Download Rupture of Right Sinus of Valsalva Aneurysm with Alternating

Unusual Ruptured Sinus of Valsalva Aneurysm
Case Report
Acta Cardiol Sin 2009;25:52-5
Rupture of Right Sinus of Valsalva Aneurysm
with Alternating Shunting into Right Atrium and
Right Ventricle
Ming-Shyan Lin,1 Chia-Pin Lin,1 Jaw-Ji Chu,2 Yuan-Chang Liu3 and Pao-Hsien Chu1
Sinus of Valsalva aneurysm (SVA) is a rare cardiac anomaly that may be congenital or acquired. We report the case
of a 29-year-old female who presented with a three-month history of exertional dyspnea. Cardiac auscultation
revealed a grade III/VI continuous murmur along the right sternal border. Transthoracic echocardiography revealed
alternating jet-flows into the right atrium (RA) and right ventricle (RV) during different phases of the cardiac cycle.
The aortography revealed periodic movement of a jet of contrast medium, which was due to a long windsock SVA
swimming between RA and RV. The aneurysm descended into the tricuspid orifice during diastole, and was possibly
lifted by the leaflet of the tricuspid valve closure during systole. The patient underwent open-heart surgery to close
the aneurysm ostium and directly suture the right atrial fistula.
Key Words:
Sinus of Valsalva aneurysm · SVA rupture · Right atrium · Right ventricle
the left sinuses.2 The incidence of SVA in males is 2-3
times higher than in females. The most common associated cardiovascular lesions are ventricular septal defects
(VSDs) and aortic valve insufficiencies.3 Structural defects are also one of the anomalies observed in association with congenital SVAs, including the bicuspid aortic
valve, pulmonary stenosis, cocarctation, tetralogy of
Fallot, patent ductus arteriosus and atrial septal defects
(ASDs).4 SVA can be diagnosed by transthoracic echocardiography (TTE), but this technique has some limitations in terms of confirmation.
We report an unusual and interesting case in which
the presence of alternating jet flows initially revealed
rupture of right SVA that was descending from the right
atrium (RA) to the right ventricle (RV) along with a
small ASD; this finding was finally confirmed at surgery. We focus on echocardiographic features and provide surgical findings to describe the condition.
INTRODUCTION
Congenital sinus of Valsalva aneurysm (SVA) is
caused by dilation, usually of a single sinus of Valsalva
due to deficiencies in tissue elasticity.1 Abnormal development of the bulbous cordis has been associated with
SVA development. Acquired etiologies include trauma,
infection, endocarditis, syphilis, Marfan syndrome, and
senile dilation of sinus and left coronary sinus. A recent
Mayo Clinic study and reviews have revealed that approximately 70% of SVAs originate from the right sinus
of Valsalva, 26% from the non-coronary, AND 5% from
Received: May 11, 2008
Accepted: July 29, 2008
1
Division of Cardiology, Department of Internal Medicine; 2Division
of Thoracic and Cardiovascular Surgery, Chang Gung Memorial Hospital; 3Department of Medical Imaging and Intervention, Chang Gung
Memorial Hospital, Chang Gung University College of Medicine,
Taipei, Taiwan.
Address correspondence and reprint requests to: Dr. Pao-Hsien Chu,
First Cardiovascular Division, Department of Internal Medicine,
Chang Gung Memorial Hospital, 199 Tun-Hwa North Road, Taipei
105, Taiwan. Tel: 886-3-328-1200 ext. 8162; Fax: 886-3-327-1192;
E-mail: [email protected]
Acta Cardiol Sin 2009;25:52-5
CASE REPORT
This is a report of a 29-year-old female who was
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Unusual Ruptured Sinus of Valsalva Aneurysm
ent directions; the jet flow going into the RV during diastole disappeared during systole, and an alternating
blood flow into the RA appeared (Figures 1A and 1B).
Ventricular septal defect (VSD) could not be ruled out
completely due to the absence of obvious bulging of the
sinus of Valsalva or vegetations. The patient refused to
undergo transesophageal echocardiography (TEE) even
after a comprehensive description of the procedure was
provided. Computed tomography angiography (CTA)
was performed, and it revealed a tubular structure from
the anterior base of the right sinus of Valsalva, with a
maximum diameter of 6 mm. The tract ran posteriorly
and became an aneurysm-like structure (17 ´ 14.5 mm),
located within the RA and attached to the tricuspid valve
(TV) (Figures 1C and 1D). Cardiac catheterization revealed elevated pulmonary artery pressure (S/D 33/11,
mean 23 mmHg) and oxygen saturation step-up at the
RA and RV. [SVC: 56% (High), 59% (Low), IVC: 46%
(Low), 59% (High), RA: 73% (High), 81% (Middle),
healthy previously and presented refractory cough, progressive dyspnea and intermittent palpitations during the
preceding 3 months. Murmur over the right parasternal
border was also noted at a local clinic, and the patient
was then transferred to our hospital for further examination.
Physical examination revealed an engorged jugular
vein (9 cm above the Lewis angle) and mild leg edema.
A continuous heart murmur was heard over the right upper and lower sternal borders, and crackles were also
heard over the bilateral basal lung fields. A hemogram
revealed normocytic anemia (hemoglobin 12.6 g/dL),
and biochemical profile revealed normal renal functions
(creatinine 0.9 mg/dL) and negative cardiac enzymes
creatine kinase-MB (CK-MB) 6 ng/mL and troponin-I,
0.13 ng/mL. At admittance, the patient’s vital signs were
37.3 °C, 105 bpm, 17 breaths/min and 108/60 mmHg.
Chest X-rays revealed mild pulmonary congestion without cardiomegaly. TTE revealed two jet flows of differ-
Figure 1. Color flow Doppler images in short-axis view demonstrating alternating bidirectional turbulent flow to the right atrium during systole
(A), and ventricle during diastole. (B) Computed tomographic angiography images reveal a tubular structure from the anterior base of the right sinus
of Valsalva, with a maximum diameter of 6 mm. (C) The tract ran posterior and became an aneurysm-like structure, sized 17 × 14.5 mm; it was
located within the RA and attached to the tricuspid valve. (D) The aortogram demonstrated mild aortic regurgitation and sinus of Valsalva aneurysm
presence, with jets into the right ventricle during diastole (arrowheads). (E) The left ventricle angiography revealed absent existence of ventricular
septal defect but showed contrast leaking into the right atrium during systole (arrowheads). (F) (AV = aortic valve; LA = left atrium; RA = right
atrium; RV = right ventricle; TV = tricuspid valve; RC = right coronary cuspid; SVA=sinus of Valsalva aneurysm).
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Acta Cardiol Sin 2009;25:52-5
Ming-Shyan Lin et al.
signs.6 These murmurs are loud, stentorian and continuous; they vary in intensity during systole and diastole,
and are best heard at the base of the heart. The murmurs
start in the systole and continue to the diastole, peaking
near S2. The intensity of the murmurs may be diminished at around the second heart sound, only to increase
again during diastole, creating a ‘to-and-fro’ cadence.
Possible differential diagnoses include patent ductus
arteriosus, ruptured SVA, cervical venous hum, mammary soufflé of pregnancy, coronary arteries, great vessel, or hemodialysis AV fistulas.
Echocardiography remains the best non-invasive diagnostic tool, and it can detect the wall of the aneurysm
and the disturbed flow within the aneurysm or at the site
of perforation.7,8 TEE may provide more precise and optimal information than TTE if performed prior to surgery. If the aneurysm is ruptured, color flow Doppler
mapping can show a continuous turbulent jet within the
aneurysm that would drain into the receiving chamber. If
the aneurysm has ruptured into the RA or RV, the flow
would be continuous during both systole and diastole according to pressure gradient. If an aneurysm is not observed with TTE, further study with TEE, MRI, CT, or
aortography should be performed to obtain complementary data for the SVA diagnosis.
In our case, SVA could not be identified easily due
to the location, we could visualize one jet flow into the
RV during the early to mid-diastole, and another jet flow
into the RA cavity was noted during systole. Interestingly, we found that two jet flows originated from the
same location but had different directions that differed
with the phase of the cardiac cycles. Since the major jet
flow was into the RV and occurred during diastole, the
possibility of a VSD (systolic phase) could be excluded.
Sequential cardiac catheterization confirmed the diagnosis of an SVA running from the RA to RV without a
VSD. By using LV angiography and aortography, we
could describe the dynamic movement of the alternating
jet flows from a long windsock SVA from the RA to RV
through the TV, and the aneurysm descended into the
tricuspid orifice during diastole, and was lifted by the
leaflet of the TV during systole.
From a surgical viewpoint, our case is unique because SVA was suspected on the basis of an interesting
echocardiographic feature; an SVA that communicated
with both cardiac chambers, namely, RA and RV, or an
74% (Low), RV: 88% (In), 89% (Body), and 83% (Out)].
Furthermore, transcardiac shunting was suspected, and
the shunt ratio (Qp/Qs) was 3.2. Aortography revealed
an aortocardiac shunt between the RA and RV, suggesting a ruptured SVA (Figures 1E and 1F).
During surgery, a right SVA that ruptured into the
junction of TV and RA was noted and an incidental
small ASD was also found (Figure 2). The SVA was
closed with a Gore-Tex patch on the aortic side and direct closure using 4-O prolene running sutures along the
right atrial side. After successful surgery, the patient’s
symptoms improved, and regular outpatient department
follow-ups were conducted.
DISCUSSION
A recent review concerning the frequency of sinus
and cardiac chamber involvement in aneurysm rupture
has been published. According to a Mayo Clinic study
and previous reviews (11 studies), aneurysms usually
rupture into the RV (50-77%) or RA (37%), leading to
aortocardiac fistulas, while the right coronary sinus
aneurysms mostly (73%) ruptured into the RV.2 The Oriental population has a higher incidences of SVA orientation from the right coronary cuspid that ruptures into RV
than western population. 5 These rare aneurysms are
eventually diagnosed once they rupture, typically fistulazing into another cavity (pericardium, atrium, RV, or
right outflow tract) and manifesting symptoms and
Figure 2. Exposure of the ruptured windsock aneurysm, protruding
into the right atrium (arrow), located at the lower right atrium (RA) just
above the level of the tricuspid valve.
Acta Cardiol Sin 2009;25:52-5
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Ming-Shyan Lin et al.
TV involvement if TEE is unavailable.
SVA in RA along with a VSD were suspected. However,
one long and large wind-sock SVA located at the atrial
side near the TV junction, descending into the RV during
the cardiac cycle, was finally confirmed. Tricuspid regurgitation is a serious consideration if there is TV involvement, and may increase the risk of endocarditis and
the difficulty of surgical repair. CTA provides early diagnosis of SVA and precise location of the SVA before surgical evaluation. For example, in this study, CTA revealed that the SVA was located along the atrium near
the junction of the TV without destruction or involvement of the TV. In time, CTA will be a perfect pre-operative tool for evaluating TV involvement and identifying
the location of the rupture hole; however, if SVA is suspected, images of better quality focus around the TV can
be obtained.
To the best of our knowledge, there were only six
cases of SVA presented with suspected jet flows into
both chambers, due to bilateral rupture in three cases9-11
or alternating jet flows in others,12-14 from 1987 to 2008.
Of the six cases, only two were acquired due to endocarditis; the others were congenital.9,11 In acquired aneurysms, secondary degeneration of the elastic connective
tissue occurs as a result of atherosclerosis or infection.
There are many interesting clinical features for cases
with direct or indirect involvement of both chambers.
SVA with echocardiographic features of dual jet flows
into both chambers will be classified to two major types;
one is direct dissection or rupture of the SVA into both
chambers, the other is a long and large sac within one
chamber, and protruding into the other.
SVA is a rare congenital anomaly that requires early
surgical correction following its diagnosis and recognition
of typical heart failure signs after physical examination.
The repair procedure for an unruptured SVA is individualized, and it has a high success rate and low mortality.15
In conclusion, this is an unusual case of SVA with
interesting echocardiographic features since this SVA
was a wind sac that swam between both chambers and
was combined with an ASD that was undetected prior to
surgery. CTA, or more recently, magnetic resonance imaging could provide sufficient additional information
about aneurysm location, communicating chambers, and
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