Key result
Pulmonary vasodilators linked to improved NYHA class and modestly increased exercise tolerance in unrepaired ToF.
Why the study?
The use and long-term effects of pulmonary vasodilators in adults with unrepaired tetralogy of Fallot, pulmonary atresia, and aortopulmonary collaterals with segmental pulmonary arterial hypertension are not well described.
Do pulmonary vasodilators improve symptoms and exercise tolerance in adult patients with unrepaired tetralogy of Fallot, pulmonary atresia, aortopulmonary collaterals, and segmental pulmonary arterial hypertension?
Case Report (n=3)
Do pulmonary vasodilators improve symptoms and exercise tolerance in adult patients with unrepaired tetralogy of Fallot, pulmonary atresia, aortopulmonary collaterals, and segmental pulmonary arterial hypertension?
Pulmonary vasodilators may provide long-term symptomatic improvement and stability without significant side effects in adult patients with unrepaired tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collaterals.
Should not change practice based on this case report alone; leaves open the role of pulmonary vasodilators in unrepaired tetralogy of Fallot.
We present the use of pulmonary vasodilators in three adult patients with unrepaired tetralogy of Fallot, pulmonary atresia, aortopulmonary collaterals, and segmental pulmonary arterial hypertension. Patients improved by 1-2 NYHA classes with modest exercise-tolerance increase, and remained stable without side effects during 2.5, 10, and 14 years. Literature review revealed five studies with pulmonary vasodilators in heterogeneous, mostly repaired patient populations.
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Apostolopoulou et al. (2017) conducted a case report in Tetralogy of Fallot with pulmonary atresia, aortopulmonary collaterals, and segmental pulmonary arterial hypertension (n=3). Pulmonary vasodilators was evaluated on NYHA class, exercise tolerance, and clinical stability. Pulmonary vasodilators improved NYHA class by 1-2 classes and modestly increased exercise tolerance in three adult patients with unrepaired tetralogy of Fallot, who remained stable for up to 14 years.
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