Page 24 - Journal of Structural Heart Disease Volume 4, Issue 3

81 Original Scienti c Article
Figure 2. Cardiac MRI with feraheme contrast of a patient with Pulmonary Artery Aneurysm demonstrating severe dilation of the main pulmonary artery. Panel A. Left anterior oblique view. Panel B. Frontal view.
three layers of the vessel wall” [4]. There have been several suggested de nitions for pulmonary artery aneurysm. Nguyen et al [5] de ned a PAA as the“focal dilation of the artery beyond its maximal normal cal- iber”. There is no clear consensus on the upper limit of normal size for the main pulmonary artery in adult patients. In this study, we considered PAA to be a di- lation of the main and/or proximal branch pulmonary artery of ≥ 2.5 cm. The histopathologic characteristics of PAA include fragmentation and loss of elastin bers (Figure 1). Patients with PAA are generally asymptom- atic and the diagnosis is often made incidentally on chest or cardiac imaging. This study seeks to describe the imaging modalities utilized to make the diagnosis of PAA and monitor for growth or complications, the natural history of this condition and review the exist- ing literature. Moreover, commonly utilized imaging modalities for the diagnosis and follow-up of PAA, such as echocardiography and cross-sectional imag- ing with computed tomography (CT) and/or magnet- ic resonance imaging (MRI), are compared (Figure 2).
Methods
A retrospective review of the Ahmanson/UCLA Adult Congenital Heart Disease Center database was performed to identify a cohort of patients with PAA. The criteria for patient inclusion were dilated main and/or branch pulmonary artery ≥ 2.5 cm, lack of signi cant pulmonary valvular disease, absence of congenital cardiac shunt and/or pulmonary hyper- tension, and absence of a clear secondary etiology. Transthoracic echocardiographic and cross-sectional imaging (CT and/or MRI) imaging data were gathered and compared. The rst recorded PAA size from each imaging modality was compared to the most recent PAA size allowing estimation of rate of growth over time.
Results
Eleven patients with PAA were identi ed from a total of 4,857 patients in the Ahmanson/UCLA Adult Congenital Heart Disease database. Patients with
Obeid K. et al.
Pulmonary Arteriopathy without Valvular Anomalies