Author + information
- Received November 23, 2012
- Accepted November 27, 2012
- Published online May 21, 2013.
A 39-year-old man with an unrepaired tetralogy of Fallot presented with exertional dyspnea and cyanotic spells. Examination revealed lip cyanosis and clubbing of fingers (A). Chest radiograph showed lacy reticular vascularity in bilateral lower lungs and notching of the fourth and fifth right posterior ribs (B, arrows). Cardiac magnetic resonance study revealed right ventricular hypertrophy, a ventricular septal defect, and an overriding aorta (C, Online Video 1), and an angiogram demonstrated pulmonic stenosis and left pulmonary arterial atresia (D, Online Video 2). There were substantial major aortopulmonary collateral arteries (D, Online Video 2, small arrows) perfusing the left and right lower lungs. The right subclavian artery was stenotic with collateral arteries (D, large arrows, Online Video 2) arising proximally with an extrapulmonary intercostal course to the perfuse right lung, which caused unilateral rib notching. The well-developed collateral circulation resembled multiple “palliative shunts,” which alleviated his symptoms for decades.
- Received November 23, 2012.
- Accepted November 27, 2012.
- American College of Cardiology Foundation