Key result
Stopping pulmonary vasodilators in congenital heart disease-associated PAH worsens exercise tolerance, reversed by reintroduction.
Why the study?
The optimal duration and necessity of continuous pulmonary vasodilator therapy in PAH secondary to congenital heart disease after corrective surgery is unclear.
Does cessation of pulmonary vasodilators worsen exercise tolerance in adult patients with severe pulmonary hypertension after corrective surgery for atrial septal defect?
Case Report (n=1)
Does cessation of pulmonary vasodilators worsen exercise tolerance in adult patients with severe pulmonary hypertension after corrective surgery for atrial septal defect?
Long-term combination of pulmonary vasodilators without interruption is crucial for maintaining exercise tolerance in patients with PAH related to congenital heart disease.
Pulmonary arterial hypertension secondary to congenital heart diseases is a subset of patients who need pulmonary vasodilators (PVDs). We are reporting an adult female who continued to have severe pulmonary hypertension after corrective surgery for the atrial septal defect. She showed improvement in symptoms and effort tolerance assessed by 6-min walk distance, but on cessation of therapy, showed worsening of exercise tolerance which improved after reintroduction. This report stresses the importance of the need for long-term combination of PVDs without interruption.
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Sathyamurthy et al. (2023) conducted a case report in Pulmonary arterial hypertension secondary to congenital heart diseases (n=1). Pulmonary vasodilators vs. Cessation of therapy was evaluated on Symptoms and effort tolerance assessed by 6-min walk distance. Cessation of pulmonary vasodilators in a patient with PAH related to congenital heart disease led to worsening of exercise tolerance, which improved upon reintroduction.
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