Acute rupture of a sinus of Valsalva aneurysm into the right atrium: a case report and a narrative review

Abstract

Background: Sinus of Valsalva aneurysm (SVA) is a rare cardiac anomaly which has potential for spontaneous rupture into other cardiac chambers or the pericardial space (depending on its location). A ruptured SVA has a very poor prognosis with high morbidity and mortality. The development of a shunt between the sinus of Valsalva and right-sided cardiac chambers results in a continuous murmur on examination. Our case report is a case of SVA rupture into the right atrium.

Case presentation: In this case report, we describe a 23-year-old patient with an acute onset of chest pain, shortness of breath, palpitations and dizziness starting 2 days prior to presentation to the emergency department. The patient was initially treated for presumed pulmonary embolism overnight while awaiting CTPA the next morning. However, further examination by the inpatient medical team demonstrated a continuous machinery cardiac murmur. Subsequent echocardiography demonstrated an acutely ruptured SVA with shunting to the right atrium. Emergency surgical repair resulted in an excellent outcome for the patient.

Conclusion: A thorough clinical history and physical examination is the cornerstone of all medical encounters. An SVA could be asymptomatic until acute rupture. Echocardiography is the preferred initial diagnostic tool. Additional imaging techniques can be used to confirm the diagnosis. In cases of rupture, prognosis is poor and surgical repair is always required.

Keywords: Aneurysm; Aortic aneurysm; Congenital heart disease; Sinus of Valsalva.

Fig. 1

Two-dimensional transthoracic echocardiography in parasternal long axis right ventricular inflow view; a tricuspid valve regurgitation jet on colour Doppler echocardiography. b Continuous wave Doppler through tricuspid valve regurgitation jet showing a continuous flow. RV, right ventricle; RA, right atrium

Fig. 2

Two-dimensional transthoracic echocardiography in parasternal short axis with colour compare view demonstrating a shunt from the right coronary sinus to the right atrium. RV, right ventricle; RA, right atrium; LA, left atrium; RCC, right coronary cusp; NCC, non-coronary cusp; LCC, left coronary cusp

Fig. 3

Transthoracic echocardiography suprasternal view with pulsed Doppler echocardiography demonstrating diastolic flow reversal in the descending thoracic aorta without evidence of aortic regurgitation

Fig. 4

Two-dimensional transoesophageal echocardiography in mid-oesophagus short axis with colour compare view showing a left-to-right shunt from the right sinus of Valsalva to the right atrium. RVOT, right ventricular outflow tract; RV, right ventricle; RA, right atrium; LA, left atrium; RCC, right coronary cusp; NCC, non-coronary cusp; LCC, left coronary cusp

Fig. 5

Reconstructed computed tomography pulmonary angiography sagittal view image shows intravenous contrast in the communication between the aortic root and probable right ventricle (red arrow). RV, right ventricle; RA, right atrium; AV, aortic valve

Fig. 6

Intraoperative view; a Right sinus of Valsalva aneurysm and fistula. b Repair of aneurysm. SVA, sinus of Valsalva aneurysm