Successful treatment of neonatal pulmonary thrombosis in congenital nephrotic syndrome

A 21-year-old adult patient with Tetralogy of Fallot presented acutely unwell with a 3-month history of general malaise following dental treatment. Following initial complete repair, he subsequently underwent two right ventricular outflow tract reconstructions with conduits. On admission, transthoracic echocardiography and peripheral blood cultures were suggestive of streptococcal endocarditis. Sequential computed tomography pulmonary angiography demonstrated peripheral emboli with progressive central pulmonary artery filling defects suggestive of thrombi and potential vegetations (Fig 1a and b). Urgent surgery was performed for uncontrolled sepsis and increasing hypoxia. Peri-operative transesophageal echocardiogram confirmed previous findings (Fig 1c; Supplementary Video 1). Having resected the conduit, we performed a peripheral pulmonary embolectomy and resected an abundance of the infected material from the pulmonary arterial tree under low-flow hypothermia; we then proceeded with a jugular venous valved conduit (Contegra®, Medtronic Inc, Minneapolis, Minnesota, United States of America) replacement and tricuspid valve annuloplasty. He made a slow but steady recovery and was discharged home in good health