Rupture of sinus of valsalva aneurysm into left atrium in a patient who is a candidate for hepatic transplantation
Veysel Tosun1, Necmettin Korucuk2, Unal Guntekin3 1 Department of Cardiology, Sanliurfa Education and Research Hospital, Sanliurfa, Turkey 2 Department of Cardiology, Medical Park Hospital, Antalya, Turkey 3 Department of Cardiology, Faculty of Medicine, Akdeniz University, Antalya, Turkey
Date of Web Publication
Correspondence Address: Veysel Tosun Department of Cardiology, Sanliurfa Education and Research Hospital, Yenice Boulevard, Eyyubiye, Sanliurfa Turkey
Source of Support: None, Conflict of Interest: None
Sinus of valsalva aneurysm (SVA) is a rare cardiac disease. The most common complication of SVA is rupture into the right atrium or right ventricle. Rupture into the left chambers is very rare. Patients with ruptured SVA are likely to die of heart failure or endocarditis. We present a 29-year-old man who was hospitalized for hepatic transplantation with rupture of SVA. Transthoracic echocardiography and transesophageal echocardiography showed rupture of a noncoronary SVA into the left atrium. Mitral valve infective endocarditis developed and surgery was planned for the patient, but the patient died due to multiple organ dysfunction syndrome.
Keywords: Hepatic transplantation, left atrium, noncoronary sinus, sinus of valsalva aneurysm
How to cite this article: Tosun V, Korucuk N, Guntekin U. Rupture of sinus of valsalva aneurysm into left atrium in a patient who is a candidate for hepatic transplantation. J Cardiovasc Echography 2018;28:130-2
How to cite this URL: Tosun V, Korucuk N, Guntekin U. Rupture of sinus of valsalva aneurysm into left atrium in a patient who is a candidate for hepatic transplantation. J Cardiovasc Echography [serial online] 2018 [cited 2021 Oct 26];28:130-2. Available from: https://www.jcecho.org/text.asp?2018/28/2/130/232570
Sinus of valsalva aneurysm (SVA) is a rare cardiac anomaly. SVA is usually asymptomatic but may lead to symptomatic presentation related to compression of adjacent structures or intracardiac shunt caused by rupture. Rupture of dilated sinus causes intracardiac shunt toward the right atrium, right ventricle, or other cardiac chambers.
Noncoronary SVA rupture cases which are opened to the right or left cardiac chambers are very rare in the literature and generally rupture into the right atrium. We present a very unusual case of noncoronary SVA rupture into the left atrium during the evaluation of operation preparations before hepatic transplantation.
A 29-year-old male patient who underwent hepatic transplantation in 2009 due to autoimmune hepatitis was consulted while hospitalized in the gastroenterology clinic due to hepatic rejection. The patient was considered for hepatic transplantation again. Minimal shortness of breath and orthopnea were available on physical examination. Vital signs were within normal limits. A loud, superficial, systodiastolic murmur in all foci and a palpable thrill along the right and left lower parasternal border were detected. Hepatic failure findings such as edema and ascites were seen in patients. Other laboratory findings except hypoalbuminemia (albumin: 2.6 g/dL), thrombocytopenia (platelets: 56,000/mm3), and hyperbilirubinemia (total bilirubin: 15.6 mg/dL, direct bilirubin: 11.2 mg/dL) were within normal limits. A transthoracic (TTE) and transesophageal echocardiography (TEE) showed a ruptured aneurysm originated from the noncoronary sinus of valsalva. Color Doppler imaging showed turbulent flow from the aortic aneurysm toward the left atrium next to the anterior leaflet of mitral valve [Figure 1] and [Videos 1], [Videos 2]. Mild aortic and moderate mitral regurgitation were observed. Continuous wave Doppler demonstrated a continuous high-velocity jet (4.0 m/s) along the atrial surface of anterior mitral leaflet. The patient was evaluated at the cardiology and cardiovascular surgeon's council, and surgical operation was recommended for the patient, but he refused surgery. Because of the necessity of cardiac operation, hepatic transplant preparations were delayed and the patient was discharged 1 week after hospitalization. Three months later, he was admitted to the gastroenterology clinic with elevated hepatic enzymes (peak value of alanine aminotransferase was 1895 U/L and peak value of aspartate aminotransferase was 2356 U/L), ascites and diffuse edema, and a fever of 39°C. In addition, laboratory values showed an increase in the infection parameters (peak value of C-reactive protein was 89 mg/dL and peak value of white blood cell was 33,000/mm3) and a decrease in the complement values (C3: 6.6 mg/dL and C4: 18 mg/dL). Four blood cultures taken from two peripheral brachial veins yielded the viridans group streptococcus. A bedside portable echocardiography was performed in the Intensive Care Unit and showed two gross vegetations on the atrial surface of anterior mitral leaflet and posterior mitral leaflet. Ampicillin (200 mg/kg/day in 4 doses) and gentamicin (3 mg/kg/day in 1 dose) were initiated intravenously for infective endocarditis immediately. In the following days, septic shock developed in the patient and he died due to multiple organ dysfunction syndrome.
Figure 1: (a) A ruptured noncoronary sinus aneurysm protruded into the left atrial cavity by transthoracic echocardiography. (b) Continuous turbulent flow from the noncoronary sinus aneurysm toward the left atrium by color Doppler transthoracic echocardiography. (c) A ruptured noncoronary sinus aneurysm protruded into the left atrial cavity by transesophageal echocardiography. (d) Continuous turbulent flow from the noncoronary sinus aneurysm toward the left atrium by color Doppler transesophageal echocardiography
SVA can be congenital or acquired. A congenital SVA has usually a silent clinic but may vary from a mild, asymptomatic dilatation detected in routine TTE to symptomatic presentation related to the compression of adjacent structures or intracardiac shunts. Approximately 61% of SVA originate from the right sinus of valsalva, while SVAs originating from noncoronary (26%) and left sinuses (12%) are exceedingly rare. In this actual review, rupture or fistula from the noncoronary sinus to the left atrium was extremely rare (1.5%).
A deficiency of normal elastic tissue and abnormal development of the bulbus cordis have been associated with the development of SVA. Other disease processes that involve the aortic root (e.g., atherosclerotic aneurysms, syphilis, endocarditis, cystic medial necrosis, and chest trauma) may also produce SVA, although these usually involve multiple sinuses.
SVA rupture may lead to intracardiac shunts. Physiopathological consequences of SVA rupture depend on the volume of flow and the cardiac chamber with which it communicates. The perforation of acutely high flow does not allow for hemodynamic compensation and causes the development of a sudden cardiac insufficiency. A small, gradual, and progressive perforation can be tolerated temporarily. In our case, hepatic failure due to rejection was present and the patient was receiving hepatic cirrhosis treatment, including diuretic treatment. The development of pulmonary edema may be prevented or delayed due to treatment.
TTE is a prominent tool to detect as many as 75% of all patients with SVA. Color Doppler imaging technique identifies easily an SVA rupture. TEE provides information regarding SVA and allows precise identification of structural anomalies and shunt locations for perioperative assessment., Cardiac catheterization is considered the gold standard, but not necessary in most cases. Magnetic resonance imaging and computed tomography have a high diagnostic power. Cardiac catheterization was considered for our patient, but he refused.
Urgent surgical intervention is recommended in all patients with SVA rupture. An unruptured but symptomatic or enlarging SVA should also be considered for surgical repair with an operative mortality rate of <2%., Perioperative medical management consists of relieving heart failure symptoms, treating arrhythmia if present, and treating endocarditis if present. Surgery was planned for our patient, but he had refused the operation. After 3 months, he had admitted to the hospital with fever due to infective endocarditis. He died due to septic shock and multiple organ failure following days.
In the 47 years Mayo Clinic experience, endocarditis might be the initial presenting finding in 6% of patients and might be a complication of SVA. Endocarditis was also reported as an uncommon postoperative complication in that overview. In our case, there was no doubt in favor of infective endocarditis at initial evaluation. After a 3-month period, infective endocarditis developed as a complication of SVA and the patient died before surgery.
In this case, we identified a patient with rupture of a noncoronary SVA into the LA and infective endocarditis with gross vegetation on the atrial surface of anterior and posterior mitral leaflet by TTE and TEE. Accelerating the operation period in such patients is particularly important to reduce the complications of SVA.
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The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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