509 The importance of transesophageal echocardiography in the therapeutic approach of a patient with Hakim-Adams syndrome and right atrial mass

Abstract

Abstract Hakim-Adams Syndrome (HAS) or symptomatic normotensive hydrocephalus is a rare clinical entity (prevalence of 1:25 000 in the general population), characterized by the presence of triad: progressive dementia, ataxia (Parkinson-like) and urinary incontinence. The only efficient therapy for HAS is the implantation of ventriculo-atrial or ventriculo-peritoneal shunts, in order to drain the excess cerebrospinal fluid (CSF). However, long-term maintenance of these devices may predispose to severe complications (e.g. infection, thrombosis). We report the case of a 38 years old patient who suffered a head trauma with subarachnoid hemorrhage and subsequent hydrocephalus, for which a shunt between left lateral cerebral ventricle and right atrium of the heart has been placed. The patient presented progressive resting dyspnea, chest pain and intermittent fever. The initial transthoracic echocardiography revealed a highly mobile mass in the right atrium, without being able to specify the exact place of insertion. In addition, severe pulmonary hypertension and major tricuspid regurgitation were observed. The computed tomography also revealed bilateral pulmonary embolism with areas of pulmonary infarction. The clinical features and echocardiography aspects suggested a possible infective endocarditis of tricuspid valve, therefore a transesophageal echocardiography was necessary. This exploration revealed the presence of a ∼ 40 mm pangliform mass originating from the inter-atrial septum, near the superior vena cava opening in the right atrium, without any link with the tricuspid valve. Basically it was a thrombus, an endoluminal molding of the shunt used to drain CSF. Given the coexistence of pulmonary embolism and the intra-cavitary mass with high emboligenic potential, the heart team in collaboration with neurosurgeons decided to administer heparin, with good clinical and biological evolution. The association between Hakim-Adams syndrome and pulmonary embolism secondary to intra-shunt thrombosis is a very rare clinical entity. The case particularity is also determined by the initial suspicion of infective endocarditis, a diagnosis infirmed only through transesophageal echocardiography. This also guided the further therapeutic approach, considering that anticoagulants are a first-line choice in pulmonary embolism, but are not recommended in endocarditis.