Case Report: Ostium secundum atrial septal defect with unilateral lung hypervascularity revealing associated right pulmonary artery stenosis

Abstract

Background: Atrial septal defect (ASD) is often an isolated disease, but its association with other abnormalities can make diagnosis challenging. Careful analysis of simple complementary exams can help precise anatomical diagnosis ensuring suitable treatment. The aim of this article is to report, from a case report and literature review, diagnostic challenges and the contribution of simple complementary exams, such as chest X-ray, for the diagnostic orientation of an ASD associated with peripheral pulmonary artery stenosis, as well as therapeutic particularities. Case report: We report the case of a girl born in 2007, with history of dyspnoea and recurrent bronchitis in whom a loud systolic murmur was detected fortuitously at the age of 2 years. Her clinical examination was otherwise normal. The electrocardiogram recorded sinus rhythm, incomplete right bundle branch block, and right ventricular hypertrophy. Chest X-ray showed moderate cardiomegaly and hypervascularity of the left lung field contrasting with reduced blood flow to the right lung. Doppler echocardiography revealed a wide ostium secundum ASD, right chamber volume overload and right pulmonary artery stenosis. The latter was confirmed by CT angiography and right cardiac catheterization.  The patient underwent percutaneous right pulmonary artery dilation with stent placement. Control chest X-ray noted bilateral hypervascularity of the lung. The ASD was closed percutaneously one year later. The outcome was uneventful. Conclusion: The combination of ASD with pulmonary artery stenosis limits pulmonary hyperflow. In our case, this stenosis was tight and sat on the right branch of the pulmonary artery reducing significantly blood flow to the ipsilateral lung. Careful chest X-ray analysis may suggest diagnosis, which can be confirmed by ultrasounds and if necessary, by further examination, allowing treatment adaptation. To our knowledge, this association is very rare and no similar case has been reported.