Pericardial Involvement in Hereditary Hemorrhagic Telangiectasia

Abstract

Hereditary haemorrhagic telangiectasia is a rare disease characterized by cutaneo-mucous and visceral arteriovenous malformations. Cardiac involvement is uncommon and was presented primarily by hyper-output heart failure. Hemorrhagic pericardial effusion, although is extremely rare, can occur during HHT. We report the case of a 48-years-old woman which was hospitalized in 2015 in internal medicine department, Sfax, Tunisia in 2015 for anicteric cholestasis. She noticed a personnel and familial history of recurrent epistaxis. Biologic findings revealed anemia and moderate cholestasis. Viral investigations and immunologic tests were negative. Abdominal tomography showed multiple arterio-venous shunts of the liver. Liver involvement due to Rendu Osler Weber disease was retained. She was treated by ferrous iron, but she was lost to follow up. She was presented in February 2021, with severe anemia (5 g/dL). Physical examination revealed signs of global heart failure. Biological investigations found anemia, inflammatory biological syndrome, cytolysis and cholestasis. Heart ultrasound revealed an abundant pericardial effusion. Only 500cc of hemorrhagic fluid could be aspirated before the needle became blocked. Unfortunately, one week after, re-accumulation of pericardial fluid and worsening occurred. She underwent a partial surgical pericardial excision with pleuropericardial opening. Analysis of the fluid ruled out any infectious cause of this effusion.  Histological examination confirmed the vascular dysplasia with signs of hemorrhage and inflammation. The patient was discharged 1 month after surgery with no other bleeding episodes. For her anemia, she received a transfusion of red blood cells. Then, the patient was treated by iron treatment.