scholarly journals Marfan syndrome: diagnosis and treatment of cardiovascular lesions

2021 ◽  
pp. 63-67
Author(s):  
V.I. Kravchenko ◽  
◽  
I.M. Kravchenko ◽  
I.A. Osadovska ◽  
V.D. Lybavka ◽  
...  

Purpose – to analise of results, long-term experience of surgical treatment of cardiovascular diseases in patients with Marfan syndrome to improve quality and prolong life. Materials and methods. A conducted prospective analysis of consecutive patients (292) with Marfan syndrome treated at the institute during 1980–2018. The age of patients was 7–57 years, mean 31.6±9.4 years. Among them, men – 220 (75.3%), women – 72 (24.7%). Marfan syndrome was diagnosed according to the criteria of Gent Nosology (2010). 24 (8.2%) patients were not operated on for various reasons. The remaining 268 (91.8%) were managed surgicaly: 257 – ascending aortic aneurysm; 4 – abdominal; 7 patients – only mitral valve insufficiency. Results. Hospital mortality was 9.3% (25 patients). Long-term results were studied in 224 (92.2%) patients from all who had been discharged from the clinic within 6 months – 20 years on average 63 months. Good long-term results were admitted in 145 (64.7%), satisfactory in 41 (18.3%), unsatisfactory in 14 (6.3%) patients. 24 (9.9%) patients died in the long term observation. Conclusions. Aortic aneurysms in patients with Marfan syndrome are formed at a young age. The most common cause sof death in the unoperated cases are rupture of aneurism or heart failure. The «gold-standarg» remains the Bentall De Bono operation. The patients who underwent surgical treatment requires observation during all-life period. The research was carried out in accordance with the principles of the Helsinki Declaration. The study protocol was approved by the Local Ethics Committee of these Institutes. The informed consent of the patient was obtained for conducting the studies. The authors declare no conflicts of interests. Key words: Marfan syndrome, aortic aneurysm, aortic dissection.

Circulation ◽  
2001 ◽  
Vol 104 (Supplement 1) ◽  
pp. I-8-I-11 ◽  
Author(s):  
E. Braunberger ◽  
A. Deloche ◽  
A. Berrebi ◽  
F. Abdallah ◽  
J.A Celestin ◽  
...  

Circulation ◽  
2001 ◽  
Vol 104 (suppl 1) ◽  
pp. I-8-I-11 ◽  
Author(s):  
E. Braunberger ◽  
A. Deloche ◽  
A. Berrebi ◽  
A. Fayssoil ◽  
J.A Celestin ◽  
...  

1981 ◽  
pp. 466-472
Author(s):  
V. Gallucci ◽  
V. Valfré ◽  
G. Caruso ◽  
G. Panizzon ◽  
G. Aru ◽  
...  

1998 ◽  
Vol 15 (3) ◽  
pp. 205-211 ◽  
Author(s):  
G. Faggioli ◽  
A. Stella ◽  
A. Freyrie ◽  
M. Gargiulo ◽  
S. Tarantini ◽  
...  

1998 ◽  
Vol 115 (1) ◽  
pp. 84-93 ◽  
Author(s):  
Sylvain Chauvaud ◽  
Jean François Fuzellier ◽  
Rémi Houel ◽  
Alain Berrebi ◽  
Serban Mihaileanu ◽  
...  

2011 ◽  
Vol 49 (4) ◽  
pp. 486-491 ◽  
Author(s):  
L.K. Dosen ◽  
R. Haye

Background: Results of surgical treatment of nasal septal perforation are usually evaluated using closure of the perforation as criterion of success. Patients, however, may still have symptoms. Aim: To assess the long-term results of surgical treatment of nasal septal perforation with bilateral, posterior based mucoperichondrial septal flaps using a four-point symptom score to ultimately improve treatment and selection criteria. Methodology: Patients were seen 6 months postoperatively. Questionnaires were sent to 116 surviving patients in 2008-2009. The response was 104. Patients reporting moderate or severe symptoms were seen as outpatients. Results: Between 1987 and 2004, 126 patients were surgically treated using posterior based bilateral mucoperichondrial septal flaps. Sixteen patients had a reperforation during the first 3 months, and another 3 several years later. There was no correlation between early outcome and diagnosis, preoperative size of the perforation, gender or severity of preoperative crusting. There was an increased rate of reperforation with increasing age. Complications seen at the 6 months` follow-up of patients with closed perforations were lachrymal duct stenosis, partial vestibular stenosis, hypoesthesia, crusting and septal deviation, most of which were treatable. Long-term observation mean 10 years) of the same patients showed the following moderate or severe symptoms: crusting, obstruction and bleeding, mainly in men. Obstruction was often due to various forms of perennial rhinitis, sometimes to crusting and more rarely to septal deviation. Crusting was the only independent symptom. There was no correlation between crusting and diagnosis, preoperative size of the perforation, age or severity of preoperative crusting. Conclusions: Results of the surgical technique using posterior based bilateral mucoperichondrial septal flaps for treatment of nasal septal perforations were good, but depend on surgical expertise and age of the patient. Long-term results from other studies will be a guide to choose the proper surgical procedure to minimize the number of late symptoms. Prosthetic treatment cans be an alternative. Patients with return of symptoms should seek further advice.


1981 ◽  
Vol 29 (06) ◽  
pp. 394-398 ◽  
Author(s):  
G. Köveker ◽  
E. de Vivie ◽  
K. Hellberg ◽  
W. Ruschewski ◽  
B. Heisig

2011 ◽  
Vol 49 (4) ◽  
pp. 486-491
Author(s):  
L.K. Dosen ◽  
R. Haye

BACKGROUND: Results of surgical treatment of nasal septal perforation are usually evaluated using closure of the perforation as criterion of success. Patients, however, may still have symptoms. AIM: To assess the long-term results of surgical treatment of nasal septal perforation with bilateral, posterior based mucoperichondrial septal flaps using a four-point symptom score to ultimately improve treatment and selection criteria. METHODOLOGY: Patients were seen 6 months postoperatively. Questionnaires were sent to 116 surviving patients in 2008-2009. The response was 104. Patients reporting moderate or severe symptoms were seen as outpatients. RESULTS: Between 1987 and 2004, 126 patients were surgically treated using posterior based bilateral mucoperichondrial septal flaps. Sixteen patients had a reperforation during the first 3 months, and another 3 several years later. There was no correlation between early outcome and diagnosis, preoperative size of the perforation, gender or severity of preoperative crusting. There was an increased rate of reperforation with increasing age. Complications seen at the 6 months` follow-up of patients with closed perforations were lachrymal duct stenosis, partial vestibular stenosis, hypoesthesia, crusting and septal deviation, most of which were treatable. Long-term observation mean 10 years) of the same patients showed the following moderate or severe symptoms: crusting, obstruction and bleeding, mainly in men. Obstruction was often due to various forms of perennial rhinitis, sometimes to crusting and more rarely to septal deviation. Crusting was the only independent symptom. There was no correlation between crusting and diagnosis, preoperative size of the perforation, age or severity of preoperative crusting. CONCLUSIONS: Results of the surgical technique using posterior based bilateral mucoperichondrial septal flaps for treatment of nasal septal perforations were good, but depend on surgical expertise and age of the patient. Long-term results from other studies will be a guide to choose the proper surgical procedure to minimize the number of late symptoms. Prosthetic treatment cans be an alternative. Patients with return of symptoms should seek further advice.


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