Pulmonary arterial hypertension in patients with prior atrial switch procedure for d-transposition of great arteries (dTGA)

Pulmonary arterial hypertension (PAH) is an uncommon complication of atrial switch repair (Mustard or Senning) for d-transposition of the great arteries (dTGA), often difficult to diagnose by trans-thoracic echocardiography. This patient population is unique in that heart failure and elevated filling pressures are common after atrial switch repairs. Most studies evaluating the use of PAH therapies have excluded this group of patients.MethodsOur echocardiography database was reviewed for patients with a diagnosis of dTGA status post-atrial switch operation treated with pulmonary vasodilator therapy (monotherapy or combination).ResultsSix patients (2 male, 4 female) were identified from 104 patients (67% male). Mean age at atrial switch was 22.1 months; mean age of PAH diagnosis was 29.3 years. Functional class improved from a baseline mean of 3.3 to 1.8 on treatment (p = 0.001). No adverse events were associated with treatment. 3 of 4 patients initially referred for heart–lung transplant no longer require transplantation.ConclusionsPAH is a late complication of the atrial switch procedure for dTGA, affecting 5.7% of our dTGA atrial switch population, with a higher incidence in female patients. In this cohort, pulmonary arterial vasodilator therapy was well tolerated and improved functional status.