Chapter 32

    This is a pathology where no left to right shunt exists, but a significant dilatation of the main pulmonary artery and at times, its major branches, occurs with otherwise normal vascularization of both lungs and normal heart.
    These patients may present systolic click, wide splitting of S₂, and occasionally pulmonic systolic murmur.
    Despite the normal physiology, patients often complain of recurrent dyspnea.

    The chest roentgenograms will depict the pulmonary artery dilatation with otherwise normal cardiac silhouette. The electrocardiogram and catheterization are normal.

    The entity should be distinguished from the dilatation seen in Marfan's SyndromQ which is usually associated with aortic arch disease, and from secondary enlargement to pulmonary hypertension.

    Eventually Idiopatic Dilatation of the Pulmonary Artery can lead to regurgitation or aneurysmatic dilatation. In general, however, the disorder courses almost asymptomatic and has a typically benign evolution.