Pulmonary Arterial Aneurysm with Thrombus Formation and Atrial Septal Defect with Pulmonary Hypertension

ABSTRACT

Introduction@#Pulmonary arterial aneurysm (PAA) is a rare condition with varying symptomatology. Patients are often referred due to incidental finding of intrathoracic mass on chest radiograph. Common causes include structural cardiac and vascular abnormality, pulmonary hypertension, infection, vasculitis, connective tissue disease, and trauma.@*Case presentation@#A 37-year-old female came in due to shortness of breath associated with easy fatigability, exertional dyspnea, orthopnea, and occasional palpitations. Workup revealed 2D echocardiography findings of atrial septal defect (ASD) with left to right shunt; dilated right atrium and right ventricle; severe pulmonary hypertension; dilated main pulmonary artery. Contrast-enhanced computed tomography (CT) angiography showed PAA in the right main trunk, left and right pulmonary artery with thrombus formation. Cardiac catheterization and hemodynamic study were intended prior to definitive surgical treatment however patient did not opt to proceed. She was managed with warfarin, bisoprolol, digoxin, and trimetazidine. In spite of symptom relief, the patient still needs regular follow-up for reassessment to monitor disease progression.@*Discussion@#Pulmonary arterial aneurysm (PAA) is a rare complication of ASD, a common congenital heart disease. In this case, it presented as a mediastinal mass with nonspecific symptoms diagnosed after routine chest x-ray, verified through echocardiogram. Contrast-enhanced CT angiography ruled out aortic aneurysm and provided additional information of the extent of thrombus formation. However, pulmonary angiography still remains the diagnostic gold standard. Due to the rarity of the disease, optimal treatment of PAA is still uncertain. It is reasonable, however, to treat conservatively for asymptomatic patient with no significant pulmonary hypertension. A more aggressive surgical approach has been advocated for patients with complications.@*Conclusion@#It is important to emphasize that PAA can present with non-specific manifestations thus high index of suspicion is critical for the diagnosis. It is also vital for early detection of the aneurysm and correction of the etiology to prevent disease morbidity and mortality.