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Coarctation of the Aorta

Title               

Ñ    Coarctation of the Aorta

Definition

Ñ    Narrowing of the aorta

Ñ    At or just distal to the point of insertion of the ductus arteriosus

Pathogenesis

Ñ    Decreased renal perfusion results in systemic hypertension due to activation of the renin angiotensin system

Ñ    Severe narrowing of the aorta and decreased blood flow leads to collateral formation involving the periscapular and intercostal arteries

Clinical Features

History

Ñ    Asymptomatic for many years

Sex

Ñ    Males > Females

CVS

Ñ    Claudication

Ñ    Cold legs

CNS

Ñ    Nose bleeds

Ñ    Headaches

Examination

CVS

Ñ    Hypertension in the upper limbs

Ñ    Weak and delayed pulses in the lower limbs

Ñ    Enlarged collaterals in the back:

May be visible and palpable

Ñ    Heaving apex beat

Ñ    Systolic thrill in the suprasternal region

Ñ    Ejection-systolic click:

Due to bicuspid aortic valve

Ñ    Fourth heart sound:

Due to ventricular hypertrophy

Ñ    Mid or late systolic murmur due to turbulent flow through the coarctation

Ñ    Systolic murmur at the base of the heart

Ñ    Early diastolic murmur:

Due to aortic regurgitation:

·          Dilated aorta

Ñ    Systolic or continuous bruits over collateral vessels

Associated features

Ñ    Turner’s syndrome

Ñ    Noonan’s syndrome

Ñ    Bicuspid aortic valve

Ñ    Patent ductus arteriosus

Ñ    Ventricular septal defect

Ñ    Mitral stenosis

Ñ    Mitral regurgitation

Ñ    Circle of Willis aneurysms

Investigations

Imaging

Chest X-ray

Ñ    Dilated aorta indented at the site of the coarctation

Ñ    Rib notching:

Dilated collateral intercostal arteries eroding bone

CT, MRI

Ñ    Accurately demonstrates coarctation and quantifies flow

Aortography

Ñ    Demonstrates the defect

Electrophysiology

ECG

Ñ    Left ventricular hypertrophy

Management 

Cure

Surgery

Ñ    Surgery for complex situations and or aneurysms

Ñ    Resection with end-to-end anastomosis, interposition of a graft if the coarctation is extensive

Ñ    Balloon dilatation either as primary therapy or for recurrence

Ñ    Stenting for anatomically suitable coarctation

Prognosis

Ñ    Depends on extent and duration of pre-operative hypertension

Ñ    Problems that can occur after surgery are:

Recoarctation

Persistent hypertension

Aortic valve disease

Coronary artery disease

Aortic or cerebral aneurysm

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