Ruptured Sinus of Valsalva Aneurysm: An Old Picture in the Modern Era

Case Presentation. A 48-year-old male with severe mental retardation, non-verbal and able to follow simple commands, presented to his primary care physician with one and a half months of progressive shortness of breath and lower-extremity edema. Examination revealed a loud, “machinery-like” continuous murmur which was best heard at the base of the heart. A transthoracic echocardiogram (TTE), which was technically difficult, revealed severe tricuspid regurgitation with septal leaf prolapse, mild aortic incompetence (AI) and moderate-to-severe pulmonary hypertension (56 mmHg). Left and right ventricular (RV) size and function were reported as normal. He went on to have a transesophageal echocardiogram (TEE) 11 days later due to ongoing symptoms despite medical therapy. The TEE showed a sinus of Valsalva aneurysm (SVA) with perforation into the RV and right atrium (RA) just behind the septal leaflet of the tricuspid valve and a significant left-to-right shunt. The RV was moderately dilated at this stage, but with normal function. The patient was then referred to our institution for surgical evaluation. He underwent cardiac catheterization, which included right-heart catheterization, a shunt “oximetry run” and aortic-root angiography. The shunt run demonstrated a severe step-up in oxygenation of the RV and the RA, with a calculated Qp:Qs ratio of 3.2:1. There was severe pulmonary hypertension, with a measured pulmonary pressure of 66 mmHg. Aortic-root angiography in a LAO cranial position (Figure 1) clearly showed severe reflux of contrast from the right coronary sinus into the RV and RA, as well as mild AI. The patient was then transferred to radiology where a 64 multidetector computed tomography (MDCT) scan was performed in order to more accurately demonstrate which chambers were involved, namely whether the rupture involved only the RV or included the RA. The MDCT images (Figure 1) clearly demonstrated rupture into the RV and not the RA, as opposed to the angiographic images, which appeared to involve the RA — most likely an element of tricuspid regurgitation of the contrast, and not a direct connection between the coronary sinus and the RA. The patient was sent for surgery the following day where a pericardial patch was applied to the fistula and a subpulmonic ventricular septal defect (VSD) was repaired, thereby confirming MDCT as being superior to both angiography and echocardiography in showing the true anatomy of this ruptured SVA. SVAs are rare (0.1–3.5% of all congenital heart defects) and usually clinically silent, diagnosed in the setting of a rupture. Most originate from the right coronary sinus,¹ with the right ventricle being the most common receiving chamber (80­–90%). VSD frequently coexists and is a predisposing factor for AI. Echocardiography (TTE and TEE) and cardiac catheterization are the mainstay modalities of diagnosis,² with magnetic resonance imaging and helical CT only now gaining favor as additional diagnostic tools to better visualize regional anatomy. Surgical intervention remains the primary treatment modality,³ with percutaneous catheter closure being a newer alternative. Surgical repair is highly successful, with life expectancy approximating that of the normal population (10-year survival 90–95%). The risk of a recurrent fistula or VSD is minimal.

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