The Immediate and Follow-up Results of Transcatheter Occlusion of the Ruptured Sinus of Valsalva Aneurysm With Duct Occluder

Abstract: Background. Percutaneous transcatheter closure is an alternative strategy to traditional conventional surgical repair for ruptured sinus of Valsalva aneurysm (RSVA). The immediate and follow-up results of transcatheter occlusion in 13 patients were reported. Methods. From February 2004 to June 2009, a total of 13 patients (9 males, 4 females), ages 18-38 years, were involved in the report. The diagnosis of RSVA was made based on a combination of several imaging modalities. None of the patients had other associated congenital heart disease, and all underwent local anesthesia. Transthoracic echocardiography was used during the procedure. All patients received aspirin (100 mg/day) and clopidogrel (75 mg/day) for a 6-month period after the procedure. Enalapril (5-20 mg/day) was administered to the patients with heart failure and/or cardiac dilatation. Chest radiography, electrocardiogram, and transthoracic echocardiography were undertaken at intervals of 1, 6, 12, 24, 36, 48, and 60 months during the follow-up. Results. The size of the duct occluder selected was up to 1-3 mm larger than the maximal diameter of the RSVA opening site. The devices were successfully deployed without any complications. On follow-up, no severe arrhythmia occurred; there was no device embolization, residual shunt, RVOT obstruction, new aortic regurgitation, or rupture site. Compared with the preoperative results, the cardiothoracic ratio and left ventricular were significantly decreased in the patients with cardiac dilatation (0.54 ± 0.05 vs 0.50 ± 0.04, P<.05 and 54.11 ± 2.32 vs 50.11 ± 2.47 mm, P<.01, respectively). Conclusions. Transcatheter closure is a safe and effective alternative in the treatment of RSVA. The mid-term follow-up outcomes are good.   

J INVASIVE CARDIOL 2014;26(2):55-59

Key words: intervention, catheter, sinus of Valsalva aneurysm, follow-up

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The occurrence of sinus of Valsalva aneurysm (SVA) is more rare in the West than in Asian countries.¹ The unruptured aneurysm of sinus of Valsalva is usually asymptomatic. However, the hemodynamic effects are profound when it ruptures into one of the four chambers of the heart, and nearly 80% patients are symptomatic. Surgical repair has traditionally been the mainstay of treatment for ruptured SVA (RSVA), but some problems limit its application, such as penetrating wounds and relatively long hospital stay, etc. RSVAs have been treated with percutaneous transcatheter closure to avoid sternotomy and cardiopulmonary bypass in the critically ill and hemodynamic stable patients since 1994.^2-6 We reported the immediate and follow-up results of transcatheter treatment of RSVA in 13 patients.

Methods

Patients. This study was conducted according to the principles expressed in the Declaration of Helsinki and approved by our institutional review board (Department of Scientific Research, Southwest Hospital, the Third Military Medical University, Chongqing, China). All patients provided written informed consent for the collection of samples, subsequent analysis, and publication of these case details.

From February 2004 to June 2009, thirteen RSVA patients (9 male, 4 females), ages 18-38 years, were identified and accepted for transcatheter closure, due to the rupture of congenital sinus of Valsalva aneurysm. The defects were identified and evaluated by auscultation of a continuous murmur, electrocardiograms, chest radiographs, and echocardiography. The detailed clinical data of all 13 patients are shown in Table 1. Heart failure (New York Heart Association class II-IV) and/or enlargement of the heart were found in 10 patients, while 5 patients had asymptomatic presentation. Sinus tachycardias were demonstrated in 9 patients, and incidental ventricular and/or atrial premature beats were found in 8 patients by the electrocardiogram. Associated cardiac lesions included mild aortic insufficiency in 3 patients and mild mitral insufficiency in 5 patients.

Procedure. Under local anesthesia, the introducing sheaths were inserted into the right femoral artery and vein. Heparin (100 units/kg) was administered after puncture. All patients underwent routine left and right heart catheterization and the pulmonary pressures were recorded. Then, the left ventricular and aortic pressures were obtained by a 6 Fr pigtail catheter. Aortograms were performed to delineate the origin and size of the fistulae to determine the narrowest opening size. Aortic insufficiency was also assessed. Transthoracic echocardiography was performed to monitor the procedure. The distance between the aortic opening site of the RSVA and the right or left coronary ostium was also measured by aortogram and echocardiography. Selective coronary arteriography was not routinely carried out.

A 6 Fr Judkins right catheter was advanced from the right femoral artery to the ostium of the RSVA over a 0.032˝ guidewire (260 cm) and advanced through the defect to the superior vena cava or left pulmonary artery. The wire was exteriorized through the right femoral vein to form an arterial venous loop; an arteriovenous rail was then established. A 6-10 Fr delivery sheath was then advanced from the femoral vein into the ascending aorta along the guidewire. Based on the measurement of the aortogram, the proper duct occluder (AGA Medical or Starway Medical Supplies, Ltd), which was 1-3 mm larger than the narrowest diameter of the openings of aneurysm measured on aortogram, was advanced along the sheath. The device was deployed in the correct location using fluoroscopy for guidance. To ensure that the aortic valve and coronary ostium were not compromised before detaching the device from the delivery cable, the position of the device was confirmed with both angiography and transthoracic echocardiography. A repeated aortogram and echocardiography were performed routinely after the detachment to evaluate device position, residual shunt, and aortic valve function. Transthoracic echocardiography was performed on the third day after the closure. All patients received aspirin (100 mg/day) and clopidogrel (75 mg/day) for a 6-month period, and follow-up chest x-ray, electrocardiogram, and transthoracic echocardiography were undertaken at intervals of 1, 6, 12, 24, 36, 48, and 60 months after the procedure. The cardiothoracic ratio (CTR) and the chambers of the heart were measured. Enalapril 5-20 mg/day was administered to the patients with heart failure and/or cardiac dilatation before the operation, and used continuously for 3 years in the cases with cardiac dilatation after the closure.

Results

As shown in Table 1, none of patients had associated ventricular septal defect, and all patients underwent local anesthesia. Two-dimensional and color Doppler echocardiographic monitoring were used during the procedure. Eight of 13 originated from the right coronary sinus, while the non-coronary sinus ruptured in 4 cases. The drainage site of the RSVA was in the right ventricle in 8 patients and right atrium in 4 patients. Case #12 was an exception: the aortogram and echocardiography revealed that the left coronary sinus was ruptured into the right ventricular outflow tract (RVOT; Figure 1). The maximal diameter (Dmax) of the aortic opening site of the RSVA measured by aortogram ranged from 6-12 mm. Following the procedure, the mean pulmonary artery pressure decreased from 24.92 ± 8.09 to 21.77 ± 6.25 mm Hg (P>.05). The procedure time ranged from 45 to 130 minutes (average, 82 ± 26 minutes). In all patients, the defect was crossed retrogradely from the aortic side and the duct occluder was deployed by antegrade venous approach. No complications were found in the procedure except the paroxysmal premature beats and tachycardia. 

The 3-day follow-up echocardiography showed that the device was well positioned in the aneurysm and the trace residual shunt disappeared. During the follow-up period (1-60 months; median, 43 months), 5 out of 8 patients with incidental ventricular and/or atrial premature beats before operation had no further arrhythmias. As for the 9 patients with enlarged hearts, compared with the preoperative results, the CTRs were significantly reduced (0.54 ± 0.05 vs 0.50 ± 0.04; P<.05). Echocardiography in the latest follow-up showed neither residual shunts nor new aortic insufficiencies, and there was no device embolization, infective endocarditis, new rupture sites, or RVOT obstruction. The left ventricles of hearts were significantly reduced in the 9 patients with cardiac dilatation during the follow-up (54.11 ± 2.32 vs 50.11 ± 2.47 mm; P<0.01); the other chambers of the heart (left atria, etc) also had a tendency to revert to the normal values (31.22 ± 3.93 mm, 19.00 ± 1.80 mm, and 31.56 ± 3.50 mm vs 36.44 ± 6.78 mm, 20.33 ± 2.12 mm, and 34.22 ± 3.87 mm, respectively, P>.05) (Table 1).

Patients did not exhibit hemorrhagic complications due to antiplatelet drugs. In the latest follow-up, 5 of 8 patients who were taking enalapril with heart failure no longer exhibited symptoms, whereas 3 patients experienced a dramatic improvement in heart function. Having rapidly recovered from heart failure and exhibited normal-sized heart, case #11 stopped taking enalapril before being discharged. One week after operation, case #5 regained normal heart function and a normal heart size (Figure 2), and the echocardiography showed that the aortic and/or mitral insufficiency had disappeared. The patient stopped taking enalapril 1 month after the occlusion. 

Discussion

Sinus of Valsalva aneurysms are rare congenital anomalies that are more prevalent in Asian countries than in the West.¹ Although acquired aneurysms of the aortic sinus have been described postoperatively,^7-9 the medical histories, typical saccular shape, and aneurysm locations in our patients suggested congenital etiology. In addition, RSVA is frequently associated with other congenital defects such as ventricular septal defect, patent ductus arteriosus, or bicuspid aortic valve,^6,10-12 but none of these congenital defects were found in our study. Just 5 of 13 had mitral insufficiency and/or aortic insufficiency. Since the mitral and/or aortic regurgitation disappeared rapidly after the hearts returned to normal size in some patients with enlarged hearts (eg, case #5), the valvular insufficiency might be related to enlargement of the left ventricle. However, the regurgitation could increase the enlargement of the heart at the same time.

The right coronary sinus is the most common site (75%-90%) in which the RSVA may occur, and the right ventricle is the most common drainage chamber.^13,14 Conventionally, with an operative mortality rate less than 2%, surgical repair of the RSVA has been the mainstay of therapy.^10,15,16 Since the first case of ruptured SVA closure with a Rashkind umbrella was reported in 1994,² the transcatheter technique has been performed with different devices to close RSVA.^6,8,9,17,18 In the present study, we chose to use a ductal occluder to close the RSVA since the shunt was large, and the shape of the ductal occluder was best suited to close these saccular aneurysms. All the operations were successful and no complications occurred. Thus, transcatheter closure was a safe and effective treatment for the RSVA and could be an alternative to surgery in selected cases. In these patients, #12 was a special case; sinus of Valsalva aneurysms occurred in the left coronary sinus and the drainage site of the RSVA was the RVOT. Although Cullen¹⁸ reported the treatment of an RSVA draining into the RVOT with an atrial septal occluder, closure in the RVOT may induce the obstruction of RVOT and persistent hemolysis.¹⁷ To avoid the RVOT obstruction, a 12 mm ductal occluder was deployed in the site of RSVA. Echocardiography monitoring did not detect the obstruction of RVOT during the procedure and the follow-up, and hemolysis did not occur after transcatheter closure. In this case, there was an acute curve between the right ventricle and drainage site of the RSVA, so it was very difficult to advance the delivery sheath and occluder.

The unruptured aneurysm of sinus of Valsalva is usually asymptomatic, and the symptoms may occur when the SVA is too large (we had a 26-year-old patient with old myocardial infarction of the inferior wall and the right coronary artery was obliterated by a giant unruptured aneurysm originated from the right coronary sinus). Upon rupture, the patients suffer from biventricular increases in volume and develop congestive heart failure. Ten out of 13 patients have heart failure and/or cardiac dilatation; the ratio is very high. Of these 10 patients, 8 already knew that they had abnormalities of the heart several years before symptoms began (3-9 years), 3 were diagnosed through echocardiography, while 5 exhibited abnormal cardiac auscultation. Due to poverty and poor medical conditions, the patients were not willing to go to the hospital before they exhibited symptoms or their conditions became more severe. None of these patients had gusty chest pain. Case #11 had dyspnea and chest pain occasionally on exertion, a low diastolic blood pressure of the aortic root (118/45 mm Hg) was detected before the occlusion and it increased to 112/60 mm Hg after the procedure. The patient stopped taking enalapril before hospital discharge and no longer exhibited these symptoms during the follow-up. Thus, his symptoms may have been related to the reduction of coronary perfusion during the relaxing period.  

Although many reports^2,3,6,8 have emphasized the benefits of transesophageal echocardiography and selective coronary arteriography for transcatheter closure of intracardiac lesions, we think that this may complicate the procedure. In our series, there is usually a distance between the rupture site and the ostium of the coronary artery. Aortogram, fluoroscopy, and transthoracic echocardiography are enough for selecting the ductal occluder size, locating and releasing the devices, and evaluating the function of the aortic valves. 

On follow-up (1-60 months), there were no hemorrhagic complications, residual shunts, new aortic regurgitations, or RVOT obstructions. The arrhythmias had disappeared. For the 9 patients who took enalapril orally, with enlarged hearts before transcatheter closure, the hearts decreased significantly in size and their cardiac functions were significantly improved. These results show that the treatment is successful and that transcatheter occlusion is safe and effective for patients with cardiac dilatation. The correction of abnormal hemodynamics played a crucial role in the improvement of cardiac structure and function in case #5. However, since the pulmonary artery pressures descended from 27.22 ± 8.11 mm Hg to 24.11 ± 5.99 mm Hg (P>.05) following the procedure in the 9 patients who took enalapril with cardiac dilatation, and 6 patients still exhibited enlarged hearts in the latest follow-up, it is difficult to confirm whether the reduction of chambers in these patients was a result of enalapril or the improvement of hemodynamics after closure, or both; this is probably multifactorial as a result of decreasing the shunt or afterload reduction. Because there were no adverse reactions to enalapril during the follow-up, and there is considerable evidence that ACEI can improve heart function and cardiac remodeling through a variety of ways in patients with heart failure,^19-21 out of the 4 patients who completed 5 years of follow-up, 2 patients with heart failure and/or cardiac dilatation are taking enalapril. Since this was a small-scale study and the control group’s relevance was absent, the value of enalapril is limited and a long-term follow-up is mandatory. 

However, RSVAs may have multiple drainage sites, and transcatheter closure can’t prevent the occurrence of new rupture sites in the same sinus of Valsalva.¹⁴ For these patients, we do not currently have enough experience to help us choose reinterventional therapy or thoracic surgery treatment. Additionally, a unified proposal should also be formulated above all in the selection, dose, and usage of antithrombotic drugs after transcatheter closure. Although many problems still need to be solved, the catheter approach is safe, feasible, and effective for the ruptured SVA. Long-term follow-up on a large scale may help us to obtain the associated experiences and resolve these problems.

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