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Congenital heart diseases

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Congenital heart diseases

  1. 1. CONGENITAL HEART DISEASES Iman Sulaiman Al-Hatmi 85569 Done by:
  2. 2. Objectives  Classification of CHD  Pathophysiological changes in circulation that occurs after birth  Innocent murmur vs. pathological murmur  Maternal illness that can result in CHD  Appropriate investigations  Management options  Indications for infective endocarditis prophylaxis
  3. 3. Case Scenario A 5-year old boy brought by his mother to the health center for pre school check up. The child has bee seen previously in the health center for immunization and for minor acute respiratory infection. At this visit the physical examination reveals a systolic murmur for the first time.
  4. 4. History  Cardinal cardiac symptoms: congestive heart failure (FTT, breathlesness, poor feeding, cyanosis, sweating, syncope …)  PMH: rheumatic fever, recurrent chest infections  FHx: of hypertrophic cardiomyopathy, congenital heart disease or unexplained childhood/early adulthood death is of importance.  Perinatal Hx: of premature birth, maternal diabetes, drug or toxin ingestion and intrauterine infection is relevant.
  5. 5. Physical Examination  Dysmorphism and congenital anomalies. Presence of anomalies of other organ systems associated with CHD in as many as 25% of cases.  Child's growth and development  CVS:  Cyanosis, cool periphries. arterial pulse, perfusion, delay , BP, HR, edema  Precordiam:  Inspection, palpation  Auscultation in the four different areas ( heart sounds, murmurs, and for additional sounds, clicks)  A crucial in children is to characterise the second heart sound and its components.  Auscultation over the back, the axillae and the neck.  Variations of murmurs and heart sounds with position (supine, sitting, standing)
  6. 6. Circulatory changes at birth In the fetus:
  7. 7. Circulatory changes at birth With 1st breaths:
  8. 8. Definition  CHD: group of anatomic abnormalities of the heart that are generally present at birth.  Ranges from asymptomatic to fatal  The most common group of structural malformations in children.  Occur in 8 per 1000 births
  9. 9. Incidence and spectrum of congenital heart disease in Oman  Hospital-based study analysed the incidence and spectrum of congenital heart disease (CHD) in the Sultanate of Oman from 1994 to 1996.  CHD was detected in 992 of 139,707 live births (incidence 7.1/1000 live births).  The common CHDs were VSD(24.9%), ASD(14.4%) and PDA (10.3%).  The frequency of AV septal defects (5.9%) was higherthan reported from other countries.  Age at diagnosis was under 1 month in 38% and 1-12 months in 40%.  Cyanotic CHDwas found in 21.7% of the whole group and 35% of neonates. Incidence and spectrum of congenital heart disease in Oman. Subramanyan R, Joy J, Venugopalan P, Sapru A, al Khusaiby SM. Ann Trop Paediatr. 2000 Dec;20(4):337-41.
  10. 10. Causes of CHD Cause example Maternal disorder Rubella infection SLE DM Maternal drugs Warfarin therapy Fetal alcohol syndrome Sodium valproate Phenytoin Chromosomal abnormalities Down syndrome(trisomy 21) Edward’s syndrome(trisomy 18) Patau’s syndrome(trisomy 13) Turner’s syndrome(45XO) William’s syndrome (chromosome 7 microdeletion) Chromosome 22q11.2 deletion
  11. 11. Presentation  Antenatal cardiac ultrasound diagnosis  Heart murmur (most common)  Cyanosis  Heart failure  Shock (severe L heart obstruction)
  12. 12. Heart murmurs  Vast majority of children with murmurs have a normal heart, ‘Innocent murmur’, from turbulent flow in great vessels outflow tracts.  2 types of innocent murmur:  Ejection murmur  turbulent blood flow in the ventricle, outflow tract or great vessels  not associated with structural abnormalities  Venous hum  turbulent blood flow in head & neck veins  continuous low pitched rumble heard beneath either clavicle. Increase on inspiration, louder after exercise.  Distinguished from PDA by its disappearance on lying flat/ with compression of jugular veins on same side.
  13. 13. Cont. 7 S’s : Soft (low amplitude) Short Systolic Single (no click/gallop) Sensitive (to posture) left Sternal edge aSymptomatic  Hallmarks of inno ce nt ejection murmur:  Soft blowing systolic ( usually from R side pulmonary outflow in 2nd L interspace) OR short buzzing ( usually from L side aortic blood flow in 4th L interspace)  Localized to L sternal edge  No diastolic component  Normal heart sound with no added sounds  No parasternal thrill  No radiation  Asymptomatic pt
  14. 14. Cyanosis  Concentration of reduced Hb > 5 g/dL  Cardiac cyanosis may be caused by:  Reduced pulmonary blood flow  Duct-dependent pulmonary circulation relies on blood flowing from L to R across ductus arteriosus  Abnormal mixing of systemic venous & pulmonary blood (transposition of great arteries)  Most infants with cyanotic heart disease in 1st few days of life are duct-dependent  The key to early survival is Maintenance of ductal patency with IV prostaglandin ‘PGE’
  15. 15. Classification AcyanoticAcyanotic CyanoticCyanotic 1-Left- to –right shunt •Ventricular septal defect (VSD) •Persistant ducus arteriosus (PDA) •Atrial septal defect(ASD) •Atrioventricular septal defect AVSD(endocardial cushion defect) 2-Obstructive lesions •Pulmonary stenosis (PS) •Aortic stenosis (AS) •Coarctation of the aorta (COA) Cyanotic (right-to-left shunt( •Teratology of fallot •Transposition of great artieries •Truncus arteriosus •Tricuspid atresia •Total anomalous pulmonary venous drainage
  16. 16. Left-to-right shunt breathlessness, feeding difficulty, recurrent chest infections
  17. 17. Atrial Septal Defect
  18. 18. ASD Due to failure of septal growth or excessive reabsorption of tissue. 2 main types: Secundum ASD  defect in the center of the atrial septum involving the foramen ovale. Partial AV septal defect (primum ASD)  defect of the AV septum characterized by:  An inter-atrial communication between the bottom end of the atrial septum and the AV valves (primum ASD)  Abnormal AV valve which has 3 leaflets and tend to leak ( regurgitant valve)
  19. 19. Cont.  Symptoms:  Rarely symptomatic  Recurrent chest infections/wheeze  HF  Physical signs:  Fixed splitting of S2 .. ?  Ejection systolic murmur over the pulmonary area ..?  Mid-diastolic murmur at the left lower sternal border .. ?
  20. 20. ASD.. Investigations  Chest X-ray:  Cardiomegaly  Right atrial enlargement  Enlarged pulmonary arteries  Increased pulmonary vascular markings  ECG:  Croee-sectional echocardiography
  21. 21. ASD.. Management  Children with significant ASD (shunt is still present at around 3yr of age) will require treatment :  Secundum ASD by cardiac cathetrization with insertion of an occlusion device  Partial AVSD by surgical correction Treatment undertaken at 3-5 yrs of age to prevent RHF & arrhythmias in later life.
  22. 22. Ventricular Septal Defect
  23. 23. Ventricular septal defect  The most common CHD (30%)  Occur when there is a defect anywhere in the vetricular septum usually perimembranous (adjacent to tricuspid valve)or muscular septum.  The size of the VSD affects the clinical presentation  Small VSD: smaller than aortic valve, up to 3mm  Large VSD: same/bigger than aortic valve. Small VSD Large VSD
  24. 24. Small VSD Clinical features: Symptoms:  Asymptomatic Physical signs:  May have thrill at lower sternal edge  Loud pansystolic murmur at lower left sternal edge Investigations: •Chest X-ray: -Normal •ECG: -Normal •Echocardiography: -Show the defect - Doppler echocardiography to asses the hemodynamic effect Management:  Most of these lesions will close spontaneously by 3 years of age ( conformed by disappearance of murmur, normal ECG, normal echocardiogram)
  25. 25. Large VSD Clinical features: Symptoms: ( 2-4 wks)  HF with breathlessness, poor feeding, diaphoresis & FTT  Recurrent chest infection • Physical Signs: -Soft pansystolic murmur or no murmur -Apical –mid diastolic murmur -Loud and palpable P2 -Left parasternal heave -Tachypnea ,Tachycardia -Enlarged liver
  26. 26. Cont. Investigations: Chest X-ray:  Cardiomegaly  Enlarged pulmonary arteries  Increased pulmonary vascular marking  Pulmonary congestion • ECG: – Biventricular hypertrophy • Echocardiogram: – Demonstrates the anatomy of the defect, hemodynamic effects , severity of pulmonary hypertension. cardiomegaly Increased pulm markings Enlarged pulm arteries
  27. 27. Cont. Management:  Anti-heart failure therapy: diuretics, digoxin, ACE inhibitors  If symptoms are controlled and pulmonary HTN dose not develop> follow up  Continued poor growth and pulmonary HTN despite therapy > surgical closure
  28. 28. Patent Ductus Arteriosus
  29. 29. PDA  The ductus arteriosus allows blood to flow from the pulmonary artery to the aorta during fetal life. This changes to the opposite after birth.  In term infants, it normally closes shortly after birth. Failure of the normal closure of it by a month post term is due to a defect in the constrictor mechanismof the duct.  In preterm infants, the PDA is not from CHD but due to prematurity.
  30. 30. Cont. Clinical features: Symptoms:  Small PDA: asymtomatic  Large PDA: HF Physical signs:  High volume collapsing pulse  Widened pulse pressure  Loud S2  Continuous murmur (beneath left clavicle) Investigation: Small PDA> normal X-ray and ECG Large PDA:  Chest X-ray:  Cardiomegaly  Pulmonary congestion  ECG:  Left ventricular hypertrophy  If pulmonary HTN> right ventricular hypertrophy  Echocardiography Management: Closure with a coil or occlusion device introduced via a cardiac catheter at about 1 year of age.
  31. 31. Complete AV septal defect
  32. 32. cAVSD  A combination of several closely associated heart problems that result in a large defect in the center of the heart:  Defects include holes such as ASD, VSD and abnormalities of the AV valves.  Blood can move freely among the four heart chambers, mixing oxygen-rich (red) blood with oxygen-poor (blue) blood.  The condition is common in children with    Down syndrome (about 20%).
  33. 33. Cont. Clinical features:  Pulmonary HTN  As the pressure in the lungs rises, blood within the heart will eventually "shunt" through the septal openings from right heart to the left. This allows oxygen-poor (blue) blood to reach the body, and cyanosis will be noted. Investigations: Chest X-ray:  Cardiomegaly with enlargement of all chamber ECG:  Superior axis  Combined ventricular hypertrophy Echocardiography Treatment:  Treat HF medically & surgical repair at 3-6 months of age.
  34. 34. Summary.. L-to-R shunt lesion Symptoms signs management ASD-secundum None Ejection systolic murmur at ULSE Catheter device closure at 3-5 yrs ASD-partial None, HF Fixed split S2 Surgery at 3 yrs VSD-small (80- 90%) None Pansystolic murmur at LLSE None VSD-large(10- 20%) HF Loud P2, soft murmur, tachypnoea, hepatomegaly Diuretics, captopril Surgery at 3-6 months PDA-tem None Continous murmur at ULSE +/- bounding pulses Coil/device closure at cardiac catheter PDA-preterm None, HF Systolic murmur at ULSE +/- bounding pulses Fluid restriction, surgical ligation
  35. 35. Outflow Obstruction
  36. 36. Pulmonary Stenosis
  37. 37. PS  Site: Valvar (most), supravalvar, or subvalvar  The valve may have only two or one leaflets  The leaflets that are partially fused together  Three leaflets, but thick and partly or completely stuck together narrowing of the valve
  38. 38. Pathophysiology The right ventricle pump harder and at a higher pressure to propel blood through the valve Right ventricular hypertrophy Pulmonary valve is mildly to moderately narrowed
  39. 39. severe stenosis in a neonate Right ventricle cannot eject sufficient volume of blood flow into the pulmonary artery Right ventricular pressure becomes extremely high cyanosi s Lead to right-to-left shunting through a patent foramen ovale/atrial septal defect delay the closure of the foramen ovale
  40. 40. Cont. Clinical features:  Symptoms:  Mild PS: asymptomatic  Moderate- severe PS:  exertional dyspnea and easy fatigability.  Newborn with severe stenosis may be more symptomatic and even cyanotic because of right-to- left shunting at the atrial level.  Physical signs:  Ejection systolic murmur over the pulmonary area that radiate to the back  Systolic thrill over the pulmonary area  S2 widely splitting  Left parasternal heave (RV hypertrophy)  Ejection click that audible in inspiration
  41. 41. Cont. Investigations: Mild PS > normal X-ray and ECG Severe PS:  Chest X-ray:  normal or post-stenotic dilatation of the main pulmonary artery - ECG:  RVH Management: Indicated for moderate to severe cases Treatment measures designed to maintain the patency of the ductus arteriosus > used as palliative measure to maintain or increase pulmonary blood flow in infants with sever pulmonary stenosis Trans-catheter balloon dilatation is the treatment of choice (when pressure gradient across pulmonary valve > 64mmHg)
  42. 42. Aortic stenosis
  43. 43. AS  Valvular, subvalvular or supravulvalar  Failure of :  development of the three leaflets  Resorption of tissue around the valve
  44. 44. Cont. Clinical features: Mild- moderate AS > asymptomatic Severe AS: Symptoms: Easy fatigability Excertional chest pain Syncope Infants> symptoms of HF Physical signs:  Ejection systolic murmur over the aortic area and radiating to the neck  Carotid thrill (always)  Apical ejection click
  45. 45. Cont. Investigations:  Chest x-ray:  Normal or prominent left ventricle with post- stenotic dilation of the ascending aorta  ECG:  LVH  Echocardiography Managements: The degree of aortic stenosis frequently progress with growth and age > regular clinical and echocardiographic assessments. Balloon valvotomy > children with Sx on exercise or who have high resting pressure gradient (>64mmHg) across aortic valve. Balloon dilatation in older children is safe, but in neonates much more difficult & dangerous.
  46. 46. Coarctation of Aorta
  47. 47. COA  Narrowing of the aorta at the junction of the arch with descending aorta preductal or postductal(98%).  2X more common in males  25% of patients with Turner’s Syndrome have coarctation of aorta  Associated Defects:  Bicuspid aortic valve (most common associated defect seen in 50%)  VSD  ASD
  48. 48. Cont. Clinical features: Symptoms:  Less severe: no symptoms  In severe cases the blood supply to the descending aorta comes through ductus arteriosus> when it close in the first week of life baby present with shock, respiratory distress & poor feeding.  HF in neonatal period  Older children are usually asymptomatic but may present with leg discomfort with exercise , headache,  Physical signs:  Radio-femoral delay  Weak pulse in the lower extremities  Bounding pulse of the arms and carotid vessels.  Fall in systolic BP in the lower extremities compared to the upper extremities.  Systemic HTN in the upper extremities  Loud aortic S2  Systolic ejection murmur  Systolic ejection click
  49. 49. Cont. Investigations: CXR : rib notching with large collaterals ECG: LVH Managements: Infants: intravenous infusion of prostaglandin E1 , inotropic agents, diuretics. Balloon angioplasty and stenting Surgical repair
  50. 50. Summary Lesion Signs Management Aortic stenosis • Murmur: upper R sternal edge • carotid thrill Ballon dilatation Pulmonary stenosis •Murmur: upper L sternal edge • no carotid thrill Ballon dilatation Coarctation of aorta • systemic HPT •Radio-femoral delay Stent insertion or surgery
  51. 51. Cyanotic CHD  Cyanotic (right-to-left shunt)  Occurs when the systemic venous return crosses from the right side to the left side of the heart and returns to the body without going through the lungs.  Examples:  Teratology of fallot  Transposition of great artieries  Truncus arteriosus  Tricuspid atresia  Total anomalous pulmonary venous drainage
  52. 52. Teratology of fallot
  53. 53. Teratology of fallot
  54. 54. Cont. Clinical features: Symptoms:  Vary widely (cyanosis) and depend mainly on the severity of pulmonary stenosis.  Infancy:  cyanosis  SOB on exertion (feeding )  hyper-cyanotic spells : periods of increasing cyanosis associated with inconsolable crying , fast breathing and irritability (may lead to unconsciousness , anoxic seizures, MI, cerebrovascular accidents, death)  Older children: effort intolerance and squatting
  55. 55. Cont.  Physical signs:  Central cyanosis  Clubbing of the fingers and toes  Loud harsh ejection systolic murmur initially and then with increasing severity of pulmonary stenosis the murmur become shorter and softer.  Single second heart sound (A2)
  56. 56. Cont. Investigations: Chest X-ray:  Boot-shaped heart ( caused by small main pulmonary artery and upturned apex secondary to RVH)  Pulmonary oligemia> decreased pulmonary vascular marking ECG:  RVH  LAD Echocardiography
  57. 57. Cont. Management: Hypoxic spells:  Oxygen administration  Placing the child in the knee- chest position  Morphine > to relaxe the pulmonary infundibulum and for sedation  Alpha-adrenergic agonist> increase the systemic vascular resistance  Occurance of cyanotic spell indicate the need for surgical intervention :  Complete surgical repair> VSD closure and removal or patching of the pulmonary stenosis  Palliative shunt surgery> between the subclavian artery and pulmonary artery
  58. 58. Truncus arteriosus  There is a common arterial trunk. The trunkal valve is often very abnormal. Presents with cyanosis and heart failure in the first few weeks, as pulmonary vascular
  59. 59. Totally anomalous pulmonary venous drainage (infradiaphragmatic(  All four pulmonary veins drain to the right side. Below the diaphragm they are always obstructed. Infant presents in first days with cyanosis, circulatory and respiratory failure and collapse.
  60. 60. Infective endocarditis prophylaxis whom to give?  In Yasmeen’s presentation of IE in medicine ^_~
  61. 61. References  Karen J, Robert M, Hal B , Richard E. Nelson Essential of Pediatrics. 6th edition. P537- 543  Joshi S, Wali Y. Practical Pediatrics.1st edition. P279-283  Lissauer T, Clayden G. Illustrated textbook of Pediatrics. 3rd edition. P290-300  Porth C. Essential of Pathophysiology. 2nd edition. P 411-415
  62. 62. Thank You

Hinweis der Redaktion

  • No definite etiological factors can be identified in the vast majority of children and the disease is believed to be multifactorial in origin
  • Abnormal communication between the right and the left side of the heart
    Pressure is greater on the left side> blood flows from the left to right side of the heart
    Excess blood entering the lungs > pulmonary congestion (symptoms like breathlessness, feeding difficulty, recurrent chest infections
  • Foramen ovale > enable the fetal heart to transport the oxygenated umbilical venous blood to the left side of the heart
  • - Fixed splitting> due to overload of the right ventricle with prolonged ejection into the pulmonary trunk >right ventricular SV equal in both inspiration and expiration
    Ejection systolic murmur > due to increased flow to the RV because of the shunt
    Mid-diastolic murmur at the left lower sternal border > because of increased volume passing across the tricuspid valve
  • Non-specific features
  • Mid diastolic murmur> increase the flow across the mitral valve
    Palpable P2 > pulmonary HTN
    Parasternal heave: indicate right vetricular enlargement or left atrial enlargement.
  • X-ray> enlargment of left ventricle+ may be right ventricle( because of pulmonary hypertention due to increased flow/ increased pulmonary vascular resistance)
  • ACE inhibitors> captopril
    Some VSDs especially muscular defect can be closed with devices placed at cardiac catheterization
    Best age of surgical closure is 6-12 monthes
  • Widened pulse pressure> after the infants pulmonary vascular resistance falls the PDA cause continuous runoff of aortic blood into the pulmonary artery > decrease in aortic diastolic and mean arterial pressure> widening of pulse pressure
    * Increased flow across the mitral valve > mid-diastolic murmur at the apex
  • Increased pulmonary circulation > pulmonary HTN
  • The ductus arteroisus may provide the vital accessory route for perfusing the lungs in infant
    When pulmonary stenosis is extreme > increased pressure in the right side> delay the closure of the foramen ovale
  • Upright T wave in V1 indicate RVH in children
  • Pulse delay > due to blood bypassing the obstruction via collateral vessels in the chest wall and hence the pulse in the legs is delayed.
    Normally the systolic BP in the legs is 10-20 mmHg higher than the arm.
  • Rib –notching > due to the development of large collateral intercostal arteries running under the ribs posteriorly to bypass the obstruction
  • 10% of all CHD
    Blue baby
  • Infants initially may be acyanotic > if the PS become more severe the amount of right –to- left shunt at the VSD will increase > more cyanotic
    hyper-cyanotic spells = tet spells
    Crying , feeding, defecating> increase the pulmonary vascular resistance > increase right-to- left shunt> decrease pulmonary blood flow
    squatting : sitting with bent hip and knee joints> help to increase systemic vascular resistance > promoting pulmonary blood flow
    Placing child in the knee-chest position > increase systemic vascular resistance
  • Pulmonary oligemia> due to reduced pulmonary blood flow