Abstract
Residual pulmonary hypertension (PH) in congenital heart defects (CHD) is a condition characterized by persistent or progressive high pressure in the pulmonary artery (PA) following radical correction of CHD. The clinical course and pathophysiology of residual PH after radical CHD correction are similar to idiopathic pulmonary arterial hypertension (PAH). Despite advancements in PAH-specific therapies (endothelin receptor antagonists, soluble guanylate cyclase stimulators, prostanoids), the three-year survival rate of patients does not exceed 68%. Additionally, some patients exhibit refractory disease progression with decompensated right ventricular failure and fatal outcomes.
As a palliative intervention for this patient population, the Potts pulmonary-aortic anastomosis has been proposed. This surgical technique involves creating a connection between the left PA and the descending aorta. The procedure reduces suprasystemic PH, mitigates symptoms of right ventricular failure, and improves patients’ functional status, though it is associated with moderate hypoxemia in the lower body. We present a clinical case of the successful use of the Potts pulmonary-aortic anastomosis in a female patient with residual PH and severe progressive disease refractory to maximal medical therapy.
