P886 Shoots from the whip: catheter tip migration and tromboembolic comlications of ventriculoatrial shunt for hydrocephalus in von Hippel Lindau disease - multimodality findings

Abstract

Abstract We report a rare nowadays case of 35 yo patient with von Hippel Lindau (VHL) disease and pulmonary hypertension as a severe complication of ventriculo-atrial (VA) shunt insertion in the treatment of hydrocephalus. The patient was admitted to our department with signs of heart failure such as large decrease in exercise tolerance, increasing dyspnea, fatigue, ankle swelling manifested since few months. In the past, the patient was diagnosed with multiple cerebral hemangiomas of the cerebellum and the spinal cord and increased intracranial pressure due to obstructive hydrocephalus as a sequel to a mass effect from haemangioblastoma. He underwent surgery of the posterior cranial cavity tumor and ventriculo-atrial shunting. Electrocardiogram showed sinus tachycardia, right axis deviation, and right ventricle hypertrophy. Particular transthoracic and 3D transesophageal echocardiography showed enlarged right chambers with right overload (A) and severe tricuspid regurgitation (B) with pulmonary hypertension. The distal end of the VA shunt was seen in the right atrium (C). The tip was migrating in some cycles of the heart into the right ventricle inflow tract (D). It moved like a cracking whip and was knocking against the tricuspid leaflets. A pulmonary scan (230 MBq of the Tc99m intravenously) was then undertaken to confirm the presence of the pulmonary embolism and showed in both lungs numerous segmental and subsegmental perfusion deficits. Multidetector CT pulmonary angiogram revealed marked mosaic attenuation in bilateral lung fields as well as abridgment of subsegmental pulmonary arteries (E). Conventional pulmonary angiography showed on the right side arteries A9 and A10 obstructed, A4 amputated, A6 bifurcation at division into subsegmental branches (F). All the diagnostic tests confirmed CTEPH and medical therapy, including anticoagulation, was started. Removal of the catheter was planned. After 3 months The patient was qualified for pulmonary artery angioplasty. In conclusion, however, VA shunts inserted for the treatment of hydrocephalus are known to be a risk factor for pulmonary hypertension the pathogenetic process that causes pulmonary hypertension after shunt insertion is unclear. Therefore routine echocardiography and/or lung scanning is necessary for early diagnosis and treatment. Abstract P886 FIGURES A-F