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.