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Ann Thorac Surg 1998;65:768-770
© 1998 The Society of Thoracic Surgeons

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Original Articles: Cardiovascular

Surgical Management of Aortopulmonary Window

Department of Cardiac Surgery, Royal Group of Hospitals, Belfast, Northern Ireland, United Kingdom

Accepted for publication September 22, 1997.

Mr Gladstone, Royal Victoria Hospital, Grosvenor Rd, Belfast BT12 6BA, Northern Ireland.

	Abstract

Top Abstract Introduction Material and Methods Results Comment References

 
Background. Aortopulmonary window is a rare anomaly, and a variety of surgical techniques have been described for its closure.

Methods. We treated 6 infants with aortopulmonary window between 1993 and 1995. Three had associated type A interrupted aortic arch, and another had a muscular ventricular septal defect. The diagnosis was made by echocardiography, confirmed by cardiac catheterization in 4 infants. In 1 very sick neonate with interrupted arch, diagnosis of the window was considerably delayed. In 4 patients, we closed the window by using a flap of pulmonary artery, which was reconstructed without using a patch. In 2 neonates with interrupted arch we anastomosed the mobilized descending aorta directly to the aortic defect of the aortopulmonary window, closing the pulmonary artery with a pericardial patch.

Results. There were no hospital deaths, and all patients are in New York Heart Association functional class I at a mean follow-up of 30 months. Echocardiography shows no significant distortion of the great vessels.

Conclusions. The techniques described achieve excellent results using only autologous tissues with the potential for normal growth.

	Introduction

Top Abstract Introduction Material and Methods Results Comment References

 
Aortopulmonary window (APW) is a rare congenital cardiac anomaly first described by Elliotson in 1830 in an autopsy study [1]. We report our experience of 6 patients with APW who presented for surgical treatment. In this series all repairs were accomplished using autologous tissue.

	Material and Methods

Top Abstract Introduction Material and Methods Results Comment References

 
Between February 1993 and September 1995, 6 patients presented with APW and subsequently underwent repair in the Cardiac Surgical Unit of the Royal Victoria Hospital, Belfast (Table 1).

Initial diagnoses were made by two-dimensional echocardiography and were subsequently confirmed by cardiac catheterization in 4 patients.

In the first patient of the series, there was considerable delay in diagnosing the APW. This patient presented in a state of circulatory collapse and the initial diagnosis was of severe aortic coarctation. At emergency operation, type A IAA was diagnosed and treated by direct anastomosis between descending aorta and arch. Postoperatively, he was in renal failure for several days, and his subsequent slow recovery and persistent cardiac failure were initially attributed to his poor preoperative status, as no additional intracardiac anomaly was seen on echocardiography. The clinical picture was further complicated by the development of severe infection with respiratory syncytial virus, requiring prolonged ventilatory support. The diagnosis of APW was eventually made at 3.5 months of age, and shortly thereafter he underwent a further successful operation and made a good recovery.

In patient 2, whose cardiac failure was well controlled on medical therapy, operation was delayed because of low birth weight. Two of the remaining 4 patients (patients 3 and 5) required preoperative ventilation, but all of the operations were performed semielectively once the patients had been stabilized on medical therapy.

The surgical approach adopted in 4 patients was to close the APW using a simple pulmonary flap technique. After institution of cardiopulmonary bypass with moderate hypothermia, either the APW itself or the main pulmonary artery (PA) distal to it was occluded, followed by ascending aortic cross-clamping and administration of cold cardioplegia solution.

A semicircular flap of arterial wall was created by incising the main PA anteriorly a few millimeters lateral to the APW, and then extending this incision to diametrically opposite points at the superior and inferior margins of the defect (Fig 1). Care was taken to ensure that there was no anomalous coronary orifice that might be compromised by the repair. The flap was then sutured to the posterior margin of the APW using continuous polypropylene (Fig 2).

View larger version (32K): [in this window] [in a new window]   Line of incision on the main pulmonary artery. (Ao = ascending aorta; PA = main pulmonary artery.)

View larger version (34K): [in this window] [in a new window]   The pulmonary flap is sutured to the posterior margin of the aortopulmonary window. (Ao = ascending aorta; PA = main pulmonary artery.)

View larger version (35K): [in this window] [in a new window]   The cut edge of the pulmonary artery is sutured to the adventitia of the aortic wall. (Ao = ascending aorta; PA = main pulmonary artery.)

	Results

Top Abstract Introduction Material and Methods Results Comment References

 
All patients made a good recovery from operation. All are well from the cardiac viewpoint, and taking no cardiac medication at a mean follow-up of 30 months. Each has been studied by echocardiography at follow-up on at least one occasion, but none has yet been subjected to further invasive investigation. These studies show no evidence of PA narrowing and normal flow patterns.

Patients who required repair of interrupted aortic arch show no evidence of anastomotic narrowing on two-dimensional imaging. Doppler measurements in the descending aorta show maximum flow velocities that give small calculated gradients (mean 13 mm Hg). In our experience, patients with such findings do not show significant gradients on invasive measurement.

	Comment

Top Abstract Introduction Material and Methods Results Comment References

 
Aortopulmonary window was first described by Elliotson [1] and is a result of incomplete septation between the ascending aorta and the PA. Richardson and colleagues [2] described three types of APW, depending on the distance between the defect and the plane of the semilunar valves. All of our patients had type I APW according to their classification.

In our series, 4 of 6 patients had associated cardiac anomalies, in three patients type A IAA. This association has previously been reported in 26% of cases, in a review of the literature, by Kutsche and van Mierop [3].

Aortopulmonary window may be readily diagnosed by echocardiography. However, as our first patient demonstrated, the findings can be easily misinterpreted on multiple examinations if the operator is not alert to the diagnosis. The finding of type A IAA in isolation should be treated with special suspicion. Any doubt about the diagnosis may be resolved by cardiac catheterization.

Since Gross [4] first successfully ligated an APW in 1948, several surgical techniques and modifications have been described, with or without extracorporeal circulation, through a transaortic or transpulmonary approach, and with or without the use of a patch [5][6][7][8][9][10][11]. Matsuki and associates in 1992 [12] and Messner and colleagues in 1994 [13] have described similar pulmonary flap techniques for closure of APW, although they both used pericardium to repair the pulmonary artery.

If the pulmonary flap is based across the diameter of the defect, as we describe, the length of the flap does not need to be as great as if it is more anteriorly and narrowly based. We have found that our repair gives a normal cylindric shape to the aorta using only a small amount of PA wall. All of our patients showed marked dilatation of the main PA and direct closure was achieved without causing significant distortion. We believe that patching should only be necessary in special situations where a larger pulmonary flap is required to close a more complex defect, or to direct blood to an anomalous coronary origin.

In the 2 patients in whom APW and IAA were diagnosed together at presentation, we anastomosed the descending aorta directly to the aortic defect of the APW. This option, which was also reported by Burke and Rosenfeld in 1994 [14], in a patient with anomalous origin of the right PA, seems to achieve good results without significant compression of the right pulmonary artery or left main bronchus, despite the more proximal connection to the ascending aorta.

In conclusion, APW is an uncommon anomaly that can be easily overlooked if not sought specifically. It is often associated with other cardiac anomalies, especially type A interruption of the aortic arch, which is almost never found in isolation.

The techniques we describe are simple and effective means of repairing AP window with and without interrupted aortic arch. The absence of foreign materials should give good growth potential with minimal distortion of the vessels and excellent functional results.

	References

Top Abstract Introduction Material and Methods Results Comment References

 

1. Elliotson J Case of malformation of the pulmonary artery and aorta. Lancet 1830;1:247-251.
2. Richardson JV, Doty DB, Rossi NP, Ehrenhaft JL The spectrum of anomalies of aortopulmonary septation. J Thorac Cardiovasc Surg 1979;78:21-27.[Abstract]
3. Kutsche LM, Van Mierop LHS Anatomy and pathogenesis of aortopulmonary septal defect. Am J Cardiol 1987;59:443-447.[Medline]
4. Gross RE Surgical closure of an aortic septal defect. Circulation 1952;5:858-863.[Abstract/Free Full Text]
5. Scott HW, Sabiston DC Surgical treatment for congenital aorticopulmonary fistula. Experimental and clinical aspects. J Thorac Surg 1953;25:26-28.
6. Morrow AG, Greenfield LJ, Braunwald E Congenital aortopulmonary septal defect. Clinical and hemodynamic findings, surgical technique and results of operative correction. Circulation 1962;25:463-476.[Abstract/Free Full Text]
7. Putman TC, Gross RE Surgical management of aortopulmonary fenestration. Surgery 1966;59:727-735.[Medline]
8. Wright JS, Freeman R, Johnston JB Aortopulmonary fenestration. A technique of surgical management. J Thorac Cardiovasc Surg 1968;55:280-283.[Medline]
9. Johansson L, Michaelsson M, Wasterholm CJ, Aberg T Aortopulmonary window: a new operative approach. Ann Thorac Surg 1978;25:564-567.[Abstract/Free Full Text]
10. Ravikumar E, Whight CM, Hawker RE, Celermajer JM, Nunn G, Cartmill TB The surgical management of aortopulmonary window using the anterior sandwich patch closure technique. J Cardiovasc Surg (Torino) 1988;29:629-632.[Medline]
11. Shatapathy P, Madhusudhana Rao K, Krishnan KV Closure of aortopulmonary septal defect. J Thorac Cardiovasc Surg 1987;93:789-791.[Medline]
12. Matsuki O, Yagihara T, Yamamoto F, Nishigaki K, Uemura H, Kawashima Y New surgical technique for total-defect aortopulmonary window. Ann Thorac Surg 1992;54:991-992.[Abstract/Free Full Text]
13. Messner BJ Pulmonary artery flap for closure of aortopulmonary window. Ann Thorac Surg 1994;57:498-501.[Abstract/Free Full Text]
14. Burke RP, Rosenfeld HM Primary repair of aortopulmonary septal defect, interrupted aortic arch, and anomalous origin of the right pulmonary artery. Ann Thorac Surg 1994;58:543-545.[Abstract/Free Full Text]

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