Pediatric and Congenital Heart Disease Session 6: Pediatric and Congenital Cases
5 - Superior vena cava stenosis in an adult congenital heart disease patient presenting with a brain abscess.
Saturday, February 15, 2020
8:36 AM – 8:43 AM
Location: Salon J2
Description of Clinical Presentation: A 23-year-old female with a history of superior sinus venosus defect (SVD) repair at 2 years of age, presents with headache and two episodes of syncope. She is found to have a brain abscess. Further evaluation shows complete occlusion of the superior vena cava (SVC) right atrial (RA) junction with retrograde flow into the brachiocephalic vein and back into the left lung via anomalous drainage of the left upper pulmonary vein and pulmonary venovenous (V-V) collaterals. SVC stenosis is relieved successfully by percutaneous stent placement.
Diagnostic Techniques and Their Most Important Findings: Agitated saline injection on transthoracic echocardiogram (TTE) reveals complete opacification of the left chambers and no residual intracardiac shunt (figure 1). Cardiac MRI/ MRA with 3D reconstructions shows complete occlusion of the SVC-RA junction with retrograde flow into the brachiocephalic vein. The brachiocephalic vein then drains into the left atrium via an anomalous left upper pulmonary vein and pulmonary V-V collaterals (figure 2). The remainder of the pulmonary veins drained normally into the left atrium. Cardiac catheterization confirmed the cardiac MRI/MRA findings and the SVC obstruction is relieved successfully via placement of a covered CP stent.
Learning Points from this
Case: SVC stenosis has been described after the surgical repair of SVD. The stenosis can be progressive culminating with the development of collaterals. In our case, the intrapulmonary collateralization was possible because of the partial anomalous pulmonary venous drainage. It leads to a systemic-to-pulmonary shunt, ultimately predisposing to the development of brain abscess. It is important for the imager to assess for SVC stenosis after SVD repair. The SVC can be difficult to assess by TTE in adults with congenital heart disease and further cross-sectional imaging is required. The diagnosis was made by cardiac MR imaging leading to timely and successful management of the patient.