Abstract
In this rare case report of giant unruptured sinus of Valsalva aneurysm (SOVA), a 17-year-old male presented with sudden onset syncope due to complete heart block (CHB). An emergency evaluation was done with the help of transthoracic echocardiography, transesophageal echocardiography, and cardiac catheterization with support of temporary pacemaker. The obvious distorting effects of a giant SOVA dissecting into interventricular septum were CHB, significant regurgitation of tricuspid and mitral valve, mild regurgitation aortic valve and biventricular dysfunction. The case was treated by repair of SOVA and posterior mitral ring annuloplasty. CHB improved to sinus rhythm on 11th day after surgery. On follow-up, tricuspid valve regurgitation improved to mild regurgitation and he continued to have mild aortic regurgitation.
<Learning objective: Sinus of Valsalva aneurysm (SOVA) may present with cardiac emergency without rupture. A giant unruptured SOVA competes for space with neighboring intra cardiac structures. The distorting effects are erosion into interventricular septum, complete heart block (CHB), valvular and ventricular dysfunction. Transthoracic and transesophageal echocardiography provide enough information for emergency surgery. Sometimes, evaluation may need cardiac catheterization and computed tomography. Immediate surgery saves life. CHB may improve on follow-up.>
Keywords: Sinus of Valsalva aneurysm, Complete heart block, Temporary pacemaker, Biventricular dysfunction, Mitral ring annuloplasty
Introduction
A congenital sinus of Valsalva aneurysm (SOVA) is usually clinically silent. The presentation may vary from asymptomatic dilatation of coronary cusp detected in routine 2-dimensional echocardiography to symptomatic presentations related to the compression of adjacent structures or intracardiac shunting caused by rupture of the SOVA mostly into the right side of the heart [1]. The compressive features are tricuspid valve regurgitation, right ventricular outflow tract obstruction and rarely complete heart block (CHB). Dissection or erosion into interventricular septum is one of the rarest complications of SOVA [1]. Most of the large or giant SOVA rupture on follow-up [2]. The symptomatic presentation is almost always a surgical emergency. Sometimes catheter-based closure of ruptured SOVA also can be attempted with suitable anatomy.
Case report
A 17-year-old, apparently healthy male presented with sudden onset of syncope. There were no clinical findings of Marfan syndrome or Ehlers–Danlos syndrome. A 12-lead electrocardiogram showed complete heart block (CHB) (Fig. 1A). He was immediately supported by temporary pacing. Routine investigation reports were in the normal limit. Transthoracic (Fig. 1B,C, Video 1) and transesophageal echocardiographic (TTE, TEE) (Fig. 2A–C, Video 2) assessment revealed a giant unruptured SOVA (5.2 cm × 3 cm) of right coronary sinus eroding far into interventricular septum. The apparent protrusion into right ventricular outflow tract (RVOT) and cranial displacement of septal leaflet tricuspid valve (TV) were apparent on TTE and TEE. RVOT gradient was 40 mmHg. There was more than moderate tricuspid valve regurgitation. There was more than grade II aortic valve regurgitation due to poorly coapting right coronary cusp. Mitral valve regurgitation was moderate to severe due to relatively small left ventricular cavity that can be explained by giant aneurysm in the interventricular septum (IVS). The aortic root angiogram in left-anterior oblique 42 degree projection showed unruptured SOVA of right coronary sinus eroding far distal into IVS, with diastolic opacification (Fig. 2D, Video 3). There was no step-up of oxygen during hemodynamic study. Surgical repair of SOVA and posterior ring annuloplasty of mitral valve (Fig. 3A) were done successfully in emergency. The case was followed with temporary epicardial pacing wire. CHB improved to sinus rhythm (Fig. 3B) on the 11th post-operative day. Tricuspid regurgitation improved after surgery. There was mild residual aortic regurgitation on follow-up.
Fig. 1.
(A) 12-lead electrocardiogram showing complete heart block at presentation with syncope; (B) Transthoracic echocardiography (TTE) of the left ventricular outflow tract in parasternal long axis (PLAX) which is marked by green arrow demonstrates the unruptured right sinus of Valsalva aneurysm (SOVA); (C) TTE (parasternal short-axis, yellow arrow) shows the large SOVA bulging into the right ventricular outflow tract.
Fig. 2.
(A) Transesophageal echocardiography (TEE) demonstrates sinus of Valsalva aneurysm (SOVA) in the proximal half of interventricular septum (IVS) in zero degree with more than moderate mitral regurgitation with color Doppler interrogation; (B) TEE in 115 degree (aortic root and IVS long axis) with color Doppler interrogation shows mild aortic regurgitation (AR); (C) TEE in 115 degree (aortic root and IVS long axis) shows giant SOVA with apparent dissecting and wedging into IVS and significant right ventricular outflow tract protrusion. All the specified pathology has been marked by bold yellow colored arrows; (D) Aortic root angiogram in left-anterior oblique (LAO), 30 degree projection shows diastolic opacification of dissecting aneurysm of right coronary cusp (RCC) into IVS and temporary pacemaker lead in right ventricle.
Fig. 3.
(A) Posterior ring annuloplasty of mitral valve for more than moderate mitral valve regurgitation; (B) 12 Lead electrocardiogram shows the improvement of complete heart block to sinus rhythm on the 11th day of surgery.
Discussion
SOVA is a consequence of weakness of the elastic lamina at the junction of the aortic media and aortic annulus fibrosis in either congenital or acquired groups. It is associated with ventricular septal defect in approximately 40% of patients. The incidence of isolated SOVA is rare. It is located to right coronary sinus in 60–85%, noncoronary sinus in 10–30%, and left coronary sinus in 5% of cases with male preponderance (4:1). It accounts for 1% of congenital cardiac defect in grown-up children. The average age of presentation is 30 years and age range is 11–64 years. The one-year follow-up result with medical management is dismal in symptomatic patients with or without rupture. The rupture of SOVA is more frequent into right ventricular outflow tract (60–90%) [1]. The symptomatic aneurysmal dilatation of sinuses is rare in children [3], [4]. In general, the rough incidence is 0.09% in the general population. It may be congenital or acquired. The clinical presentation depends upon degree of compression of neighboring structures, competition for intracardiac space, rupture, and dissection into IVS. Therefore, the spectrum of presentation in the descending order of frequency includes rupture, incidental detection without rupture, infective endocarditis, thromboembolism, valvular regurgitation, CHB, and ventricular dysfunction. The associated cardiac defects sometimes include bicuspid aortic valve (9%), ventricular septal defect (34%), and aortic regurgitation (44%) [2], [5]. It is sometimes associated with RVOT obstruction or tricuspid valve regurgitation due to encroachment into ROVT and inlet of right ventricle. Unruptured aneurysm may cause distortion and obstruction in the immediate neighboring structures leading to asymptomatic to acute presentation such as severe aortic valve regurgitation, CHB, and acute coronary syndrome. The possible explanation for CHB associated with large aneurysm eroding IVS is compression of bundle of His when aneurysm of right coronary sinus grows caudally distorting or eroding into membranous part of IVS. That is why the aneurysm should be excluded from aortic root or excised carefully, to relieve the His bundle from compression which may help the CHB to improve to normal sinus rhythm. A significantly large aneurysm in the IVS may result in biventricular dysfunction and atrioventricular valve regurgitation as seen in this case. Ruptured aneurysm is usually present with acute heart failure due to significant left to right shunt. Echocardiogram is the standard imaging technique. Sometimes the anatomical and hemodynamic details need cardiac catheterization and cardiac computed tomography. Ruptured SOVA can be closed by surgery as a first option or by device as off label. The symptomatic unruptured aneurysm always needs surgery. The incidence of unruptured SOVA is 5% as reported in a large surgical series consisting of 160 cases [6]. But acute presentation without rupture that needs immediate intervention is rare. It is still rare to come across unruptured giant aneurysm with CHB, and those cases reports are anecdotal [7], [8]. Our case is unique because of the presence of the constellations of distortive effect of a giant unruptured SOVA which included CHB, both atrioventricular valve regurgitation, aortic valve regurgitation, obstruction of RVOT, and biventricular dysfunction and all of the distortions improved after surgical exclusion of SOVA from right coronary cusp and mitral ring anuloplasty.
Conflict of interest
None declared.
Footnotes
Supplementary material related to this article can be found, in the online version, at http://dx.doi.org/10.1016/j.jccase.2015.09.002.
Appendix A. Supplementary data
The following are the Supplementary data to this article:
Transthoracic echocardiography of the left ventricular outflow tract in parasternal long axis view (PLAX) demonstrates a large unruptured right sinus of Valsalva aneurysm.
Transesophageal echocardiography demonstrates sinus of Valsalva aneurysm occupying the proximal 2/3rd of interventricular septum in left ventricular long-axis view (115 degree) and color Doppler shows aortic valve regurgitation.
Aortic root angiogram in left-anterior oblique-cranial 48 degree showing obvious diastolic filling of sinus of Valsalva aneurysm far distal into interventricular septum. There is no filling of right or left ventricle suggesting no evidence of rupture consistent with no step up of saturation on hemodynamic study. The temporary pacemaker lead is seen in right ventricle via inferior vena cava.
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Associated Data
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Supplementary Materials
Transthoracic echocardiography of the left ventricular outflow tract in parasternal long axis view (PLAX) demonstrates a large unruptured right sinus of Valsalva aneurysm.
Transesophageal echocardiography demonstrates sinus of Valsalva aneurysm occupying the proximal 2/3rd of interventricular septum in left ventricular long-axis view (115 degree) and color Doppler shows aortic valve regurgitation.
Aortic root angiogram in left-anterior oblique-cranial 48 degree showing obvious diastolic filling of sinus of Valsalva aneurysm far distal into interventricular septum. There is no filling of right or left ventricle suggesting no evidence of rupture consistent with no step up of saturation on hemodynamic study. The temporary pacemaker lead is seen in right ventricle via inferior vena cava.