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Obstructive lesions
Coarctation of Aorta
Definition
 Coarctation of Aorta consists of a discrete
narrowing in the proximal thoracic aorta, just
opposite to the insertion of the ductus arteriosus.
 It may be long segment stenosis
Types
1. Simple coarctation :
it can be with or without the presence of
PDA
2. Complex Coarctation:
coexist with VSD, ASD, TGA, aortic
stenosis, AVSD
3. Other associated anomalies:
variations in brachiocephalic artery
anatomy
Development of collateral arterial
circulation
berry aneurysms of the circle of Willis
Etiology
1. Abnormal migration of ductus smooth muscle
cells into the periductal aorta, with subsequent
constriction & narrowing of the aortic lumen
2. Develop as a result of hemodynamic
disturbances that reduce the volume of blood
flow through the fetal aortic arch and isthmus
Epidemiology
Common with 6-8% of CHD”S
Male > female- 2:1
Present in 35% of female with turners syndrome
(X0)
Turners syndrome
Pathophysiology
Blood from right ventricle
↓
Pulmonary artery
↓ (with PDA)
Descending arch of aorta
↓
Supplied to the body
If PDA closes
Narrowing or stenosis of aorta
↓
Left ventricular overload or hypertrophy
↓
Left atrial overload
↓
Pulmonary congestion
↓
Pulmonary artery hypertension
↓
Right ventricular hypertrophy
↓
Congestive cardiac failure
Clinical features
1) Asymptomatic with minor defect
2) Feeding difficulty, poor weight gain
3) Dyspnoea on exertion
4) Failure to thrive
5) Pitting edema
6) Gallop rhythm
7) Heart murmur
8) Fatigue, weakness
9) Frequent epistaxis
10) Cramps and intermittent
claudication
11) Headache
12) Over growth of upper limbs
and chest
13) Dilated and tortuous
collaterals may be seen over
the inter scapular area in
older children called as ‘
SUZMAN SIGN’.
Collaterals- Suzman sign
14) Tachycardia, Tachypnoea
15) Diaphoresis
16) Hepatomegaly
17) BP & pulse of upper and lower limb vary
Upper limb may have full noting, bounding pulse
where as lower extremities pulse may not be palpable.
BP will have the variation of 20mm of Hg
from upper to lower limb.
Diagnosis
A. ECG
B. Echocardiography: stenosis, lesions
C. X-ray: ventricular enlargement, ‘E’ or ‘3’ sign on
barium swallow, due to precoarctational, post
coarctational dilatation & middle narrowed
coarctation.
Management
Medical :
Ionotropic support and diuretic therapy
Prostaglandin infusion
Surgical :
End to end anastomosis
Prosthetic patch aortoplasty
Subclavian flap aortoplasty
Percutaneous balloon angioplasty
 Following surgery re-coarctation, systemic HTN can arise.
Which could be corrected with balloon angioplasty.
Patch aortoplasty
Complications
 CCF
 Aortic rupture
 Aortic aneurysm
 CVA
 Rupture of berry intracranial aneurysm
 Rupture of an intercostal aneurysm
 Dissection of aorta
Aortic stenosis
 Obstruction to the left ventricular outflow tract
which may be at the level of the aortic valve,
above the valve ( supravalvular) or below the
valve (infravalvular)
 Constitutes 8% of CHD
Hemodynamics
Blood from the left ventricle
↓through stenosed valve
Aorta
↓
But stenosed valve of the aorta creates more
pressure in the left ventricle
↓
Left ventricular hypertrophy
↓
MI & pulmonary edema
CCF, & Infective endocarditis
Clinical features
During infancy:
 Decreased cardiac output
 Feeble peripheral pulses
 Pale look
 Dusky & Cool skin
In older children:
 Chest infections
 Chest pain or angina
 Dyspnea on exertion
 Fatigue
 Narrow pulse pressure
 Fainting episodes (syncope)
 Exercise intolerance
Diagnosis
 Pulse is low volume & there is a thrill in the
suprasternal notch
 Auscultation reveals a harsh, low-pitched systolic
ejection murmur, maximal at the second right
intercostal space.
 Chest X-ray- dilated aorta, enlargement of left
ventricle
 ECG-T wave inversion
 Echo- identifies level of obstruction
Prevention & Treatment
 Restriction of physical activities like athletics,
outdoor games, strenuous activities and
competitive sports. Because it can be a deadliest
disease causing death within no time.
 Balloon dilatation or balloon aortic
valvuloplasty is a non-operative relief of the
lesion. Done through femoral
catheterization. Only contraindication is
aortic regurgitation.
Surgery
 Aortic valvulotomy
 Aortic valve replacement
Pulmonic stenosis
 There is obstruction to flow of blood from the right
ventricle to lungs.
 Accounts for 2% of CHD.
 90% are at the level of the valve remaining
supravalvular and subvalvular
(infundibular)
Hemodynamics
Blood from the right ventricle
↓ through stenosed valve
Pulmonary artery
↓
Because of obstruction to the output
and to maintain the cardiac output
right ventricle undergoes hypertrophy
↓
Rt. Sided CCF
Clinical features
 Child may be generally asymptomatic but may
have decreased exercise tolerance with fatigue
and dyspnea.
 With severe obstruction :
Dyspnea
Cyanosis,
Precordial pain
 On physical examination: the patients ate
characteristically described as having a round
face and hypertelorism. Port-wine angiomatous
malformation over the skin. Turner’s phenotype
without chromosomal abnormalities is associated
with pulmonic stenosis.
Diagnosis
 Auscultation reveals a systolic ejection murmur
over pulmonic area
 Chest X-ray- enlargement of right ventricle and
main pulmonary artery
 ECG- rt. Ventricular hypertrophy
 2D Echo -Level of obstruction
Treatment
 At cardiac catheterization if the right ventricular
pressure is 75% or more of the systemic systolic
pressure child can be operated.
 Balloon pulmonary valvuloplasty

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Coarctation of aorta

  • 3. Definition  Coarctation of Aorta consists of a discrete narrowing in the proximal thoracic aorta, just opposite to the insertion of the ductus arteriosus.  It may be long segment stenosis
  • 4.
  • 5. Types 1. Simple coarctation : it can be with or without the presence of PDA 2. Complex Coarctation: coexist with VSD, ASD, TGA, aortic stenosis, AVSD 3. Other associated anomalies: variations in brachiocephalic artery anatomy Development of collateral arterial circulation berry aneurysms of the circle of Willis
  • 6. Etiology 1. Abnormal migration of ductus smooth muscle cells into the periductal aorta, with subsequent constriction & narrowing of the aortic lumen 2. Develop as a result of hemodynamic disturbances that reduce the volume of blood flow through the fetal aortic arch and isthmus
  • 7. Epidemiology Common with 6-8% of CHD”S Male > female- 2:1 Present in 35% of female with turners syndrome (X0)
  • 9. Pathophysiology Blood from right ventricle ↓ Pulmonary artery ↓ (with PDA) Descending arch of aorta ↓ Supplied to the body
  • 10. If PDA closes Narrowing or stenosis of aorta ↓ Left ventricular overload or hypertrophy ↓ Left atrial overload ↓ Pulmonary congestion ↓ Pulmonary artery hypertension ↓ Right ventricular hypertrophy ↓ Congestive cardiac failure
  • 11. Clinical features 1) Asymptomatic with minor defect 2) Feeding difficulty, poor weight gain 3) Dyspnoea on exertion 4) Failure to thrive 5) Pitting edema 6) Gallop rhythm 7) Heart murmur 8) Fatigue, weakness 9) Frequent epistaxis
  • 12.
  • 13. 10) Cramps and intermittent claudication 11) Headache 12) Over growth of upper limbs and chest 13) Dilated and tortuous collaterals may be seen over the inter scapular area in older children called as ‘ SUZMAN SIGN’.
  • 15. 14) Tachycardia, Tachypnoea 15) Diaphoresis 16) Hepatomegaly 17) BP & pulse of upper and lower limb vary Upper limb may have full noting, bounding pulse where as lower extremities pulse may not be palpable. BP will have the variation of 20mm of Hg from upper to lower limb.
  • 16. Diagnosis A. ECG B. Echocardiography: stenosis, lesions C. X-ray: ventricular enlargement, ‘E’ or ‘3’ sign on barium swallow, due to precoarctational, post coarctational dilatation & middle narrowed coarctation.
  • 17. Management Medical : Ionotropic support and diuretic therapy Prostaglandin infusion Surgical : End to end anastomosis Prosthetic patch aortoplasty Subclavian flap aortoplasty Percutaneous balloon angioplasty
  • 18.  Following surgery re-coarctation, systemic HTN can arise. Which could be corrected with balloon angioplasty.
  • 20.
  • 21. Complications  CCF  Aortic rupture  Aortic aneurysm  CVA  Rupture of berry intracranial aneurysm  Rupture of an intercostal aneurysm  Dissection of aorta
  • 22. Aortic stenosis  Obstruction to the left ventricular outflow tract which may be at the level of the aortic valve, above the valve ( supravalvular) or below the valve (infravalvular)  Constitutes 8% of CHD
  • 23. Hemodynamics Blood from the left ventricle ↓through stenosed valve Aorta ↓ But stenosed valve of the aorta creates more pressure in the left ventricle ↓ Left ventricular hypertrophy ↓ MI & pulmonary edema CCF, & Infective endocarditis
  • 24. Clinical features During infancy:  Decreased cardiac output  Feeble peripheral pulses  Pale look  Dusky & Cool skin
  • 25. In older children:  Chest infections  Chest pain or angina  Dyspnea on exertion  Fatigue  Narrow pulse pressure  Fainting episodes (syncope)  Exercise intolerance
  • 26. Diagnosis  Pulse is low volume & there is a thrill in the suprasternal notch  Auscultation reveals a harsh, low-pitched systolic ejection murmur, maximal at the second right intercostal space.  Chest X-ray- dilated aorta, enlargement of left ventricle  ECG-T wave inversion  Echo- identifies level of obstruction
  • 27. Prevention & Treatment  Restriction of physical activities like athletics, outdoor games, strenuous activities and competitive sports. Because it can be a deadliest disease causing death within no time.
  • 28.  Balloon dilatation or balloon aortic valvuloplasty is a non-operative relief of the lesion. Done through femoral catheterization. Only contraindication is aortic regurgitation.
  • 29. Surgery  Aortic valvulotomy  Aortic valve replacement
  • 30. Pulmonic stenosis  There is obstruction to flow of blood from the right ventricle to lungs.  Accounts for 2% of CHD.  90% are at the level of the valve remaining supravalvular and subvalvular (infundibular)
  • 31. Hemodynamics Blood from the right ventricle ↓ through stenosed valve Pulmonary artery ↓ Because of obstruction to the output and to maintain the cardiac output right ventricle undergoes hypertrophy ↓ Rt. Sided CCF
  • 32. Clinical features  Child may be generally asymptomatic but may have decreased exercise tolerance with fatigue and dyspnea.  With severe obstruction : Dyspnea Cyanosis, Precordial pain
  • 33.  On physical examination: the patients ate characteristically described as having a round face and hypertelorism. Port-wine angiomatous malformation over the skin. Turner’s phenotype without chromosomal abnormalities is associated with pulmonic stenosis.
  • 34. Diagnosis  Auscultation reveals a systolic ejection murmur over pulmonic area  Chest X-ray- enlargement of right ventricle and main pulmonary artery  ECG- rt. Ventricular hypertrophy  2D Echo -Level of obstruction
  • 35. Treatment  At cardiac catheterization if the right ventricular pressure is 75% or more of the systemic systolic pressure child can be operated.  Balloon pulmonary valvuloplasty