Transcript Congenital Aneurysm of the Sinus of Valsalva

Sinus Valsalva Aneurysm
Seoul National University Hospital
Department of Thoracic & Cardiovascular Surgery
Sinus Valsalva Aneurysm
Definition
• Thin walled, saccular or tubular outpouchings, usually
always in the right sinus or adjacent half of the
noncoronary sinus.
• They generally have an intracardiac course, but may
protrude into the pericardial space and they may
rupture into the right (or rarely left) heart chambers
to form an aorta-cardiac fistula.
• This defect may result from absence of normal elastic
tissue and media in this region.
• Congenitally weak area gradually enlarges under aortic
pressure to form an aneurysm, although the age at
which this occurs is uncertain
Sinus Valsalva Aneurysm
 History
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1st description by Hope in 1839
1st important paper published by Thurman in 1840
Suggested ruptured as congenital by Abbott in 1919
Reviewed the subject of congenital and acquired lesion
by Jones and Langley in 1949
1st diagnosis of rupture during life by Venning in 1951
1st. successful repair with CPB in 1956 at Mayo Clinic
& University of Minnesota using CPB
Aneurysm of Sinus Valsalva
Clinical features
1 Etiology ; congenital but other possibly acquired
* Endocarditis, syphilis, Behcet’s disease, atherosclerosis,
Cystic medial necrosis, penetrating injury
* Incomplete fusion of proximal & distal bulbous chordous
* Anatomic defect in the elastic tissue
* Deficiency of the conal septum
2 Rupture or fistula
1) Incidence : rare ( 0.2 ~ 0.5% of open heart surgery),
75% ~ 80% are male
2) Site
* Right coronary sinus to right ventricle : 65%
* Noncoronary sinus to right atrium : 25%
* Left coronary sinus to left atrium : rarely
3) Aortico-left ventricle tunnel
: exceedingly rare form
Sinus Valsalva Aneurysm
Etiology
1 Separation of the aortic media of the sinus from the media adjacent
to the hinge line of the AV valve cusp resulted from the absence
of normal aortic elastic tissue and media in two region.
2 Congenitally weak area gradually gives way under aortic pressure to
form an aneurysm.
3 The aneurysm appears an excavation of the sinus which protrudes
into the underlying cardiac chamber.
4 In Asians, the basic abnormality is sited leftward and toward the
commissural area between Rt. and Lt. cusp.
5 Acquired lesions caused by medionecrosis, syphilis, atherosclerosis,
endocarditis, or penetrating injury are more diffuse, involving more
of sinus or multiple and often ascending aorta, and projecting outside
the heart.
Sinus Valsalva Aneurysm
Pathophysiology
• Thinning of the aorta medial layer in the wall of a sinus
of Valsalva results in an aneurysmal dilation, which
may extend and rupture into a corresponding cardiac
chamber, forming an aortocardiac fistula.
• Aneurysms usually arise from the right coronary sinus
and extend into the right ventricle or right atrium.
• Aneurysmal rupture into the right heart results in a
large left-to-right shunt , which, in turn, can lead to
congestive heart failure.
• Unruptured aneurysms extending into the right heart
may cause tricuspid valve stenosis/incompetence, right
ventricular outflow tract obstruction, or complete heart
block.
Sinus Valsalva Aneurysm
Histologic view
Unruptured aneurysm of right sinus Valsalva
Aneurysm is walled by atrophic muscular tissue of RVOT
Sinus Valsalva Aneurysm
Associated cardiac anomalies
• VSD occurs in 30 to 50%, but may be a little higher in
surgical patients.
• Aortic valve abnormalities & incompetence are common,
and when VSD is present, AR usually results from a
prolapsed cusp , and when VSD is not present , AR
usually arises from other valve abnormalities
• Pulmonary stenosis is uncommon, but small gradients
are common.
• Others are uncommonly, but any defects including COA,
PDA, ASD, subaortic stenosis & TOF are present.
Sinus Valsalva Aneurysm
Natural History
1 Unruptured aneurysms uncommonly cause symptoms, by
protrusion into RA and RV, heart block as well as ventricular
tachycardia may result.
2 Rupture of aneurysm tends to take place in the 3rd or 4th
decade of life.
3 Once symptoms develop, the heart failure worsens and, without
surgical treatment, most patient die within one year.
Clinical presentation is usually within the 3rd decade of life
4 When a VSD coexists, AV is usually at least mildly incompetent,
by the time 15 to 20 years, a fixed fibrous deformity of the
prolapsed leaflet occurs.
Sinus Valsalva Aneurysm
 Clinical features
•
The SVA produce TV dysfunction or RVOT obstruction.
•
80% of the persons with sinus Valsalva aneurysm are male.
•
Rupture produces acute symptoms in about 35% and gradual
onset of effort dyspnea in 45% and no symptoms in 20%.
•
In a few patients, death occurs within days, but in most there is
improvement, followed by recurrent symptoms.
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The frequency of symptoms may be related to the size of the
Fistula.
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Rupture is heralded not only by pain & dyspnea but also by
appearance of murmur, widened pulse pressure.
Sinus Valsalva Aneurysm
Daigram of aneurysm
• Unruptured aneurysm of right sinus Valsalva with VSD
Sinus Valsalva Aneurysm
Daigram of aneurysm
• Ruptured right Sinus Valsalva Aneurysm with VSD
Sinus Valsalva Aneurysm
Rupture site
Arrows indicate common sites of rupture of sinus of Valsalva aneurysm
M ; membraneous septum NC ; noncoronary sinus
V ; atrioventricular septum C ; conal septum
Sinus Valsalva Aneurysm
RVOT obstruction
Sinus Valsalva Aneurysm
Rupture
1
The sinus of origin is the main determinant of the direction of projection
and rupture.
2
Gradually develops a more localized windsock, in an unknown
percent of cases ultimately rupture into an adjacent low pressure
chamber and rarely outside chamber.
3
When the aneurysm coexists with a VSD(30-50%), the windsock usually
projects into the RV.
4
In about one fourth, there is no windsock or any suggestion of
aneurysm formation, but rather, a direct fistulous communication.
5
Typical windsock deformity may be more common from right sinus
lesion, and a direct fistula in noncoronary sinus to RA lesion.
.
Sinus Valsalva Aneurysm
Sinus Valsalva Aneurysm
Sinus Valsalva Aneurysm
Sites of rupture or fistula
1 Aneurysm of the right sinus may originate more centrally and
project into the outlet of RV, but leftward portion into region of
membranous septum.
2 Aneurysms from the noncoronary sinus usually originate from
its anterior portion and rupture into the RA, but in rare cases
into RV, posterior portion may rupture into the pericardium.
3 Rarely, right or noncoronary sinus aneurysm rupture into LV.
4 Aneurysms from left coronary sinus rupture into the LA, LV,
but rarely into LV due to thick wall and high pressure.
5 Aneurysms rupturing into areas adjacent to TV may be a cause
of heart block or RBBB.
Sinus Valsalva Aneurysm
Indications for operation
1 When ruptured or is associated with VSD or
with a VSD and AR, prompt operation is
advisable.
2 Unruptured aneurysm that are producing
hemodynamic derangements should be
repaired.
3 Small or moderate-sized unruptured aneurysm
probably should not be repaired surgically.
Sinus Valsalva Aneurysm
Techniques of operation
1 Ruptured aneurysm of right sinus Valsalva without
VSD
2 Ruptured aneurysm of the sinus of Valsalva into the
RA without VSD
3 Ruptured aneurysm of the right sinus of Valsalva
associated with VSD
* Repair by excision of aneurysm and reconstruction
* Repair by closing the origin of aneurysm
* Repair the associated VSD and valve
Sinus Valsalva Aneurysm
Techniques of operation
• Repair of ruptured aneurysm of right sinus Valsalva with VSD
Sinus Valsalva Aneurysm
Techniques of operation
• Repair of unruptured aneurysm of right sinus Valsalva
Sinus Valsalva Aneurysm
Techniques of operation
• Noncoronary sinus
of Valsalva aneurysm
extending into the
right atrium
Sinus Valsalva Aneurysm
Techniques of operation
• Noncoronary sinus
of Valsalva aneurysm
extending into the
right atrium
• VSD patch closure in
case of VSD
Sinus Valsalva Aneurysm
Techniques of operation
• David-V valve-sparing root replacement using a De Paulis
Gelweave Valsalva graft
Sinus Valsalva Aneurysm
 Results of operation
1 Survival
2 Risk factors for premature late death
1) severe aortic incompetence
2) left ventricular enlargement
3) aortic valve replacement
3 Functional status
Persistent or worsening aortic valve incompetence
accounts for most of functional disability
4 Complications
1) Reoperation
2) Heart block
Aorta–right Atrial Tunnel
Clinical features
• Aorta–right atrial tunnel (ARAT) is a very rare
abnormal tubular extracardiac communication
between the ascending aorta and the right atrium.
• The first case was described in 1980 by Otero Coto
and colleagues
• Embryologic background and cause for this anomaly
are not clear.
• Probable cause seems to be a congenital deficiency of
the elastic lamina in the aortic media
• The tunnel-like vascular extracardiac communication
between the aortic root and the right atrium arose from
any of the 3 sinuses of Valsalva.
Aorta–right Atrial Tunnel
Clinical features
• This aorto–right atrial communication behaves like a
left-to-right shunt at the atrial level.
• The most common symptoms were shortness of breath,
palpitation, and recurrent respiratory tract infections.
• On physical examination, all patients had a continuous
murmur at the right parasternal border.
• The single diagnostic feature is demonstration of this
distinct tunnel arising from one of the aortic sinuses of
Valsalva and having an extracardiac course and
entering into the right atrium
• Treatment options are simple ligation or ligation with
implantation of coronary ostium or coil embolization.