PULMONARY ARTERY DILATATION IN ASYMPTOMATIC YOUNG MALES

Abstract

SESSION TITLE: Fellows Pulmonary Vascular Disease Posters SESSION TYPE: Fellow Case Report Posters PRESENTED ON: October 18-21, 2020 INTRODUCTION: Idiopathic dilatation of the Pulmonary artery (IDPA) is a diagnosis of exclusion with an incidence of less than 0.01% in autopsy samples.1 Even though it was first reported by Wessler et. Al in 1923 1,2 still very little is known about the etiology and pathogenesis of the condition. Some postulate it to be due to the weakness of the arterial wall, and some believe it is due to the unequal division of the truncus arteriosus communis. 2 With increasing emphasis on imaging modalities to aid in the diagnosis of pathological conditions, it is common practice to comment on pulmonary vasculature when imaging the chest. Dilatation of the pulmonary arteries is found in many cardio-pulmonary disease states. Therefore, known likely conditions have to be excluded before incidental pulmonary artery (PA) dilation is labeled as idiopathic. CASE PRESENTATION: In this review, we present three young asymptomatic or minimally symptomatic male patients aged between 18-30 years old who were incidentally found to have pulmonary artery (PA) dilatation on imaging. They were referred to either pulmonary or cardiology service from September 2017 to February 2019 for evaluation. All three patients had normal findings on physical examination. They underwent further invasive or advanced imaging to rule out systemic diseases and pulmonary hypertension. The diameter of the main pulmonary artery was measured 3.6, 2.5 and 3.4 cm on transthoracic echocardiogram (TTE) with estimated pulmonary artery systolic pressure (PASP) of 20 and 28.9 mmHg in the first two patients. The PASP was unmeasurable by TTE in the third patient. CT scan of the chest confirmed the dilatation of the PA, ranging between 3.4-3.6 centimeters. The patients underwent right heart catheterization and they were all found to have normal pulmonary artery pressures. DISCUSSION: Pulmonary artery dilation can be due to a variety of reasons. When found on imaging modalities, the pathologic causes, most importantly pulmonary hypertension should be the primary focus of the diagnostic workup in the general population. Idiopathic dilatation of the pulmonary artery (IDPA) is a rare congenital anatomic variant in the PA structure 3 and is a diagnosis of exclusion that does not require therapy. 3 As it is shown in our case series and is conventionally accepted, it appears that the incidence of IDPA among the young asymptomatic population is higher than previously expected. Pulmonary artery dilatation in these patients is unlikely to be a sign of pulmonary hypertension. CONCLUSIONS: Individualized monitoring of these patients by periodic symptom assessment, clinical examination, and non-invasive modalities such as echocardiography should prevent the need for unnecessary invasive procedures such as right heart catheterization. Reference #1: Sharma RK, Talwar D, Gupta SK, Bansal S. Idiopathic dilatation of pulmonary artery. Lung India. 2016;33(6):675-677. doi:10.4103/0970-2113.192869 Reference #2: Malviya A, Jha PK, Kalita JP, Saikia MK, Mishra A. Idiopathic dilatation of pulmonary artery: A review. Indian Heart J. 2017;69(1):119-124. doi:10.1016/j.ihj.2016.07.009 Reference #3: Dilatation of the Pulmonary Artery, Idiopathic. NORD (National Organization for Rare Disorders). Accessed May 6, 2020. https://rarediseases.org/rare-diseases/dilatation-of-the-pulmonary-artery-idiopathic/ DISCLOSURES: No relevant relationships by Rahul Bhardwaj, source=Web Response No relevant relationships by Ahmet Copur, source=Web Response No relevant relationships by Seyed Kamran Hejazi Kenari, source=Web Response No relevant relationships by Eric Shafer, source=Web Response