Pulmonary Hypertension in Patients after Repair of Transposition of the Great Arteries

One well-documented complication of unrepaired d-transposition of the great arteries (d-TGA) is progressive pulmonary vascular disease. It is generally accepted that pulmonary vascular disease will not develop if d-TGA repair is performed early in life. Herein, we report a number of repaired d-TGA, especially in older patients with Mustard/Senning procedures, in whom pulmonary vascular disease developed years after their original repair. The etiology of this phenomenon is unknown, warranting further investigation. Our case reports highlight an overlooked, possible long-term complication following d-TGA repair. From this experience, we recommend that evidence of progressive pulmonary vascular disease may need to be regularly sought during the follow-up of patients after d-TGA repair, especially Mustard/Senning repairs, as early treatment of pulmonary hypertension may improve long-term clinical outcomes.