Coarctation of the aorta (CoA) is the 5th most common congenital heart defect.
CoA typically manifests as a discrete constriction of the aortic isthmus.
The majority of patients affected present in infancy with varying degrees of heart failure, which reflect predominantly the severity of the aortic narrowing.
Some patients may not present until later in childhood or adolescence, with upper extremity hypertension, either due to less severe initial narrowing or to the development of collateral circulation bypassing the coarctation.
Tx options include surgery, balloon angioplasty, and stenting.
Although early surgery may prevent/delay the onset of hypertension, approximately 30% will be hypertensive by adolescence.
HTN is the single most important outcome variable in patients with CoA
HTN present in young children is often under-recognized or not treated aggressively enough, screening for cardiovascular & renovascular anomalies is essential
Untreated CoA has significant early mortality, with mean age of death ~30-40
References
Hijazi K. Coarctation of the aorta: From fetal life to adulthood. Cardiology Journal. 18(5):487-95, 2011
