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Unruptured aneurysms of the sinus of valsalva into the pulmonary artery presenting with right ventricular outflow tract obstruction

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Abstract

Congenital aneurysms of the sinus of Valsalva are rare lesions. These can rupture into any cardiac chamber, depending upon the site of origin of the aneurysm, forming aortocardiac fistulas. We encountered a 30-yearold male, with aneurysm of the right sinus of Valsalva, which through the infundibulum, projected into the pulmonary artery, and presented with right ventricular outflow tract obstruction (RVOTO) Case Report Congenital aneurysms of the sinus of Valsalva are rare lesions. These aneurysms follow predictable intracardiac course, usually rupturing into adjacent lowpressure chamber. Patients present either with aortocardiac fistulas with large shunts and congestive cardiac failure or with signs of ventricular septal defect (VSD) and/or aortic incompetence (A1). It is extremely rare for an aneurysm to rupture into the pulmonary artery or to project into the pulmonary artery to cause right ventricular outflow tract obstruction. We encountered a 30-year-old male, who presented with dyspnea on exertion (NYHA class II) and palpitations. Clinical examination revealed signs of right ventricular overload. A grade 1V/V1 systolic murmur was found in the left parastemal area in the 2 nd and 3 rd interspaces. Echocardiography revealed obstruction to the right ventricular outflow tract at the valvar level. There was no VSD or AI. The patient was posted for cardiac catheterization and possible balloon dilatation of the RVOTO, however on cardiac catheterization a 4 cm large aneurysm arising from the right sinus of Valsalva was found obstructing the pulmonary artery at the level of the valve annulus.
IJTCVS Karbhase et al 145
2003; 19: 145–146 SOV aneurysms
Congenital aneurysms of the sinus of Valsalva are
rare lesions. These can rupture into any cardiac chamber,
depending upon the site of origin of the aneurysm,
forming aortocardiac fistulas. We encountered a 30-year-
old male, with aneurysm of the right sinus of Valsalva,
which through the infundibulum, projected into the
pulmonary artery, and presented with right ventricular
outflow tract obstruction ( RVOTO)
Case Report
Congenital aneurysms of the sinus of Valsalva are
rare lesions. These aneurysms follow predictable
intracardiac course, usually rupturing into adjacent low-
pressure chamber. Patients present either with
aortocardiac fistulas with large shunts and congestive
cardiac failure or with signs of ventricular septal defect
(VSD) and /or aortic incompetence (A1). It is extremely
rare for an aneurysm to rupture into the pulmonary
artery or to project into the pulmonary artery to cause
right ventricular outflow tract obstruction.
We encountered a 30-year-old male, who presented
with dyspnea on exertion (NYHA class II) and
palpitations. Clinical examination revealed signs of right
ventricular overload. A grade 1V/V1 systolic murmur
was found in the left parasternal area in the 2nd and 3rd
interspaces. Echocardiography revealed obstruction to
the right ventricular outflow tract at the valvar level.
There was no VSD or AI.
The patient was posted for cardiac catheterization
and possible balloon dilatation of the RVOTO, however
on cardiac catheterization a 4 cm large aneurysm arising
from the right sinus of Valsalva was found obstructing
the pulmonary artery at the level of the valve annulus.
Unruptured aneurysms of the sinus of valsalva into the
pulmonary artery presenting with right ventricular outflow
tract obstruction
JN Karbhase, M.Ch, P Sinha, MS, P Joshi, M.Ch, C Doshi, MS, KN Nagle, M.Ch.
BYL Nair Charitable Hospital, Dept. of CTVS, Mumbai
The patient underwent an elective operation.
Through a median sternotomy ascending aortic and
bicaval cannulation was done and cardiopulmonary
bypass started. A vent was placed through the right
superior pulmonary vein. Aorta was cross-clamped and
a transverse aortotomy was made. Warm sanguinous
direct intracoronary cadioplegic infusion was used to
arrest the heart. The aneurysm found to be arising form
the right sinus of Valsalva, and was projecting through
the infundibulum into the pulmonary artery. Aneurysm
Address for correspondence:
Dr. J.N. Karbhase
Prof. & Head, CTVS, 2nd Floor, College Building
BYL Nair Charitable Hospital, Mumbai – 08
email –dr-pranavas@yahoo.com
©IJTCVS 097091341931103/003
Fig. 2. Right ventriculogram showing filling deffect in outflow tract.
Fig. 1. Left ventriculogram showing opacification of the aneurysm.
Case reports
030-02.p65 8/24/2003, 12:40 PM145
146 Karbhase et al IJTCVS
SOV aneurysms 2003; 19: 145–146
sac was everted excised and the defect repaired with a
PTFE patch. The aorta was closed. The patient was
weaned off cardiopulmonary bypass. The postoperative
recovery was uneventful. The postoperative
echocardiogram showed no residual RVOTO. There was
no residual AI.
Comment
Congenital aneurysms of the sinus of Valsalva are
extremely rare lesions with an incidence of 0.1%1. they
are almost five times more common in Asians2. Three
quarters of the patients are males4.
Two thirds of the aneurysms arise from the right
aortic sinus, one quarter from the noncoronary sinus
and remaining from the left aortic sinus1.
Associated lesions are common in congenital sinus
of Valsalva aneurysm1. Aortic imcompetence occurs in
almost 30% to 75% of cases and ventricular septal defects
occur in 30% to 50% of cases3. The aneurysm usually
follows a predictable intracardiac course4. Owing to the
central nature of the aortic root they can rupture into
any cardiac chamber. However rupture into the
pulmonary artery or the pericardium is rare1. The
incidence of rupture into the pulmonary artery is less
than 1%1, and there is only one case report so far5. This
is because of the anatomical relationship of the aortic
root to the pulmonary artery, the pulmonary artery being
separated from the aorta by the infundibulum. Another
report of an unruptured sinus of Valsalva into the
pulmonary artery has been made4.
There was no residual RVOTO following successful
surgical repair of the lesion. There is 17% risk of
development of late AI requiring aortic valve
replacement3, hence such patients must be kept in close
long-term observation.
References
1. Kirklin JW, Barratt Boyes BE. Congenital aneurysms of the sinus
of Valsalva In: Kirklin JW, Barratt Boyes BE, eds, Cardiac surgery.
2nd ed. New York: Churchill Livingstone, 1993; 825–39.
2. Chu Sh, Hung CR, How SS, et al. Ruptured aneurysms of the
sinus of Valsalva in Oriental patients. J Thorac Cardiovas Surg.
1990; 99: 288–98.
3. Azakie A, David TE, Peniston CM, et al. Ruptured sinus of
Valsalva aneurysm: Early recurrence and fate of the aortic valve.
Ann Thorac Surg. 2000; 70: 1466–71.
4. Shiraishi S, Watarida S, Katsuyama K, et al. Unruptured
aneurysm of the sinus of Valsalva into the Pulmonary artery.
Ann Thorac Surg. 1998; 65: 1458–59.
5. Heilman KJ, Groves BM, Cammpbell D, et al. Rupture of left
sinus of Valsalva aneurysm into the pulmonary artery. J Am Coll
Cardiol. 1985; 5: 1005–07.
Fig. 3. Right ventriculogram showing outflow tract obstruction.
030-02.p65 8/24/2003, 12:40 PM146
... Their most frequent complication is the aneurysm rupture into the adjacent cavities, mostly to right ventricle ( fig.1) followed to right atrium (3)(4)(5)(6)(7). Other complications include compression of the adjacent structures, causing obstruction of the left or right ventricular outflow tract, (8,9) compression of the coronary arteries leading to angina, or myocardial infarction (10). Rarely dissection by SOVA of the interventricular septum may occur (11). ...
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A sinus of Valsalva aneurysm may occur as a congenital or acquired defect at the junction of the aortic media and the surrounding fibrous tissue. They are generally asymptomatic until a complication sets in, thus can cause compression and or rupture of adjacent cardiac structures, resulting in symptoms which prompt urgent management. This condition may be managed surgically or percutaneously. We aim to evaluate the outcomes in surgical versus percutaneous closure of ruptured sinus of Valsalva. This Systematic review and meta-analyses statement included 254 patients, stratified in two groups, percutaneous group (70 patients) and surgical group (184 patients). Overall poor outcome occurred in 31 patients OR 0.75 (P= 0.51, I2=53%). Sub-analysis showed an occurrence of residual shunt (OR 1.71, P=0.47, I2=0%), aortic regurgitation “de novo” (OR 1.11, P=0.87), sinus of Valsalva aneurysm recurrence (OR4.28, P=0.31), pericardial effusion (OR 0.74, P=0.71, I2=0%), with a mortality OR 0.46 (CI 95% [0.08-2.75], P=0.39, I2=0%). The present study showed that when compared to surgical treatment the percutaneous closure of ruptured sinus of Valsalva is safe and effective, being an excellent alternative in selected patients.
... 7-11 Approximately 11.5 to 53% of SVAs are unruptured. [7][8][9][10][11] As in these cases, the SVA may protrude into the adjacent cardiac chamber, create right 12,13 or left ventricular outflow tract obstructions, 14 cause myocardial infarction or ischaemia 15,16 by compression of the coronary arteries by the aneurysm, or rarely, dissect into the interventricular septum. 3,4,7,[17][18][19][20][21] The mean age of SVA reported by Choudhary 7 was 27.51 ± 3.21 years, and in most other reported series, 2,[9][10][11]17,18 it was similar. ...
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Sinus of Valsalva aneurysms (SVA) are considered rare congenital anomalies. Apart from a few reported cases of SVA in African patients, there is little information on the clinical presentation of this entity in Africans. We describe the clinical and echocardiographic features of four consecutive African patients in whom the diagnosis of SVA was established over the last six years at the echocardiography laboratory of our institution. In this report we compare the clinical and echocardiographic features in our patients with those of others published in the literature.
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Full-text available
Abstract Introduction: The sinus of Valsalva aneurysms are relatively rare diseases. Are more frequent in the eastern population than in the western population. Little is known about its frequency in African countries. Methods: The authors conducted a retrospective study of patients referred to the echocardiography laboratory of our hospital, where the diagnosis of aneurysm of the sinus of Valsalva was done in the period from May 2001 to September 2014 Results 10 patients were found, seven were male . The average age was 25.8±11.4 years. The most common clinical presentation was cardiac failure in six patients, followed by atypical chest pain in two. The sinus of Valsalva most often affected was the right coronary sinus in seven patients (70%), followed by the left coronary sinus in two patients. The most common complication was dissection of the interventricular septum in 6 patients (60%), followed by rupture of the right cavities in two patients. Eight patients were operated: four in Angola, three in South Africa and one in Italy. Two patients died. CONCLUSIONS: 1) the sinus of Valsalva aneurysms are relatively rare diseases; 2) Achieve younger individuals; 3) dissect more often to the interventricular septum; 4) have a good prognosis when treated quickly; 5) They must always be on the list of differential diagnosis in young patients with heart failure, heart murmurs or arrhythmias. Keywords: Sinus of Valsalva aneurysm. Echocardiography; Transesophageal echocardiography
Article
Between 1964 and 1987, a total of 57 cases of ruptured aneurysm of the sinus of Valsalva underwent surgical correction at the National Taiwan University Hospital. This represents 0.96% of all cardiac operations. The origin of ruptured aneurysm of the sinus of Valsalva was the right coronary sinus in 46, the noncoronary sinus in nine, and the left coronary sinus in two. The aneurysms ruptured into the right ventricle in 44, into the right atrium in 11, into the left ventricle in one, and into both the right ventricle and right atrium in one. Associated congenital cardiac anomalies included ventricular septal defect in 30 patients, aortic regurgitation in 20, and infundibular pulmonic stenosis and coarctation of the aorta in one each. Operative death occurred in two patients (3.5%) and one patient had a successful reoperation. The remainder did well following surgery. To compare the differences between Oriental and Western countries in ruptured aneurysm of the sinus of Valsalva, 361 cases (195 Oriental patients versus 166 Western) were collected from the literature. Analyses of these cases revealed that ruptured aneurysm of the sinus of Valsalva in Oriental patients compared with Western series is characterized by a higher incidence (5 times), more aneurysms originating from the right coronary sinus (87.9% versus 63.6%), more aneurysm rupturing into the right ventricle (84.2% versus 56.6%), a higher incidence of association with ventricular septal defect (mainly supracristal) (59.0% versus 34.6%), less incidence of association with other congenital cardiac abnormalities (4.1% versus 21.5%), very few instances of rupturing into cardiac chambers other than the right ventricle and right atrium, and less incidence of occurrence in the extremities of ages (the youngest was 7 years in Oriental patients versus 11 months in the Western series). In other words, ruptured aneurysm of the sinus of Valsalva in Oriental patients is more or less a simple and uniform disease entity in contrast to the more diverse and protean pathologic profiles encountered in Western series. However, both Oriental patient and Western patient series have similar incidences of combination with aortic regurgitation (24.6% versus 20.0%), with 40.4% of Oriental patients and 60.6% of Western patients presenting with intact ventricular septum. Therefore the pathogenetic mechanisms of ruptured aneurysm of the sinus of Valsalva may at the same time contribute to the development of aortic regurgitation.
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Congenital sinus of Valsalva aneurysm is an uncommon lesion that frequently presents after rupture in adult life. This report describes a patient with a left sinus of Valsalva aneurysm that ruptured into the main pulmonary artery, a previously unreported anatomic variant. Anatomic and clinical features of previously reported cases of ruptured sinus of Valsalva aneurysm are reviewed.
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Congenital aneurysms of the sinus of Valsalva are rare lesions. Because the aortic root is central, the aneurysm call rupture into any cardiac chamber, and virtually all combinations of sinus and chamber fistulas have been described. Rupture into the pulmonary artery, however, is very rare. We encountered a 14-year-old boy with conal ventricular septal defect and right coronary cusp prolapse with an unruptured aneurysm of the sinus of Valsalva into the pulmonary artery. (C) 1998 by The Society of Thoracic Surgeons.
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We reviewed our experience with congenital ruptured sinus of Valsalva aneurysms (RSVA) to determine patterns of early recurrence and the fate of the aortic valve (AV). Over a 28-year period, RSVA was identified in 34 patients, (mean age 31.6 years). Primary closure of the RSVA was performed in 10 patients, and a patch employed in 24. Aortic insufficiency was present in 24 patients. AV replacement (AVR) was performed in 5 patients; AV repair in 6. Follow-up of 9.2 +/- 8.3 years (6 months to 24 years) was complete in all but 2 patients. Five early fistula recurrences (in 4 patients) correlated with primary rather than patch closure (p < 0.03). Kaplan-Meier survival at 10 years is 90 +/- 7%. Freedom from reoperative AVR at 10 years is 83 +/- 9%. Late AVR was performed in 6 patients for progressive aortic insufficiency due to bicuspid valve (n = 3), cusp disease of affected sinus (n = 2), or aortic root dilatation (n = 2). Patch closure of the RSVA should be routinely employed. A bicuspid valve may be associated with the late need for AVR.
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Sinus of Valsalva aneurysm is a rare congenital defect and the diagnosis is usually made after it ruptures. Very few cases of non-complicated sinus of Valsalva aneurysm have been published. The anomaly most often involves the right coronary sinus and less frequently the non-coronary sinus. A case with left sinus of Valsalva has not been reported. Since the aortic valve occupies a central position in the base of the heart, rupture of a sinus of Valsalva aneurysm can occur in any of the four heart chambers. Rupture into the pulmonary artery is very rare and is mostly associated with other congenital cardiopathies.
Rupture of left sinus of Valsalva aneurysm into the pulmonary artery Fig. 3. Right ventriculogram showing outflow tract obstruction
  • Kj Heilman
  • Groves
  • Bm
  • D Cammpbell
Heilman KJ, Groves BM, Cammpbell D, et al. Rupture of left sinus of Valsalva aneurysm into the pulmonary artery. J Am Coll Cardiol. 1985; 5: 1005–07. Fig. 3. Right ventriculogram showing outflow tract obstruction. 030-02.p65 8/24/2003, 12:40 PM146
Right ventriculogram showing outflow tract obstruction. 030-02
  • Fig
Fig. 3. Right ventriculogram showing outflow tract obstruction. 030-02.p65 8/24/2003, 12:40 PM