Asian Cardiovasc Thorac Ann 2001;9:229-231
© 2001 Asia Publishing EXchange Pte Ltd
Residual Aortopulmonary Window Closure With Amplatzer Septal Occluder
Bagrat G Alekyan, MD,
Vitaly A Boukharin, MD,
Vladimir P Podzolkov, MD,
Manolis G Poursanov, MD
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Department of Interventional Cardiology and Angiology Bakoulev Scientific Center for Cardiovascular Surgery Russian Academy of Medical Sciences Moscow, Russia
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For reprint information contact: Bagrat G Alekyan, MD Tel: 7 095 414 7547 Fax: 7 095 414 7708 email: b_alekyan{at}mtu-net.ru Department of Interventional Cardiology and Angiology, Bakoulev Scientific Center for Cardiovascular Surgery, Russian Academy of Medical Sciences, 135 Roublevskoye Shosse, Moscow 121552, Russia.
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Abstract
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A 15-year-old female presented with fatigue and dyspnea on exertion due to a residual left-to-right shunt across an aortopulmonary window that had been surgically closed 11 years earlier. The residual opening was located distal to the coronary ostia, the semilunar valves, and the pulmonary artery bifurcation. An Amplatzer septal occluder was used to completely close the residual defect, thereby avoiding reoperation.
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INTRODUCTION
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Aortopulmonary windows are usually repaired early in life to eliminate left heart volume overload and congestive heart failure, and prevent the development of pulmonary vascular obstructive disease and subacute bacterial endocarditis. Two case reports described closure of small aortopulmonary windows using Rashkind double umbrella devices.1,2 We previously reported successful closure of a residual aortopulmonary window in 1998.3 A similar case was treated by Richens and colleagues.4 This report describes closure of a residual shunt after surgical treatment of an aortopulmonary window, using an alternative device, the Amplatzer septal occluder (AGA Medical Corp., Golden Valley, MN, USA).
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CASE REPORT
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A 15-year-old female presented with fatigue and dyspnea on exertion 11 years after surgical repair of an aorto-pulmonary window. Presurgical heart catheterization had revealed pulmonary artery pressure of 100/40 mm Hg (mean, 55 mm Hg), systemic pressure of 100/56 mm Hg (mean, 68 mm Hg), pulmonary resistance of 7.5 Wood's units, and a pulmonary-to-systemic flow ratio of 2.2. Surgery had been performed under cardiopulmonary bypass and hypothermia. The aortopulmonary window had a diameter of 2 cm and was located distal to the aortopulmonary septum. The defect was sutured with a heterologous pericardial patch. The total circulatory arrest time was 31 minutes. Shortly after the operation, a con-tinuous murmur was heard, indicating residual shunting.
Although the patient had presented with symptoms, she had no signs of congestive heart failure, and her blood pressure was 110/60 mm Hg. The peripheral pulses were bounding (80 beats per minute). A continuous murmur was again heard at the upper left sternal border. A chest radiograph showed increased pulmonary vascular markings. A transthoracic echocardiogram demonstrated that the residual shunting was small and located at the superior edge of the aortic patch originally placed to close the aortopulmonary window, distal to the semilunar valves and the pulmonary artery bifurcation. The right ventricular systolic pressure was estimated as 45 mm Hg. Taking into consideration the small size of the residual defect and its favorable position, transcatheter closure using an Amplatzer septal occluder was recommended instead of surgery, and informed consent for the procedure was obtained.
The procedure was performed under general anesthesia. The main pulmonary artery systolic pressure was assessed as 50 mm Hg, and the pulmonary-to-systemic flow ratio was 0.45. Multiple angiograms confirmed the small size and favorable position of the residual defect (Figure 1
). A 4-mm diameter angioplasty balloon was advanced from the main pulmonary artery across the residual defect. The balloon was inflated under low pressure to accurately measure the diameter of the defect (3.4 mm). A 4-mm diameter Amplatzer septal occluder was selected to close the residual defect. An 8F delivery system was introduced via the right femoral vein and advanced from the main pulmonary artery across the defect to deploy the occluder. Transthoracic echocardiography confirmed that the occluder was in the optimal position with no residual shunting and no interference with the semilunar valves. The fluoroscopy time was 17 minutes. Aortography after deployment showed complete closure of the residual defect (Figure 2) and no obstruction to coronary artery flow. Oxymetry revealed no residual shunting. The main pulmonary artery systolic pressure was decreased to 42 mm Hg. Pressure measurements and pulmonary arteriography showed that the right pulmonary artery remained patent. Heparin 100 units•kg–1 and first-generation cephalosporins were given during and after the procedure. Post-procedure auscultation revealed no murmur. Follow-up transthoracic echocardiography showed no residual shunting, normal semilunar valve function, and no obstruction to pulmonary artery flow. The patient was discharged 3 days after the procedure with instructions to complete a 6-day course of prophy-lactic antibiotic therapy and to take aspirin 5 mg•kg–1 daily for 3 months.
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Figure 1. Contrast injection via a catheter advanced from the pulmonary artery to the ascending aorta across a small residual defect in the patch originally placed to close the window. Angiography showed the position and small size of the residual defect (arrow).
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Figure 2. Injection of dye into the ascending aorta after deployment of a 4-mm Amplatzer septal occluder. This angiogram showed the device in a satisfactory position with no residual shunt across the aortopulmonary window.
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DISCUSSION
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Aortopulmonary windows are rare congenital cardio-vascular abnormalities found in only 0.2% to 0.6% of patients with congenital heart disease.5–7 The majority of aortopulmonary windows are large and located in close proximity to the semilunar valves, the pulmonary artery bifurcation, or both.8 Only 12% of aortopulmonary windows examined in the autopsy series of Ho and colleagues8 were located in an intermediate position distal to both semilunar valves and the pulmonary artery bifurcation, which is considered suitable for transcatheter closure.
Indeed, the 2 cases of aortopulmonary window that were closed with Rashkind double umbrella devices, like the residual defect in this report, were small and located distal to the coronary ostia, the semilunar valves, and the pulmonary artery bifurcation, in patients who had no associated anomalies of the pulmonary or coronary arteries.1,2 Nonetheless, it is reasonable to offer trans-catheter therapy to this small group of patients to avoid the morbidity and expense associated with sternotomy, cardiopulmonary bypass, and circulatory arrest.
The recanalization of aortopulmonary windows after surgical repair is a rare complication. At the same time, reoperation carries a higher risk of hemorrhage due to repeat sternotomy and the presence of tissue adhesions. The positive experience with this type of occluder suggests that the risks are minimal as there is an opportunity to withdraw the device even when it is completely opened.
The Rashkind device is no longer used due to its high rate of complications (device arm fractures, high incidence of residual shunting, difficult implantation technique).9,10 The Amplatzer septal occluder has some advantages over the Rashkind device in being self-centering with a small introduction system, simple placement technique, waist diameter of 4 to 38 mm, "stem-like" placement into a defect with secondary thrombosis, and the potential to be retrieved and repositioned.10 The Amplatzer septal occluder has a somewhat higher profile than double-disk occluders such as the Rashkind device. The Amplatzer device proved entirely suitable for use in the teenaged patient described here. However, it is possible that lower profile devices will prove more suitable for use in smaller patients who receive Rashkind devices to close their aortopulmonary windows; this is because lower profile devices may be less likely to obstruct pulmonary artery flow.1,2
It is appropriate to stress that among the abnormalities associated with aortopulmonary windows is anomalous origin of a coronary artery adjacent to the margin of the window.6,8 It is imperative that the relationship between the origins of the coronary arteries and the aortopulmonary window be clearly defined before undertaking trans-catheter closure of an aortopulmonary window so as to avoid placing a device across a coronary ostium and causing myocardial ischemia.
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REFERENCES
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Alekyan BG, Poursanov MG, Vedernikova L, Mitina I, Putiato N. The first case of successful closure of a recanalized defect of aortopulmonary septum with Amplatzer septal occluder: case report. Grudnaya I Serdechno-sosudistaya Chirurgia 1998;5:71–3.
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Kutsche LM, Van Mierop LH. Anatomy and pathogenesis of aorticopulmonary septal defect. Am J Cardiol 1987; 59:443–7.[Medline]
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Ho SY, Gerlis LM, Anderson C, Devine WA, Smith A. The morphology of aortopulmonary windows with regard to their classification and morphogenesis. Cardiol Young 1994;4:146–55.
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Masura J, Gavora P, Formanek A, Hijazi ZM. Transcatheter closure of secundum atrial septal defects using the new self-centering Amplatzer septal occluder: initial human experience. Cathet Cardiovasc Diagn 1997;42:388–93.[Medline]
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Abstract
INTRODUCTION
