What are the typical clinical presentations of aortic coarctation?

Clinical Presentation of Aortic Coarctation

Aortic coarctation presents with upper extremity hypertension, lower extremity hypoperfusion, diminished or delayed femoral pulses, and a blood pressure gradient >20 mmHg between the arms and legs. 1

Cardinal Physical Examination Findings

Blood Pressure Abnormalities:

• Upper extremity hypertension (>140/90 mmHg in adults) with relative lower extremity hypotension 1
• Arm-to-leg systolic blood pressure gradient >20 mmHg is diagnostic 1, 2
• The location of coarctation determines whether there is differential blood pressure between right and left arms (if near left subclavian origin) 1

Pulse Abnormalities:

• Radio-femoral pulse delay—a pathognomonic finding where the femoral pulse is felt after the radial pulse 1
• Diminished amplitude or absent femoral pulses 1, 3
• Hyperdynamic carotid pulsations 1

Auscultatory Findings:

• Systolic murmur or bruit in the left interscapular region (between the shoulder blades) 1
• Continuous murmurs over parasternal areas (from mammary artery collaterals) and around the left scapula 1
• Suprasternal thrill may be palpable 1
• Systolic ejection sound if bicuspid aortic valve is present (occurs in 50% of cases) 1

Collateral Vessel Signs:

• Palpable periscapular collateral vessels 1
• Continuous murmurs from extensive collateral circulation 1

Symptom Presentation by Severity

Severe Cases (Typically Present in Infancy):

• Heart failure symptoms 1
• Absent or markedly diminished femoral pulses 3

Mild-to-Moderate Cases (May Present in Adulthood):

• Headache 1
• Nosebleeds (epistaxis) 1
• Dizziness and tinnitus 1
• Exertional symptoms: shortness of breath, leg fatigue, claudication, leg cramps 1
• Cold feet 1
• Abdominal angina 1
• Many patients remain asymptomatic and are discovered incidentally during evaluation for hypertension 4, 5

Associated Cardiac Abnormalities to Assess

Bicuspid Aortic Valve (Present in >50% of Cases):

• Listen for systolic ejection click and murmur of aortic stenosis 1
• Early diastolic decrescendo murmur if aortic regurgitation present 1

Left Ventricular Hypertrophy:

• Important marker of hemodynamically significant disease 1
• May be evident on ECG or physical examination (sustained apical impulse) 1

Other Associated Lesions:

• Ventricular septal defect 1, 3
• Subaortic stenosis 1
• Mitral valve abnormalities (parachute mitral stenosis) 1

Imaging Clues on Initial Studies

Chest X-Ray Findings:

• Rib notching of ribs 3-8 (from collateral vessels)—pathognomonic but may be absent 1
• "Figure 3 sign"—indentation at coarctation site with pre- and post-stenotic dilation 1
• Prominent ascending aorta shadow along right sternal border 1
• Widening of left subclavian artery 1

Echocardiographic Doppler Pattern:

• Continuous forward diastolic flow in descending aorta (diastolic "run-off" phenomenon)—the most reliable sign 1
• Decreased pulsatility and absent early diastolic flow reversal in abdominal aorta 1
• Note: Doppler gradients alone are unreliable for quantification, especially with extensive collaterals 1

Life-Threatening Complications of Untreated Coarctation

Acute Cardiovascular Events:

• Aortic dissection or rupture 1, 4
• Heart failure 1
• Ruptured intracranial (berry) aneurysm—10% prevalence in adults with coarctation vs. 2% in general population 1

Chronic Complications:

• Premature coronary artery disease 1, 6
• Stroke 1, 6
• Infective endocarditis 1, 6
• Distal hypoperfusion 1

Critical Diagnostic Pitfalls to Avoid

Collateral Circulation Masking:

• Extensive collaterals may reduce the measured gradient across coarctation, falsely suggesting mild disease 1, 7
• Blood pressure gradient may underestimate anatomic severity when collaterals are well-developed 1, 7

Measurement Technique Errors:

• Always measure blood pressure in both arms and at least one leg to avoid missing the diagnosis 1
• Use appropriately sized cuffs for accurate measurements 4

Post-Repair Considerations:

• Persistent or recurrent hypertension occurs in 20-25% after repair, even with anatomically successful correction 7, 6
• Exercise-induced hypertension may be present despite normal resting blood pressures 1
• Recoarctation occurs in ~10% after surgical repair and ~8% after balloon dilation 1