Abstract
Aneurysms of the sinus of Valsalva are rare, with dissecting aneurysms of the sinus of Valsalva that extend into the interventricular septum being even more rare. This report describes a young patient with syphilis and a quadricuspid aortic valve who experienced a spontaneously dissecting aneurysm of the sinus of Valsalva and the basal interventricular septum.
Keywords Dissecting aneurysm, Sinus of Valsalva, Interventricular septum, Tertiary syphilis
A 27-year-old man presented to the emergency room after 3 days of chest and abdominal pain and vomiting. Six months earlier, he began experiencing occasional chest pains and became fatigued, with progressive worsening in the previous 2 months. One month before presentation, he was admitted to another hospital and diagnosed with pleural effusion and syphilis. Chest drainage was successful, and the patient recovered. After discharge, he was prescribed benzathine penicillin G infusion for 20 days. Initial physical examination in the emergency room of our hospital showed that his blood pressure was 116/38 mmHg, his pulse was 80 beats/min with arrhythmia, and he showed significant cardiac enlargement, although no murmur was heard in the five valve auscultation areas. His serum brain natriuretic peptide concentration was 4950 ng/L (reference range: 0–88 ng/L) and his troponin concentration was slightly elevated. Screening for syphilis showed a 1:16 titer on a toluidine red unheated serum test and a positive result on a T. pallidum passive particle agglutination assay. Electrocardiography revealed a premature ventricular beat.
Transthoracic echocardiography showed a large aneurysm of the sinus of Valsalva located on the left side of the aortic root and close to the right ventricle. The aneurysm wall dissected and extended into the basal segment of the interventricular septum (Figure A and B, movie clip S1). Color Doppler flow imaging showed low-speed shunting through the perforation from the sinus of Valsalva into the dissecting aneurysm (Figure C). Three-dimensional echocardiography image showed a intimal tear in the aortic root (Figure D). In addition, a quadricuspid aortic valve was observed (movie clip S2). There was severe aortic regurgitation, and no vegetation was observed on the aortic valves. Contrast-enhanced computed tomography (CT) confirmed a giant aneurysm of the left sinus of Valsalva measuring 9.5×7.5 cm (Figure E, F and G).
Subsequent open heart surgery confirmed the dissecting aneurysm of the sinus of Valsalva and the involvement of the basal interventricular septum. Aortic wall thickening with calcification and a quadricuspid aortic valve were coincidentally observed. The aortic valves were restricted and insufficient. The aortic valve, the aortic root, and the ascending aorta were replaced by a mechanical valve and artificial graft, and the right coronary artery was implanted into the artificial graft. Intraoperative transesophageal echocardiography showed that the mechanical aortic valve worked well. The patient’s postoperative course was uneventful and he was discharged in good condition. Histopathologic analysis of the aorta revealed focal thickening of the arterial intima with fibrous tissue hyperplasia, multiple focal destruction of the medial structure with chronic infiltration of inflammatory cells, and scattered lymphoid follicles in the peripheral membrane with small vascular hyperplasia (Figures H and I). These results were consistent with meso-aortitis syphilitica. Follow-up after 3 months showed that the patient was asymptomatic.