A 26 year old man presents to his physician with a chronic cough. The man is a smoker, and states that he also gets frequent headaches and aches in his legs when he exercises. Chest x-ray demonstrates notching of his ribs. Which of the following undiagnosed congenital defects may be responsible for these findings?
Coarctation of the aoa occurs in two patterns. In the infantile type, the stenosis is proximal to the inseion of the ductus aeriosus (preductal); this pattern is associated with Turner's syndrome. In the adult form, the stenosis is distal to the ductus aeriosus (postductal) and is associated with notching of the ribs (secondary to continued pressure from the aoa on them), hypeension in the upper extremities, and weak pulses in the lower extremities. Headache, cold extremities, and lower extremity claudication with exercise are typical if the patient is symptomatic (many adults with mild distal coarctation may remain asymptomatic for years). Upper extremity hypeension with weak pulses in the lower extremities, and a midsystolic (or continuous) murmur over the chest or back may be the only obvious signs in some. Note that the chronic cough is probably related to the man's smoking, and is not caused by the coarctation. Eisenmenger's syndrome is a shift from a left-to-right shunt to a right-to-left shunt secondary to pulmonary hypeension. Tetralogy of Fallot and transposition of great aeries cause cyanosis and are usually diagnosed in infancy. Ref: Karamlou T.B., Welke K.F., Ungerleider R.M. (2010). Chapter 20. Congenital Hea Disease. In F.C. Brunicardi, D.K. Andersen, T.R. Billiar, D.L. Dunn, J.G. Hunter, J.B. Matthews, R.E. Pollock (Eds), Schwaz's Principles of Surgery, 9e.
Coarctation of the aoa
Eisenmenger's syndrome
Tetralogy of Fallot
Transposition of great vessels