Obstructive Lung Diseases: Fellow Case Report Poster - Obstructive Lung Disease |

Idiopathic Pulmonary Artery Aneurysm: A Case Report of a Rare Disease FREE TO VIEW

Siddique Chaudhary, MD; Joseph Ramzy, MD; Susan Smith, MD
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Mclaren, Flint, MI

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;150(4_S):901A. doi:10.1016/j.chest.2016.08.1001
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SESSION TITLE: Fellow Case Report Poster - Obstructive Lung Disease

SESSION TYPE: Affiliate Case Report Poster

PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM

INTRODUCTION: Pulmonary artery aneurysm (PAA) is defined as dilation of the proximal pulmonary artery or one of its branches. It is difficult to diagnose and often is an autopsy finding. It is classified as central, which arises from the main pulmonary artery, or peripheral, which arises from segmental or sub-segmental arteries. Pulmonary hypertension is an important risk factor for development and rupture of PAA. We present a case of idiopathic PAA which has remained stable for past 10 years.

CASE PRESENTATION: A 32 year old male with a history of PAA presented to the ER with complaints of atypical chest pain. Physical exam findings and laboratory studies were unremarkable. Contrast-enhanced CT scan of the chest revealed aneurysmal dilation of the right pulmonary artery with a clot in it. This was compared to multiple previous CT scans and did not show any changes. Review of past records revealed that 10 years ago, the patient was admitted for similar complaints, found to have a right hilar mass on X-ray and chest CT suspicious for neoplasm. Thoracotomy was done for biopsy and patient was found to have a 4x3 cm right PAA. Echocardiography showed normal pulmonary artery pressure. On this visit VQ scan was done which was negative for pulmonary embolism. Patient was discharged after 2 days with outpatient follow-up.

DISCUSSION: PAA is a rare condition with an incidence of 1 in 14,000 autopsies. Unlike aortic aneurysm it has equal prevalence in both genders and occurs in younger patients. The likely pathology seems to be increased hemodynamic stresses combined with underlying vessel wall weakness. It is commonly associated with congenital heart defects (shunts, PDA or VSD), pulmonary artery hypertension and connective tissue disorders such as Marfan’s syndrome and infections like syphilis and TB. It typically presents with hemoptysis, chest pain, cough and/or dyspnea but is also not infrequently discovered as an incidental finding. The advent of contrast-enhanced CT, MRI and echocardiograms have assumed a primary role in diagnosis. Complications may include pulmonary hypertension, thrombus formation, bronchial compression or aneurysmal rupture. Management is surgical coupled with treatment of the underlying disorders.

CONCLUSIONS: Controversy still exists regarding indications for surgical management, with some pursuing conservative approach while others in favor of surgical intervention even in asymptomatic patients. One third of the patients die from rupture of the aneurysm emphasizing the need for follow up and prompt intervention if it enlarges or become symptomatic. Therefore, close follow-up is required.

Reference #1: Deterling RA, Clagett OT. Aneurysm of the pulmonary artery: Review of the literature and report of a case. Am Heart J. 1947;34(4):471-499.

Reference #2: Arom KV, Richardson JD, Grover FL, Ferris G, Trinkle JK. Pulmonary artery aneurysm. Am Surg. 1978;44(10):688-692.

Reference #3: Deb SJ, Zehr KJ, Shields RC. Idiopathic pulmonary artery aneurysm. Ann Thorac Surg. 2005;80(4):1500-1502.

DISCLOSURE: The following authors have nothing to disclose: Siddique Chaudhary, Joseph Ramzy, Susan Smith

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