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Interventional Cardiology Journal
ISSN: 2471-8157
2
n d
E d i t i o n o f E u r o S c i C o n C o n g r e s s o n
Heart Disease and
Interventional Cardiology
F e b r u a r y 2 5 - 2 6 , 2 0 1 9
P a r i s , F r a n c e
Heart and World Cardiology 2019
M
arfan syndrome is the most common genetic disorder of connective
tissue. One complication that threatens the lives of patients is
progressive dilatation of the ascending aorta with development of aortic valve
regurgitation or the emergence of dissection, often leading to sudden death.
Until now, these patients were operated only after dilatation of the ascending
aorta causing hemodynamically significant regurgitation of the aortic valve.
The surgery consisted of the replacement of the ascending aorta and aortic
valve or valve sparing procedure. This new method is a preventive operation.
The method involves creating a custom made external support of the root and
the ascending portion of the aorta. Based on the CT examination, prosthesis
Extent is created. The surgery is performed from the longitudinal median
sternotomy without cardiopulmonary bypass. Entire aortic root is dissected
to its origin from the left ventricle, ostia of the coronary arteries are encircled,
the prosthesis is pulled underneath and fixed to the root and then sutured
longitudinally. It is interesting that the prosthesis was developed and as the
world’s first has it sewn on himself (Mr Tal Golesworthy), 13 years ago in
Oxford. Neither him nor the other 100 patients operated in this department
with this disease had dilation or dissection throughout the study. It is because
the prosthesis grows over time into the aortic wall thereby enforces it while
maintaining the elastic properties of the wall. At our institute, we have so far
experience with operations of 20 patients, with good results. This operation
moves the care of patients with Marfan syndrome to qualitatively higher level
Biography
Jan Pirk has completed his Graduation from the Faculty
of General Medicine, Charles University in Prague. After
completion of his Graduation, he worked at the District Hospital
in Nymburk from 1972 until 1974. He has been working in the
Institute of Clinical and Experimental Medicine (IKEM) from
1974 until now. From 1991 to May 2017, he was the Head
of the Clinic of Cardiovascular and Transplantation Surgery
and since 1995 he has been the Head of the Cardiocentre. In
1990-1991, he worked as a Consultant at Odense University
Hospital in Denmark. He is a Member of a number of national
and international scientific organizations. In his free time, he is
mostly engaged in sports and likes theater.
Extent: a completely new method of treatment of patients
with Marfan syndrome
Jan Pirk, Kočková R, Krebsová A and Malý J
IKEM, Czech Republic
Jan Pirk et al., Interv Cardiol J 2019, Volume: 5
DOI: 10.21767/2471-8157-C1-005