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JOURNAL OF CLINICAL CARDIOLOGY

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CASE REPORT OPEN ACCESS

VOLUME 5 | ISSUE 2 | DOI: HTTPS://DOI.ORG/10.33696/CARDIOLOGY.5.054

A CASE OF RUPTURED SINUS OF VALSALVA ANEURYSM

ALLA ADELKHANOVA1,*, BUSHRA UROOJ AHMED1, STEPHANIE ANDREA VALENCIA1

 * 1Mount Sinai Hospital, Chicago, IL, USA
   

+ Affiliations - Affiliations


*Corresponding Author

Alla Adelkhanova, alladelkhanova@hotmail.com

Received Date: May 16, 2024

Accepted Date: May 27, 2024

Citation

Adelkhanova A, Ahmed BU, Valencia SA. A Case of Ruptured Sinus of Valsalva
Aneurysm. J Clin Cardiol. 2024;5(2):54-58.


Copyright
© 2024 Adelkhanova A, et al. This is an open-access article distributed under
the terms of the Creative Commons Attribution License, which permits
unrestricted use, distribution, and reproduction in any medium, provided the
original author and source are credited.


ABSTRACT

Introduction: Sinus of Valsalva aneurysm (SVA) is an abnormal dilation of one or
more aortic sinuses located between the aortic valve annulus and the sinotubular
junction. SVA rupture can cause shunting into cardiac chambers creating
hemodynamic compromise and has a high morbidity and mortality requiring prompt
recognition and treatment.

Case presentation: A 62-year-old male with past medical history of heart
failure, COPD, hypertension, hyperlipidemia, schizophrenia, and dementia,
presented with a two-day onset of shortness of breath. BNP was elevated and
chest X-ray showed bilateral pleural effusions and volume overload. The patient
had a TTE (transthoracic echocardiogram) performed on May 2023 which showed
normal ejection fraction, diastolic flattening of ventricular septum consistent
with RV volume and pressure overload; atrial shunt, moderate TR, moderate
free-flowing pericardial effusion; systolic pulmonary artery pressure of 77
mmHg. TEE (transesophageal echocardiogram) was performed and showed a small
right-to-left atrial level shunt, a ruptured non-coronary sinus of Valsalva
aneurysm into the right atrium with continuous systolic and diastolic left to
right shunt on spectral and color Doppler interrogation. During the
hospitalization TTE was obtained which re-demonstrated the aneurysmal dilation
of the noncoronary sinus of Valsalva with protrusion into the right atrium,
associated with rupture and shunting into the right atrium. Patient underwent
repair of sinus of Valsalva aneurysm with fistula to right atrium using bovine
pericardium patch, repair of atrial septal defect as well as coronary artery
bypass grafting and was ultimately discharged.

Conclusion: A ruptured sinus of Valsalva aneurysm is a rare condition which can
cause heart failure with volume overload as well hemodynamic compromise. This
condition has high morbidity and mortality, therefore must be promptly
identified, and treated surgically. 


KEYWORDS

Sinus of Valsalva aneurysm, Rupture, Atrial shunt, Echocardiography

ABBREVIATIONS

SVA: Sinus of Valsalva Aneurysm; TTE: Transthoracic Echocardiogram; TEE:
Transesophageal Echocardiogram

LEARNING OBJECTIVES

 1. To identify a ruptured sinus of Valsalva aneurysm on transesophageal
    echocardiogram.
 2. To identify symptoms produced by ruptured sinus of Valsalva aneurysm.

INTRODUCTION

A sinus of Valsalva aneurysm (SVA) is a rare cardiac anomaly, accounting for
only 1% of congenital cardiac anomalies [1]. The condition is described as an
abnormal dilation of one or more of the aortic sinuses located between the
aortic valve annulus and the sinotubular junction that ensues from the dysplasia
of the vascular media [2]. It can mainly arise from a congenital defect of the
aortic media or may be acquired following trauma, connective tissue disorders,
endocarditis, atherosclerosis, or syphilis [3]. Unruptured sinus of Valsalva
aneurysms are usually clinically silent, but greater availability of diagnostic
imaging in recent years has led to an increased number being diagnosed
incidentally on echocardiography or cardiac MRIs (magnetic resonance imaging)
[4]. A SVA can rupture into adjacent cardiac chambers presenting with sudden
hemodynamic collapse and has a very poor prognosis with high morbidity and
mortality and should prompt urgent cardiothoracic evaluation for consideration
of repair [5].

We present a case of a patient with acute onset of symptoms due to a ruptured
sinus of Valsalva aneurysm.

CASE PRESENTATION

History of presentation

A 62-year-old male with a past medical history of heart failure, COPD,
hypertension, hyperlipidemia, schizophrenia and dementia, presented with two-day
onset of shortness of breath. The patient reported that the shortness of breath
started suddenly, and he felt as if he could not catch his breath. He also
endorsed orthopnea, paroxysmal nocturnal dyspnea, and leg swelling. He denied
chest pain. It was unclear whether the patient was compliant with his home
medications, one of which was Furosemide.

Investigations

On physical examination the patient was volume overloaded, with crackles over
bilateral lung fields and 1+ pitting edema on bilateral lower extremities, there
was a pan systolic murmur over the aortic, tricuspid, and mitral valves.

Laboratory investigations were significant for B-type natriuretic peptide (BNP)
of 502 pg/mL (1-100 pg/mL), high sensitivity troponins were elevated and peaked
at 277 pg/mL (0-20 pg/mL).

Chest X-ray showed bilateral pleural effusions and volume overload (Figure 1).



Figure 1. Chest X-ray demonstrating bilateral pleural effusions and volume
overload.

Patient had a TTE (transthoracic echocardiogram) performed on May 2023 which
showed normal ejection fraction, right ventricle (RV) mildly increased size and
normal function, diastolic flattening of ventricular septum consistent with RV
volume and pressure overload; atrial shunt, moderate TR with tricuspid
regurgitant peak velocity of 4.1 m/sec, moderate free-flowing pericardial
effusion; systolic pulmonary artery pressure 77 mmHg (Figure 2). He was
scheduled for an outpatient TEE (transesophageal echocardiogram) which was
performed in December 2023. It demonstrated a small right-to-left atrial level
shunt, a ruptured non-coronary sinus of Valsalva aneurysm into the right atrium
with continuous systolic and diastolic left to right shunt on spectral and color
Doppler interrogation (Figure 3). Patient was scheduled to follow up in
cardiology and cardiothoracic surgery clinic, however he did present to his
appointments.



Figure 2. TEE (midesophagel long axis aortic valve view) demonstrating ruptured
non-coronary sinus of Valsalva. NCC: Non-Coronary Cusp; LCC: Left Coronary Cusp;
RCC: Right Coronary Cusp.



Figure 3. TEE (midesophagel long axis aortic valve view) doppler demonstrating
shunting into right atrium.

During the hospitalization a repeat TTE was obtained which re-demonstrated the
aneurysmal dilation of the noncoronary sinus of Valsalva with protrusion into
the right atrium, associated with rupture and shunting into the right atrium.

Patient underwent left and right heart catheterizations, which showed
obstructive CAD (coronary artery disease) with a 40% pLAD (proximal left
anterior descending artery), 70% pRCA (proximal right coronary artery) and a
diffuse 70-80% mRCA (mid right coronary artery). The LVEDP (left ventricular end
diastolic pressure) was 21 mmHg with a mean pulmonary artery pressure of 29
mmHg. A shunt study demonstrated an abnormal 12% step-up increase in oxygen
saturation from the inferior vena cava to the right atrium indicating a left to
right atrial shunt. Aortogram demonstrated coronary sinus aneurysm with a shunt
from the aorta into the right atrium.

Management

Cardiothoracic surgery was consulted, and the patient was prepped and underwent
repair of sinus of Valsalva aneurysm with fistula to right atrium using bovine
pericardium patch, repair of atrial septal defect as well as coronary artery
bypass grafting of the right posterior descending artery using reverse saphenous
vein graft. During the surgery chest tubes as well as a temporary pacing wires
were placed. Patient was extubated on post operative day 1 and weaned off
Dopamine drip. Chest tubes were removed on post operative day 2 and 3 and
patient was downgraded from surgical ICU to medical step down. Hospital course
was complicated by post operative atrial fibrillation/flutter managed with
amiodarone drip.

Repeat TTE showed EF 55-60%, mild mitral regurgitation with small residual flow
through the surgical site from the aorta to the right atrium as well as mild
regurgitation of the aortic valve and small pericardial effusion.

Outcome

Patient was discharged to a skilled nursing facility with outpatient follow up
with cardiology and cardiothoracic surgery.

DISCUSSION

When a right or noncoronary sinus ruptures, a fistula forms between the aorta
and either the right atrium or the right ventricular outflow tract, creating a
left to right shunt, which can lead to right ventricular overload and
right-sided heart failure. Left sinus of Valsalva aneurysm ruptures are
clinically less significant, causing communication to the left atrium or left
ventricular outflow tract [1-3]. The diagnosis depends on imaging tools such as
echocardiography, computed tomographic angiography (CTA), magnetic resonance
imaging (MRI), and coronary angiography (CAG). Both TTE and TEE are initial
diagnostic tools as they are fast, noninvasive, inexpensive, and real-time ways
to assess the characteristics of aneurysmal dilation, cardiac chamber
involvement, and cardiac function. Spectral color Doppler imaging is also
crucial in aiding the diagnosis as it helps to reveal the persistent turbulent
flow between the aneurysm and the receiving chamber [4]. As seen in our case,
initial TTE showed an atrial shunt and a TEE with color Doppler visualized the
ruptured noncoronary sinus of Valsalva aneurysm into the right atrium with
systolic and diastolic flows. Our patient also underwent cardiac catheterization
which is also commonly conducted, and considered gold standard for the diagnosis
of SVA, as it assesses coronary anatomy before surgery and demonstrates elevated
PAO2 saturations in the right side of the heart, solidifying the presence of a
left to right shunt. Though CTA and MRI can offer precise visualization of
cardiac anatomy, their use is limited due to risks of ionizing radiation, cost
[4,5].

Once presence of a left to right shunt from a ruptured aneurysm of sinus of
Valsava is diagnosed, urgent surgical evaluation is recommended as mortality
rate is alarmingly high. Ruptured SVAs produce serious hemodynamic changes from
continuous shunting from the aorta into the receiving chamber, and patients who
are left untreated die within one year [6]. Patients presents with acute heart
failure symptoms like those seen in our patient and congestive heart failure is
known to be the main cause of death [7]. Tricuspid stenosis and regurgitation,
as seen in our patient, has also been reported [8]. There have also been cases
of right ventricular outflow obstruction, coronary artery compression with
infarction, conduction disturbances, endocarditis, and thrombus within the
aneurysmal cavity [9]. Based on our patient’s presentation, his sudden
decompensation was likely from progression of his rupture leading to worsening
shunt due to his lack of prompt follow-up. Surgical interventions have good
outcomes with operative mortality being between 1.9% and 3.6%, with a 15-year
survival post-procedure being around 90% [10]. In our patient, given the need
for a CABG, a surgical approach (as opposed to percutaneous closure repair) was
considered ideal as the likelihood of SVA recurrence is relatively uncommon with
surgical intervention. Cardiac conduction anomalies can be sometimes seen with
surgical intervention as compared to the percutaneous approach, which was seen
in our case, as the patient developed atrial flutter post-operation. However,
some studies show similar outcomes in both surgical and percutaneous
interventions, with both needing for close monitoring [11,12].

CONCLUSION

A ruptured sinus of Valsalva aneurysm is a rare condition which can cause heart
failure with volume overload as well hemodynamic compromise. This condition has
high morbidity and mortality, therefore must be promptly identified, and treated
surgically.

DISCLOSURES

The authors have nothing to disclose.

FUNDING

No external funding was received.

CONSENT

Informed patient consent was obtained prior to starting the case report.


REFERENCES

1. Feldman DN, Roman MJ. Aneurysms of the sinuses of Valsalva. Cardiology. 2006
Aug 1;106(2):73-81.

2. Serban AM, Bătrâna N, Cocoi M, Ianoș R, Moț Ș, Kovacs E, et al. The role of
echocardiography in the diagnosis and management of a giant unruptured sinus of
Valsalva aneurysm. Medical Ultrasonography. 2019 May 10;21(2):194-6.

3. Sun Z, Guo J, Wang H, Cui T. Aortic sinus aneurysm rupture into the right
atrium in a 29‐year‐old patient. Journal of Clinical Ultrasound. 2019
Jun;47(5):319-21.

4. Wang ZJ, Zou CW, Li DC, Li HX, Wang AB, Yuan GD, et al. Surgical repair of
sinus of Valsalva aneurysm in Asian patients. The Annals of Thoracic Surgery.
2007 Jul 1;84(1):156-60.

5. Wierda E, Koolbergen DR, de Mol BA, Bouma BJ. Rupture of a giant aneurysm of
the sinus of Valsalva leading to acute heart failure: a case report
demonstrating the excellence of echocardiography. European Heart Journal-Case
Reports. 2018 Sep;2(3):yty090.

6. Feldman DN, Roman MJ. Aneurysms of the sinuses of Valsalva. Cardiology. 2006
Aug 1;106(2):73-81.

7. Diwakar A, Patnaik SS, Hiremath CS, Chalam KS, Dash P. Rupture of sinus of
valsalva–A 15 years single institutional retrospective review: Preoperative
heart failure has an impact on post operative outcome?. Annals of Cardiac
Anaesthesia. 2019 Jan 1;22(1):24-9.

8. Chen J, Liang HN, Wu L, Dong SH, Li JH. Right sinus of Valsalva aneurysm
spontaneously dissecting into the interventricular septum in a rare case of
Behcet’s disease. European Heart Journal-Cardiovascular Imaging. 2019 May
1;20(5):601.

9. Doost A, Craig JA, Soh SY. Acute rupture of a sinus of Valsalva aneurysm into
the right atrium: a case report and a narrative review. BMC Cardiovascular
Disorders. 2020 Feb 18;20(1):84.

10. Stróżyk A, Kołaczkowska M, Fijałkowska J, Siondalski P, Fijałkowski M. Sinus
of Valsalva rupture in a patient with a mechanical aortic prosthesis: aneurysm
dissecting into the interventricular septum. Polish Heart Journal (Kardiologia
Polska). 2018;76(12):1742.

11. Vural KM, Şener E, Taşdemir O, Bayazıt K. Approach to sinus of Valsalva
aneurysms: a review of 53 cases. European Journal of Cardio-Thoracic Surgery.
2001 Jul 1;20(1):71-6.

12. Johari MI, Deraman MA, Mohamed MS, Daud AB. An unusual cause of recurrent
syncope: sinus of Valsalva aneurysm. Cureus. 2021 Sep 4;13(9):e17707.


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A CASE OF RUPTURED SINUS OF VALSALVA ANEURYSM

Introduction: Sinus of Valsalva aneurysm (SVA) is an abnormal dilation of one or
more aortic sinuses located between the aortic valve annulus and the sinotubular
junction. SVA rupture can cause shunting into cardiac chambers creating
hemodynamic compromise and has a high morbidity and mortality requiring prompt
recognition and treatment.



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