Congenital Heart Disease

1. CONGENITAL HEART DISEASE
DR P J MWANYIKA

2. DEFINITION
• Defect in the structure of the heart and great
vessels which is present at birth
• More common in premature infants
• Commonest congenital malformations
10:1000 live births (1%)
• All diagnosed by echocardiography

3. Aetiology
Multifactorial
• Maternal illness (diabetes mellitus, phenylketonuria,
and systemic lupus erythematosus)
• Maternal infections (Rubella, Seizure disorder)
• Drugs (lithium, thalidomide)
• Known teratogens
• Chromosomal abnormality (especially trisomies): 7% of
CHD – Down’s syndrome in 5% of CHD. Also Edwards,
Patau, Turner – ?22q11 microdeletion - another 5%

4. Classification
Left-to-Right Shunt Lesions
• Atrial Septal Defect (ASD)
• Ventricular Septal Defect (VSD)
• Atrioventricular Septal Defect (AV Canal)
• Patent Ductus Arteriosus (PDA)

5. Classification
Right to left shunt
• Tetralogy of fallot
• Transposition of great Arteries
• Total anomalous Pulmonary Venous Return
• Truncus Arterious

6. Classification
Obstructive Heart lessions
• Pulmonary Stenosis
• Aortic Stenosis
• Coarctation of the Aorta

7. Atrial Septal Defect
• ASD is an opening in the atrial septum permitting free
communication of blood between the atria. Seen in 10% of all
CHD.
• Different anatomic types:
– Secundum ASD (80% of ASDs; located in the region of the fossa
ovalis and its surrounding)
– Primum ASD (15%, synonym: partial atrioventricular septal defect
(AVSD), located near the crux, AV valves are typically malformed
resulting in various degrees of regurgitation (esp. Mitral)
– Sinus venosus defect (5%, located near the superior vena cava
(SVC) entry, associated with partial or complete connection of right
pulmonary veins to SVC/right atrium (RA)

8. ASD

9. Hemodynamics of ASD
• Red blood crosses from LA to RA
• Increased O2 saturation in RA and increased RA
volume
• Increased O2 saturation in RV and increased RV
volume
• Increased blood in pulmonary circuit, but normal
pulmonary artery pressures and resistance

10. Clinical Findings
• Some patients remain asymptomatic throughout
life
• Easy fatiquability and mild growth failure
• RV precordial bulge with RV heave LLSB (due to
enlarged RV)
• murmur: systolic ejection murmur due to
increased blood flow across the pulmonary valve
- not due shunting across the ASD, this is a low
velocity shunt and does not make noise
• S2 (second heart sound): widely split - due to
delayed emptying of the volume-overloaded RV

11. Clinical findings
• Arrhythmias (Atrial fibrillation, flutter) – Hypertrophy
and dilatation of RA
• Increase of transpulmonary flow – reactive higher
pulmonary vascular resistance
that results into severe PAH (only in 5%) and
bidirectional shunt (Eisenmenger syndrome)
• Paradoxical embolism (thrombus from lower limb
veins through ASD to systemic circulation e.g. CNS)

12. Diagnosis
• Imaging
• ECG
• ECHO
• Cardiac Catheterization

13. Imaging
• Radiographs shows cardiac enlargement
• Increased pulmonary vascular markings

14. Electrocardiography (ECG)
• Right axis deviation
• Hypertrophy RV, RA

15. Echocardiogram (ECHO)
• Dilated RV
• Direct visualization of the ASD
• Demonstration of left to right shunt through
the defect by color flow Doppler

16. Cardiac Catherization
• Rarely needed for diagnostic purpose
• Reveals a significant increase in oxygen at the
atrial level.
• Normal pulmonary artery pressure
• Pulmonary to Systemic blood flow vary from
1.5:1 to 4.1

17. Treatment
• Surgical or Catheterization laboratoey closure recommended for Ostium
Secundum ASDs in which ratio of pulmonary to systemic is 2:1
• Closure is elective between ages 1 and 3years. (ASD rarely close
spontaneously after age 3)
• ASD primum – connected with cleft of anterior mitral leaflet with mitral
regurgitation (mostly significant) and in Sinous Venous ASDs surgical
treatment usually necessary
AIM: To prevents development of pulmonary vascular disease
• Unrepaired ASDs result in pulmonary vascular disease in 5 - 10% of
patients
• Unrepaired there is a risk of paradoxical emboli – stroke

18. Ventricular Septal Defect
• VSD – is an abnormal opening in the
ventricular septum, which allows free
communication between the Rt & Lt
ventricles. Accounts for 25% of CHD.

19. Ventricular Septal Defect
• 4 Types
• Perimembranous (or membranous) – Most
common.
• Infundibular (subpulmonary or supracristal VSD) –
involves the RV outflow tract.
• Muscular VSD – can be single or multiple.
• AVSD – inlet VSD, almost always involves AV
valvular abnormalities.

20. Ventricular Septal Defect
Defect size more important than location
• Small to medium sized defects – the size
restricts the amount of left to right shunting
• Large defect – nonrestrictive, no resistance to
flow across the defect
• Shunt amount and direction determined by
the relative resistances of the systemic and
pulmonary circuits

21. Large VSD
• Red blood crosses from LV to RV through VSD resulting in
increased O2 sat in RV
• Large hole allows equalization of LV and RV pressure
(increased RV and PA pressure) not pressure restrictive
• Increased blood in pulmonary circuit and increased
pressure and O2 saturation in pulmonary artery
• Increased LA volume due to increased blood returning from
pulmonary circuit
• Increased blood in LV- results in LV enlargement
• Increased LV radius - myocardial fibers lengthen
• With significant LV dilation, the myocardium cannot
develop sufficient tension to maintain pressure -
Congestive Heart Failure (CHF)

22. CHF due to Large VSD
• LV volume overload (increased preload)
• - LV dilation
• - increased LV radius (R)
• -as LV radius increases LV tension (T) must increase to maintain pressure
(P) (LaPlace relationship: T= P X R)
• With continued LV dilation the myocardium cannot develop sufficient
tension to maintain the pressure volume relationship
• - congestive heart failure
• Compensatory mechanisms to maintain myocardial performance and
cardiac output
• - stimulation of the sympathetic adrenal system increased catecholamine
release
• - increased catecholamine release
• - increased force of contraction and heart rate
• - Myocardial hypertrophy

23. Small VSD
• Small defects are pressure restrictive - do not
allow equalization of LV and RV pressures
• Shunt is left to right because LV systolic
pressure greater than RV systolic pressure
• Pressure restrictive
Normal pulmonary artery pressures
L > R shunt is small

24. Clinical findings
• Small - moderate VSD, 3-5mm, are usually
asymptomatic.
• Small defects located predominantly in the
muscular septum with slight hemodynamic
impairment
• Moderate – large VSD, almost always have
symptoms and will require surgical repair.

25. Clinical Findings
• If the defect is large and pulmonary vascular
resistance is not significantly elevated (L-t-R-
shunt)– growth failure, CHF, repeated lower
respiratory tract infections (begin at 1-
2month)
• If the defect is large and pulmonary vascular
resistance is very high (Eisenmenger’s
reaction) – shortness of breath, dyspnea on
exertion, chest pain, cyanosis;

26. Clinical Findings
• Pansystolic/holosystolic murmur - loud, harsh,
blowing heard best over the LLSB, frequently
is accompanied by thrill (depending upon the
size of the defect) +/-more prominent with
small VSD, may be absent with a very large
VSD.

27. Diagnosis
• Imaging
• Electrocardiography
• Echocardiogram

28. Imaging
• In patients with small VSDs, the chest radiograph
is usually normal, although minimal cardiomegaly
and a borderline increase in pulmonary
vasculature may be observed.
• In large VSDs, the chest radiograph shows gross
cardiomegaly with prominence of both ventricles,
the left atrium, and the pulmonary artery
• Pulmonary vascular markings are increased, and
frank pulmonary edema, including pleural
effusions, may be present.

29. ECG
• The electrocardiogram shows biventricular
hypertrophy; P waves may be notched or
peaked.

30. ECHO
• Echocardiogram shows the position and size
of the VSD

31. Treatment
• The natural course of a VSD depends to a large degree on
the size of the defect.
• 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 than
membranous VSDs
• In large VSD, medical management has two aims: to control
heart failure and prevent the development of pulmonary
vascular disease.
• Indications for surgical closure of a VSD include patients at
any age with large defects in whom clinical symptoms and
failure to thrive cannot be controlled medically

32. Patent Ductus Arteriosus
• PDA – Persistence of the normal fetal vessel
that joins the PA to the Aorta.
• Normally closes in the 1st wk of life.
• Female:Male ratio of 2:1
• Often associated w/coarctation & VSD.
• PDA is also associated with maternal rubella
infection during early pregnancy
• It is a common problem in premature infants

33. Hemodynamic PDA
• Shunting of red blood from the aorta to pulmonary artery
• Increased pulmonary blood flow, increased pulmonary
artery pressure and increased O2saturation in the
pulmonary artery
• Low diastolic blood pressure due to “run-off” into the
pulmonary artery
• Increased blood in the pulmonary circuit – this results in
increased blood returning to the LA and increased LA
pressure. Because of poor gas exchange in the lung as a
result of CHF, there is a low LA saturation
• Increased blood in the LV results in LV enlargement and
increased LV end diastolic pressure (LVEDP) and CHF
develops

34. Clinical Findings
• A small patent ductus does not usually have any
symptoms associated with it.
• A large PDA will result in heart failure similar to
that encountered in infants with a large VSD.
• Retardation of physical growth may be a major
manifestation in infants with large shunts.
• The apical impulse is prominent and, with cardiac
enlargement, is heaving. A thrill, maximal in the
2nd left interspace, is often present and may
radiate toward the left clavicle, down the left
sternal border, or toward the apex.

35. Clinical Findings
• The classic continuous murmur is described as
being like machinery or rolling thunder in quality.
It begins soon after onset of the 1st sound,
reaches maximal intensity at the end of systole,
and wanes in late diastole. It may be localized to
the 2nd left intercostal space or radiate down the
left sternal border or to the left clavicle.
• In patients with a large left-to-right shunt, a low-
pitched mitral mid-diastolic murmur may be
audible at the apex as a result of the increased
volume of blood flow across the mitral valve.

36. Diagnosis
• Imaging
• Electrocardiography
• Echocardiography
• Catherization and Angiography

37. Imaging
• In simple PDA, the radiographic appearance
depends on the size of the shunt.
• If the shunt is relatively small, the heart is not
enlarged.
• If the shunt is large, both left atrial and LV
enlargement may be present. The aorta and
the main pulmonary artery segment may be
prominent.

38. Electrocardiography
• The ECG may be normal or may show LVH,
depending on the size of the shunt. In patients
with pulmonary hypertension caused by
increased blood flow, biventricular
hypertrophy usually occurs. In those with
pulmonary vascular obstructive disease, pure
right ventricular hypertrophy (RVH) occurs.

39. Echocardiography
• Direct visualization of the ductus and
confirmation of the direction and degree of
shunting

40. Cardiac catheterization and
angiography
• Children with PDA never require a cardiac
catheterization for diagnostic purposes.
However, children > 5 kg with a PDA routinely
have the ductus closed in the catheterization
laboratory. Device closure is the sole
indication for cardiac catheterization in PDA.

41. Treatment
• Irrespective of age, patients with PDA require surgical or
catheter closure.
• In patients with a small PDA, the rationale for closure is
prevention of bacterial endarteritis or other late
complications.
• In patients with a moderate to large PDA, closure is
accomplished to treat heart failure or prevent the
development of pulmonary vascular disease, or both.
• Once the diagnosis of a moderate to large PDA is made,
treatment should not be unduly postponed after adequate
medical therapy for cardiac failure has been instituted.

42. Atrioventricular Defect
• Atrioventricular septal defect (AVSD) results from
incomplete fusion of the embryonic endocardial
cushions.
• The endocardial cushions help to form the “crux” of
the heart, which includes the lower portion of the
atrial septum, the membranous portion of the
ventricular septum, and the septal leaflets of the
tricuspid and mitral valves.
• AVSD accounts for about 4% of all congenital heart
disease. 60% of children with Down syndrome have
congenital heart disease, and of these, 35–40% has
AVSD.

43. Atrioventricular Defect
• Types of AV septal defects
-partial
-complete.
• In partial AVSD, there are two separate AV valve
orifices and usually a cleft in the left-sided valve
• In Complete AVSD valves are located in the
midportion of the defect, both atrial and
ventricular components of the septal defect are
present and the left- and right-sided AV valves
share a common ring or orifice.

44. AVSD
• Partial AVSD behaves like an isolated ASD with
variable amounts of regurgitation through the
cleft in the left AV valve
• The complete form causes large left-to-right
shunts at both the ventricular and atrial levels
with AV valve regurgitation. If there is
increased pulmonary vascular resistance, the
shunts may be bidirectional.

45. AVSD

46. Clinical findings
• The partial form may produce symptoms similar
to ostium secundum ASD.
• Patients with complete AVSD usually are severely
affected. HF often develops in infancy, and
recurrent bouts of pneumonia are common.
• Audible murmur due to relatively equal systemic
and pulmonary vascular resistance (PVR) in a
complete form.
• There is both rightand left-sided cardiac
enlargement. S2 is loud, and a pronounced
diastolic flow murmur may be heard at the apex
and the lower left sternal border.

47. Clinical findings
• If severe pulmonary vascular obstructive
disease is present, there is usually dominant
RV enlargement. S2 is palpable at the
pulmonary area and no thrill is felt. A
nonspecific short systolic murmur is heard at
the lower left sternal border. No diastolic flow
murmurs are heard.
• In severe cases predominant right-to-left
shunting causes cyanosis.

48. Diagnosis
• Imaging
• ECG
• ECHO
• Cardiac Catheterization and Angiography

49. Imaging
• Cardiac enlargement is always present. In the
complete form, all four chambers are
enlarged. Pulmonary vascular markings are
increased.
• In patients with pulmonary vascular
obstructive disease, only the main pulmonary
artery segment and its branches are
prominent and the peripheral pulmonary
vascular markings are usually decreased.

50. ECG
• Extreme left axis deviation with a
counterclockwise loop in the frontal plane.
• First-degree heart block occurs in over 50% of
patients.
• Right, left, or combined ventricular
hypertrophy is present depending on the
particular defect and the presence or absence
of pulmonary hypertension.

51. ECHO
• Echocardiography is the diagnostic test of choice
• Defected anatomy can be well visualized by two-
dimensional echocardiography.
• Both AV valves are at the same level, compared with
the normal heart in which the tricuspid valve is more
apically positioned.
• The size of the atrial and ventricular components of the
defect can be measured.
• AV valve regurgitation can be detected. The LV outflow
tract is elongated (gooseneck appearance), which
produces systemic outflow obstruction.

52. Cardiac Catheterization and
Angiography
• Cardiac catheterization is not routinely used to
evaluate AVSD but may be used to assess
pulmonary artery pressures in older infant
with down syndrome as this group is
predisposed to early-onset pulmonary
hypertension
• Angiocardiography reveals the characteristic
gooseneck deformity of the LV outflow tract in
the complete form.

53. Treatment
• Surgery is always required. In the partial form,
surgery carries a low mortality rate (1–2%), but
patients require follow-up because of late-
occurring LV outflow tract obstruction and mitral
valve dysfunction.
• The complete form carries a higher mortality
rate.
• Complete correction in the first year of life, prior
to the onset of irreversible pulmonary
hypertension, is obligatory.

54. Tetralogy of Fallots
• ToF is the most common cyanotic cardiac lesion and
accounts for 10% of all congenital heart disease.
• A single embryologic abnormality causes multiple
morphologic problems.
• Abnormality in the septation of the conus and truncus
arteriosus with anterior deviation of the conus into the RV
outflow tract causes
-Narrowing of the right ventricular outflow tract
- VSD
- Dextroposition of the aorta with override of the
ventricular septum.
• The RV hypertrophies occur because it pumps against
systemic resistance across a (usually) large VSD.

55. TOF
• The VSD is usually located in the membranous ventricular
septum but may be surrounded completely by muscular
tissue.
• Obstruction to RV outflow may be primarily at the
infundibular level (in 25–50% of cases), at the valvular level
alone (rarely), or at both levels (most commonly)
• Obstruction to RV outflow plus a large VSD results in a
right-to-left shunt at the ventricular level and arterial
desaturation
• Although the patient may be deeply cyanotic, the amount
of pressure the RV can develop is limited to that of the
systemic (aortic) pressure because the VSD is unrestrictive.
• The RV is able to maintain this level of pressure without
developing heart failure.

56. Clinical Findings
• Clinical findings vary with the degree of RV outflow
obstruction.
• Minimally cyanotic or acyanotic for the patients with mild
obstruction. Often, cyanosis is not present at birth, but with
increasing hypertrophy of the right ventricular
infundibulum and patient growth, cyanosis occurs later in
the 1st yr of life.
• In infants with severe degrees of right ventricular outflow
obstruction, neonatal cyanosis is noted immediately.
Initially pulmonary blood flow may be dependent on flow
through the ductus arteriosus. Severe cyanosis and
circulatory collapse may occur when the ductus close.
• Children with long-standing cyanosis who have not
undergone surgery may have dusky blue skin, gray sclerae
with engorged blood vessels, and marked clubbing of the
fingers and toes

57. Clinical Findings
• Dyspnea occurs on exertion with squatting position for
the relief of dyspnea
• Hypoxic, “blue,” or “tet” spells particularly during the
1st 2 yr of life.
• Delay in growth and development and puberty in
patients with severe untreated tetralogy of Fallot,
particularly when oxygen saturation is chronically
<70%.
• The left anterior hemithorax may bulge anteriorly due
to right ventricular hypertrophy.
• Systolic thrill along the left sternal border in the 3rd
and 4th parasternal border

58. Clinical Findings
• The systolic murmur most intense at the left
sternal border, generally ejection in quality at
the upper sternal border and holosystolic
toward the lower sternal border.

59. Diagnosis
• Laboratory
• Imaging
• ECG
• ECHO
• Catheterization and Angiography

60. Laboratory
• Hemoglobin, hematocrit, and red blood cell
count are usually elevated secondary to
arterial desaturation.

61. Imaging
• Normal size heart.
• RVH shown by an upturning of the apex (boot-
shaped heart).
• The main pulmonary artery segment is usually
concave and, if there is a right aortic arch, the
aortic knob is to the right of the trachea.
• Decreased pulmonary vascular markings

62. ECG
• The QRS axis is rightward, ranging from +90 to
+180 degrees.
• The P waves are usually normal.
• RVH is always present, but RV strain patterns
are rare.

63. ECHO
• Reveal thickening of the RV wall, overriding of the
aorta, and a large subaortic VSD.
• Obstruction at the level of the infundibulum and
pulmonary valve can be identified, and the size of
the proximal pulmonary arteries can be
measured.
• The anatomy of the coronary arteries should be
visualized, as abnormal branches crossing the RV
outflow tract are at risk for transection during
surgical enlargement of the area.

64. Catheterization and Angiography
• Cardiac catheterization reveals a right-to-left
shunt at the ventricular level in most cases.
• Presence of varied degree of arterial desaturation
• The RV pressure is at systemic levels and the
pressure tracing in the RV is identical to that in
the LV if the VSD is large.
• The pulmonary artery pressure is invariably low.
Pressure gradients may be noted at the valvular
level, the infundibular level, or both.
• RV angiography reveals RV outflow obstruction
and a right-to-left shunt at the ventricular level.

65. Treatment
• Medical palliation
• Surgical correction

66. Medical palliation
• IV prostaglandin E1 (0.01–0.20 μg/kg/min), a
potent and specific relaxant of ductal smooth
muscle, causes dilatation of the ductus
arteriosus and usually provides adequate
pulmonary blood flow until a surgical
procedure can be performed
• Long-term oral β-blocking agents (propranolol
0.5–1 mg/kg every 6 hr) may delay surgery

67. Surgical Correction
• Corrective open heart surgery performed in early
infancy and even in the newborn period in
critically ill infants but range from birth to 2 years.
During this surgery, the VSD is closed and the
obstruction to RV outflow removed. Surgical
mortality varies from 1–2%.
• Surgical palliation is by insertion of a GoreTex
shunt from the subclavian artery to the ipsilateral
pulmonary artery (modified Blalock-Taussig
shunt). It has a very low likelihood of mortality

68. Complications of TOF
• Before correction, patients with the tetralogy
of Fallot are susceptible to serious
complications such as
-Brain Abscess
-Bacteria Endocarditis

69. Transposition of great arteries
• Second most common cyanotic congenital heart
disease, accounting for 5% of all cases of
congenital heart disease.
• Male female ratio is 3:1.
• It is caused by an embryologic abnormality in the
spiral division of the truncus arteriosus in which
the aorta arises from the RV and the pulmonary
artery from the LV
• Survival is impossible without mixing between
the two circuits hence PDA and the patent
foramen ovale are critically important

70. ToGA

71. Clinical Findings
• Many neonates are large (up to 4 kg) and
profoundly cyanotic without respiratory
distress or a significant murmur
• Infants with a large VSD may be less cyanotic
and they usually have a prominent murmur.
The findings on cardiovascular examination
depend on the intracardiac defects.
Obstruction to outflow from either ventricle is
possible, and coarctation must be ruled out.

72. Imaging
• Usually non diagnostic
• “egg on a string” appearance because aorta
is directly anterior to the main pulmonary
artery, giving the image of a narrow
mediastinum.

73. ECG
• ECG in transposition is of little help, as it will
frequently look normal.

74. ECHO
• Demonstrate the anatomy and physiology
well.
• Visualization of aorta from the RV and the
pulmonary artery arises from the LV.
• Associated defects, such as a VSD, RV or LV
outflow tract obstruction, or coarctation, may
be seen

75. Treatment
• Early corrective surgery is recommended.
• The arterial switch operation (ASO) is
performed at age 4–7 days. The arteries are
switched in the anterior chest, and the
coronaries are separately reimplanted.
• Small associated VSDs may be left to close on
their own, but large VSDs are closed at this
time. The atrial septum is also closed.
• Survival after the ASO is greater than 95%

76. Truncus Arteriosus
• Truncus arteriosus accounts for less than 1%
of congenital heart malformations.
• A single great artery arises from the heart,
giving rise to the systemic, pulmonary, and
coronary circulations.
• A VSD is always present.
• Is due to failure of the division of the common
truncus arteriosus into the aorta and the
pulmonary artery embryologically

77. Clinical Findings
• Patients with high pulmonary blood flow are
usually acyanotic and present in HF.
• Hyperactive precordium.
• A loud holosystolic murmur is audible at the
left lower sternal border
• Patients with decreased pulmonary blood flow
are cyanotic early. The most common
manifestations are growth retardation, easy
fatigability, and HF

78. Imaging
• Boot-shaped heart, absence of the main
pulmonary artery segment, and a large aorta
that has a right arch 30% of the time.
• The pulmonary vascular markings vary with
the degree of pulmonary blood flow.

79. ECG
• The axis is usually normal. RVH or combined
ventricular hypertrophy is commonly present.

80. ECHO
• Shows override of a single great artery (similar
to ToF, but no second great artery arises
directly from the heart). The origin of the
pulmonary arteries and the degree of truncal
valve abnormality can be seen.

81. Angiography
• Cardiac catheterization is not routinely
performed but may be of value in older infants
in whom pulmonary vascular disease must be
ruled out.

82. Treatment
• Surgery is usually performed in the neonatal
period invoving closure of VSD and pulmonary
artery (type 1) or arteries (types 2–3) are
separated from the truncus as a block, and a
valved conduit is fashioned from the RV to the
pulmonary circulation.

83. Tricuspid Atresia
• No outlet from the right atrium to the right
ventricle
• Entire of systemic venous return enters the left
side of the heart by means of the foramen ovale
or an associated atrial septal defect
• The left atrium receives both systemic venous
return and pulmonary venous return. Complete
mixing occurs in the left atrium, resulting in
variable degrees of arterial desaturation

84. Clinical Findings
• Cyanosis in early infant
• Poor growth and development
• Exhaustion during feedings, tachypnea, and
dyspnea
• Finger clubbing in older children
• Murmur for those with VSD

85. Imaging
• The heart is slightly to markedly enlarged
• The size of the right atrium varies from
moderately to massively enlarged, depending
on the size of the communication at the atrial
level
• Decreased pulmonary vascular markings

86. ECG
• Left axis deviation
• P waves are tall and peaked
• LVH or LV dominance

87. ECHO
• Shows absence of the tricuspid valve, the
relationship between the great arteries, and
the size of the pulmonary arteries.

88. Cardiac Catheterization and
Angiocardiography
• Reveals a large right-to-left shunt at the atrial
level
• Increased right atrial pressure
• LV and systemic pressures are normal
• The catheter can’t passed through the
tricuspid valve from the right atrium to the RV

89. Treatment
• Management of patients with tricuspid atresia
depends on the adequacy of pulmonary blood
flow.
• Severely cyanotic neonates should be
maintained on an intravenous infusion of
prostaglandin E1 (0.01–0.20 μg/kg/min) until a
surgical aortopulmonary shunt procedure can
be performed to increase pulmonary blood
flow.

90. Coarctation of the Aorta
• 6% of all congenital heart disease
• Male: Female 3: 1
• Narrowing occurs in the thoracic portion of
the descending aorta distal to the takeoff of
the left subclavian artery in the juxtaductal
region
• May be feature of Turner’s Syndrome
• Associated with bicuspid aortic valve in 70% of
patients

91. Pathophysiology
Preductal or infantile-type Coarctation )
• Ascending aortic blood flows through the narrow
segment to reach the descending aorta, = left
ventricular hypertension and hypertrophy result.
• In the 1st few days of life, the patent ductus
arteriosus may serve to widen the juxtaductal
area of the aorta and provide temporary relief
from the obstruction. In these acyanotic infants,
net left-to-right ductal shunting occurs.

92. Pathophysiology cont…
Postductal Coarctation
• In the presence of transverse arch hypoplasia = right
ventricular blood is ejected through the ductus to
supply the descending aorta, Perfusion of the lower
body is then dependent on right ventricular output.
• The ductal right-to-left shunting is manifested as
differential cyanosis, with the upper extremities being
pink and the lower extremities being blue.
• Such infants may have severe pulmonary
hypertension, high pulmonary vascular resistance and
signs of heart failure

93. Pathophysiology cont…
• In infancy, coarctation of the aorta results in
the development of an extensive collateral
circulation, from the branches of the
subclavian, the superior intercostal, and the
internal mammary arteries to create channels
for arterial blood to bypass the area of
coarctation.

94. CoA

95. Clinical Findings
• Decrease or absence of femoral pulses
• CHF in the first month of life for an infant with
coarctation alone, or in combination with VSD,
ASD, or other congenital cardiac anomalies
• Pulse discrepancy between the arms and legs
• Upper limb (arm) hypertension
• An ejection systolic murmur of grade II/VI
intensity is often heard at the aortic area and the
lower left sternal border along with an apical
ejection click from the bicuspid aortic valve also
heard in the left axilla and the left back

96. Imaging
• Findings on roentgenographic examination
depend on the age on the effects of
hypertension and collateral circulation.
• In infants with severe coarctation, cardiac
enlargement pulmonary congestion.
• During childhood - 1st decade, the heart
tends to be moderately enlarged because of
left ventricular prominence.

97. Imaging cont….
• The enlarged left subclavian artery produces a
prominent shadow in the left superior
mediastinum.
• Notching of the inferior border of the ribs
from pressure erosion by enlarged collateral
vessels is common by late childhood.
• An area of pre and poststenotic dilatation of
the descending aorta (figure 3" sign)

98. Notching of the rib

99. ECG
• ECGs in older children may be normal or may
show LVH.
• ECGs usually shows RVH in infants with severe
coarctation.

100. ECHO
• Visualization of the coarctation
• If the ductus arteriosus is patent,
echocardiography may not detect the coarctation
• Associated lesions such as a bicuspid aortic valve
or mitral abnormalities may suggest the presence
of a coarctation.
• Evidence of left ventricular hypertrophy in older
patients.
• Neonates and young infants display right or
biventricular hypertrophy.

101. Cardiac Catheterization and
Angiocardiography
• Cardiac catheterization and angiocardiography
are rarely performed for infants or children
with coarctation unless dilation of the coarct
during catheter study is planned.

102. Treatment
• Infants with coarctation of the aorta and CHF may
present in extremis secondary to LV dysfunction and
associated low cardiac output.
• Prostaglandin infusion (0.025–0.1 g/kg/min) to reopen
the ductus arteriosus and inotropic support.
• Infant undergo corrective repair when stablelized
• Primary balloon aortoplasty in infants with coarctation
canbe done
• Modified end-to-end anastomosis technique or the left
subclavian approach, has a high success rate, but there
is a 10–15% rate of recoarctation after repair.

103. Complications
• Systemic hypertension
• Premature coronary artery disease
• Heart failure
• Hypertensive encephalopathy,
• Intracranial hemorrhage.
• Infective endocarditis or endarteritis
• Aneurysms of the descending aorta or of the
collateral