Coarctation of the Aorta

Case Presentation

Jonar Zareh
CASE – Mr. AC
Case overview
3-yo boy presented to paeds cardiac clinic for review of his coarctation repair.

No significant past medical history.

Born 40/40, NVD, BW 4030g (>90%ile). APGARs 9 + 9.

Original Clinical Picture:

Routine examination: absent femoral pulses, pre- & post-ductal oxygen saturations
(96% & 100% respectively).

Echocardiogram:
Narrowing of the proximal aortic arch (3mm) with closed ductus arteriosus.

Management:
Transferred to WCH for review and surgical repair of the coarctation.
Coarctation of the Aorta (CoA)
- Coarctation = “Narrowing”
- Commonly in the distal aortic arch, in close proximity of the ductus arteriosus
and left subclavian artery.
- 5-7% of all congenital cardiac defects.
- In Australia, 2000 babies born with congenital heart disease per year (Blue et al.,
2012).

Incidence of CoA
3 cases per 10,000 live births
• In 2007-08, 1.3 per 100,000 hospitalisations for CoA (AIHW, 2008).

Australian Institute of Health and Welfare. (2008). National Hospital Morbidity Database. Australian Government: Canberra.
1
Risk Factors & Associations
•More common in males
• M>F = 1.5:1

•Genetic? Familial link suggested

•Associated with genetic syndromes including Turner, DiGeorge, PHACE, Noonan

•Associated with bicuspid aortic valve (50-75%), VSD, PDA and aortic arch
hypoplasia
 Pathophysiology
• Underlying mechanism not fully understood.
• 3 main theories (Dijkema et al., 2017):
1. Abnormal development during
embryogenesis.
2. Reduced intrauterine flow through aorta
-> aortic underdevelopment.
3. Aberrant ductus arteriosus tissue in
aortic wall -> when PDA regresses, aorta
also constricts nearby.

Narrowing of the aorta -> increased flow proximal

and decreased flow distal to the narrowing

Compensatory mechanisms
• Myocardial hypertrophy & collateral blood flow
Clinical Signs & Symptoms
Neonates
•Asymptomatic if PDA present or mild narrowing
•Differential cyanosis - cyanosis in lower extremities
•Severe stenosis: when ductus arteriosus closes -> shock and multi-organ failure

Childhood
•Exertional chest pain, cold lower extremities, reduced exercise capacity
•Hypertension at a young age or treatment-resistant
• Upper extremity systolic hypertension
• Headache, epistaxis

Signs on physical examination:

•Differential BP in upper and lower limbs (>20mmHg)
•Reduced lower extremity pulses
•Systolic ejection murmur
• LSB and back
Diagnostics
- Prenatal diagnosis challenging.

- Transthoracic Doppler echocardiography = Gold Standard

- Sensitivity 78%, specificity 92% (Anuwutnavin et al., 2016)

Torok RD, Campbell MJ, Fleming GA & Hill KD. (2015). Coarctation of the aorta: Management from infancy
to adulthood. World J Cardiol, 7, 765-75.
Management
• Main goal: complete repair of the coarcted segment with minimal
complications.
• Type of repair depends on age of patient at diagnosis and severity of
coarctation.
• PGE1 infusion for critical coarctation.

Surgical repair
• End-to-end anastomosis (most common)
• Subclavian flap repair

Catherisation
• Balloon angioplasty
• Stent placement

The Royal Children’s Hospital Melbourne. Coarctation of the Aorta. Date accessed 20/8/19:
<https://www.rch.org.au/cardiology/parent_info/Coarctation_of_the_Aorta/>
Prognosis

Complications of CoA (Long-Term)

Local Re-coarctation, aneurysm,
dissection, rupture,
endocarditis, fistulae, heart
failure
Aortic Valve Bicuspid valve, stenosis,
regurgitation
Cerebral Berry aneurysms, intracranial
haemorrhage
Systemic Secondary hypertension,
reduced exercise capacity

•Long-term follow-up of LV function, valvular function, BP and regular imaging of

heart and aorta critical.
 Best type of repair
• Alaei et al. (2011) demonstrated in infants <1yo, a significantly lower rate of
recurrent re-coarctation in surgery group (17%) vs. balloon angioplasty (BA)
(20%).

• Lower re-CoA in 6mo infants with to end-to-end-anastomosis compared to

subclavian flap surgery. (Bigdelian & Sedighi, 2016).

• Re-CoA risk with BA increases with younger age at intervention (>50% Re-CoA
rate <1yo) (Gewillig et al., 2012). Usually performed after 3-6mo of age.

• In patients aged 6-12 years, stenting confers lower incidence of acute

complications compared to BA and surgery. (Forbes, et al., 2011).

• Cochrane systematic review by Padua et al. (2012) reported no difference in post-

operative mortality in stent placement vs. surgical treatment.
CASE continued
- Our patient underwent and end-to-end anastomosis via median sternotomy. No
surgical complications.

Currently:
- Normal growth & development.
- Asymptomatic.

Exam:
- Bright, responsive
- Systolic murmur 1-2/6 – likely innocent murmur
- BP: R arm 102/54, L arm 104/59. HR 90
- Equal radial, brachial, femoral pulses

Echo:
- Normal aortic valve structure & function, normal aorta.

Plan: 2-year review.

Summar
y
- CoA is a congenital heart condition with narrowing of the proximal thoracic
aorta.

- Early identification is important to minimise severe complications

- Different types of repair for CoA

- Surgical repair preferred option in neonates – lowest risk of re-coarctation.
- Current techniques minimise complications.
- BA recommended for re-CoA after surgical repair.

- Long-term complications include hypertension and re-coarctation, so lifelong

reviews are warranted.
References
• Anuwutnavin, S., Satou, G., Chang, R., DeVore, G.R., Abuel, A. and Sklansky, M. (2016).
Prenatal Sonographic Predictors of Neonatal Coarctation of the Aorta. J Ultrasound Med, 35,
2353-64.
• Bigdelian, H. and Sedighi, M. (2016). Repair of aortic coarctation in infancy: A 10-year clinical
experience. Asian Cardiovascular and thoracic Annals, 24, 417-421.
• Blue, G.M., Kirk, E.P., Sholler, G.F., Harvey, R.P. and Winlaw, D.S. (2012). Congenital heart
disease: current knowledge about causes and inheritence. Med J Aust, 197:3, 155-159.
• Forbes T.J., Kim D.W., Du W., Turner D.R., Holzer R., Amin Z., Hijazi Z., Ghasemi A, Rome
J.J., Nykanen D., Zahn E., Cowley C., Hoyer M., Waight D., Gruenstein D., Javois A.,
Foerster S., Kreutzer J., Sullivan N., Khan A., Owada C., Hagler D, Lim S., Canter J. and
Zellers T. (2011). Comparison of surgical, stent, and balloon angioplasty treatment of native
coarctation of the aorta: an observational study by the CCISC (Congenital Cardiovascular
Interventional Study Consortium). J Am Coll Cardiol., 58, 2664-74.
• Wong, S.C., Burgess, T., Cheung, M. and Zacharin, M. (2014). The prevalence of turner
syndrome in girls presenting with coarctation of the aorta. J Pediatr, 164:2, 259-63.
• Torok RD, Campbell MJ, Fleming GA & Hill KD. (2015). Coarctation of the aorta:
Management from infancy to adulthood. World J Cardiol, 7, 765-75.
• Australian Institute of Health and Welfare. (2008). National Hospital Morbidity Database.
Australian Government: Canberra.
• Dijkema, E.J., Leiner, T. and Grotenhuis, H.B. (2017). Diagnosis, imaging and clinical
management of aortic coarctation. Heart, 103, 1148-55.
• Padua, L.M.S., Garcia, L.C., Rubira, C.J. and Carvalho, P.E. (2012). Stent placement versus
surgery for coarctation of the thoracic aorta. Cochrane Database of Systematic Reviews, 5.