Asian Cardiovasc Thorac Ann 2001;9:111-114
© 2001 Asia Publishing EXchange Pte Ltd
Aortopulmonary Window Repair: 15-Year Surgical Experience
Khawar Aizaz, MBBS,
Howaida Al Qethamy, MBBS,
Khalid Al Jubair, MBBS,
Yahya Al Faraidi, MBBS,
Mohammed R Al Fagih, MBBS
Department of Cardiac Surgery
Prince Sultan Cardiac Center
Riyadh, Saudi Arabia
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For reprint information contact: Khawar Aizaz, MBBS Tel: 966 1 478 3000 Ext. 8557 Fax: 966 1 476 0543 email: kzazs{at}yahoo.com Department of Cardiac Surgery (F-277), Prince Sultan Cardiac Center, P.O. Box 7897, Riyadh 11159, Saudi Arabia.
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Abstract
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Aortopulmonary window is an uncommon anomaly. Early surgery is recommended before permanent pulmonary vascular changes develop. Results were reviewed in 18 patients who underwent aortopulmonary window repair between January 1985 and December 1999. A transaortic approach was employed in 12, a transpulmonary approach was used in 3, the pulmonary artery flap technique was performed in 2, and an aortopulmonary window was simply ligated in 1 patient. Concomitant repair of all associated anomalies was carried out, except in 2 patients who had interrupted aortic arch repaired 6 days before aortopulmonary window repair. There was no hospital mortality. During a mean follow-up of 43 months (range, 6 to 144 months), there was no late death and all patients were in New York Heart Association functional class I, except 3 who required reoperation: 2 had pulmonary artery confluence stenosis 5 to 10 years after aortopulmonary window repair; and 1 required transfer of the right coronary artery from the pulmonary artery to the aorta 28 months after aortopulmonary window repair. Surgical repair of aortopulmonary window, even when associated with other cardiac anomalies, carries a low risk. Early surgical treatment achieved excellent immediate and long-term results.
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Introduction
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Aortopulmonary window (APW) is a rare cardiac anomaly in which there is a localized absence of the conotruncal septum.1 Anatomically, it consists of a communication between the ascending aorta and the main pulmonary artery, with separated aortic and pulmonary valves.
The lesion was first described in an autopsy study by Elliotson2 in 1830, and its successful closure was initially reported by Gross3 in 1952. Richardson and colleagues4 described 3 types of APW, depending on the distance between the defect and the plane of the semilunar valves. Type I defects are between the ascending aorta and main pulmonary artery, just above the sinus of Valsalva. Type II defects are more cephalad between the ascending aorta and the origin of the right pulmonary artery from the main pulmonary artery. A type III defect is anomalous origin of the right pulmonary artery from the aorta.4
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Patients and Methods
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From January 1985 to December 1999, 18 patients with APW were operated upon at the Prince Sultan Cardiac Center, Riyadh, Saudi Arabia. There were 10 males (56%) and 8 females; their ages ranged from 2 weeks to 72 months (mean age, 11.13 months), with 13 (72%) under 1 year of age, of whom 4 were less than 1 month old. Weights ranged from 2.07 to 14.5 kg (mean, 4.97 kg), and 12 patients (67%) were less than 5 kg (Table 1
). Two patients with associated type A interrupted aortic arch presented during the neonatal period in a state of circulatory collapse. Both of these patients required preoperative intubation and ventilation, and were supported with inotropics and prostaglandin E1 infusion. The remaining patients had mild degrees of cardiac failure and presented with recurrent pulmonary infection and/or failure to thrive. Electrocardiograms and chest radiographs were studied in all cases. Standard right and left heart catheterization with cineangiography was performed in 14 of the 18 patients. Angiographic findings and associated cardiac anomalies are listed in Table 1.
Surgical repair was carried out through a median sternotomy. Fifteen patients underwent correction with the use of cardiopulmonary bypass (CPB), moderate hypothermia, aortic crossclamping, and a single dose of antegrade cold blood cardioplegia (25 to 30 mL•kg–1). Two patients had APW repair using CPB and normo-thermia without aortic crossclamping, and the APW was doubly ligated without CPB in 1 patient. The operation was performed via a transaortic approach with repair of the defect using a Gore-Tex patch (WL Gore, Flagstaff, AZ, USA) in 12 patients. A transpulmonary approach using a Gore-Tex or Dacron patch to close the defect was undertaken in 3 patients. The pulmonary artery flap technique (direct closure of the aortic defect with a pulmonary arterial wall patch and repair of the resultant defect in the pulmonary artery with an autologous pericardial patch) was performed in 2 patients. Con-comitant repair of all associated anomalies was carried out, except in the 2 patients with interrupted aortic arch; as both were in a state of circulatory collapse at presentation, a staged repair was considered safest. Repair of interrupted aortic arch was undertaken through a standard left thoracotomy as the first stage, thereby avoiding the adverse effects of CPB in these sick neonates. APW repair was then carried out as a second stage 6 days later in both cases. The operative data are summarized in Table 2.
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Results
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There were no operative or late deaths. Three patients (17%) required reoperation as shown in Table 3. All 3 made an uneventful recovery after reoperation. Follow-up ranged from 6 to 144 months (mean, 43 months). The complications are listed in Table 4. Follow-up data were obtained in all patients from the records of clinical examinations and echocardiography findings documented at the time of the last visit to the outpatient clinic. Fifteen of the 18 patients (excluding the 3 who required reoperation) were in New York Heart Association functional class I, with no residual shunts.
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Discussion
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APW clinically mimics a large patent ductus arteriosus or ventricular septal defect with pulmonary hypertension. However, pulmonary vascular resistance in these patients tends to increase very rapidly. Prompt diagnosis and treatment is therefore required.4 The spectrum of APW is wide so several surgical techniques and modifications have been described, with or without CPB, through a transaortic, transpulmonary, or trans-window approach, and with or without the use of a patch.5–11 In 1992, Matsuki and colleagues12 described the pulmonary artery flap technique for closure of APW. Using the same principle, we recently started using the pulmonary artery flap technique to repair APW. Our method differs in the use of CPB without aortic crossclamping. The APW was isolated by careful dissection, taking particular care of the left main coronary artery that can be intimately related to the posteroinferior surface of the defect. A side-biting vascular clamp was placed across the defect towards the pulmonary artery immediately after institution of CPB. Normothermia was maintained and the heart was kept beating. The window was divided, taking along with it the patch of pulmonary arterial wall, which is oversewn, thereby closing the defect on the aortic side. The side-biting vascular clamp was removed, the defect in the pulmonary artery was repaired using an autologous pericardial patch, and the patient was weaned off CPB. This technique avoids narrowing of the aorta and pulmonary artery, allows complete separation of the aorta and pulmonary artery, prevents any residual shunts, and avoids complications associated with a synthetic patch on the aorta (aneurysmal dilatation). We used this technique in 2 patients, and because of the advantages and simplicity, it will be our procedure of choice in future to repair APW, especially for a type I defect. Larger defects may require aortic crossclamping as placing the side-biting vascular clamp across a larger APW can lead to hemorrhage. In addition, this technique allows exact tailoring of the pulmonary artery patch to close the aortic defect, thereby avoiding a redundant and aneurysmal patch.
APW is an uncommon anomaly often associated with other cardiac defects, especially type A interrupted aortic arch (in 26% of cases).13 Despite reports of successful single-stage repair of APW and interrupted aortic arch, we believe that a two-stage repair is safer in neonates who present with severe heart failure and circulatory collapse.14,15 Surgical repair of APW, even when associated with other cardiac anomalies, carries a low risk. Early surgical treatment prevents development of obstructive pulmonary vascular disease and achieves good immediate and long-term results.
Presented at the 8th Annual Meeting of The Asian Society for Cardiovascular Surgery, Fukuoka, Japan, September 6–8, 2000.
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References
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Abstract
Introduction
