SCMR 22nd Annual Scientific Sessions
Description of Clinical Presentation:
Previously healthy 16 years old male presented with acute onset of lower extremity and facial edema, underwent extensive work-up which revealed hypoalbuminemia and elevated fecal alpha 1-antitrypsin leading to the diagnosis of protein losing enteropathy (PLE). Despite medical treatment, the edema and hypoalbuminemia progressed requiring further imaging work up with an intrahepatic dynamic contrast magnetic resonance lymphangiogram (IH-DCMRL) for evaluation of his PLE. IH-DCMRL is performed by ultrasound guided percutaneous needle placement within the intrahepatic lymphatics with dynamic magnetic resonance imaging (MRI) during the lymphatic injection of gadolinium.
Diagnostic Techniques and Their Most Important Findings:
IH-DCMRL demonstrated lymphatic perfusion of the duodenum with intraluminal leakage of lymphatic contrast into the duodenum with a dilated thoracic duct (Figure 1). Additionally, the central veins were dilated suggesting increased central venous pressure which prompted further hemodynamic evaluation with cardiac catheterization. Cardiac catheterization findings were consistent with constrictive pathology demonstrating elevated ventricular filing pressures with equalization of the right and left ventricular end-diastolic pressures, elevated wedge pressures and elevated pulmonary vascular resistance index, and low cardiac index. On further evaluation with cardiac MRI, small right and left ventricular volumes with dilation of the superior and inferior vena cava and pericardial thickening were noted, and viability imaging showed pericardial hyperenhancement (Figure 2). The patient underwent pericardiectomy with surgical and pathologic findings consistent with constrictive pericarditis.
Learning Points from this Case: