History and exam

Key diagnostic factors

common

hypertension presenting at a young age or resistant to treatment

Most commonly presents with upper extremity hypertension. Typically, aortic coarctation is congenital. It is commonly detected in the newborn period or first decade of life with diminished lower extremity pulses or upper extremity systolic hypertension. Occasionally, it can be undiagnosed until adulthood. These patients may have a history of longstanding difficult-to-treat hypertension.

diminished lower extremity pulses

May present with diminished or absent lower extremity pulses.

differential upper and lower extremity blood pressure (BP)

Typically have a gradient between the BP in the arms and the legs.

Other diagnostic factors

common

systolic ejection murmur

Audible over the left sternal border and back.

male sex

Aortic coarctation has a male predominance.[11]

uncommon

genetic syndrome

Over 50% of patients with Turner syndrome have cardiovascular abnormalities. Coarctation of the aorta and bicuspid aortic valve, alone or in combination, constitute >50% of the cardiac malformations.[12]

Patients with DiGeorge syndrome have cardiac defects including tetralogy of Fallot, interruption of aortic arch, aortic coarctation, truncus arteriosus, and ventricular septal defects. PHACE syndrome is rare, in which the association of Posterior fossa malformations, Haemangioma, and Arterial, Cardiovascular, and Eye abnormalities may be seen.[13] These patients may have coarctations that may be complex and long-segment in nature.[14]

claudication

Leg pain on walking due to reduced blood flow.

headache

Rarely, can present with a severe headache due to an intracranial haemorrhage from an associated berry aneurysm in the circle of Willis.

systolic ejection click

If associated with a bicuspid aortic valve.

other cardiac anomalies

Hypoplastic left heart syndrome is a group of cardiac anomalies characterised by underdevelopment of the left side of the heart with severe hypoplasia of the ascending aorta. Shone complex is a multilevel left heart obstruction including supravalvar mitral ring, parachute mitral valve, subaortic stenosis, and aortic coarctation.

Risk factors

strong

male sex

Aortic coarctation has a male predominance.[11]

young age

Typically, aortic coarctation is congenital. It is commonly detected in the newborn period or first decade of life with diminished lower extremity pulses or upper extremity systolic hypertension.

Occasionally, it can be undiagnosed until adulthood. These patients may have a history of long-standing difficult-to-treat hypertension.

Turner's syndrome

Over 50% of patients have cardiovascular abnormalities. Coarctation of the aorta and bicuspid aortic valve, alone or in combination, constitute >50% of the cardiac malformations.[12]

DiGeorge's syndrome

Cardiac defects include tetralogy of Fallot, interruption of aortic arch, aortic coarctation, truncus arteriosus, and ventricular septal defects.

hypoplastic left heart syndrome

A group of cardiac anomalies characterised by under-development of the left side of the heart with severe hypoplasia of the ascending aorta.

Shone's complex

Multi-level left heart obstruction including supra-valvar mitral ring, parachute mitral valve, subaortic stenosis, and aortic coarctation.

PHACE syndrome

A rare syndrome in which the association of Posterior fossa malformations, Haemangioma, and Arterial, Cardiovascular, and Eye (PHACE) abnormalities may be seen.[13] These patients may have coarctations that may be complex and long-segment in nature.[14]

weak

family history

Recent evidence shows a familial link with left ventricular outflow tract obstructive lesions including aortic coarctation.[4][5]

Use of this content is subject to our disclaimer