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

TL;DR

This paper presents a rare case of a child with idiopathic dilatation of the pulmonary artery and highlights the need for more research and data on this condition.

Contribution

The paper contributes a pediatric case report and a literature review on the rare condition of idiopathic dilatation of the pulmonary artery.

Findings

A 3-year and 11-month-old female was diagnosed with IDPA based on imaging showing significant dilation of the pulmonary artery.

There is a significant gap in guidance for managing and understanding IDPA, emphasizing the need for more case reports and data collection.

Abstract

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.

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.

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.

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