Sinus of Valsalva aneurysm (SVA) is an uncommon cardiac abnormality and can be serious complication of infection, trauma, or after cardiac surgery or procedure. Clinical features and symptoms are inextricably linked to aneurysm progression and the site of rupture.
We present two rare cases of ruptured SVA both associated with infective endocarditis (IE): one is rupturing into left ventricle (LV) and another is dissecting into interventricular septum (IVS).
This case highlights expanding clinical view of SVA complicated by IE using echocardiography and hemodynamic assessment to guide surgical planning.
Sinus of Valsalva aneurysm (SVA) is a cardiac structural disorder that refers to dilation of an aortic sinus which located between the aortic valve annulus and the sinotubular junction. 1 It can be acquired or congenital. SVA complicated by infective endocarditis (IE) is extremely rare because in most cases SVA are congenital. Due to infection, SVA has potential for spontaneous rupture into adjacent cardiac structure depending on its location. Here, we present two rare cases of ruptured aortic sinus aneurysm both associated with IE: one is rupturing into left ventricle(LV) and another is dissecting into interventricular septum (IVS).
A 59-year-old male was transferred to our hospital presented with progressive chest tightness, dyspnea and short of breath when working. Two weeks ago he was treated in local hospital with anti-infective for suspected infective endocarditis with moderate aortic regurgitation. There was no relevant past medical history or significant family history.
Physical examination are as follows. His vital signs included a blood pressure of 125/ 84 mmHg with a pulse rate of 106 beats per minute, a respiratory rate of 16 per minute and oxygen saturation of 96% on room air. Cardiac auscultation revealed a continuous diastolic murmur best heard in the left second intercostal space radiating to the carotid arteries. Laboratory investigation showed normal blood cell count, serum electrolytes, and renal and liver function test results. Blood cultures were negative. Electrocardiogram (ECG) showed sinus tachycardia.
Transthoracic echocardiography (TTE) showed a slightly dilated left ventricle of 56 mm in end-diastolic diameter and a normal ejection fraction of 64%. A saccular left SVA (3.6*2.7*2.5cm) was observed which bulged into the left ventricular outflow tract (Figure 1A). Color-flow Doppler showed abnormal flow into the left ventricular outflow tract, suggesting a ruptured SVA (Figure 1B-C). Transesophageal echocardiography (TEE) confirmed a ruptured SVA from the left coronary sinus into the left ventricular outflow tract during systole and diastole (Figure 1D). A few small vegetations (with size about 3mm) were attached to the around of communication. An aortic regurgitating jet of large diameter in the left ventricular outflow tract was also noted (Figure 1E). There was no associated cardiac anomaly.
In view of the threat of complications by the ruptured SVA, vegetations embolism and the severe aortic regurgitation, surgery was performed. The patient underwent resection of the aneurysm, removal of vegetation and aortic valve replacement using a 25mm biological aortic prosthesis (Figure 1F). Intraoperative TEE showed the bioprosthetic valve functioning normally. At routine outpatient follow-up 4 weeks later, he was reported no further symptoms, resolution of his cardiac murmur and successful repair on repeat transthoracic echocardiography.
A 39-year-old male patient without a history of known health problems was admitted to our emergency department presented with acute onset dyspnea and short of breath for four days. He denied chest pain, palpitations, dizziness and fever.
The blood pressure, heart rate, electrocardiogram and biochemical examinations were within the normal range. Electrocardiogram and blood analysis were normal.
An urgent echocardiography revealed a saclike right SVA dissecting into the IVS (Figure 2A). There was a 6mm vegetation attached to the SVA (Figure 2B). In diastole, the dissected of IVS was filled with blood and expanded into the left ventricular out flow tract. Color Doppler showed turbulent flow at the site of communication (Figure 2C). TEE revealed multiple dissection of IVS arising from dilated right SVA (Figure 2D). It communicated with the aortic root from the anterolateral of the aortic annulus (Figure 2E). A trileaflet aortic valve was noted with severe aortic regurgitation(Figure 2F). There were no associated atrial or ventricular septal defect.
The patient subsequently received successful aortic valve replacement using 23mm mechanical valve and underwent Dacron patching. Intraoperative TEE showed the mechanical valve functioning normally. Postoperative course was uneventful and the patient was discharged in a satisfactory condition.
SVA is a rare cardiac disease that caused by weakening of the elastic lamina and muscular tissue in the aortic wall behind the sinus of Valsalva. 2,3 The great mass of the cases are congenital and secondary to connective tissue diseases such as Marfan’s syndrome or multifarious others. However, acquired cases of SVA generally due to infective endocarditis (IE), atherosclerosis, traumatic, the abuse of drugs or alcoholism, medial cystic necrosis and degenerative diseases.4 Our two patients are both associated with IE confirmed by the intraoperation and histopathology. Infection may lead to Sinus of Valsalva dilatation and aortic valve cusps deformation that subsequently to SVA rupture and IVS dissection. Progression of the disease may also increasingly predisposed to recurrent endocarditis. 5The diagnosis of endocarditis mainly depends on finding vegetations and positive blood culture. Due to the influence of blood collection time, quantity and antibiotic application, it has been reported that the positive rate of blood culture is in between 50% and 90%. In our two cases, multiple blood cultures were also negative. Positive blood cultures can help clinical selection of antibiotics, but negative results cannot easily rule out the diagnosis of endocarditis.
Most SVA originates from the right coronary sinus, followed by the non-coronary sinus, the left coronary sinus is least common.6SVA is generally asymptomatic. But enlarged coronary sinus protrude into the adjacent cardiac structure, lead to neighboring cavity obstructions, myocardial infarction or ischemia by compress of the coronary arteries, or extremely rare dissect into the IVS. Strenuous activity, emotional excitement, infection, trauma, iatrogenicity(cardiac catheterization) can induce SVA rupture. The majority of right SVA rupture into the right ventricular outflow tract (RVOT) followed by the RV and right atrium (RA). SVA dissecting into the IVS as exemplified in our case2 always originates from the right coronary sinus and is usually associated with conduction abnormalities and aortic regurgitation. According to its dissecting nature, it can cause congestive heart failure when rupture into the ventricular cavity. Less ruptured noncoronary sinus aneurysms rupture into RA, RV and pericardial cavity. Left SVA typically ruptures into the LV, left atrium (LA) or pericardial cavity is the rarest as demonstrated in our case1.
Clinical features and symptoms are inextricably linked to the aneurysm progression and the location of rupture. An unruptured aneurysm manifest generally clinically silent until it increases in size and compresses nearby structures or develops thromboembolic complications.7SVA rupture is an imperative and serious situation. It can manifest as sudden hemodynamic collapse, acute coronary syndrome, acute heart failure, cardiac tamponade and even sudden death which depends on size of the ruptured orifice, acuity of the rupture and the receiving chamber.8,9In our two cases, embolic events associated with endocarditis also should be taken seriously. Coronary angiography is the gold standard for diagnosing for SVA, however, this is not required in our case. Echocardiography is regarded as the preferred initial examination tool in general. The location, size, and relationship with adjacent structures of dissecting aneurysm, vegetations and any abnormality in the cardiac can be detected by it. Vortex flow in the dissecting cavity, aortic regurgitation, and communication between dissecting cavity and left or right ventricle can be easily showed by color Doppler. The collapse of the aneurysm during systole and blood filled in the aneurysm during diastole, continuous flow in systole and diastole is a useful finding in a ruptured SVA. These features were demonstrated by echocardiography in our two patients.
Subaortic aneurysm, ventricular septal defect, coronary arteriovenous malformation ,fistula and abscess should to be differential diagnosis from SVA. The crucial distinction is that SVA involves one or more sinus(es) of Valsalva structural anomalies in the aortic root. Echocardiographic findings of SVA are crucial for correct diagnosis and early surgical intervention is the optimum treatment for SVA to prevent further rupture and exacerbation of symptoms. According to the authors, the prognosis of the two patients we reported maybe better and the operation is simpler if operation is undertaken in the absence of endocarditis.
In conclusion, SVA though rare rupture into LV cavity and the IVS combined with infections as illustrated in our two cases, Early diagnosis of SVA and surgical intervention are crucial to patient survival. Echocardiography plays a useful role in accurate diagnosis, perioperative evaluation, and follow-up.
SVA, Sinus of Valsalva aneurysm; LV, left ventricle; IVS, interventricular septum; IE, infections endocarditis; RV, right ventricular ; ECG, Electrocardiogram; TTE, Transthoracic echocardiography; TEE, transesophageal echocardiography; RVOT, right ventricular outflow tract; RA, right atrium; LA, left atrium
Ethics approval and consent to participate
The study was approved by the Institutional Review Board at the First Affiliated Hospital of Zhejiang University (Hangzhou, China). The procedures were conducted according to the principles of the Helsinki Declaration.
Consent for publication
Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
Availability of data and materials
The authors declare that they have no competing interests.
This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.
HG wrote the manuscript. YC and YM helped to conceive the study. LX carried out the intraoperative transesophageal echocardiography. ZH and YM carried out the echocardiography. ZL revised the manuscript. All authors have read and approved the final manuscript.
This study was supported by grants from Scientific Research Fund of Zhejiang Provincial Education Department (No. Y201738814).