Pulmonary arteriovenous malformations: strategies for detection, treatment and follow-up.
- Conditions
- Hereditary haemorrhagic telangiectasia (HHT)Trans thoracic contract echocardiography (TTCE)Pulmonary arteriovenous malformations (PAVMs)Magnetic resonance imaging (MRI)Computed tomography (CT)
- Registration Number
- NL-OMON25616
- Lead Sponsor
- St. Antonius Hospital
- Brief Summary
Recent publications: 1. Velthuis S, Buscarini E, Mager JJ, Vorselaars VMM, van Gent MWF, Gazzaniga P, Manfredi G, Danesino C, Diederik AL, Vos JA, Gandolfi S, Snijder RJ, Westermann CJJ and Post MC. Predicting the size of pulmonary arteriovenous malformations on chest computed tomography: a role for transthoracic contrast echocardiography. European Respiratory Journal 2014; [Epub ahead of print] 2. Vorselaars VMM, Velthuis S, Mager JJ, Snijder RJ, Bos W-J, Vos JA, Strijen MJL and Post MC. Direct haemodynamic effects of pulmonary arteriovenous malformation embolisation. Netherlands Heart Journal 2014; [Epub ahead of print] 3. de Gussem EM, Lausman AY, Beder AJ, Edwards CP, Blanker MH, Terbrugge KG, Mager JJ and Faughnan ME. Outcomes of Pregnancy in Women With Hereditary Hemorrhagic Telangiectasia. Obstetrics & Gynecology 2014; 123(3):514-520 4. van Gent MWF, Velthuis S, Post MC, Snijder RJ, Westermann CJJ, Letteboer TGW and Mager JJ. Hereditary hemorrhagic telangiectasia: how accurate are the clinical criteria? American Journal of Medical Genetics Part A 2013; 161(3):461-466. 5. Velthuis S, Buscarini E, van Gent MW, Gazzaniga P, Manfredi G, Danesino C, Schonewille WJ, Westermann CJ, Snijder RJ, Mager JJ and Post MC. Grade of pulmonary right-to-left shunt on contrast echocardiography and cerebral complications; a striking association. Chest 2013; 144(2):542-548. 6. Velthuis S, Vorselaars VM, van Gent MW, Westermann CJ, Snijder RJ, Mager JJ and Post MC. Role of transthoracic contrast echocardiography in the clinical diagnosis of hereditary hemorrhagic telangiectasia. Chest 2013; 144(6):1876-1882.
- Detailed Description
Not available
Recruitment & Eligibility
- Status
- Recruiting
- Sex
- Not specified
- Target Recruitment
- 1500
All consecutive persons screened for HHT and/or PAVMs from 2004 until present at the St.
Antonius Hospital will be included in the dataset.
Patients without HHT and without PAVMs and no family history of HHT
Study & Design
- Study Type
- Not specified
- Study Design
- Not specified
- Primary Outcome Measures
Name Time Method In a retrospective cohort we will study the accuracy of the current clinical diagnostic criteria, compared to<br>genetic testing in correlation with age. We will study the additional value of different pulmonary shunt grading on TTCE, not only<br>in the screenings algorithm, but also in follow-up of untreated PAVMs. The potential spontaneous increase or decrease of<br>pulmonary shunting on TTCE after five years will be evaluated. In a prospective analysis we will compare the diagnostic<br>accuracy of MRI to CT in both the detection and follow-up of (treated) PAVMS. We will determine potential differences in<br>long-term results of PAVM embolotherapy with either coils or plugs and determine potential patterns in PAVM feeding arteries<br>involved in persistent perfusion after embolotherapy. A comparison of HHT versus sporadic PAVMs and embolisation results<br>will be made.
- Secondary Outcome Measures
Name Time Method Survival and cause of mortality will also be analysed and correlated to demographic and clinical characteristics to<br>find prognostic factors of survival.