DR J P SONI Professor and Head of the Department Paediatrics Division of Paediatric Cardiology DR S N Medical College Jodhpur Doc_jpsoni@yahoo.com

1. Congenital Malformation of aorta
DR J P SONI
Professor and Head of the Department Paediatrics
Division of Pediatric Cardiology
DR S N Medical College
Jodhpur
Doc_jpsoni@yahoo.com

2. P D A
Most neonates have a patent ductus arteriosus in the first 8 hours of life with spontaneous
closure occurring in 42 % at 24 hours of age, in 90 % at 48 hours of age and in almost all
infants at 96 hours
In a classic description still used today, Desmond and colleagues organized these changes into three stages:
•The first period of reactivity (0-30 minutes) characterized by an increase in heart rate, irregular
respirations and fine crackles in the chest with grunting and nasal flaring
•A period of decreased responsiveness (30 minutes to 3 hours) with rapid shallow respirations, lower heart
rate, decreased muscle activity interspersed with jerks and twitches and sleep
•A second period of reactivity (2-8 hours) in which exaggerated responsiveness, tachycardia, labile heart
rate, abrupt changes in tone and color, and gagging and vomiting are commonly seen

3. Acyanotic CHD’s
ACYANOTIC
CHDS
PURE
ACYANOTIC
POTENTIALLY
CYANOTIC

4. Acyanotic
CHD
Without shunt(normal
or decreased flow)
Right side of heart
PULMONARY
STENOSIS
Left side of heart
AORTIC STENOSIS
COARCTATIONOF
AORTA
L-> R SHUNT ↑ PBF
ASD
VSD
P.D.A.
Aorto-pulmonary
Window

5. Normal anatomy of arch
Ascending AO
Ascending AO - part of arch upto right common carotid
Proximal arch - Part between right and left carotid
Distal arch - Part between left carotid and after left
subcalivan artery
Isthmus - Part of aorta just after left subcalivan artery
When to say arch hypoplasia –
Arch diameter < 50% of aorta at the level of diaphragm
Roser Me - < baby weight +1 mm
Z score < - 2 SD

6. Fetal arch circulation
Ascending aorta, both carotid and left subclavian is perfused By left
ventricular flow and descending aorta is perfused by Ductal flow from right
ventricle. Isthmus is relatively under-perfuse water shed zone. If there is CoA,
low gradient at CoA segment. Because of CoA Segment Pressure will be on
PDA, PA, enlarge right atrium and ventricle. Part of aorta beyond isthmus will
by perfused by PDA. Thus fetal echo – Depiction of narrowing is not
till fetus have severe coarctation important sign is Enlarge RA & RV
( indirect Sign) in absence of Pulmonary artery narrowing and ductal
closure.

7. Fetal arch circulation
Ascending aorta, both carotid and left subclavian is perfused by
left ventricular flow and descending aorta is perfused by Ductal
flow from right ventricle. Isthmus is relatively under-perfuse
water shed zone.
If there is Critical aortic stenosis, ascending aorta will be
perfuse by retrograde blood flow from PDA to aortic arch and
there by head and upper limb.
The fetal echo – Depicts retrograde flow in ascending aorta
With enlarge RA and RV and small LV.
(Normally till 26 weeks of gestation RV = LV, There after RV is
bigger than LV).

8. Coarctation of Aorta (CoA)
CoA constitutes 6%–8% of all congenital heart defects with a prevalence of 0.24/1000 live
births. It is more common in males. It constitutes 15%–35% of cardiovascular malformations in
children with Turner syndrome. BAV is the most common associated congenital anomaly seen in
80% of cases.
It involve part of aorta between Left subclavian and PDA, Long segment of Aortic arch if involved
known as tubular hypoplasia of aortic arch.
Clinical presentation depends on the age at presentation. Neonates and infants often present
with congestive heart failure. There may be associated aortic arch hypoplasia. Older children and
adults present with upper-limb arterial hypertension.
The diagnosis is now more because of fetal ECHO and habit to palpate femoral pulse
regularly of all neonates at birth and before discharge. Otherwise the diagnosis is
incidental in older patients while they are being investigated for hypertension.

9. Diagnostic workup
Clinical assessment:
Weak and delayed femoral pulses in comparison with brachial / radial pulse are the key to the
diagnosis. The diagnosis may be missed in neonates due to PDA.
Measuring blood pressure in all four limbs shows upper-limb hypertension and is a useful guide
to the gradient across the CoA segment.

10. X-ray chest
Can be normal in asymptomatic infants and young children.
Symptomatic neonates show pulmonary venous congestion with moderate-to-severe cardiomegaly,
mainly due to dilation of the RV and the right and left atria.
In children and young adults, heart size is normal or only mildly enlarged. Pulmonary vascular
markings are normal unless there is an associated left-to-right shunt.
The combination of a dilated left subclavian artery proximal to the coarctation and a dilated
aorta distal to the coarctation produce a figure of 3 silhouette on the X-ray. Notching of the
ribs is a classic radiologic sign caused by collateral flow through dilated, pulsatile posterior
intercostal arteries. Rib notching seldom appears before 5–6 years of age.
Typical coarctation distal to the left subclavian artery results in bilateral notching of the
posterior aspects of third to eighth ribs.

11. ECG
Right-axis deviation with right ventricular hypertrophy is the typical finding in symptomatic
neonates and infants, Because lower part of body is perfuse by PDA, load is on RA, LA is
responsible for perfusion of head and neck only, thus no load on LV.
The finding of left ventricular hypertrophy in infancy, especially if associated with strain
pattern (ST-segment and T wave depression), strongly suggests associated aortic stenosis (AS)
or associated myocardial disease.
ECG in older children and adolescents reflects the effects of long-standing left ventricular
pressure overload in the form of left ventricular hypertrophy and left atrial enlargement.
Prominent, coved ST-segment depression with deeply inverted T waves indicates coexisting BAV
with stenosis.
Prominent left precordial q waves suggest left ventricular volume overload, which may be due to
significant regurgitation through a BAV.

12. Fetal arch circulation
Ascending aorta, both carotid and left subclavian is perfused By left
ventricular flow and descending aorta is perfused by Ductal flow from right
ventricle. Isthmus is relatively under-perfuse water shed zone. If there is CoA,
low gradient at CoA segment. Because of CoA Segment Pressure will be on
PDA, PA, enlarge right atrium and ventricle. Part of aorta beyond isthmus will
by perfused by PDA. Thus fetal echo – may Depicts narrowing of aorta
but important sign are Enlarge RA & RV in absence of
Pulmonary artery narrowing and ductal closure.

13. sign of fetal coarctation
Indirect sign : Enlarge right atrium and ventricle
Direct sign : Isthmus Z score < 1.2
Ascending aorta Z score < 1.1
Isthmus/Ductal ratio <0.74
Isthmus and ductal angle <125 Degree
Doppler : may or may not demonstrate gradient across coarctation because blood flow is less
Interrupted aortic arch depict small LV and ascending aorta with intrruption.

14. Neonate with CoA will present with pallor- ness, breath less, increased heart rate, gallop rhythm, poor pulses
Particularly of lower limbs in case of CoA, and weak all pulses in case of severe aortic stenosis.
In case of CoA ECHO will demonstrate narrowing of aorta at the site of COA with turbulent blood flow
Along with PDA flow in neonate. Echo also Demonstrate posterior self with gradient of at least 20 mm Hg.
If self is anterior, pulses are good and gradient is < than 20 mmHg, CoA is not very important.

15. Echocardiography
Fetal echo shows dilated Right atrium and Ventricle and small LV, three vessel view – aorta is small in size as
compare to PA & narrowing of isthmus part of aorta. DAO is perfused by PDA.
It provides accurate assessment of site and severity of obstruction, ventricular hypertrophy and function.
Associated findings such as arch hypoplasia, intracardiac defects (such as VSD and BAV), and presence or
absence of PDA can be easily diagnosed by echocardiography.
Color-flow Doppler assists in localizing the site of obstruction, particularly in cases where two-dimensional (2D)
imaging is difficult. Subcostal views in young children show blunted Doppler flow in descending thoracic aorta.

16. Bicuspid aortic valve Coarctation of aorta

18. Coarctation Of Aorta

19. Neonate with Post-ductal CoA with PDA reverse flow

23. Trisomy 18 b/o pooja

24. Trisomy 18 CoA b/o pooja

25. Inlet VSD, BAV, BPV, Pseudo CoA

26. Pseudo - CoA

27. Co-arctation of aorta with PDA

28. Preductal Coarctation of aorta with PDA

29. Preductal Coarctation of aorta with PDA

30. PDA STEAL
D
A
O
D
A
O
PDA FLOW
Preductal Coarctation of aorta with PDA

31. Coarctation of aorta

32. PM & Sub Ao VSd CoA DSVC

33. Coarctation of aorta, reversal in DAO through PDA

35. Interrupted aortic arch

36. Interrupted aortic arch AMBA

37. VSD, PDA & interrupted aortic arch
bicuspid aortic valve

38. interrupted aortic arch
VSD, PDA & interrupted aortic arch & bicuspid aortic valve

39. Interrupted aortic arch

41. Proximal type II A P window

42. PA
A
O
R
T
A
d TGA WITH AP WINDOW
type IV intermediate

43. Right pulmonary artery from DAO

44. TRUNCUS ARTERIOSUS TYPE II

45. TRUNCUS ARTERIOSUS TYPE II

46. RV
LV LV
AO
PA PA
PA
PA
AO
AO
PA
LA
LA
RV
RV
RA
Truncus arteriosus II

47. Double Aortic Arch

48. TOF Double Aortic Arch PA MAPCA

49. Double aortic arch

50. Double aortic arch

55. 2D color dopopler study depicting muscular VSD, narrow and early origin of left pulmonary artery, right side
descending aorta and normal both coronary artery.

56. CT thorax revealed normal Left aortic arch with right descending aorta, circumflex course of isthmus, right carotid, left carotid, left
subclavian artery and narrowing of right subclavian artery at it’s origin, as first, second, third and fourth branch of aorta respectively.

57. Chest computed tomography angiogram with the arrows pointing to the anatomic structures at the
level of the vascular ring such as the ascending aorta, circumflex course of isthmus of aorta,
Descending right aorta, and retro-trachea-esophageal transverse aortic arch.

58. CT depicting left pulmonary artery taking origin much early than normal

59. ANEURYSM OF ABDOMINAL AORTA

60. ANEURYSM OF ABDOMINAL AORTA

61. CTA/cardiac magnetic resonance imaging (cMRI) may be required in select cases, especially in
adults when anatomy is unclear on echo. These tests are also recommended for follow-up
imaging in adults with coarctation, who have had surgical or catheter intervention.
Cardiac catheterization:
Required if an intervention is planned. Pressure gradient across the CoA segment may be
diminished in cases with left ventricular dysfunction and low cardiac output, in the presence of
a large PDA, multiple left-sided obstructive lesions in series, or well-developed collateral
circulation that decompresses the aorta proximal to coarctation.

62. Indications for intervention
i. Patients with CoA gradient ≥20 mmHg (Class I)
ii. Patients of CoA presenting with left ventricular dysfunction, even though the gradient
across is <20 mmHg, where left ventricular dysfunction is considered to be due to tight
CoA (Class I).
iii. Patients with gradient <20 mmHg, but having upper limb hypertension, left ventricular
hypertrophy, or significant collateral formation (Class IIa).
iv. Patients with hypertension who have >50% narrowing at the site of CoA, relative to aortic
diameter at diaphragm on CTA/cMRI/angiography, irrespective of pressure gradient (Class
IIa).
v. v. Intervention is not indicated if Doppler gradient across coarctation segment is <20
mmHg with normal left ventricular function and no upper-limb hypertension (Class III).

63. Ideal age for intervention
i. With left ventricular dysfunction/congestive heart failure or severe upper-limb
hypertension (for age): Immediate intervention (Class I)
ii. Normal left ventricular function, no congestive heart failure, and mild upper-limb
hypertension: Intervention beyond 3–6 months of age (Class I)
iii. No hypertension, no heart failure, normal ventricular function: Intervention at 1–2 years
of age (Class I)
iv. In patients with significant CoA associated with a sizable VSD, both defects should be
repaired in a single stage (Class I).

64. Mode of intervention
i. Neonatal presentation: Surgery (Class I). Aortic arch hypoplasia, if associated, should
also be repaired.
ii. ii. Critically ill neonates who are considered high risk for surgery (shock-like syndrome
and severe left ventricular dysfunction): Balloon angioplasty to tide over the crisis (Class
IIa)
iii. iii. Infants with native coarctation: Surgery (Class I) or balloon angioplasty (Class IIa)
iv. iv. Infants with re-coarctation: Balloon angioplasty (Class I)

65. v. Children <25 kg with native coarctation: Balloon angioplasty (Class I) or surgery (Class IIa)
vi. Children <25 kg with re-coarctation: Balloon angioplasty ± stenting (Class I)
vii. Children >25 kg and adults with native coarctation: Catheter-based stenting (Class IIa)
viii. Children >25 kg and adults with re-coarctation: Catheter-based stenting (Class I)
ix. Elective endovascular stenting of aorta is contraindicated in children <10 years of age
(Class III).

66. Indications of using a covered stent (provided the anatomy is suitable)
i. Native coarctation where risk of rupture of aorta is high
a. BAV with ascending aorta dilation
b. Nearly atretic isthmus (<3 mm diameter)
c. Turner syndrome
d. Age >60 years
e. Marfan syndrome
f. ii. Recoarctation with aneurysm or pseudoaneurysm at the site of CoA.
All patients with CoA must be advised to maintain good oro-dental hygiene.

67. Important determinants of long-term prognosis
Residual or recurring coarctation
status of aortic valve (if bicuspid)
aneurysms of the ascending aorta or aneurysm at the intervention site
premature coronary artery disease
and berry aneurysms of the circle of Willis

68. Untreated patients who have survived infancy have a 25% survival at 46 years and 10% survival at
56 years.
The risk of residual hypertension and early atherosclerotic cardiovascular disease is increased with
late repair.
The prevalence of residual hypertension is only 6% in repairs performed between 1 and 5 years of
age in comparison with 30%–50% in patients repaired at an older age.

69. Follow-up recommendations
i. Lifelong follow-up is required.
ii. Annual follow-up initially; later, every 2–3 years if no residual lesions
iii. Clinical assessment should include measuring upper- and lower-limb blood pressure.
Echocardiography to be done at each follow-up to exclude any residual issues and to assess for
other abnormalities, such as BAV.
iv. Beyond 5 years of age, echo alone may not be sufficient for evaluation. cMRI or CTA is
recommended 3–5 yearly or earlier. cMRI is preferable in postsurgical and postballoon angioplasty
patients while CTA is preferred after endovascular stenting.
v. Beta-blockers are the preferred drugs for control of hypertension.
vi. IE prophylaxis is needed for 6 months after surgery and intervention.
However, all patients are advised to maintain good oro-dental hygiene after this period also.