Images in cardiovascular medicine: septic pulmonary thromboemboli in an adolescent with Tetralogy of Fallot.

A 14-year-old adolescent man from the Republic of Congo, with no personal medical history, presented with progressive shortness of breath and exercise limitation. On clinical examination, he had a 3/6 systolic murmur and regular cardiac rhythm, and cyanotic fingers and clubbing, as well. Pulmonary auscultation was normal. No recent episode of fever or shivering or peripheral sign of endocarditis were reported. Pulse oxymetry showed oxygen saturation at 64%. The diagnosis of Tetralogy of Fallot was subsequently established. Laboratory results showed a normal white blood cell count with no abnormalities. The initial echocardiogram showed a large perimembranous septal defect of 1.5-cm diameter, an aortic override, a predominant right-to-left ventricular shunt, a severe infundibular pulmonary stenosis, and right ventricular hypertrophy. Left ventricular diameter was 39 mm at end-diastole, and the left ventricular ejection fraction was normal. A 4-mm mobile element was unexpectedly visualized at the level of the pulmonary valve. An ECG-gated multidetector pulmonary computed tomography angiogram with iodinated contrast injection was then performed to explore this unusual finding. Preoperative transesophageal echocardiography was not considered as appropriate for this young, poorly cooperating adolescent patient. It revealed a 28 14 mm hypodense intraluminal filling defect attached to the pulmonary valve (Figures 1 and 2). This mass, suggesting a thrombus or vegetation, nearly occluded the pulmonary trunk. In addition, multiple bilateral pulmonary emboli were seen with images suggestive of mycotic aneurysms (Figure 3). The right inferior lobar artery was occluded. There were also pulmonary nodules in relation to distal pulmonary arteries with peripheral ground-glass opacities, indicative of peripheral pulmonary abscesses (Figure 4). Bronchial arterial collaterals were well developed. Pulmonary arterial pressure measurement by catheter was avoided because of the major risk of material migration. Surgery was indicated to remove and analyze the mass from the pulmonary arterial trunk, and, depending on the resultant pulmonary arterial pressure, to repair the Tetralogy of Fallot. Intraoperative transesophageal echocardiography was performed to confirm the diagnosis (Movie I in the online-only Data Supplement). The intervention was performed through a median sternotomy, and pulmonary arterial pressure was isosystemic (105/64) by direct measurement. Hypothermic cardiopulmonary bypass with antegrade cold blood cardioplegia was used. Incision of the pulmonary artery showed the mass, which seemed thrombotic, attached to a bicuspid pulmonary valve. All of the abnormal material was excised from the main pulmonary artery without removing the pulmonary valve and sent for pathological and bacterial examination (Figure 5). After closure of the main pulmonary artery and weaning from cardiopulmonary bypass, the pulmonary arterial pressure remained isosystemic (108/67). Infundibular pulmonary stenosis protected this patient from pulmonary overcirculation as we can see in Eisenmenger syndrome, but multiple distal thromboemboli led to irreversible secondary pulmonary Figure 1. Computed tomography angiography, axial view. Large near-obstructive filling defect of the pulmonary trunk corresponding to a septic thrombus attached to the pulmonary valve (arrow).