Spontaneous Rupture of Sinus of Valsalva Aneurysm Presenting as Perivalvular Hematoma.

Acute rupture of sinus of Valsalva often presents as an acute emergency with significant hemodynamic compromise whereas contained rupture of sinus of Valsalva with a perivalvular hematoma formation is rarely seen. We describe the case of a 63-year-old male who presented with acute shortness of breath and was found to have rupture of sinus of Valsalva aneurysm (SVA) with a perivalvular hematoma and severe aortic regurgitation. We also review the presentation, diagnosis, and management of SVAs.

INTRODUCTION

Acute rupture of sinus of Valsalva aneurysm (RSVA) often presents as an acute emergency with significant hemodynamic compromise whereas contained rupture of sinus of Valsalva (SVA) with a perivalvular hematoma formation is rare. We describe the case of a 63-year-old male who presented with acute shortness of breath and was found to have RSVA with perivalvular hematoma and severe aortic regurgitation. While RSVA is well described, contained rupture of the sinus of Valsalva has been rarely reported in medical literature.[1]

CASE REPORT

A 63-year-old African American man with no medical history presented to the emergency room with sudden onset dyspnea. He denied any chest pain but reported cocaine use several days ago. Examination revealed a BP of 211/105 mmHg, fine bibasilar crackles on auscultation, and a 2/6 holosystolic murmur at the left sternal border. Chest X-ray showed pulmonary edema. He was intubated for respiratory failure and started on nitroglycerine drip for hypertensive emergency. The laboratory investigations revealed a Pro-BNP of 14,704 pg/ml and troponin of 0.153 ng/ml which peaked at 10.9 ng/ml. Electrocardiogram (EKG) revealed normal sinus rhythm with left ventricular hypertrophy with some ST-segment depressions in lateral leads. Acute coronary syndrome treatment was initiated with intravenous heparin drip. Cardiac catheterization revealed three-vessel disease and severe aortic regurgitation. Transthoracic echocardiogram revealed dilated aortic root measuring 4.8 cm with severe aortic regurgitation. There was left SVA with thickening of the intervalvular fibrosa area, suspicious for perivalvular hematoma versus aortic root abscess [Figures 1–3]. Computed tomography (CT) of the heart confirmed a left SVA with extravasation of contrast inferiorly and posteriorly. There was hypodense material around the left sinus of valsalva, likely representing hemorrhage [Figures 4–6]. He was taken to the operating room for aortic valve replacement with coronary artery bypass grafting. Based on the examination of the aorta and aortic root intraoperatively, the tissue was deemed unsuitable for replacement. A bovine patch repair was performed with the evacuation of a large amount of hematoma from the area around the left sinus of Valsalva along with surgical coronary revascularization. Examination of pathology specimen confirmed the tissue as hematoma. Postoperatively, the patient remained on ventilator requiring full respiratory support. He subsequently had a massive middle cerebral artery stroke with hemorrhagic conversion; midline shift; and mass effect during follow-up and expired after 14 days.

Figure 1

Transthoracic echo parasternal long-axis view showing hematoma at left coronary sinus

Figure 3

Color Doppler image parasternal long-axis view showing blood flow around hematoma

Figure 4

Three-dimensional reconstructed computed tomography image showing extravasation of contrast from the left sinus of Valsalva aneurysm into the hematoma around left coronary cusp

Figure 6

Sagittal oblique computed tomography image at the level of sinuses of Valsalva demonstrating contrast extravasation into the hematoma around left coronary cusp

Color Doppler image parasternal long-axis view showing aortic regurgitation

Double oblique axial computed Tomography image of the aortic root at the level of sinuses of Valsalva demonstrating contrast extravasation into the hematoma around left coronary cusp

DISCUSSION

Epidemiology, pathogenesis, and clinical features

SVA is rare with an incidence ranging from 0.09% to 1.8%.[23] SVA most commonly involves the right coronary sinus (65.6%), followed by the noncoronary sinus (28%) and the left coronary sinus (6.5%).[4] SVA can be congenital or acquired. SVA may be associated with cardiac anomalies such as ventricular septal defect (53.3%), right ventricle outflow tract stenosis (7.5%), and aortic valvular malformations (5.2%).[2] Acquired SVA is usually secondary to conditions affecting the aortic wall, degenerative diseases, or trauma.

Unruptured SVA usually remains asymptomatic and may be detected incidentally. Rarely, an unruptured aneurysm may present as a cardiac murmur, or with exertional dyspnea, palpitations, angina, myocardial infarction, arrhythmias, or thromboembolic stroke.

Right-sided SVA can rupture into the right ventricle or the right atrium, and the left-sided SVA into the left ventricle or the left atrium. Other variations include rupture into the pericardium and pulmonary trunk. Ruptured SVA symptoms include acute onset dyspnea, chest pain, and symptoms of heart failure. The natural history of asymptomatic SVAs is not well described, and there is no consensus for follow-up of asymptomatic lesions.

Imaging studies

Transthoracic echocardiogram with Doppler is the first line imaging modality. It allows imaging the extent of the aneurysmal sac, severity of the aortic valvular regurgitation and provides information about chamber dimensions, and the presence of associated anomalies. Color flow imaging can show continuous turbulent vortex in unruptured SVA, and turbulent systolic and diastolic shunt into the receiving chamber in cases of rupture. Transesophageal echocardiogram (TEE) is helpful in delineating small ruptures.[5] With multiplane transducers, three-dimensional (3D) anatomy is better appreciated which is important for planning surgical repair.[5] 3D TEE technologies in real time can track catheters and devices and is a useful guide for percutaneous closure.

With increasing use of CT scans, an SVA may be incidentally detected. The typical SVA appears as a thin-walled tubular or crescent-shaped outpouching contiguous with the aortic root, with discontinuity of its wall suggesting rupture.[6] EKG gated, contrast-enhanced multidetector-row computed tomography (MDCT) with its high image resolution can be used to specifically delineate SVA and aortic root anatomy.[7]

Cardiac magnetic resonance imaging (CMRI) can provide a comprehensive assessment of cardiac anatomy, functional assessment with bright blood imaging (steady-state free precession sequences) and flow (velocity-encoded phase contrast sequences).[8] Bright blood images reviewed as cine loops enable dynamic assessment of SVA morphology including its impact on related valves and outflow tracts. CMRI is particularly useful when there are associated cardiac anomalies. The advantage of CMR over MDCT lies in its ability to provide physiological and flow information, making it more useful in cases of SVA rupture.

Ruptured SVA can be treated either through surgical approach or percutaneous transcatheter closure devices. Surgical closure is preferred in cases with worse aortic regurgitation and more complex-associated lesions and multiple site of rupture.[9]

Percutaneous approach is typically used in patients with mild or no aortic regurgitation, simple associated defects (muscular ventricular septal defects, secundum atrial septal defect, small patent ductus arteriosus), or in patients who are at prohibitively high surgical risk due to comorbidities.[9] Repair is performed by either primary suture repair or patch repair. Aortic valve replacement or repair may be needed in cases with aortic root or valvular abnormalities.[910] The early perioperative mortality for surgical closure is low, at 1.5%.[9] Life expectancy after successful term survival after surgical closure reflects that of the general population.[10] Long-term survival data after percutaneous closure are not available although the rate of reoperation has been found to be 2.9%.[9]

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Conflicts of interest

There are no conflicts of interest.