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.
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