Rupture of Sinus of Valsalva with Endocarditis and Aortic Root Abscess

A ruptured sinus of Valsalva aneurysm rarely accompanies the aortic valve endocarditis. A 40 year old female presented with low threshold exertional dyspnea and fever. Transthoracic and transesophageal echocardiography showed ruptured sinus of Valsalva aneurysm in right ventricle with multiple vegetations at the aortic root. Initial blood cultures were negative and an empirical antibiotic therapy was started. Patient was operated for surgical repair of sinus of Valsalva aneurysm. An aortic root abscess with rupture of sinus of Valsalva was found at operation. However, postoperatively she developed sepsis and multiorgan failure leading to her death.


Introduction
A Sinus of Valsalva Aneurysm (SOVA) is an enlargement of the aortic root area between the aortic valve annulus and the sinotubular ridge.In a normal heart, both the left and right sinus contain their respective coronary artery ostia, whereas the posterior sinus is a non coronary sinus [1].
SOVA is a congenital or acquired cardiac defect which is found in roughly 0.09% of the general population [2].Less commonly, it is associated with endocarditis, atherosclerosis, trauma, syphilis, or aortic dissection.Its incidence is five times higher in Asian countries with male/female ratio as 4:1 [2,3].
The anatomic setting of a SOVA usually predicts the clinical outcome of aneurysm rupture.Rupture of the right and non coronary sinuses typically results in communication between the aorta and the right ventricular outflow tract or the aorta and the right atrium.Symptoms include substernal chest pain, abdominal pain and mild to severe dyspnea.In many cases, patients may experience symptoms of acute heart failure, cardiac tamponade, hemodynamic instability and even cardiac death [4].
Diagnosis is confirmed by transesophageal echocardiography or catheterization [5].[6,7,8].Aortic root abscess is the most severe sequela of infective endocarditis and its surgical management is a complicated procedure because of the high risk of morbidity and death [9].SOVA with aortic root abscess has rarely been reported.

Case Report
A 40 year old normotensive and normoglycemic women presented in emergency department of a tertiary care cardiac hospital in Rawalpindi, Pakistan on May 23rd 2017.She complained of shortness of breath, fever and pain in right hypochondrium for last 15 days.Her past history was insignificant.On clinical examination, she was anemic, febrile and looked lethargic.She had a temperature of 101 °F, pulse rate of 101/minute and respiratory rate of 30/minute.Her jugular venous pressure was raised with prominent right sided facial veins.A loud continuous sawing murmur was heard throughout the first and second heart sounds.ECG showed sinus tachycardia.Arterial blood gas analysis revealed oxygen desaturation.Laboratory investigations showed Haemoglobin (Hb) 7.1g/dl, White Cell Count (WBCs) count of 16.1x 10 9 /l with normal hepatic and renal profile.Mantoux test, Antinucleur Antibody and VDRL were negative.X-rays chest showed markedly dilated right vena caval shadow.Initial blood culture did not yield any growth.Transthoracic and Transesophageal echocardiography showed aneurysmal tissue of aortic root with small multiple vegetations 2-3mm.Intravenous antibiotics for culture negative endocarditis were started which included intravenous inj.Ceftriaxone 1g every 12 hourly, inj Vacomycin 500mg every 12 hourly and inj.Levofloxacin 500 mg once daily.At operation, there was rupture of SOVA with small aortic root abscess at its base.The aortic root abscess was excised and necrotic tissue was removed.Reconstruction of aortic root was done with bovine pericardial patch.

Discussion
Sinus of Valsalva Aneurysm (SOVA) is a rare disorder.It is usually congenital, but other origins have been described.It may be asymptomatic or it may present as angina or with symptoms of valvular insufficiency or outflow obstruction [5].It can become complicated with rupture or infection [6][7][8]10].
Once ruptured, SOVA often leads to hemodynamic instability.Transesophageal echocardiography or catheterization is required to confirm the diagnosis [5].
Infective endocarditis has been reported in patients with ruptured SOVA.Both bacterial endocarditis leading to rupture of sinus of Valsalva and sinus of Valsalva rupture leading to infective endocarditis has been cited in literature [6,7].However, SOVA with aortic root abscess has rarely been reported.In our patient, there was ruptured sinus of Valsalva aneurysm and a small aortic root abscess with small multiple vegetations 2-3 mm in size.The prognosis of infective endocarditis depends upon whether the infected valve is native or prosthetic [14,15,16].The type & virulence of the microorganism and resistance of the host are important in determining the fate of IE; it's complications like paravalvular abscess, cardiac fistulas and severe destruction of the native aortic valve.A mechanical valve is often infected in its sewing ring and the infection also extends into surrounding structures [9].The microbiology of the IE also depends on the nature of valve i.e. native or prosthetic.Native-valve endocarditis causing perianular abscess involves aortic valve more often than mitral valve [17].
An uncontrolled aortic root abscess is often complicated by a burrowing abscess, a cardiac fistula or a rupture into a cardiac chamber, a pseudoaneurysm, or an arrhythmia that can lead to hemodynamic instability.Early and extensive surgical resection of aortic root abscess followed by its reconstruction can be life saving as antibiotics alone are usually ineffective to stop the destruction of cardiac tissue by the abscess [9].
In our study, the presence of sepsis, multiorgan failure & coagulopathy lead to the fatal outcome of the case.A large, multicenter, international study showed that 22% of patients with aortic valve IE had perianular abscess which was more frequent in prosthetic valves than with native valves (40% vs 19%) [18].SOVA complicated with endocarditis or sepsis has 4-5 times greater risk of perioperative death [13].The operative mortality rate for the surgical treatment of aortic root abscess varies from 3.9% to 25% [18,19].Early intervention for IE with extended sequelae is known to have better outcome.Surgeons therefore opt for an early operation if life-threatening sequelae do not develop [20].Most urgent surgical series have high mortality rates, ranging from 55% to 77% [14,21].

Conclusion
Although rupture of sinus of Valsalva is a rare condition in young to middle aged patients.It can be life threatening condition due to hemodynamic instability and can lead to acute heart failure or sudden death.Bacterial endocarditis and aortic root abscess can complete SOVA which should be confirmed by transesophageal echocardiography or cardiac catheterization.Urgent Surgical repair and appropriate antibiotic therapy should be considered to decrease morbidity& mortality due to the disease and its complications.

Figure 1 :
Figure 1: Transoesophageal echocardiography at mid esophageal position at 135 degree left ventricle long axis view showing the colour M mode cut section at aortic sinuses level shows an abnormal aliasing at 43.9 cm/sec aliasing velocity, below the right sinus of Valsalva into RV.

Figure 2 :
Figure 2: Transoesophageal echocardiography at mid esophageal position at 135 degree left ventricle long axis view showing the 2D colour imaging modality with clear flow from right sinus of Valsalva in to RV.

Figure 3 :
Figure 3: Transoesophageal echocardiography at mid esophageal position at 105 degree left ventricle long axis view showing 2D image of clear rupture right sinus of Valsalva.

Figure 4 :
Figure 4: Transoesophageal echocardiography at mid esophageal position at 105 degree left ventricle long axis view.The 2D n colour Doppler image shows clear flow from rupture right sinus of valsalv into RV and small mobile vegetation attached at right coronary cusp on the aortic side measuring 6 mm.

Figure 5 :
Figure 5: Transoesophageal echocardiography at mid esophageal position at 105 degree left ventricle long axis view.The 2D n colour Doppler image shows clear flow from rupture right sinus of valsalv into RV and small mobile vegetation attached at right coronary cusp on the aortic side measuring 6 mm.