General

o       2X more common in males

Common classification

o       Infantile or preductal form

o       Adult or juxtaductal form

Adult Form

o       Adult or juxtaductal (postductal) form is more common

o       Usually localized

o       Area of coarctation is just beyond the origin of LSCA at level of ductus

Infantile Form

o       Infantile, preductal form = diffuse type

o       Long, tubular segment of narrowed aorta

§ From just distal to innominate to level of ductus

o       Intracardiac defects (VSD,ASD, deformed mitral valve) present in 50% of diffuse type

§  Also patent ductus arteriosis

Other Classifications

o       More complicated classifications take following into account:

§  Location and length of coarct

§  Patency of ductus arteriosis

§  Relationship of coarct to ductus

Associated Defects

o       Bicuspid aortic valve (most common associated defect seen in 75-80%)

o       VSD

o       ASD

o       Transposition

o       Found in 25% of patients with Turner’s Syndrome

Shone Syndrome

o       Coarctation

o       Aortic stenosis

o       Parachute mitral valve

o       Supravalvular mitral ring

Imaging findings

o       Rib Notching

§         Single best sign

§         Older the person, more likely to have rib notching

§         Majority have it over 20 years of age

§         Rib notching occurs in the high pressure circuit

§         Most often involves 4th-8th rib

§    4th-8th do anastomose with internal mammary to form collaterals for descending aorta

§         Rib Notching–Unilateral

·         Isolated Right sided notching occurs when LSCA is involved in actual coarctation

·         Isolated Left sided notching can occur if there is an aberrant RSCA which arises from below coarct

o       Figure 3 Sign

§         Caused by (in order) either a dilated LSCA or aortic knob,  “tuck” of coarct itself, and poststenotic dilatation

§         Occurs in 1/3–1/2 of patients with coarct

§         Matched by “reverse 3” or “E” on barium-filled esophagus

o       Convexity of left side of mediastinum just above aortic knob 2° to dilated LSCA

o       Convexity of ascending aorta in 1/3

Clinical Findings–Infancy

o       Severe CHF most common from  2nd to 6th week of life

o       Weak or absent leg pulses

o       Lower BP in the legs than in the arms

o       EKG

§         RV hypertrophy because RV assumes most of the cardiac output during fetal life in these patients

Clinical Findings-Children and Adults

o       Differential pulses in arms (bounding) and legs (weak)

o       EKG

§         LVH

Echocardiographic Findings

o       2D echo can demonstrate coarcts from suprasternal notch

o       Echo is most helpful in excluding associated hypoplastic left heart syndrome

Complications

o       Heart failure in neonate

o       Subarachnoid bleeds from ruptured Berry aneurysms

o       Dissection of aorta

o       Infective endocarditis

o       Mycotic aneurysm