whom clinical symptoms and failure to thrive cannot be controlled medically – infants between 6 and 12 mo of age with large defects associated with pulmonary hypertension, even if the symptoms are controlled by medication – patients older than 24 mo with a Qp : Qs ratio greater than 2 : 1. – Patients with supracristal VSD of any size. • Severe pulmonary vascular disease is a contraindication to closure of a VSD
- 25. Acyanotic CHD… • Complications - Infective endocarditis - Recurrent lung infection - Heart failure - Pulmonary HTN - Acquired pulmonary stenosis
- 26. PROGNOSIS • A significant number (30–50%) of small defects close spontaneously, most frequently during the 1st 2 yr of life. • Small muscular VSDs are more likely to close (up to 80%) than membranous VSDs are (up to 35%). • It is less common for moderate or large VSDs to close spontaneously (up to 8% may close completely) • The results of primary surgical repair are excellent
- 29. Atrioventricular septal defect • AVSD results from incomplete fusion the the endocardial cushions, which help to form the lower portion of the atrial septum, the membranous portion of the ventricular septum and the septal leaflets of the triscupid and mitral valves. • Accounts for 4-5% of CHD.
- 30. • Complete AVSD most common in downs syndrome, unlike partial which is common in non downs syndrome patients. • Complete AVSD also occur in patients with heterotaxy syndromes(more common with asplenia than with polysplenia).
- 31. • Complete Form • Low primum ASD continuous with a posterior VSD. • Cleft in both septal leaflets of TV/MV. • Results in a large L to R shunt at both levels. • TR/MR, Pulm HTN w/ increase in PVR. • Incomplete Form • Any one of the components may be present. • Most common is primum ASD, cleft in the MV & small VSD. • Hemodynamics are dependent on the lesions.
- 32. Management • Surgery is always required. • Treat congestive symptoms. • Pulmonary banding maybe required in premature infants or infants < 5 kg. • Correction is done during infancy to avoid irreversible pulmonary vascular disease.
- 33. Patent ductus arteriosus PDA is one of the most common congenital cardiac defects. It is a connection between the aorta and the pulmonary artery. Common in premature babies and congenital rubella syndrome Female-to-male ratio is 3:1
- 34. Acyanotic CHD… Clinical Manifestation Asymptomatic in small PDA Wide pulse pressure Bounding pulse large Continuous or machinery M at 2nd Left ICS
- 35. Acyanotic CHD… Diagnosis - Clinical - Chest X-ray - ECG - Echocardiography
- 36. Treatment - Medical…indomethacin - Surgical closure Complications - Infective Endocarditis/Endarteritis - CHF - Embolization - Pulmonary HTN Prognosis - Small PDA - normal life - Large PDA - CHF
- 38. 2. The Obstructive Lesions • Pulmonary Stenosis • Aortic Stenosis • Coarctation of the Aorta
- 39. • 2.1 Pulmonic Stenosis • 4 types – Valvular stenosis – Isolated infundibular stenosis – supravalvular pulmonary stenosis – branch pulmonary artery stenosis
- 40. Hemodynamics • RV pressure hypertrophy RV failure. • RV pressures maybe > systemic pressure. • Post-stenotic dilation of main PA. • W/intact septum & severe stenosis R-L shunt through PFO cyanosis. • Cyanosis is indicative of Critical PS.
- 41. Clinical features • Depends on the severity of obstruction. • Asymptomatic with mild PS < 30mmHg. • Moderate-severe PS: 30-60mmHg, > 60mmHg • RV heave • Split 2nd heart sound with a delay • Ejection click, followed by systolic murmur. • Heart failure & cyanosis seen in severe cases.
- 42. DIAGNOSIS • The electrocardiogram shows – gross right ventricular hypertrophy, frequently accompanied by a tall, spiked P wave. • Radiographic studies – cardiac enlargement with prominence of the right ventricle and right atrium. – Prominence of the main pulmonary artery segment may be seen due to poststenotic dilatation – Intrapulmonary vascularity is decreased. • echocardiogram shows – severe deformity of the pulmonary valve and right ventricular hypertrophy. • catheterization
- 43. Con’d • TREATMENT – Surgical • COMPLICATIONS – Heart failure – Infective endocarditis • is a risk but is not common in childhood. • PROGNOSIS – In untreated severe stenosis, the course may abruptly worsen with the development of right ventricular dysfunction and cardiac failure.
- 44. Con’d 2.2 Aortic Stenosis – Valvular stenosis – Subvalvular (subaortic) stenosis – Supravalvular aortic stenosis • PATHOPHYSIOLOGY. – obstruction to Left ventricular outflow increased Left ventricular systolic pressure left ventricular wall hypertrophy
- 45. Con’d • CLINICAL MANIFESTATIONS. – depend on the severity of the obstruction. • In mild stenosis – Normal pulse and apical impulse – Normal to enlarged heart size – Systolic ejection murmur • Severe aortic stenosis (critical aortic stenosis). – Heart failure, cardiomegaly, and pulmonary edema are severe. – the pulses are weak in all extremities, and the skin may be pale or grayish, urine output may be diminished. – Rarely, fatigue, angina, dizziness, or syncope may develop in an older child – Sudden death has been reported with aortic stenosis
- 46. Con’d • The physical findings are dependent on the degree of obstruction to left ventricular outflow. – If the stenosis is severe • the 1st heart sound may be diminished • 2nd sound may be split paradoxically (becoming wider in expiration). • A 4th heart sound may be audible • The typical murmur is audible maximally at the right upper sternal border and radiates to the neck and the left midsternal border.
- 47. Con’d • DIAGNOSIS – Clinical – Chest x-ray – ECG – ECHO – Left heart catheterization – Graded exercise testing
- 48. Con’d • TREATMENT. – Balloon valvuloplasty – Surgery • COMPLICATION – Heart failure – Infective endocarditis – Sudden death – Arrhythmia • PROGNOSIS – Those who survive may develop signs of left ventricular diastolic muscle dysfunction (restrictive cardiomyopathy) later in life
- 49. 2.3 Coarctation of the Aorta • Occur at any site from the arch of aorta to iliac bifurcation • Types: Preductal, ductal, Post-ductal • 98% juxta ductal, More common in males • May be a feature of turner syndrome Pathogenesis • In utero arch hypoplasia Rt heart output passes through the ductus
- 50. Acyanotic CHD… Clinical Manifestation Hypertension → mechanical obstruction → renal Differential cyanosis → pale upper extr. → cyanosed lower extr. Classic signs - Disparty in pulse & BP - Radio-femoral delay - Systolic M at LMSB & inter-scapular area
- 51. Acyanotic CHD… Diagnosis - Clinical - CXR - cardiomegaly & pulm. congestion - Notching of ribs - ECG - Echocardiography Prognosis – Untreated cases succumb by 20-40 years Complications - Cardiovascular accidents - Infective endocarditis - Aneurysms Treatment - Medical - IV PGE1 in neonatal age - Surgery
- 54. •Thank you
Editor's Notes
- #3: This overall incidence does not include mitral valve prolapse, PDA of preterm infants, and bicuspid aortic valves (present in 1-2% of adults).
- #51: If there is coarctation is in pre-ductal area, Unoxygenated blood will pass through the PDA and supply the lower extremities there will be lower saturation compared to upper extremities (differential saturation)
- #52: rib notching (due to collateral BV that erode the ribs)