coarctation of aorta

1. COARCTATION OF AORTA
REVIEW

5. AORTICA ARCH OBSTRUCTION : TYPES
• Aortic arch obstruction may be divided into
• 1. Localized coarctation in close proximity to a PDA or
ligamentum,
• 2. Tubular hypoplasia of some part of aortic arch
• 3. Aortic arch interruption.

7. LOCALIZED STENOSIS TUBULAR HYPOPLASIA

9. EVOLUTION

10. COARCTATION OF AORTA
• One definition of significant aortic coarctation requires a “ gradient of
more than 20 mm Hg across the coarctation site at angiography with or
without proximal systemic hypertension”.
• Second definition of significant aortic coarctation requires the “ presence of
proximal hypertension in the company of echocardiographic or
angiographic evidence of aortic coarctation”.
• If there is an extensive collateral circulation there may be a minimal
pressure gradient or no gradient at all and acquired aortic atresia.

11. • Coarctation occurs two to five times more commonly in males.
• Associations
• Gonadal dysgenesis (Turner syndrome) and Bicuspid aortic
valve (≥50%).
• VSD
• Mitral stenosis or regurgitation
• Of Turner syndrome patients 35% have aortic coarctation.

12. • Beyond the neonatal period the majority of patients with isolated
coarctation are asymptomatic.
• Reduced femoral pulses and/or Hypertension.
• Heart failure is uncommon because the left ventricle has a chance to
become hypertrophied, thus maintaining a normal wall stress.
• OLDER PATIENTS
• Headache
• Cold extremities
• Leg Fatigue with excerscise

13. • Presentation in adulthood again may be entirely asymptomatic, and
picked up during routine health checks, usually because of the discovery
of a murmur or unexplained hypertension.
• Indeed, coarctation of the aorta should be excluded in all new cases of
hypertension, by clinical examination of the pulses and upper and lower
limb blood pressures.

14. • Associated abnormalities include
• Intracranial aneurysms (most commonly of the circle of Willis) in 2% to
10%
• Acquired intercostal artery aneurysms.

15. • Death in patients who do not undergo repair is most often due to :
• Heart failure (usually in patients > 30 years of age),
• Coronary artery disease,
• Aortic rupture or dissection,
• Concomitant aortic valve disease,
• Infective endarteritis or endocarditis,
• Cerebral hemorrhage.

16. • Leg claudication (pain) is rare unless there is concomitant abdominal
aortic coarctation.
• Clinical examination reveals upper limb systemic hypertension, as well
as a differential systolic blood pressure of at least 10 mm Hg (brachial
artery > popliteal artery pressure).
• Radial-femoral pulse delay is evident unless significant aortic
regurgitation coexists.
• Auscultation may reveal an interscapular systolic murmur emanating
from the coarctation site and a widespread crescendo-decrescendo
systolic murmur throughout the chest wall from the intercostal collateral
arteries.

18. • Funduscopic examination can reveal “corkscrew” tortuosity of
retinal arterioles.

19. CORK SCREW ARTERIOLES

20. • ECG
• Left ventricular hypertrophy of various degrees.
• Coexisting right ventricular hypertrophy usually implies a complicated
lesion.

21. CHEST X RAY
• Characteristic posteroanterior film feature is the so-called “ figure-3
configuration of the proximal descending thoracic aorta due to both
prestenotic and poststenotic dilation”.
• Rib notching (unilateral or bilateral, second to ninth ribs) is present in
50% of cases.
• Rib notching is unilateral if the right or left subclavian arteries arise from
the aorta distal to the coarctation.
• Rib notching is noted as an erosion of the undersurface of a posterior
rib, usually at its outer third, with a sclerotic margin.

24. • ECHO
• Demonstrates a posterior shelf, a well-expanded isthmus and transverse
aortic arch (in most cases)
• High-velocity jet with diastolic persistence through the coarctation site.
• Interestingly, a slow upstroke is observed on the abdominal aortic
velocity profile compared with that seen in the ascending aorta.

27. • MRI
• Performed before intervention, particularly if balloon dilation is the
treatment of choice.
• This is the best tool for postintervention imaging surveillance and has
become routine in many centers.

28. INDICATION FOR INTERVENTION
• American Heart Association guidelines recommend 20 mmHg
catheterization gradient as indication.
• The European Society of Cardiology guidelines clarify that Doppler is
unreliable for severity assessment.
• Instead, intervention is recommended for upper-lower limb blood
pressure gradient >20 mmHg associated with either resting or
exercise-induced hypertension or left ventricular hypertrophy.

30. • True aneurysm formation at the site of coarctation repair is also a well-
recognized entity, with a reported incidence of between 2% and 27%.
• Aneurysms are particularly common after Dacron patch aortoplasty and
usually occur in the native aorta opposite the patch.
• Late dissection at the repair site is rare, but false aneurysms, usually at
the suture line, can occur.

31. STENTING IN COARCTATION
• Stenting of native coarctation of aorta has to be taken up with extreme care as there is a
possibility of dissection and rupture.
• Stenting of re-coarctation after an initial surgical repair is safer due to the presence of
fibrosis around the aortic wall.
• Stenting of native coarctation is usually done at around 3 to 4 atmospheres of pressure
in the dilating balloon.
• It is not necessary to fully dilate the coarctation in a single setting as it increases the
chance of complications.
• A small step can be left and taken up as a re procedure after 3 to 6 months when
fibrosis around the site would make the procedure safer.
• If the step is not removed by redilatation at a later date, there is a chance of strut fracture
of the stent at the site. This in turn will lead to more fibrosis and restenosis of the coarct
segment.
• Hence it is a good practice to call back and redilate after 3 to 6 months to smooth out the
lumen of the stent.

32. • While taking the measurement of the required stent, the decrease in
length of the stent during expansion has to be taken into account. The
expanded length of a 29 x 10 mm stent will be only 27 mm. Larger stents
will lose more length on expansion.
• A catheter introduced through the left radial into the left subclavian is
useful in avoiding jailing of the left subclavian orifice during coarctation
stenting.
• In case there is a leakage from the aorta after balloon dilatation, the
balloon should be reinflated to produce local tamponade and the person
shifted for surgical repair.

33. INTERVENTIONS : CATHETER TECHNIQUES
• The outcomes of transcatheter techniques may involve complications.
• After balloon dilation : aortic dissection, restenosis, and aneurysm
formation at the site of coarctation all have been documented.
• These complications have been reduced with the now increasing if not
exclusive use of primary stenting in the adults with native coarctation as
well as recoarctation.
• Medium-term outcomes of stent therapy in children have also been
favorable.

34. 2016

36. 2012

38. 2010

40. • Prior hypertension resolves in up to 50% of patients but may recur later
in life, especially if the intervention is performed at an older age.
• Systolic hypertension is also common with exercise and is not a
surrogate marker for recoarctation of the aorta.
• It may be related to residual arch hypoplasia or to increased renin and
catecholamine activity from residual functional abnormalities of the
precoarctation vessels.

41. • THANK YOU