VASCERN News
23 July 2024
Revision of Rare Vascular Tumors in the Orphanet Database
The Heritable Thoracic Aortic Diseases Working Group (HTAD WG) has been hard at work in order to release six new Pills of Knowledge (PoK) videos, just in time for the holidays!
The PoKs are currently available in English and include several that are more directed at patients, and several that are more for medical professionals. So there is something for everyone!
- Seven signs of Marfan syndrome by Professor Yskert von Kodolitsch
This PoK video presents the seven signs of Marfan syndrome in a clear and understandeable manner. It can help anyone who suspects that they may have Marfan syndrome to assess their probability of having this rare disease, using a simple scoring system. This is not a tool for self-diagnosis, but if you have one or more of these signs you may wish to consider consulting with a Marfan syndrome specialist who will be able to give you more information and either confirm or rule out a diagnosis of Marfan syndrome.
- What to know before and during pregnancy in HTAD By Professor Jolien Roos-Hesselink and Dr. Yaso Emmanuel
This PoK video gives a comprehensive overview of the issues to consider for patients with HTAD considering pregnancy. The main considerations include risk of recurrence, risk of an aortic event (during pregnancy/delivery), risks to the foetus, risks of the medications used during pregnancy and risks of further progressive disease in the mother. Appropriate advice for each patient will need to be individualized, according to each patient’s circumstances and medical history, which is why HTAD patients should talk to their cardiologist when they are considering pregnancy.
- What is aortic root replacement and when is it indicated? By Professor Zoltan Szabolcs and Dr. Kalman Benke
This PoK video presents the basics of surgical reconstruction of the aortic root. The aorta and its main complications are first briefly explained, followed by the guidelines used to indicate prophylactic surgery in HTAD patients. The two main surgical techniques – aortic root replacement (ARR) and valve sparing root replacement (VSRR) are then detailed. Warning: intraoperative images included.
- How is the aorta monitored? By Dr. Gisela Teixido-Tura
This PoK gives an overview of the anatomy of the aorta as well as the imaging techniques used to visualize the aorta. These techniques, used to monitor the aorta in HTAD patients, are detailed and their special features are given. A very good overview for anyone wishing to learn more about the imaging techniques used for the monitoring and follow-up of HTAD patients.
- What is chronic aortic dissection? By Professor Artur Evangelista
This PoK discusses chronic aortic dissection, including its diagnosis and management. The physical and psychological aspects to consider in patients who have experienced an aortic dissection are noted. The follow-up and possible complications after aortic dissection that can occur are also listed.
- What is an acute aortic dissection? By Professor Artur Evangelista
This PoK video gives an overview of acute aortic syndrome (AAS), a term which includes aortic dissection (accounting for 80% if AAS cases), intramural hematoma (IMH; 15% of AAS cases), and penetrating aortic ulcer (5% of AAS cases). The methods for classification and diagnosis, the imaging techniques used as well as the types of surgery and medical treatments recommended for AAS are also detailed.
We look forward to receiving your feedback on these new videos and will start translating the subtitles into various EU languages in the New Year!