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Coarctation of the Aorta in Children

Congenital Aortic Narrowing Distal to Left Subclavian Artery

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Çocuk Sağlığı ve Hastalıkları department. Book Appointment →

What is Coarctation of the Aorta in Children?

Coarctation of the aorta (CoA) is a congenital narrowing of the aorta, most commonly located at the juxtaductal region (just distal to the left subclavian artery near the ductus arteriosus), causing left ventricular pressure overload and reduced lower-body perfusion.

Anatomic types include discrete shelf-like coarctation, tubular hypoplasia, and complex coarctation associated with hypoplastic aortic arch.

Frequently associated with bicuspid aortic valve (50–85%), ventricular septal defect, patent ductus arteriosus, mitral valve abnormalities, and intracranial berry aneurysms.

Strong association with Turner syndrome (15–20% of girls with Turner have CoA), Williams syndrome, and other genetic disorders.

Symptoms

Critical neonatal CoA: ductal closure precipitates circulatory collapse with shock, lactic acidosis, oliguria, and lower-body cyanosis at days 2–14 of life
Older infants and children: failure to thrive, dyspnea on exertion, fatigue, leg cramps with exertion (claudication)
Adolescents/adults: incidentally detected upper extremity hypertension, headache, epistaxis, cold extremities
Differential blood pressure: upper-extremity hypertension with weak/delayed femoral pulses (radio-femoral delay) and lower-extremity hypotension
Auscultation: systolic ejection murmur in left infraclavicular and interscapular regions; continuous murmur from collateral circulation in older patients
Imaging signs: rib notching from intercostal collateral arteries (older children), '3 sign' on chest X-ray, prominent left ventricle

Risk Factors

Genetic: Turner syndrome (45,X), Williams syndrome, NOTCH1 mutations, family history of bicuspid aortic valve or aortopathy
Sporadic congenital developmental anomaly of aortic arch and ductus arteriosus
Male sex (1.5:1 to 2:1 male predominance)
Associated congenital heart defects (bicuspid aortic valve in 50–85%)
Maternal diabetes and certain teratogen exposures

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Newborn or young infant with shock, lethargy, poor feeding, or differential cyanosis
  • Child with hypertension, especially with a known syndrome (Turner, Williams)
  • Asymptomatic infant or child with cardiac murmur or weak femoral pulses
  • Adolescent with persistent unexplained hypertension, especially with bicuspid aortic valve
  • Postoperative or post-intervention patient with new symptoms: hypertension, chest pain, exercise intolerance

Treatment Methods

01
Critical neonatal CoA: prostaglandin E1 infusion 0.05–0.1 mcg/kg/min to maintain ductal patency, intensive care stabilization, urgent surgical or transcatheter intervention
02
Surgical repair: end-to-end anastomosis (extended end-to-end for hypoplastic arch), subclavian flap aortoplasty, or patch aortoplasty; preferred in neonates and infants with discrete coarctation
03
Transcatheter intervention: balloon angioplasty for native CoA in selected children/adolescents; covered stent placement in adolescents with adult-sized vessels and recoarctation
04
Postoperative paradoxical hypertension: requires aggressive blood pressure control with beta-blockers, vasodilators, and ICU monitoring; risk of mesenteric arteritis and bowel ischemia
05
Long-term blood pressure management: ACE inhibitors, beta-blockers, or calcium channel blockers; ambulatory blood pressure monitoring detects masked or exercise-induced hypertension
06
Surveillance imaging: echocardiography, MR angiography, or CT angiography every 1–3 years for restenosis, aneurysm formation, and aortic root dilation
07
Bicuspid aortic valve and aortopathy surveillance: serial imaging for ascending aortic dilation, intracranial vascular imaging considered for screening berry aneurysms
08
Endocarditis prophylaxis only if associated bicuspid aortic valve with significant regurgitation or other indications; lifelong cardiology follow-up and exercise counseling

Which Department to Visit?

You can visit our Çocuk Sağlığı ve Hastalıkları department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.