Idiopathic dilatation of the pulmonary artery: a pediatric case report and literature review.

BACKGROUND

Idiopathic dilatation of the pulmonary artery (IDPA) is a rare vascular anomaly characterized by isolated dilation of the main pulmonary artery. There remains a limited understanding of IDPA, with a limited number of reported cases in the literature.

CASE PRESENTATION: This study reported a case of a 3-year and 11-month-old female presenting with a 1-month history of cough. The girl was diagnosed with IDPA based on Coronary computed tomography angiography (CCTA) and echocardiography showing significant dilation of the main pulmonary artery (25 mm, Z-score: 5.32) and its left (16 mm, Z-score: 5.3) and right (17 mm, Z-score: 6.08) branches. A systematic analysis of IDPA case reports was then conducted, and a review of relevant literature.

CONCLUSION

There is a significant gap in available guidance regarding the management and presentation of IDPA. Due to the potential for severe complications, case reports and data collection are critical to advancing IDPA understanding, and ultimately improving management and early identification.