The presence of an atrial septal defect in pulmonary hypertension has benefits and detractions. Even in idiopathic pulmonary arterial hypertension, a significant left-to-right shunt at atrial level may increase the pulmonary arterial pressure and exacerbate the disease. However, it is well recognised that the presence of an atrial communication may be protective in subgroups with severe disease, allowing maintenance of cardiac output during times of increased pulmonary resistance. In the present paper, we present the case of a young boy with significant idiopathic pulmonary arterial hypertension and an atrial septal defect. We report our technique of septal occlusion using a device to decrease left-to-right shunting with concomitant stent insertion in that device to maintain the potential for right-to-left shunting during times of high pulmonary arterial pressure.
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