Coarctation of the Aorta 💔

Related Subjects: |Aortic Anatomy |Acute Coronary Syndrome (ACS) General |Aortic Dissection |Acute Heart Failure and Pulmonary Oedema |Aortic Regurgitation (Incompetence) |Aortic Stenosis |Aortic Sclerosis |Transcatheter aortic valve implantation (TAVI)

🫀 Coarctation of the aorta (CoA) is a congenital narrowing of the aorta, usually just distal to the origin of the left subclavian artery. This results in higher blood pressure in the arms than the legs (reversed from normal physiology). 💡 Always check BP in both arms and in the legs. Femoral pulses are weaker and delayed compared to the radial pulses. 🔊 Collateral intercostal arteries may produce bruits and cause rib notching on CXR. ⚕️ Treatment includes surgical repair or balloon angioplasty with stenting.

📖 About

• Definition: Congenital narrowing of the aorta, usually distal to the left subclavian artery.
• Prevalence: ~5% of all congenital heart disease.
• Nature: Often part of a generalized aortopathy (stiff, less compliant aorta).

🤝 Associations

• ♂️ More common in males (2–5×).
• 👩 Turner syndrome.
• 🔗 Bicuspid aortic valve (~50% cases).
• 🧠 Berry aneurysms → ↑ risk of subarachnoid haemorrhage (SAH).

⚙️ Aetiology & Pathophysiology

• 📍 Narrowing: Shelf-like ridge, usually distal to left subclavian artery (but can occur anywhere along descending aorta).
• 📈 Proximal hypertension & ↓ distal perfusion → BP gradient across narrowing.
• 🌉 Collaterals via intercostal arteries → rib notching on X-ray.
• 🪢 Aortic stiffness → persistent hypertension even post-repair.

🩺 Clinical Presentation

• Infants: Severe CoA → early HF if ductus arteriosus closes.
• Children/Adults:
  • Upper body hypertension → headaches, epistaxis.
  • Weak/absent femoral pulses (radial–femoral delay).
  • Cold legs/claudication with exercise.
  • Systolic murmur over back/scapulae (turbulent flow).
• Complications: Aortic dissection, HF, premature CAD.

🧪 Investigations

• Bloods: Baseline (FBC, U&E, LFTs).
• ECG: LVH.
• Echocardiography: Visualise LVH, bicuspid AV, site/severity of CoA.
• CXR: "3 sign" (pre & post-stenotic dilatation), rib notching (collaterals).
• MRI/CT angiography: Gold standard in older children/adults → anatomy & collaterals.
• Cardiac cath: Pressure gradient (>20 mmHg = significant).

⚠️ Complications

• Post-repair: Aneurysm at repair site, re-coarctation (esp. children).
• Long-term: Persistent hypertension, stiff aorta.
• Valve disease: Aortic stenosis/regurgitation (esp. with bicuspid AV).
• CAD: Premature due to long-standing hypertension.

💊 Management

• Medical:
  • Control HTN → β-blockers, ACEi, ARBs.
  • Treat HF in infants awaiting repair.
• Surgery:
  • Indications: symptomatic or gradient >20 mmHg.
  • Options: end-to-end resection, patch aortoplasty, subclavian flap repair.
• Balloon angioplasty/stent: Less invasive, often for recurrence or in older children/adults.
• Follow-up: Lifelong echo, BP monitoring, imaging to check repair site.

📌 Long-term Risks

• 🧠 Neurological: Berry aneurysm → SAH (screening required).
• 💉 Hypertension: Often persists post-repair.
• 🪢 Aortic dissection: Risk ↑ in adulthood.
• ❤️ CAD: Early onset due to chronic HTN.

🩺 Case 1 — Young Adult with Hypertension

A 22-year-old man is evaluated for persistent hypertension despite lifestyle changes. Exam reveals radio-femoral delay and weaker femoral pulses compared to radial. A systolic murmur is heard over the back. CXR shows rib notching, and echocardiography confirms coarctation of the aorta just distal to the left subclavian artery. Management: 🏥 Definitive repair with surgical resection or balloon angioplasty ± stent. Control BP with beta-blockers until repair. Lifelong follow-up with cardiology. Avoid: ❌ Ignoring hypertension in young patients; avoid unmonitored exercise before repair (risk of dissection/aneurysm).

🩺 Case 2 — Infant with Heart Failure

A 3-week-old infant presents with poor feeding, sweating, tachypnoea, and lethargy. Exam shows weak lower limb pulses, differential cyanosis, and hepatomegaly. Echo reveals severe coarctation of the aorta with LV dysfunction. Management: 🚑 Start IV prostaglandin E1 infusion to maintain ductus arteriosus patency, stabilise with inotropes/diuretics, and arrange urgent surgical repair. Avoid: ❌ Delaying prostaglandin infusion (closure of duct worsens shock); avoid aggressive diuresis without surgical planning.