Zusammenfassung
Ziel: Kongenitale Klappenanomalien sind häufig bei Erwachsenen mit Aortenklappenfehlern.
Neben der bikuspiden Aortenklappe findet man darunter selten unikuspide Aortenklappen
(UAV). Ziel der Studie war es, die Klappenmorphologie und die Dimensionen der thorakalen
Aorta bei 12 Patienten mit UAV im Vergleich zur trikuspiden Aortenklappenerkrankung
(TAV) mittels MRT zu beschreiben. Material und Methoden/Ergebnisse: Insgesamt wurden 288 Patienten mit Aortenkappenfehlern im MRT untersucht (1,5 T,
Sonata, Siemens Medical Solutions). Davon wurden retrospektiv 12 Aortenklappen als
unikuspide klassifiziert. Die Fläche des Aortenklappenanulus und die Dimensionen der
thorakalen Aorta wurden retrospektiv mit einer Kohorte von 103 Patienten mit TAV verglichen.
Bei allen Patienten mit UAV war die Klappenmorpholgie unikuspide unikommissural mit
einer posterioren Kommissur. Die mittlere Anulusfläche und die Diameter der Aorta
ascendens der Patienten mit UAV waren signifikant größer im Vergleich zu den Patienten
mit TAV (12,6 ± 4,7 cm2 vs. 8,7 ± 2,3 cm2 , p < 0,01 und 4,6 ± 0,7 cm vs. 3,6 ± 0,5 cm, p < 0,0001), während keine Unterschiede
in den Diametern des Aortenbogens zu finden waren (2,3 ± 0,6 cm vs. 2,3 ± 0,4 cm,
p = 0,69). Die Diameter der Aorta descendens waren bei den Patienten mit UAV geringfügig
kleiner (2,2 ± 0,5 cm vs. 2,6 ± 0,3 cm, p < 0,05). Schlussfolgerung: Die Klappenmorphologie UAV kann mittels MRT sehr gut visualisiert werden. Bei allen
Patienten mit UAV lag eine unikuspide unikommissurale Aortenklappe vor. Eine Dilatation
der Aorta ascendens > 4,5 cm ist ein häufiges Phänomen der UAV. Bei diesen Patienten
sollte die Aorta ascendens deshalb dringend mit dargestellt werden.
Abstract
Purpose: Congenitally malformed aortic valves are a common finding in adults with aortic valve
disease. Most of these patients have bicuspid aortic valve disease. Unicuspid aortic
valve disease (UAV) is rare. The aim of our study was to describe valve morphology
and the dimensions of the proximal aorta in a cohort of 12 patients with UAV in comparison
to tricuspid aortic valve disease (TAV) using magnetic resonance imaging (MRI). Materials and Methods/Results: MRI studies were performed on a 1.5 T scanner in a total of 288 consecutive patients
with aortic valve disease. 12 aortic valves were retrospectively classified as UAV.
Annulus areas and dimensions of the thoracic aorta were retrospectively compared to
a cohort of 103 patients with TAV. In UAV, valve morphology was unicuspid unicommissural
with a posterior commissure in all patients. Mean annulus areas and mean diameters
of the ascending aorta were significantly greater in UAV compared to TAV (12.6 ± 4.7
cm2 vs. 8.7 ± 2.3 cm2 , p < 0.01 and 4.6 ± 0.7 cm vs. 3.6 ± 0.5 cm, p < 0.0001, respectively), while no
differences were observed in the mean diameters of the aortic arch (2.3 ± 0.6 cm vs.
2.3 ± 0.4 cm, p = 0.69). The diameters of the descending aorta were slightly smaller
in UAV compared to TAV (2.2 ± 0.5 cm vs. 2.6 ± 0.3 cm, p < 0.05). Conclusion: In UAV, visualization of valve morphology by MRI is possible with good image quality.
Valve morphology was classified as unicuspid unicommissural in all UAV patients. Dilatation
of the proximal aorta > 4.5 cm is a frequent finding in UAV. Additional assessment
of aortic dimensions is therefore recommended in patients with UAV.
Key words
aortic valve - aorta - heart
References
1
Roberts W C, Ko J M.
Frequency of unicuspid, bicuspid and tricuspid aortic valves by decade in adults having
aortic valve replacement for isolated aortic stenosis.
Circulation.
2005;
111
920-925
2
Roberts W C, Ko J M, Hamilton C.
Comparison of valve structure, valve weight, and severity of the valve obstruction
in 1,849 patients having isolated aortic valve replacement for aortic valve stenosis
(with or without associated aortic regurgitation) studied at 3 different medical centers
in 2 different time periods.
Circulation.
2005;
112
3919-3929
3
Falcone M W, Roberts W C, Morrow A G. et al .
Congenital aortic stenosis resulting from unicommissural valve. Clinical and anatomic
features in twenty-one adult patients.
Circulation.
1971;
44
272-280
4
Fedak P W, Verma S, David T E. et al .
Clinical and pathophysiological implications of a bicuspid aortic valve.
Circulation.
2002;
106
900-904
5
Bauer M, Pasic M, Meyer R. et al .
Morphometric analysis of aortic media in patients with bicuspid and tricuspid aortic
valve.
Ann Thorac Surg.
2002;
74
58-62
6
Niwa K, Perloff J, Bhuta S M. et al .
Structural abnormalities of great arterial walls in congenital heart disease.
Circulation.
2001;
103
393-400
7
Fedak P W, Sa M P, Verma de S. et al .
Vascular matrix remodeling in patients with bicuspid aortic valve malformations: implications
for aortic dilatation.
J Thorac Cardiovasc Surg.
2003;
126
797-806
8
Nistri S, Sorbo M, Marin M. et al .
Aortic root dilatation in young men with normally functioning bicuspid aortic valves.
Heart.
1999;
82
19-22
9
Cecconi M, Manfrin M, Moraca A. et al .
Aortic dimensions in patients with bicuspid aortic valve without significant valve
dysfunction.
Am J Cardiol.
2005;
95
292-4
10
Keane M G, Wiegers S E, Plappert T. et al .
Bicuspid aortic valves are associated with aortic dilatation out of proportion to
coexistent valvular lesions.
Circulation.
2000;
102
III-35–III-39
11
Roberts W C, Ko J M.
Clinical and morphologic features of the congenitally unicuspid acommissural stenotic
and regurgitant aortic valve.
Cardiology.
2007;
108
79-81
12
Agnihotri A K, Desai S C, Lai Y Q. et al .
Two distinct clinical presentations in adult unicuspid aortic valve.
J Thorac Cardiovasc Surg.
2006;
131
1169-1170
13
Novaro G M, Mishra M, Griffin B P.
Incidence and echocardiographic features of congenital unicuspid aortic valve in an
adult population.
J Heart Valve Dis.
2003;
12
674-678
14
Johnson T R, Hoch M, Huber A. et al .
Quantifizierung der rechtsventrikulären Funktion bei angeborenen Herzfehlern: Korrelation
von 3D-Echokardiographie und MRT als sich ergänzende Methoden.
Fortschr Röntgenstr.
2006;
178
1014-1021
15
Niendorf T, Sodickson D.
Beschleunigung der kardiovaskulären MRT mittels paralleler Bildgebung: Grundlagen,
praktische Aspekte, klinische Anwendungen und Perspektiven.
Fortschr Röntgenstr.
2006;
178
15-30
16
Ruhl K M, Langebartels G, Autschbach R. et al .
Kernspintomografie zur umfassenden Untersuchung des Herzens nach Implantation von
linksventrikulären apikoaortalen Conduits.
Fortschr Röntgenstr.
2007;
179
566-571
17
Rominger M B, Kluge A, Bachmann G F.
Biventrikuläre MR-Volumetrie und MR-Flussmessungen in Aorta ascendens und Truncus
pulmonalis zur Quantifizierung von Klappeninsuffizienzen.
Fortschr Röntgenstr.
2004;
176
342-349
18
Debl K, Djavidani B, Seitz J. et al .
Planimetry of aortic valve area in aortic stenosis by magnetic resonance imaging.
Invest Radiol.
2005;
40
631-636
19
Debl K, Djavidani B, Buchner S. et al .
Assessment of the anatomic regurgitant orifice in aortic regurgitation: a clinical
magnetic resonance imaging study.
Heart.
2008;
94
e8
20
Krombach G A, Kuhl H, Bucker A. et al .
Cine MR imaging of heart valve dysfunction with segmented true fast imaging with steady
state free precession.
J Magn Reson Imaging.
2004;
19
59-67
21
Chatzimavroudis G P, Oshinski J N, Franch R H. et al .
Quantification of the aortic regurgitant volume with magnetic resonance phase velocity
mapping: a clinical investigation of the importance of imaging slice location.
J Heart Valve Dis.
1998;
7
94-101
22
Friedrich M G, Schulz-Menger J, Poetsch T. et al .
Quantification of valvular aortic stenosis by magnetic resonance imaging.
Am Heart J.
2002;
144
329-334
23
John A S, Dill T, Brandt R R. et al .
Magnetic Resonance to assess the aortic valve area in aortic stenosis. How does it
compare to current diagnostic standards?.
J Am Coll Cardiol.
2003;
42
519-526
24
Kupfahl C, Honold M, Meinhardt G. et al .
Evaluation of aortic stenosis by cardiovascular magnetic resonance imaging: comparison
with established routine clinical techniques.
Heart.
2004;
90
893-901
25
Bonow R O, Carabello B A, Kanu C. et al .
ACC/AHA 2006 guidelines for the management of patients with valvular heart disease.
Circulation.
2006;
114
450-527
26
Chan K L, Stinson W A, Veinot J P.
Reliability of transthoracic echocardiography in the assessment of aortic valve morphology:
Pathological correlation in 178 patients.
Can J Cardiol.
1999;
15
48-52
27
Brandenburg R O, Tajik A J, Edwards W D. et al .
Accuracy of 2-dimensional echocardiographic diagnosis of congenitally bicuspid valve:
Echocardiographic-anatomic correlation in 115 patients.
Am J Cardiol.
1983;
83
1469-1473
28
Espinal Jr M, Fuisz A R, Nanda N. et al .
Sensitivity and specificity of transesophageal echocardiography for determination
of aortic valve morphology.
Am Heart J.
2000;
139
1071-1076
29
Borger M A, Preston M, Ivanov J. et al .
Should the ascending aorta be replaced more frequently in patients with bicuspid aortic
valve disease?.
J Thorac Cardiovasc Surg.
2004;
128
677-683
Dr. Kurt Debl
Klinik und Poliklinik für Innere Medizin II, Universitätsklinikum Regensburg
Franz-Josef-Strauss-Allee 11
93053 Regensburg
Phone: ++ 49/9 41/9 44 72 11
Fax: ++ 49/9 41/9 44 72 13
Email: kurt.debl@klinik.uni-regensburg.de