A 35-year-old man with a history of upper gastrointestinal bleeding, beta-thalassaemia and spondyloarthropathy, under treatment with certolizumab (anti-tumour necrosis factor [TNF] drug), who came to the emergency department for congruent left lower homonymous quadrantanopsia, with no other clinical manifestations. Brain computed tomography (CT) showed a right arachnoid cyst as an incidental finding. With a murmur on auscultation, transthoracic echocardiography (TTE) was performed, diagnosing a mass on the right coronary leaflet, with a friable surface, which generated a double aortic lesion: moderate stenosis and severe insufficiency. Laboratory tests showed leukocytosis with neutrophilia. Suspecting IE, he underwent urgent surgery with implantation of a 24 mm bidisc mechanical aortic prosthesis. There were firm pericardial adhesions. The aortic valve was trivalve, dysplastic, with severe thickening of the right coronary leaflet, yellowish-parduous in colour, with necrotic material inside. The left coronary leaflet had a similar implant in proximity to the commissure with the non-coronary leaflet. The non-coronary leaflet was thickened and appeared hypoplastic.

The postoperative course was characterised by the need for reoperation due to late cardiac tamponade, without a bleeding site. Left inferior quadrantanopsia persisted, with normal fundus and brain CT with right occipital hypodensity due to subacute established infarction in the territory of the right posterior cerebral artery, without mass effect or haemorrhagic transformation. Although all microbiological cultures were negative pre-, intra- and postoperatively, empirical antibiotic therapy was maintained with vancomycin and gentamicin, which was subsequently de-escalated to ceftriaxone. The patient had not received antibiotic treatment prior to diagnosis that could have decapitated the blood cultures. Pathology showed polymorphonuclear and histiocyte aggregates surrounding an amorphous basophilic substance, without confirmation of PAS-positive material. The control TTE was unaltered, and the patient was discharged with outpatient follow-up due to complications in the healing of the surgical wound.

He was admitted again at the fourth postoperative month due to complete atrioventricular block, requiring permanent pacemaker implantation. TTE and transesophageal echocardiography (TEE) showed an oscillating mass in the left ventricular outflow tract in the most anterior aspect of the ventricular side of the prosthesis, without clear images of abscess. Presence of mild aortic regurgitation that could not be correctly defined as intra- or periprosthetic due to the acoustic shadow of the prosthesis. In the thoracic CT scan, dehiscence of the prosthesis at the level of the area between the non-coronary sinus and the right coronary sinus, with saccular dilatation of the aorta. In view of the suspicion of early prosthetic endocarditis with pseudoaneurysm, it was decided to re-intervene. Dehiscence was found in the non-coronary sinus area, with disruption of the native aortic annulus and a subannular pseudoaneurysmal cavity. After explantation of the prosthesis, the entire native annulus was affected, with devitalised tissue and sloughing, requiring complete aortic transection for a successful approach. The pseudoaneurysm was repaired with a pericardial patch, the entire circumference of the native annulus was reconstructed with the same and a new 18 mm bidisc mechanical prosthesis was implanted.


Revision for late cardiac tamponade, as in the first operation, with no bleeding point observed. He completed a new course of empirical antibiotherapy with daptomycin and cloxacillin. The case was re-evaluated jointly with Rheumatology and Infectious Diseases. Certolizumab had been withdrawn after the first operation as a possible cause of alterations in healing and immunity. All requested microbiological cultures, serology and nucleic acid detection were negative, as in the first episode. Detection of Tropheryma whipplei (T. whipplei) in the surgical specimen, saliva, faeces and duodenal biopsy was negative. Cytomegalovirus was also not detected in gastric biopsy. A prolonged platelet aggregation time was found, with no other alterations in haemostasis. Absence of autoimmunity markers. Subsequent TTE detected only moderately elevated prosthesis gradients.

Five months after the second operation, the patient attended the emergency department for progressive dyspnoea with significant congestive symptoms. TTE with very severe aortic regurgitation and reappearance of extensive periprosthetic pseudoaneurysm causing prosthesis nodding. Dilated left ventricle, but with apparently preserved systolic function. The patient underwent urgent surgery, with aortic homograft implantation in the subcoronary position and closure of the pseudoaneurysm with the same stitches. Although the intraoperative TEE showed mild aortic insufficiency, the subsequent TTE showed a probably moderate intrahomograft insufficiency, difficult to quantify as it was an eccentric jet, with no limitation in the mobility of the leaflets at its opening. Dilated left ventricle with moderate-severe depression of function. Reproduction of a cavity in the aortic root. It was decided to complete a new regimen of empirical antibiotic therapy and discharge with outpatient control to repeat complementary tests, while awaiting the results of cultures.

The TEE at one month after the last intervention showed the insufficiency with an eccentric jet in the anterior direction, at least moderate. In the aortic root there was a cavity with systolic expansion compatible with pseudoaneurysm, confirmed by thoracic CT. The polymerase chain reaction of the surgical specimen from the latter procedure was positive for T. whipplei. Given the clinical stability and complexity of the case, it was decided to start treatment for Whipple's disease with doxycycline and hydroxychloroquine, and outpatient monitoring. Currently, in the sixth postoperative month, the clinical situation is stationary, asymptomatic for dyspnoea or other cardiorespiratory symptoms. A new TTE and CT scan showed no changes with respect to previous scans, and conservative management was chosen for the time being, given the complexity of the case.

IE is a serious disease, which requires rapid management based on a diagnosis of suspicion for optimal medical and surgical treatment, with decisions made by a multidisciplinary team. It may be a consequence of previous antibiotic treatment or due to fungi and bacteria of demanding culture. Microorganisms requiring serology for diagnosis, such as Coxiella burnetii, Bartonella, Aspergillus, Mycoplasma pneumoniae, Brucella and Legionella pneumophila, and microorganisms requiring polymerase chain reaction tests such as T. whipplei, Bartonella and fungi stand out.
