1. CONGENITAL HEART DISEASES

2. ACYANOTIC HEART DISEASE

3. Acyanotic Heart Diseases
LEFT TO RIGHT SHUNTS
OBSTRUCTIVE LESIONS
REGURGITANT
LESIONS

4. Acyanotic Heart Disease Increased Volume Load LVE VSD, PDA, AVSD RVE
ASD, PAPVR
Increased Pressure Load
LVH
Coarcation of the Aorta, Mitral regurgitation
Aortic Stenosis
RVH
Pulmonary Stenosis, Mitral Stenosis

5. LEFT TO RIGHT SHUNT

6. Determinants of L to R shunting
Size Of the Defect
Relative Compliance of the Right and Left Ventricle
Relative Vascular Resistance in the Pulmomary and Systemic Circulations
The degree of the left to right shunting is dependent on the size of the defect, the relative compliance of the right and left ventricules and the relative vascular resistance in the pulmonary and systemic circulations
Size of the defect
Larger defect, increased shunting. The amount of blood added to the usual venous return to the atrium and is pumped by the right ventricle to the lungs. The ratio of pulmonary to systemic blood flow is between 2:1 and 4:1.
Relative compliance of the right and left ventricles
The right ventricle in infants is thick and less compliant, thus, left to right shunting is limited
However, when the child gets old, the right ventricular wall becomes thinner, thus increasing the left to right shunting
Relative resistance of the pulmonary and systemic circulation
The pulmonary vascular resistance drops in older patients, hence, increased left to right shunting

7. HEART FAILURE SIGNS LEFT TO RIGHT SHUNT
Communication between the pulmonic and systemic circulation
Increase blood flow to the lungs
HEART FAILURE SIGNS

8. (dilatation of the heart)
Heart Remodeling
(dilatation of the heart)
Increase Sympathetic nervous system
Increase pulmonary vascular resistance
(EISENMENGER PHYSIOLOGY)
The increase in the blood flow to the lungs - decrease pulmonary compliance and increase work of breathing

9. Left to Right Shunt Atrial Septal Defect
Atrioventricular Septal Defect
Partial Anomalous Pulmonary Venous Retur
Ventricular Septal Defect
PDA
Coronary-AV Fistula
Ruptured Sinus of Valsalva Aneurysm

10. Atrial Septal Defect Majority of the cases are sporadic
Autosomal dominant does occur as part of the Holt Oram syndrome (hypoplastic or absent radius, 1st degree heart block and ASD

11. Atrial Septal Defect Secundum
Most common form and is associated with structurally normal atrioventricular valves
Region of the fossa ovalis
May be single or fenestrated, openings ≥2cm in largest diameter are common in symptomatic older children
Majority of the cases are sporadic
Autosomal dominant does occur as part of the Holt Oram syndrome (hypoplastic or absent radius, 1st degree heart block and ASD

12. Atrial Septal Defect Enlargement of the pulmonary artery
Enlargement of the right atrium
Normal Left Ventricle and Aorta

13. Atrial Septal Defect Clinical Manifestations and findings
Wide and fixed splitting 2nd heart sound
Mild left precordial bulge
Right ventricular systolic lift on the left sternal border
Systolic ejection murmur at the left middle and upper sternal border
Loud 1st heart sound and sometimes a pulmonic ejection click
Short rumbling Mid-diastolic murmur on the lower left sternal border
Wide and fixed splitting of the 2nd heart sound is developed due to the increasing amount of right ventricular diastolic volume and prolonged ejection time, thus delayed closure of the pulmonary valve
Systolic ejection murmur is caused by increased flow across the right ventricular outflow tract into the pulmonary artery (not caused by the low-pressure flow across the ASD)
Short rumbling mid-diastolic murmur on the left sternal border due to increased blood flow across the tricuspid valve (indicates a Qp: Qs ratio of 2:L)

14. Atrial Septal Defect Diagnostics
Chest Xray:RVE, RAE, pulmonary artery is large and pulmonary vascularity is increased
ECG: normal or right axis deviation and a minor right ventricular conduction delay (rsR pattern in the right precordial leads

15. Atrial Septal Defect Diagnostics 2D echo
increased right ventricular-end-diastoic dimension and flattening and abnormal motion of the ventricular septum (anterior movement in systole or it remains straight.
ASD confirmed by pulsed and color flow Doppler

16. Atrial Septal Defect Treatment
Surgical or transcatheter device closure is advised for all symptomatic patients and also for asymptomatic patients with a Qp:Qs ratio of at least 2:1

17. Sinus Venosus Atrial Septal Defect
Upper part of the atrial septum in close relation to the entry of the SVC
May be related to Partial Anomalous Pulmonary Venous return
Sometimes the superior vena cava straddles the defect (rarely involves the IVC)
Tx: Surgical
If the superior vena cava straddles the defect, some venous blood enters the left atrium, but rarely causes clinically evident cyanosis
Anatomic correction generally requires the insertion of a patch to close the defect while incorporating the entry of anomalous veins into the left atrium
If the anomalous vein drains high in the superior vena cava, the vein can be left intact and the ASD closed to incorporate the mouth of the SVC into the leftatrium
The SVC proximal to the venous entrance is then detached and anastomosed directly to the right atrium

18. Partial Anomalous Pulmonary Venous Return
May drain into the: SVC or IVC, Right atrium, Coronary Sinus
May involve some or all of the veins from only 1 lung (right>left)
Scimitar Syndrome
An anomalous vein draining into the IVC is visible on Chest Xray as a crescentic shadow of vascular density along the right border of the cardiac silhouette

19. Scimitar Syndrome
Scimitar syndrome: ASD is not usually present but pulmonary sequestration and anomalous arterial supply to that lobe are common findings

20. Partial Anomalous Pulmonary Venous Return
Dx: 2D Echo
Cardiac catheterization
Selective pulmonary arteriography: presence of anomalous pulmonary veins
Descending aortography: anomalous pulmonary arterial supply to the right lung
Prognosis: Excellent
Tx: Surgical if large left-to-right shunting

21. Atrioventricular Septal Defects (Ostium Primum and AV Canal Defects)
Situated in the lower portion of the atrial septum and overlies the mitral and tricuspid valve
A cleft in the anterior leaflet of the mitral valve can be seen

22. Atrioventricular Septal Defects (Ostium Primum)
Pathophysiology: left to right shunt across the atrial defect and mitral (or occasionally tricuspid insufficiency
Pulmonary arterial pressure is typically normal or only mildly increased
Pathophysiology: similar to ostium secundum ASD

23. Atrioventricular Septal Defects (Ostium Primum)
Clinical Manifestations
Asymptomatic
History of exercise intolerance, easy fatigability and recurrent pneumonia (with large defects and severe mitral insufficiency)
Harsh or occasionally high-pitched apical holosystolic murmur (due to mitral insufficiency)
Cardiac enlargement and hyperdynamic precordium
Pathophysiology: similar to ostium secundum ASD

24. Atrioventricular Septal Defects (Ostium Primum)
Clinical Manifestations
Other findings: normal or accentuated 1st heart sound; wide, fixed splitting of the 2nd sound; pulmonary ejection murmur sometimes preceeded by a click; low-pitched and diastolic rumbling murmur at te lower left sternal edge or apex or both
Pathophysiology: similar to ostium secundum ASD

25. Atrioventricular Septal Defects (AV Canal Defects)
AV canal defect or endocardial cushion defect
Contiguous AV septal defects with markedly abnormal AV valves

26. Atrioventricular Septal Defects (Ostium Primum and AV Canal Defects)
Complete form: single AV valve common to both ventricles and consists of an anterior and a posterior bridging leaflet related to the ventricular septum with a lateral leaflet in each ventricle
Common to Down syndrome

27. Atrioventricular Septal Defects (AV Canal Defects)
Left or right dominant AVSD due to hypoplasia of one of the ventricles

28. Atrioventricular Septal Defects (AV Canal Defect)
Pathophysiology
L to R shunting occurs at both atrial and ventricular levels; with shunting from the left ventricle to the right atrium (due to the absence of the AV septum)
Pulmonary hypertension and increased tendency to develop pulmonary vascular resistance  right to left shunting  cyanosis (Eisenmenger syndrome)
AV valvular insufficiency

29. Atrioventricular Septal Defects (AV Canal Defect)
Clinical Manifestations
Heart failure and intercurrent pulmonary infection
Enlarged liver
Failure to thrive

30. Atrioventricular Septal Defects (AV Canal Defect)
PE findings
Cardiac enlargement
Systolic thrill at the lower left sternal border
Precordial bulge and lift
Normal or accentuated 1st heart sound
Widely split 2nd heart sound
Low pitched, mid-diastolic rumbling murmur, lower left sternal border
Pulmonary systolic ejection murmur
Harsh apical holosystolic murmur

31. Atrioventricular Septal Defects (AV Canal Defect)
CXR: prominent ventricles and atrium
2D echo
RVE with encroachment of the mitral valve echo on the left ventricular outflow tract
“gooseneck” deformity of the left ventricular outflow tract
Both valves insert at the same level
Common AV valve
Gooseneck deformity: caused by the pseudooverriding of the ascending aorta due to abnormal low position of the AV valve

32. Atrioventricular Septal Defects (AV Canal Defect)
Treatment
Ostium septum defect: Patch prosthesis for the closure of ASD and direct suture for the cleft in the mitral valve
AV septum defect: surgical operation during infancy (due to the risk of pulmonary vascular disease as early as 6-12 months of age)

33. Patent foramen Ovale Not an ASD
Left to right shunting is unusual but may occur in the presence of a large volume load or hypertensive left atrium
Does not require surgical treatment but may be a risk for paradoxical systemic embolization.
Device closure is considered if there is a history of thromboembolic stroke

34. Ventricular Septal Defect
Most common cardiac malformation (25% of CHD)
Different types:
Membranous type: most common
Infundibular types
Muscular
Supracrista

35. Ventricular Septal Defect
Determinants of the magnitude of the L to R shunting
Size
restrictive VSD (<5mm)
Level of Pulmonary resistance in relation to systemic resistance
Nonrestrictive VSD (>10mm)
In restrictive VSD, the right ventricular pressure is normal. However, the higher pressure in the left ventricle drives the shunt left to right
In nonrestrictive VSD, the right and left ventricular pressures are equalized. In these defects, the direction of shunting and the shunt magnitude are determined by the ratio of pulmonary to systemic vascular resistance

36. Ventricular Septal Defect
At birth, the pulmonary vascular resistance is elevated, thus the size of the left to right shunting is limited
Few weeks after birth, there is decrease in pulmonary resistance
With continued exposure of the pulmonary vascular bed to high systolic pressure and high flow, pulmonary vascular obstructive disease develops  Eisenmenger syndrome

37. Ventricular Septal Defect
Clinical Manifestations
Small VSDs
Asymptomatic
Loud, harsh or blowing holosystolic murmur on the left lower sternal border (frequently with thrill)
In neonates with VSD on the apical muscular septum, murmur heard on the apex
During

38. Ventricular Septal Defect
Clinical Manifestations
Large VSD
Dyspnea, feeding difficulties, poor growth, profuse perspiration, recurrent pulmonary infections and cardiac failure
Cyanosis
Prominence of the left precordium (palpable lift)
Laterally displaced apical impulse and apical thrust
Systolic thrill
During

39. Ventricular Septal Defect
Clinical Manifestations
Large VSD
Less harsh but more blowing systolic murmur
Increased pulmonic component of the 2nd heart sound
Mid-diastolic, Low pitched rumble at the apex
Increased blood flow to the mitral valve
Qp:Qs ratio ≥2:1
During

40. Ventricular Septal Defect
Diagnosis
Chest Xray
Small VSDs: normal or minimal cardiomegaly and borderline increased in pulmonary vasculature
Large VSDs
Gross cardiomegaly with prominence of both ventricles, left atrium and pulmonary artery
Pulmonary vascular markings are increased
Pulmonary edema
During

41. Ventricular Septal Defect
Clinical Manifestations
2D echo
Helpful in estimating shunt size, the degree of volume overload, the increased dimensions of chambers and presence of other valve defects (including aortic valve insufficiency or prolapse)
Pulse doppler examination calculates pressure gradient across the defect
During

42. Ventricular Septal Defect
Treatment
30-50% close spontaneously, most frequently during the 1st 2 years of life
Small muscular VSDs > membranous
During

43. Ventricular Septal Defect
Treatment
Indications for surgical closure
Large defects in whom clinical symptoms and failure to thrive cannot be controlled medically
Infants between 6 and 12 months with large defects associated with pulmonary hypertension
Patients older than 24 months with Qp:Qs ratio greater than 2:1
Supracristal VSD
Contraindication to surgery: severe pulmonary vascular disease nonresponsive to pulmonary vasodilators
Supracristal VSD: due to higher chances of developing aortic insufficienvy

44. Patent Ductus Arteriosus
Location: the aortic end is distal to the origin of the left subclavian artery and enters the pulmonary artery at its bifurcation
Associated with maternal rubella infection
Preterm: the smooth muscle in the wall is less responsive to high PO2 and less likely to constrict after birth; normal structure PDA
Term: the wall is deficient in both the mucoid endothelial layer and the muscular media
During

45. Patent Ductus Arteriosus
Clinical Manifestations
Bounding peripheral pulses and a wide pulse pressure, due to runoff of blood into the pulmonary artery during diastole
Machinery like murmur
Thrill, maximal in the 2nd left interspace with radiation toward the left clavicle, left sternal border and apex
During

46. Patent Ductus Arteriosus
Treatment
Surgical or catheter closure
During

47. Aorticopulmonary Window Defect
Consists of a communication between the ascending aorta and the main pulmonary artery
Unlike truncus arteriosus, there is presence of pulmonary and aortic valves and an intact ventricular septum
Systolic murmur with an apical mid-diastolic rumble (due to increased blood flow across the mitral valve)
Tx: surgical
During

48. Coronary-Cameral Fistula
A congenital fistula existing between a coronary artery and an atrium, ventricle or pulmonary artery
Clinical signs may be similar to PDA but diffuse
Diagnosis: doppler echocardiography and cardiac catheterization
Treatment: small fistuals may close spontaneously, larger fistulas may require catheter intervention or surgical closure of the fistula
During

49. Ruptured Sinus of Valsalva Aneurysm
Happens when one of the valsalva of the aorta is weakened by congenital or acquired disease and ruptures in to the right atrium or ventricle
Acute heart failure with new loud to and fro murmur
Left to right shunt at the area of the atrium or ventricle
Urgent surgical repair is required
During

50. Acyanotic Heart Disease Increased Volume Load LVE VSD, PDA, AVSD RVE
ASD, PAPVR
Increased Pressure Load
LVH
Coarcation of the Aorta, Mitral regurgitation
Aortic Stenosis
RVH
Pulmonary Stenosis, Mitral Stenosis

51. Increased Pressure Overload
Cardiac output is maintained
Increased wall thickness (hypertrophy)

52. Acyanotic Heart Diseases
OBSTRUCTIVE LESIONS
REGURGITANT
LESIONS
Pulmonary Stenosis
Coarctation of the Aorta
Pulmonary Venous Hypertension
Pulmonar Valve Insufficiency
Congenital Mitral Insufficiency
Mitral Valve prolapse
Tricuspid Regurgitation

53. Acyanotic Congenital Heart Disease: Obstructive Lesions

54. Pulmonary valve stenosis
The valve cusps are deformed to various degrees thus the valve opens incompletely during systole
May be severely fused or if not, may produce a dome like obstruction to right ventricular outflow tract during systole
May be a result of valve dysplasia seen in Noonan syndrome
May be also associated with Algallie syndromwe when the pulmonary stenosis is either of the valve or the branch pulmonary arteries

55. Pulmonary valve stenosis
Severity depends on the size of the restricted valve openinng
Severe: increased right pulmonary pressure
Pulmonary artery pressure is normal
Arterial oxygenation will be normal even in cases of severe stenosis except if with intracardial communication
In neonates, decreased right ventricular compliance leads to cyanosis due to right to left shunting through a patent foramen ovale

56. Pulmonary valve stenosis
Clinical Signs
Mild: sharp pulmonic ejection click after the 1st heart sound and split 2nd heart sound
Severe stenosis: The pulmonary component of the 2nd sound is inaudible; harsh systolic ejection murmur on the pulmonic area with radiation over the entire precordium, to both lung fields, neck and back
Enlarged right ventricle and right atrium
Prominence of the pulmonary artery segment due to post-stenotic dilatation

57. Pulmonary valve stenosis
Tx:
Initial treatment: Balloon valvuloplasty
Surgical

58. Aortic Stenosis
Valvular: the leaflets are thickened and the commisures are fused to varying degrees
Subvalvular (subaortic): discrete fibromuscular shelf below the aortic valve
Associated with mitral valve stenosis and coarctation of the aorta (Shone syndrome)
Supravalvular aortic stenosis
Least common; associated with Williams syndrome

59. Aortic Stenosis Clinical findings
Early systolic ejection click, best heard at the apex and left sternal edge
The click does not vary with respiration
If severe, the 1st heart sound is diminished
Paradoxical splitting of the 2nd heart sound

60. Aortic Stenosis Diagnosis Xray: Normal or prominent ascending aorta
ECG: left ventricular hypertrophy
2D echo: left ventricular hypertrophy
May shows the presence of the number of leaflets of the aortic valve and their morphology and other associated abnormalities
In neonates with critical aortic stenosis, presence of endocardial fibroelastosis (bright in 2D echo, indicative of scarring of the endocardium)

61. Aortic Stenosis Treatment
Balloon valvuloplasty: moderate to severe valvular aortic stenosis
Ross procedure: aortopulmonary translocation

62. Coarctation of the Aorta
Juxtaductal coarctation
Most common
Just below the left subclavian artery at the origin of the ductus arteriosus
Turner’s syndrome
Shone complex: when associated with mitral valve abnormalities and subaortic stenosis

63. Coarctation of the Aorta
A tubular hypoplasia of the transverse aorta starting at one of the head or neck vesssels and extending to the ductal area (preductal or infantile type coarctation)

64. Coarctation of the Aorta
Ascending aortic blood flows through the narrowed segment to reach the descending aorta
First few days of life, PDA serve to widen the juxtaductal area and provide temporary relief from the obstruction  left to right shunting
But if with severe juxtaductal obstruction and presence of transverse arch hypoplasia, right ventricular blood is ejected through the ductus to supply the descending aorta
Perfusion of the lower part of the body is then dependent on the right ventricular output. Femoral pulses are palpable. The ductal right to left shunting as cyanotic. Upper extremities being pink and lower extremities blue

65. Coarctation of the Aorta
Discrepancy in blood pressure and pulses of the amrs and legs
Radial-femoral delay
occurs when blood flow to the descending aorta is dependent on collaterals
femoral pulse is felt after the radial pulse
Ascending aortic blood flows through the narrowed segment to reach the descending aorta
First few days of life, PDA serve to widen the juxtaductal area and provide temporary relief from the obstruction  left to right shunting
But if with severe juxtaductal obstruction and presence of transverse arch hypoplasia, right ventricular blood is ejected through the ductus to supply the descending aorta
Perfusion of the lower part of the body is then dependent on the right ventricular output. Femoral pulses are palpable. The ductal right to left shunting as cyanotic. Upper extremities being pink and lower extremities blue

66. Coarctation of the Aorta
Notching of the inferior border of the ribs due to enlarged collateral vessels
Other dx:
2D echo
CT and MRI
Cardiac Catheterization
Ascending aortic blood flows through the narrowed segment to reach the descending aorta
First few days of life, PDA serve to widen the juxtaductal area and provide temporary relief from the obstruction  left to right shunting
But if with severe juxtaductal obstruction and presence of transverse arch hypoplasia, right ventricular blood is ejected through the ductus to supply the descending aorta
Perfusion of the lower part of the body is then dependent on the right ventricular output. Femoral pulses are palpable. The ductal right to left shunting as cyanotic. Upper extremities being pink and lower extremities blue

67. Coarctation of the Aorta
Tx:
Prostaglandin E: to reopen the ductus and re- establish adequate lower extremity blood flow
Surgical

68. Coarctation of the Aorta
Tx:
Prostaglandin E: to reopen the ductus and re- establish adequate lower extremity blood flow
Surgical

69. Acyanotic Congenital Heart Disease: Regurgitant Lesions

70. Pulmonary valvular Insufficiency
Usually rare
Clinical signs
Descrescendo diastolic murmur at the upper and midleft sternal border
Dx:
Chest Xray: Right ventricular enlargement and prominence of the main pulmonary artery
2D Echo

71. Mitral valve insufficiency
Usually associated with other cardiac anomalies
Isolated cases: mitral valve annulus is usually dilated, the chordae tendinae are short and may insert anomalously and the valve leaflets are deformed

72. Mitral valve insufficiency
High-pitched apical holosystolic murmur
If in severe cases: may be associated with mid- diastolic rumbling murmur
Enlarged left atrium and Left ventricular hypertrophy
Tx: mitral valvuloplasty

73. Mitral valve prolapse
Caused by billowing of one or both mitral leaflets especially the posterior cusp, into the left atrium toward the end of systole
Prolapse: defined by single or bileaflet prolapse of ≥2mm beyond the long axis of the annular plane with or without leaflet thickening
Classic: >5mm valve thickening

74. Mitral valve prolapse
Common in patients with Marfan syndrome, straight back sydrome, pectus excavatum, scoliosis, Ehlers-Danlos syndrome, Osteogenesis Imperfecta, and pseudoxanthoma elasticum

75. Mitral valve prolapse
Apical murmur is late systolic and may be preceded by a click
2D echo: posterior movement of the posterior mitral leaflet during mid-or late systole or pansystolic prolapse of both the anterior and posterior leaflets

76. Tricuspid Regurgitation
Isolated tricuspid regurgitation occurs with ebstein anomaly of the tricuspid valve
This often accompanies right ventricular dysfunction
May be related with perinatal aspyxia due to increased susceptibility of the papillary muscles to ischemic damage and subsequent transeint papillary muscle dysfunction