Pericardial Involvement in Hereditary Hemorrhagic Telangiectasia
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 durin...
Saved in:
| Main Authors: | , , , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Tehran University of Medical Sciences
2025-01-01
|
| Series: | Acta Medica Iranica |
| Subjects: | |
| Online Access: | https://acta.tums.ac.ir/index.php/acta/article/view/10957 |
| Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
| _version_ | 1823863947394023424 |
|---|---|
| author | Abir Derbel Mouna Snoussi Raida Ben Salah Faten Frikha Leila Abid Imed Frikha Sameh Marzouk Zouhir Bahloul |
| author_facet | Abir Derbel Mouna Snoussi Raida Ben Salah Faten Frikha Leila Abid Imed Frikha Sameh Marzouk Zouhir Bahloul |
| author_sort | Abir Derbel |
| collection | DOAJ |
| description |
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.
|
| format | Article |
| id | doaj-art-c451ec183ed64f73b5e1d49fab0ccc38 |
| institution | Kabale University |
| issn | 0044-6025 1735-9694 |
| language | English |
| publishDate | 2025-01-01 |
| publisher | Tehran University of Medical Sciences |
| record_format | Article |
| series | Acta Medica Iranica |
| spelling | doaj-art-c451ec183ed64f73b5e1d49fab0ccc382025-02-09T08:50:50ZengTehran University of Medical SciencesActa Medica Iranica0044-60251735-96942025-01-0162510.18502/acta.v62i5.17778Pericardial Involvement in Hereditary Hemorrhagic TelangiectasiaAbir Derbel0Mouna Snoussi1Raida Ben Salah2Faten Frikha3Leila Abid4Imed Frikha5Sameh Marzouk6Zouhir Bahloul7Department of Internal Medicine, Hedi Chaker Hospital, University of Sfax, Sfax, Tunisia.Department of Internal Medicine, Hedi Chaker Hospital, University of Sfax, Sfax, Tunisia.Department of Internal Medicine, Hedi Chaker Hospital, University of Sfax, Sfax, Tunisia.Department of Cardiology, Hedi Chaker Hospital, University of Sfax, Sfax, Tunisia.Department of Cardiology, Hedi Chaker Hospital, University of Sfax, Sfax, Tunisia.Department of Cardiovascular Surgery, Habib Bourguiba Hospital, University of Sfax, Sfax, Tunisia.Department of Internal Medicine, Hedi Chaker Hospital, University of Sfax, Sfax, TunisiaDepartment of Internal Medicine, Hedi Chaker Hospital, University of Sfax, Sfax, Tunisia. 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. https://acta.tums.ac.ir/index.php/acta/article/view/10957Rendu osler diseaseCardiac involvementPericardial effusionTamponade |
| spellingShingle | Abir Derbel Mouna Snoussi Raida Ben Salah Faten Frikha Leila Abid Imed Frikha Sameh Marzouk Zouhir Bahloul Pericardial Involvement in Hereditary Hemorrhagic Telangiectasia Acta Medica Iranica Rendu osler disease Cardiac involvement Pericardial effusion Tamponade |
| title | Pericardial Involvement in Hereditary Hemorrhagic Telangiectasia |
| title_full | Pericardial Involvement in Hereditary Hemorrhagic Telangiectasia |
| title_fullStr | Pericardial Involvement in Hereditary Hemorrhagic Telangiectasia |
| title_full_unstemmed | Pericardial Involvement in Hereditary Hemorrhagic Telangiectasia |
| title_short | Pericardial Involvement in Hereditary Hemorrhagic Telangiectasia |
| title_sort | pericardial involvement in hereditary hemorrhagic telangiectasia |
| topic | Rendu osler disease Cardiac involvement Pericardial effusion Tamponade |
| url | https://acta.tums.ac.ir/index.php/acta/article/view/10957 |
| work_keys_str_mv | AT abirderbel pericardialinvolvementinhereditaryhemorrhagictelangiectasia AT mounasnoussi pericardialinvolvementinhereditaryhemorrhagictelangiectasia AT raidabensalah pericardialinvolvementinhereditaryhemorrhagictelangiectasia AT fatenfrikha pericardialinvolvementinhereditaryhemorrhagictelangiectasia AT leilaabid pericardialinvolvementinhereditaryhemorrhagictelangiectasia AT imedfrikha pericardialinvolvementinhereditaryhemorrhagictelangiectasia AT samehmarzouk pericardialinvolvementinhereditaryhemorrhagictelangiectasia AT zouhirbahloul pericardialinvolvementinhereditaryhemorrhagictelangiectasia |