Nonsurgical Closure of Recurrent Rupture of Sinus of Valsalva Aneurysm in the Presence of Aortic Prosthesis

From the Department of cardiology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, India. The authors report no conflicts of interest regarding the content herein. Manuscript submitted September 2, 2008, provisional acceptance given Novemer 3, 2008, and final version accepted November 10, 2008. Sasidharan Bijulal, MD, DM, Assistant Professor of Cardiology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, India 695011. E-mail bijulalsasidharan@gmail.com

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J INVASIVE CARDIOL 2009;21:E42–E43
Aneurysm of the sinus of Valsalva is a rare congenital abnormality produced by weakness of tissues at the junction of the aortic media with the annulus fibrosus. Typical associations with this condition are ventricular septal defect and aortic valve regurgitation.^1,2 The treatment option used to be surgical closure,^3–6 however, there has been a growing number of reports of nonsurgical closure using a ductal occluder.^7–9 Recurrent rupture of a sinus of Valsalva aneurysm after surgical treatment has been reported in patients who previously underwent re-surgery.^10–12 However, re-surgery can be associated with technical difficulties and added risk related to scarring and adhesion from the previous surgery. We describe the nonsurgical closure of a recurrent sinus of Valsalva aneurysm rupture in a middle-aged male patient. Case Report. A 47-year-old male was evaluated for exertional breathlessness of 3 months’ duration. Eleven years previously he had undergone surgical closure of a doubly-committed ventricular septal defect with associated significant aortic regurgitation and an aneurysm of the right sinus of Valsalva, which ruptured into the right ventricular outflow tract (RVOT). He underwent patch closure of the septal defect, aortic valve replacement using a no. 28 Starr-Edwards prosthesis and patch closure of the ruptured sinus of Valsalva aneurysm. He remained symptom-free until 3 months ago. Clinical examination revealed a continuous murmur over the left sternal border and features suggestive of congestive heart failure. Echocardiography showed recurrence of the sinus of Valsalva aneurysm rupture arising from the right sinus and opening into the RVOT immediately proximal to the pulmonary valve. The right ventricular opening measured approximately 7 mm. The aortic opening was noted at the proximal edge of the patch and was away from the right coronary artery (RCA) and the aortic prosthesis. There were no vegetations and the prosthetic valve’s function was normal. The patient had no history of prolonged fever and his blood cultures were sterile. Cardiac catheterization showed an elevated filling pressure in all four chambers, severe pulmonary artery hypertension (mean pressure, 58 mmHg) and a 2:1 left-to-right shunt. Considering the added surgical risk, the amenability for nonsurgical closure and our institution’s experience with nonsurgical closure, we decided to close the defect nonsurgically. The procedure was performed under transthoracic echocardiographic guidance. Transesophageal echocardiography (TEE) performed before the procedure was not useful in profiling the defect because of its anterior location and the interference from the aortic prosthesis. Aspirin and clopidogrel were started 2 days before the procedure. Unfractionated heparin was administered and therapeutic anticoagulant levels were achieved during the procedure. Angiography in several projections showed a cul-de-sac and a 10 mm defect at the right ventricular opening of the sac (Figure 1). The aortic end of the defect measured 16 mm. The defect was crossed from the arterial side using a hydrophilic guidewire and a right coronary catheter, and subsequently, an Amplatzer extra-support exchange length wire (AGA Medical Corp., Plymouth, Minnesota) was snared from the inferior vena cava and an arteriovenous loop was created. A 9 Fr long Mullins sheath (Cook, Inc., Bloomington) was tracked over the extra-support wire from the venous side across the defect in the sinus into the ascending aorta. Next, a 16–14 mm Blockaid ductal occluder (Shanghai Shape Memory Alloy Material Co. Ltd., China) was placed in the sac across the defect under angiographic and echocardiographic guidance. An extra-support guidewire was kept inside the delivery sheath during device advancement to facilitate its advancement across the right ventricle and to prevent kinking of the delivery sheath. Optimal positioning of the device was achieved without compromising the prosthetic valve’s function and there was no residual flow (Figure 2). The aortic end of the device was well away from the RCA. The pulmonary artery mean pressure was 31 mmHg after the procedure. The post-procedure period was uneventful. Serial electrocardiography showed no changes suggestive of coronary ischemia and the patient’s cardiac enzymes were normal. He was treated with heparin along with warfarin until low-intensity anticoagulation (international normalized ratio between 2.0–2.5) was achieved and he was subsequently discharged on warfarin and dual-antiplatelet therapy consisting of 150 mg of aspirin and 75 mg of clopidogrel daily. Discussion. Due to the growing experience worldwide, the majority of congenital and acquired heart diseases can now be nonsurgically treated. This case is unique in that nonsurgical treatment for a recurrent rupture of the aortic sinus in the presence of an aortic prosthesis had not previously been reported. The reason for recurrent rupture in this patient could be either due to progression of the aortic pathology with continuing dilatation of the aortic sinus, or to patch dehiscence from hemodynamic stress. The patient had no history of bacterial endocarditis. The presence of a prosthetic valve very close to the defect is a point of concern when planning percutaneous intervention in these types of cases, but echocardiography and fluoroscopy are extremely useful in assessing optimal device positioning. In cases such as this, where the defect is anteriorly located, TEE may not be as useful as in those involving a noncoronary sinus related to shadowing produced by the prosthetic valve. Nonsurgical treatment of a sinus of Valsalva aneurysm arising from the right sinus is more technically challenging than a defect arising from a noncoronary sinus (which usually ruptures into the right atrium) because of the necessity to negotiate from the right atria into the right ventricle and then into the aorta with a large-sized long delivery sheath. The support provided by an extra-support exchange wire is useful in preventing kinking of the sheath in these situations. Alternatively, kink-resistant sheaths can be used, but they offer less maneuverability. In view of the patient’s anatomy and our experience, we decided to deploy a ductal occluder, which resembled an arterial duct. A muscular ventricular septal defect occluder was not necessary in this situation, as the pulmonary artery pressure would drop to a near-normal level once the shunt was occluded. Optimal medical management after device placement to prevent thrombus formation over the device has yet to be determined. Experience with device closure of atrial septal defects established the importance of antiplatelet therapy after implantation of an Amplatzer septal occluder. The same is applicable in this situation as well until endothelialization of the device is completed, as thrombus can embolize into the cerebral circulation. The presence of a mechanical prosthesis requires the use of oral anticoagulants. The safety of combining dual-antiplatelet therapy with vitamin K antagonists has been well documented.¹³Conclusion. Percutaneous closure can be performed successfully and safely in a recurrent rupture of sinus of Valsalva aneurysm, and morbidity and added risk of re-surgery can be avoided. Note: The Blockaid ductal occluder is a replica of the Amplatzer ductal occluder. It is made of nitinol with a Dacron patch inside and is similar to the Amplatzer ductal occluder in shape, character and deployment technique and has excellent clotting characteristics. Further details can be obtained from www.shsma.com.

References

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