CardiologyTier 2Disease (DEADMAN)

Congenital heart disease - coarctation of the aorta

Description

Narrowing of the aorta, most often just distal to the left subclavian artery at the ductal insertion
- -> upper-limb hypertension + lower-body hypoperfusion
- -> collateral circulation via internal thoracic and intercostal arteries
Not just a plumbing problem - a diffuse arteriopathy
- Hypertension, premature CAD and stroke persist after anatomically successful repair

Epidemiology

~5-8% of congenital heart disease; M>F ~2:1
~35% of women with Turner syndrome have coarctation
Bicuspid aortic valve in the majority - up to ~85%
Presents in infancy (duct-dependent) or in adulthood as hypertension

Aetiopathogenesis

Lesion

Ductal tissue extending into the aortic wall -> constriction as the duct closes
Associated lesions: bicuspid AV, VSD, PDA, mitral valve abnormality
Berry aneurysms of the circle of Willis

Mechanism of hypertension

Mechanical obstruction -> inc proximal pressure
+ dec renal perfusion -> RAAS activation
+ abnormal proximal aortic stiffness and endothelial dysfunction
-> hypertension persists in a large proportion even after successful repair

Diagnosis

Clinical

Upper-limb hypertension with radiofemoral delay
Arm-leg systolic gradient >20 mmHg is significant
Visible or palpable collaterals over the scapulae and chest wall; suprasternal thrill
Heaving, undisplaced apex
Systolic murmur heard posteriorly, left infrascapular
Underdeveloped left arm -> coarctation at or proximal to the left subclavian origin
Exercise intolerance, claudication, angina

Investigations

ECG: LVH +/- strain
CXR: rib notching from intercostal collaterals, "figure of 3" sign
TTE: gradient, aortic dimensions, LV function, bicuspid valve
CT or MR angiography of the whole aorta - anatomy, collaterals, aneurysm
Cath: peak-to-peak gradient, and the route for intervention

Management

Intervene if

Peak-to-peak catheter gradient >=20 mmHg, OR
Hypertension with significant anatomical narrowing, even at a lower gradient, when extensive collaterals are present

Modality

Percutaneous stenting - preferred in adults where anatomy allows
Surgery for long-segment disease, hypoplastic arch, or re-coarctation not amenable to stenting

Lifelong after repair

Hypertension management - persists or recurs in a large proportion
- ACEi/ARB or beta blocker
- Measure BP in the right arm; exclude re-coarctation before escalating drugs
Surveillance CT/MR for aneurysm at the repair site and re-coarctation
Bicuspid valve and ascending aorta surveillance
Screen for berry aneurysm if headaches or a suggestive family history
Pregnancy: pre-conception assessment - risk of dissection and severe hypertension

Associations

Bicuspid aortic valve - the near-constant companion
Turner syndrome
VSD, PDA, mitral valve abnormalities
Berry aneurysms of the circle of Willis
Shone complex - multiple left heart obstructive lesions

Natural history & complications

Untreated: ~75% mortality by age ~46
Death from heart failure, aortic dissection or rupture, stroke, premature CAD, endocarditis

After repair

Medical - hypertension, premature coronary disease, stroke
At the repair site - re-coarctation, aneurysm formation
Life expectancy remains reduced even after successful repair, and worse the later the repair

Monitor

BP - all four limbs
Serial aortic imaging
Aortic valve and ascending aorta

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