Article, Cardiology

An unusual cause of acute chest pain: rupture of the noncoronary sinus of Valsalva into the right atrium

Case Report

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American Journal of Emergency Medicine

journal homepage: www. elsevier. com/ locate/ajem

American Journal of Emergency Medicine 34 (2016) 2052.e1-2052.e3

An unusual cause of acute chest pain: rupture of the noncoronary sinus of Valsalva into the right atrium?

Abstract

Ruptured sinus of Valsalva aneurysm is a rare cardiac anomaly, and it can be acquired or congenital. Untreated, there is a substantial risk of complications, especially if rupture occurs with a subsequent Intracardiac shunt and rapidly worsening Cardiac insufficiency. Herein we describe a peculiar case of a ruptured aneurysm of the noncoronary sinus of Valsalva into the right atrium in a 44-year-old man with acute chest pain.

A 44-year-old man was admitted to our emergency department because of acute onset severe chest pain that started about 2 hours later after an intense emotional stress. The pain was anteriorly located in the chest without extension to the neck or jaw. Medical history was unremarkable and the only known Cardiovascular risk factor was cigarette smoking. Physical examination revealed tachycardia with a continuous systolic-diastolic cardiac murmur. Peripheral pulses were present and symmetrical. Blood pressure was 95/45 mm Hg.

Electrocardiogram (ECG) showed a sinus tachycardia (150 beats per minute) with right bundle-branch block and diffuse ST-segment depression with elevation in aVR lead (Fig. 1).

Pocket echocardiography was not diagnostic because of the high transthoracic impedance. Left ventricle was not dilated with apparently preserved global systolic function and no obvious regional wall motion abnormalities. Right chambers were enlarged. From the subcostal view, a jet in the right atrium was visualized which appeared initially to be due to a large Atrial septal defect. However, the origin of the cardiac shunt was uncertain.

The diagnosis was not clear, and we decided to exclude an acute coronary syndrome despite the anomalous presentation and some findings consistent with an acute aortic syndrome.

The patient underwent immediate coronary angiography. Coronary

arteries were normal (Fig. 2a-b), but thoracic aortogram showed a contrast jet column directed from one of the aortic sinuses of Valsalva into the right atrium (Fig. 2c).

A transesophageal echocardiogram was thus performed, and it revealed a ruptured aneurysm of the noncoronary sinus of Valsalva into the right atrium with a large shunt (Fig. 3a-c). The aortic valve

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was otherwise continent without central leaks. There were no imaging features of intimal flap in the ascending or descending part of the aorta.

Because of the worsening of the patient’s conditions, an immediate cardiac surgery was planned. Unfortunately, a cardiac arrest with pulseless electrical activity occurred 10 minutes later. The patient died despite prompt cardiopulmo- nary resuscitation.

Sinus of Valsalva aneurysms are a rare cardiac anomaly, and they can be congenital or acquired. Aneurysms can potentially arise from all 3 coronary sinuses but most frequently involve the right or the noncoronary sinuses [1]. Congenital forms range from 0.5% to 3% of all congenital cardiac defects; acquired aneu- rysms can be caused by inflammatory disease (eg, syphilis, tuber- culosis, Behcet disease), by Traumatic events, and by connective tissue diseases (eg, Marfan disease) [2]. If unrecognized and un- corrected, these aneurysms may have catastrophic consequences because of their rupture causing large intracardiac shunts with rapidly worsening cardiac dysfunction and death. Ruptured aneu- rysms of Valsalva may be exceptionally found during a routine checkup in asymptomatic patients [3].

In 1962, Sakakibara and Konno [4] proposed the first classification system for sinus of Valsalva aneurysm according to their site of origin and rupture. Following this classification, our patient had a type IV ruptured sinus of Valsalva aneurysm. In recent years, some modified Classification systems were proposed to make their use in clinical practice easier [4].

Surgical repair is the treatment of choice for ruptured aneurysms of the sinus of Valsalva, and long-term prognosis after surgery is excellent [5]. However, transcatheter closure with occluder devices has been oc- casionally reported [6].

We would like to draw attention to certain key aspects of our report, first of all the clinical presentation of our patient. The finding of a contin- uous cardiac murmur raised the suspicion of an aortic syndrome as a possible cause of the patient’s chest pain, but the hypothesis of a rup- tured sinus of Valsalva aneurysm per se was not considered in the diag- nostic workup before coronary angiography and echocardiogram. The second aspect is related to the ECG abnormalities. The finding of a right bundle-branch block with a diffuse ST-segment depression was the main reason to perform an urgent coronary angiography; however, these abnormalities could be alternatively explained as an acute and massive right hearth overload due to a ruptured sinus of Valsalva aneu- rysm into the right atrium with consequently diffuse circumferential myocardial ischemia due to coronary steal phenomenon. Similarly, Alozie and coworkers [7] described an incomplete right bundle-

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Fig. 1. Twelve-lead ECG showing sinus tachycardia, right bundle-branch block, and diffuse ST-segment depression (with elevation in aVR).

Fig. 2. a, Coronary angiography. Normal left coronary artery. b, Coronary angiography. Normal right coronary artery. c, Thoracic aortogram. Contrast jet column (white arrows) directed from the aortic root (sinus of Valsalva) into the heart.

branch block in a 19-year old man with an aorto-right atrial shunt due to a perforated sinus of Valsalva aneurysm.

Furthermore, we would like to emphasize the importance of integrating clinical history and physical findings with an oriented multimodality imaging (angiography and transesophageal echocardiography) consistent with an emergency setting as reported by Abdelsalam and coworkers [8].

Echocardiography has a pivotal role in confirming the diagnosis, defining the anatomy, and guiding the surgical therapy as confirmed by other authors [9].

We conclude by recalling that ruptured sinus of Valsalva aneurysm, even if rare, should be considered in the diagnostic workup of chest pain in the emer- gency department because early diagnosis and treatment deeply influence patient’s survival.

G.D. Sanna et al. / American Journal of Emergency Medicine 34 (2016) 2052.e12052.e3 2052.e3

Fig. 3. a, Transesophageal echocardiography. ME AV SAX view showing a big aneurysm of the noncoronary sinus of Valsalva (asterisk [*] and white arrows) protruding into the right atrium. Ao, aortic valve; RA, right atrium; RV, right ventricle; LA, left atrium. b, Transesophageal echocardiography. The noncoronary sinus ruptured into the right atrium with a “wind sock” appearance (white arrows indicating tissue flaps). c, Transesophageal echocardiography. A large shunt directed from the aortic root (noncoronary sinus of Valsalva) into the right atrium is clearly seen with Color Doppler technique.

Giuseppe D. Sanna, MD? Giuseppe Talanas, MD1 Cristiana Denurra, MD1 Pierfranco Terrosu, MD1

Unita Operativa di Cardiologia, Ospedale SS AnnunziataAOU Sassari, Via

Enrico De Nicola, 07100 Sassari, Sassari, Italy

?Corresponding author. Unita Operativa di Cardiologia, Ospedale “SS Annunziata” AOU Sassari, Via Enrico De Nicola, 07100 Sassari Sassari Italy. Tel.: +39 0792061561; fax: +39 079210512.

E-mail addresses: [email protected],

[email protected], [email protected]

1Tel.: +39 0 792 061 561

Paolo Ferrandu, MD2

Cattedra e Scuola di Specializzazione in Medicina d’Emergenza-Urgenza, Universita degli Studi di Sassari, Viale San Pietro 8

07100 Sassari, Sassari, Italy E-mail address: [email protected]

2Tel.: +39 079 228 389

Luca Bullitta, MD3 Cattedra e Scuola di Specializzazione in Malattie dell’Apparato Cardiovascolare, Universita degli Studi di Sassari, Viale San Pietro 8, 07100

Sassari, Sassari, Italy E-mail address: [email protected]

3Tel.: +39 079 228 380

References

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    Hoevelborn T, Doering J, Lindemann S, Haas CS. Newly discovered heart murmur: noncoronary sinus of Valsalva aneurysm with rupture into the right atrium and right ventricle. Circulation 2009;119:e15-6.

  2. Sakakibara S, Konno S. Congenital aneurysm of the sinus of Valsalva. Anatomy and classification. Am Heart J 1962;63:405-24.
  3. Moustafa S, Mookadam F, Cooper L, Adam G, Zehr K, Stulak J, et al. Sinus of Valsalva aneurysms: 47 years of a single center experience and systematic overview of published reports. Am J Cardiol 2007;99:1159-64.
  4. Abidin N, Clarke B, Khattar RS. Percutaneous closure of ruptured sinus of Valsalva aneurysm using an amplatzer occluder device. Heart 2005;91:244.
  5. Alozie A, Kische S, Kaminski A, Ince H. Aorto-right atrial shunt due to perforated sinus of Valsalva aneurysm in a young athlete: a rare congenital defect confirmed by various non-invasive imaging modalities. Eur Heart J 2012;33(1):138.
  6. Abdelsalam M, Bachinsky W, Pawlush D, Mumtaz M, Goldman J. Noncoronary sinus of Valsalva aneurysm rupture into right atrium. Tex Heart Inst J 2013;40(4):493-4.
  7. Guenther F, von Zur Muhlen C, Lohrmann J, Bode C, Geibel A. Rupture of an aneurysm of the noncoronary sinus of Valsalva into the right atrium. Eur J Echocardiogr 2008; 9(1):186-7.

    http://dx.doi.org/10.1016/j.ajem.2016.03.014

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