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Journal of Structural Heart Disease, June 2018, Volume 4, Issue 3:79-84
DOI: 10.12945/j.jshd.2018.040.17

Pulmonary Arteriopathy in Patients with Mild Pulmonary Valve Abnormality without Pulmonary Hypertension or Intracardiac Shunt

Karam Obeid1*, Subeer K. Wadia, MD2, Gentian Lluri, MD, PhD2, Cherise Meyerson, MD3, Gregory A. Fishbein, MD3, Leigh C. Reardon, MD2, Jamil Aboulhosn, MD2

1 Department of Biological Sciences, Old Dominion University, Norfolk, Virginia, USA
2 Department of Internal Medicine, Ahmanson/UCLA Adult Congenital Heart Disease Center, Los Angeles, California, USA
3 Department of Pathology, Ronald Reagan/UCLA Medical Center, Los Angeles, California, USA


Background: The natural history of pulmonary artery aneurysms (PAA) without pulmonary hypertension, intracardiac shunt or significant pulmonary valvular disease has not been well studied. This study looks to describe the outcome of a cohort of adults with PAA without significant pulmonic regurgitation and stenosis. Imaging modalities utilized to evaluate pulmonary artery (PA) size and valvular pathology are reviewed.

Methods: Patients with PAA followed at the Ahmanson/ UCLA Adult Congenital Heart Disease Center were included in this retrospective analysis. The criteria for patient inclusion were PAA size ≥ 2.5 cm without intracardiac shunting, more than mild pulmonary valve stenosis and regurgitation, or pulmonary hypertension. PAA size gathered from initial imaging was compared to the most recent to quantify PAA growth over time. CT, MRI, and echocardiography results were compared.

Result: Eleven patients were included; Eight females and mean age of 57 (range 25-80). Eight patients were > 50 years of age. Five patients had PAA > 4 cm and were ≥ 50 years old. PAA size increased at a mean rate of 0.5 cm over a mean follow-up of 10 years. Echocardiography demonstrated significant correlation to CT/ MRI (r=0.93, p<0.001).

Conclusion: Most PAA cases are present in patients older than 50 years. Long-term follow-up suggests a benign course without episodes of dissection or rupture despite 6/11 patients with PAA ≥ 5 cm. PA dilation progresses slowly over time and does not appear to cause secondary events. Echocardiography correlates well with magnetic resonance imaging and computed tomography and is useful in measuring PAA over time.


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Cite this article as: Obeid K, Wadia SK, Llurie G, Meyerson C, Fishbein G, Reardon L, Aboulhosn J. Pulmonary Arteriopathy in Patients with Mild Pulmonary Valve Abnormality without Pulmonary Hypertension or Intracardiac Shunt. Structural Heart Disease 2018;4(3):79-84. DOI: 10.12945/j.jshd.2018.040.17

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