The diagnosis of idiopathic dilatation of pulmonary artery is challenging because its clinical recognition is difficult and various other causes of dilated pulmonary artery need to be excluded. The clinical findings mimic various common cardiac disorders and both invasive and non-invasive investigations should be done to arrive at the diagnosis. It is a known clinical entity but etiology and pathophysiology are largely unknown. The current echocardiographic and catheterization based diagnostic criteria, may not be satisfied completely in a particular patient and need to be revisited in view of newer imaging modalities. There is paucity of information about the natural history of the disease with attendant lack of clarity in treatment guidelines. Certain cases may progress to huge dilatation and consequent serious implications. It is a rare disease and is the diagnosis of exclusion.
Malviya, A., Jha, P. K., Kalita, J. P., Saikia, M. K., & Mishra, A. (2017, January 1). Idiopathic dilatation of pulmonary artery: A review. Indian Heart Journal. Elsevier B.V. https://doi.org/10.1016/j.ihj.2016.07.009