Interatrial septal aneurysm with pulmonary arterial hypertension
Diagnosis almost certain
Presentation
Shortness of breath with abnormal echocardiography.
Patient Data
Age: 40 years
Gender: Female
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CT pulmonary angiography (without cardiac gating) demonstrates
-
interatrial septal aneurysm
- bulging of the interatrial septum from the right atrium into the left atrium seen as a saccular outpouching (length = 20 mm, base = 18 mm)
- signs of pulmonary arterial hypertension
- enlarged right atrium and ventricle
- enlarged pulmonary trunk (measuring 35 mm at pulmonary artery bifurcation)
- main pulmonary artery to ascending aorta ratio = 1.2 (normal less than 1.0)
- enlarged pulmonary arteries (right PA = 29 mm, left PA = 28 mm in diameter)
- mild bowing of the interventricular septum towards the left ventricle
- right ventricular dilatation: right ventricle–to–left ventricle diameter ratio = 2 at the midventricular level on axial images)
- moderate dilatation with tortuousity of the branchial arteries
- lungs are clear
Case Discussion
Findings are most consistent with interatrial septal aneurysm with pulmonary arterial hypertension.
On echocardiography, there was an interatrial septal aneurysm with dilated right heart chambers with no patent foramen oval (PFO) or left-sided heart disease such as mitral or aortic valve disease. On CT there is no lung disease, chronic obstructive lung disease or pulmonary emboli. All laboratory investigations were normal. These suggest that pulmonary arterial hypertension is probably of idiopathic origin.
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