Case Report. A 47-year-old male presented with a history of sudden-onset chest pain followed by progressively increasing dyspnea worsening to New York Heart Association (NYHA) Class IV for 15 days. He had previously undergone a coronary artery bypass graft (CABG) surgery for triple vessel disease 2 years ago and had a negative stress exercise echocardiogram performed 2 weeks prior to presentation. He had no risk factors except for heavy smoking. Physical examination revealed signs of florid congestive heart failure and a grade III/VI continuous murmur at the lower-left sternal edge. Chest roentgenogram showed cardiomegaly with increased pulmonary vascular markings. The electrocardiogram was unremarkable. An echocardiographic study revealed all four chambers to be mildly enlarged, a normal left ventricular ejection fraction, dilated inferior vena cava and congested liver. There was a saccular aneurysm of the noncoronary aortic sinus opening into the right atrium near the septal tricuspid leaflet with a high-velocity continuous Doppler flow consistent with a ruptured SVA. There was no aortic regurgitation (AR), tricuspid regurgitation (TR) or ventricular septal defect (VSD). On admission, routine blood investigations were normal except for very high liver enzymes and INR. After 3 days of stabilization with diuretics and inotropes, the patient was taken to the catheterization laboratory for transcatheter closure of the ruptured SVA.
Procedure. After receiving informed consent from the patient and arranging for a surgeon to be on standby, the procedure was performed under general anesthesia and transesophageal echocardiographic (TEE) guidance. The right and left femoral arteries and the right femoral vein were accessed using 6 Fr introducer sheaths. Intravenous heparin for anticoagulation and cefazolin for endocarditis prophylaxis were administered. The right heart pressures were elevated (right atrium mean 18 mmHg, right ventricle 40 x 16 mmHg, and pulmonary artery 40 x 20 mmgHg, mean 30 mmHg). The patient’s aortic pressure was 85 x 30 mmHg. An aortic root angiogram done in the LAO cranial projection with a 6 Fr pigtail catheter revealed torrential left-to-right shunt from a ruptured noncoronary SVA into the right atrium (RA) (Figure 1). The tract was about 9 mm long and measured approximately 7 mm at its aortic end and 6 mm at the RA end. Since the SVA arose from the noncoronary sinus, it was far removed from the origjns of the right and left coronary arteries. There was no AR. The same was confirmed by online TEE as well. The ruptured SVA was crossed from the arterial side using a 4 Fr Judkins right coronary catheter and a 0.035 inch angled-tip glidewire (Radiofocus guidewire M, Terumo Corp., Tokyo, Japan) which was passed through the RA into the inferior vena cava. The glidewire was exchanged for a 300 cm long, soft 0.035 inch J-tipped Noodle wire (AGA Medical, Golden Valley, Minnesota). This wire was snared with a 15 mm Goosneck snare (Microvena, Minnesota) and exteriorized out of the right femoral vein, providing a stable arteriovenous loop and allowing a 7 Fr long delivery sheath (AGA Medical) to be advanced from the right femoral vein to the RA and positioned into the ascending aorta across the ruptured SVA. The 14 x 12 mm ADO (AGA Medical), with its attached delivery cable, was inserted through the delivery sheath. The aortic disk was deployed in the ascending aorta, and the whole assembly was pulled back so that the disk completely blocked the aortic end of the SVA. After confirming precise placement on TEE, the rest of the device was deployed on the RA side. After ensuring absence of any AR or TR on TEE, the device was released from the delivery cable. Post-release angiography (Figure 2) revealed complete closure of the ruptured SVA and no AR. This result was confirmed on TEE as well. The patient’s congestive heart failure symptoms abated dramatically, the continuous murmur disappeared and the patient was discharged 4 days after the procedure on aspirin 150 mg daily. At 2-year follow up, the patient is asymptomatic and doing well. Repeat 2-dimensional and color Doppler echocardiography on the day following the procedure and at 1 year after the procedure (Figure 3) demonstrated good position of the device, no residual shunt and no AR or TR.
Discussion. Sinus of Valsalva aneurysms are rare congenital anomalies that are more prevalent in Asian countries than in the West.2 Though acquired aneurysms of the aortic sinus have been described postoperatively9 as also due to atherosclerosis,10 the typical saccular shape and location of the aneurysm in our patient suggested a congenital etiology. Acquired aneurysms tend to be more diffuse, involving more of the sinus or multiple sinuses and often the ascending aorta.3 An unruptured SVA was probably missed in our patient during admission for the previous CABG surgery. The natural history of SVA, as regards rupture, is not well known. It typically presents in the third or fourth decade, though rupture has been diagnosed across the age spectrum, including in infancy.2,11,12 Upon rupture, there is sudden onset of aortocardiac shunting resulting in dyspnea, chest pain, bounding pulses and a continuous murmur. Due to biventricular increases in volume as a result of systemic pulmonary shunting, patients develop congestive heart failure, as in the present case. Approximately 5–10% of patients present with acute endocarditis.3 In the present case, there was no such precipitating cause for the sudden rupture. Conventionally, surgical repair of the RSVA has been the mainstay of therapy. In the present case, surgery would have carried a higher than usual risk on account of previous midsternotomy for CABG surgery, congestive heart failure and the deranged coagulation parameters due to congestive hepatomegaly. Hence, a decision was made to carry out transcatheter closure with the patient’s informed consent. The situation was similar to performing transcatheter closure of acute postinfarction ventricular septal defect.
Transcatheter closure of ruptured SVA has been previously performed.4–8 It was first described by Cullen et al in 19944 using a Rashkind umbrella device in a patient with a recurrence after prior surgical repair of ruptured SVA. As in our case, it was done to avoid a repeat sternotomy. We chose to use an ADO to close the ruptured SVA since the shunt was large, and among the available devices, the shape of the ADO was best suited to close this saccular aneurysm. Unlike Fedson et al,7 we preferred to deploy the device at the origin of the SVA in the aorta and not at the site of rupture. Online TEE guidance helped to rule out any interference with the movement of aortic valve leaflets by the retention disk of the ADO. Moreover, deploying the device at the site of rupture (RA end) could have deformed (mushroomed) the retention disk in the narrow tract. It is prudent to close all fistulae at the arterial end to avoid recurrence, since closure at the venous end (exit point) could leave behind a weakened saccular tract exposed to arterial pressure with a potential to rupture at another site.
In summary, ruptured SVAs can be treated safely and effectively with percutaneous transcatheter closure to avoid sternotomy and cardiopulmonary bypass in the hemodynamically unstable patient.
Acknowledgements. We would like to acknowledge the support of our echocardiography technician, Ms. Meena Santosh, for retrieving follow-up echocardiograms, and Dr. Narendra Garach, chief of anesthesia for providing smooth anesthesia.
- Cheng TO. Nonsurgical closure of rupture of aneurysm of sinus of Valsalva. Catheter Cardiovasc Interv 2003;58:412.
- Chu SH, Hung CR, How SS, et al. Ruptured aneurysms of the sinus of Valsalva in Oriental patients. J Thorac Cardiovasc Surg 1990;99:288–298.
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- Cullen S, Vogel M, Deanfield JE, Redington AN. Rupture of aneurysm of the right sinus of Valsalva into the right ventricular outflow tract: Treatment with Amplatzer atrial septal occluder. Circulation 2002;105:1–2.
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