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Ann Thorac Surg 2003;76:628-630
© 2003 The Society of Thoracic Surgeons

How to do it

Description of a new technique for reimplanting the anomalous right pulmonary artery

Kirk McMurtry, MDa, Shubhika Srivastava, MDb, Khanh H. Nguyen, MDa*

a Department of Cardiothoracic Surgery, Mount Sinai Medical Center, New York, New York, USA
b Department of Pediatrics, Mount Sinai Medical Center, New York, New York, USA

Accepted for publication January 19, 2003.

* Address reprint requests to Dr Nguyen, Department of Cardiothoracic Surgery, Mount Sinai Medical Center, PO Box 1028, 1190 Fifth Ave, New York, NY 10029, USA
e-mail: khanh.nguyen{at}mountsinai.org


   Abstract
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 Abstract
Introduction
 Technique
 Comment
 References
 
Anomalous origin of the right pulmonary artery from the ascending aorta is a rare congenital lesion with a high mortality and morbidity if early diagnosis is not made and correction is not undertaken. We describe the repair of such a lesion using a double-trapdoor technique of pulmonary artery reimplantation.


   Introduction
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 Abstract
 Introduction
Technique
 Comment
 References
 
Anomalous origins of both the right pulmonary artery (RPA) and left pulmonary artery have been reported in the literature. Origin of the RPA from the ascending aorta is a rare congenital lesion with approximately 65 cases in the literature [1]. Early surgical repair is now the standard of treatment to prevent the deleterious effects of pulmonary overcirculation and irreversible changes. The first surgical repairs consisted of interposition grafts made of synthetic material [2]. As techniques improved, synthetic materials were abandoned in favor of primary anastomosis of the RPA to the main pulmonary artery (MPA). This anastomosis must be performed carefully to avoid tension and stricture formation. We describe a double-trapdoor technique that we believe prevents these complications and avoids the use of synthetic material.


   Technique
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A 5-day-old girl was transferred to our institution for evaluation of a murmur discovered on the second day of life. She had been born through a cesarean section because of a breech presentation. The Apgar score was 9 at 1 minute and 5 minutes. Gestational age was 40 weeks, and weight on delivery was 3,565 g. The results of physical examination were normal except for a holosystolic murmur heard best at the left sternal border. An electrocardiogram demonstrated right ventricular hypertrophy. A transthoracic echocardiogram revealed a patent ductus arteriosus and the RPA arising from the right lateral border of the ascending aorta. There was also right atrial and ventricular dilatation with moderate right ventricular dysfunction and moderate tricuspid valve regurgitation.

At operation the following day, a median sternotomy was performed and the thymus, removed. When the pericardial sac was entered, the anomalous origin of the RPA from the right lateral aspect of the ascending aorta was immediately apparent. Further dissection exposed the patent ductus arteriosus, which was doubly ligated and divided. The ascending aorta, brachiocephalic vessels, RPA, and MPA were extensively dissected from the surrounding tissues in preparation for later anastomosis. Cannulation of the ascending aorta and right atrium was used for cardiopulmonary bypass.

On the initiation of cardiopulmonary bypass, the proximal anomalous RPA was snared with a vessel loop. An aortic cross-clamp was placed, and antegrade cardioplegia was given. Aortotomy was performed with a cuff of aorta left on the RPA for use in the anastomosis to the MPA (Fig 1A, 1B). The aorta was repaired primarily using continuous monofilament suture. This step was performed prior to implantation of the anomalous RPA to minimize the duration of cross-clamping. The aortic cross-clamp was removed, and the patient was rewarmed.



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  		Fig 1. (A, B) The anomalous right pulmonary artery (RPA) is detached from the aorta, and a double trapdoor is created on the main pulmonary artery. (C, D) The anastomosis of the RPA is performed. For clarity, the aorta is not shown. (E) Completed repair.

 
The MPA was then prepared for anastomosis by creating the two trapdoors in a side-to-side fashion oriented 90 degrees to the flaps of aorta remaining on the RPA (Fig 1B–1D). The RPA was positioned posterior to the ascending aorta and anastomosed to the MPA using the double-trapdoor technique with a continuous monofilament suture (Fig 1C–1E).

The patient was weaned from cardiopulmonary bypass without difficulty. The total cross-clamp time was 11 minutes, and the total cardiopulmonary bypass time was 17 minutes. Postoperative echocardiogram showed a wide open RPA–MPA connection (Fig 2). The patient recovered uneventfully.



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  		Fig 2. Echocardiogram showing site of right pulmonary artery (RPA)–main pulmonary artery (MPA) anastomosis. (Ao = aorta; LPA = left pulmonary artery.)

 

   Comment
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 Abstract
 Introduction
 Technique
 Comment
References
 
Origin of the RPA from the ascending aorta is a rare, yet well-described congenital defect of the great vessels. Sixty-five cases have been discussed in the literature [1]. The natural history of this lesion is that of progressive heart failure with a 70% mortality rate at 1 year compared with an 84% survival rate at 1 year with surgical repair [1]. Repair of this lesion was first reported in 1961 and was accomplished with an interposition Dacron graft [2]. This technique fell out of favor, however, because of the high probability that further surgical intervention would be necessary to resize the graft as the child grew. In 1967, Kirkpatrick and associates [3] described repair of this lesion with primary anastomosis of the RPA to the MPA. With minor variations, this has been the technique most widely used [4–7]. Potential problems with primary repair include tension at the anastomosis, stretching of the RPA caused by inadequate length, and stricture at the anastomosis itself.

The trapdoor technique has been well described and has been associated with a number of procedures, such as the reimplantation of coronary arteries in anomalous coronary artery anatomy and the arterial switch operation. The double-trapdoor technique uses a portion of the ascending aorta to reconstruct the RPA–MPA anastomosis (See Fig 1B–1D). The flaps of aorta created in the process of detaching the RPA from the ascending aorta are oriented 90 degrees from the two trapdoor flaps created on the MPA.

The choice of this technique serves two functions. First, the anastomosis is much larger than a standard end-to-side anastomosis (See Fig 1C, 1D). The two trapdoors greatly enlarge the area of a standard anastomosis, and the suture line is much longer, thereby decreasing stricture resulting from a pursestring effect. The other function is that of increasing the length of the RPA by "adding" tissue to the MPA in the form of the two trapdoors and by "adding" tissue to the proximal end of the RPA in the form of the aortic flaps. With each of these four flaps oriented 90 degrees to each other, the length of the RPA can be increased by several millimeters (See Fig 1D). Further, all anastomoses are created with autologous tissue and have the potential for growth. This technique calls for extensive mobilization of the ascending aorta and the brachiocephalic arteries to minimize tension on the aortic anastomosis and compression of the RPA. The patent ductus arteriosus is divided to provide further mobility of the MPA. As already mentioned, the aorta was reanastomosed prior to implantation of the RPA. This maneuver did not seem to interfere with the implantation, and the exposure was very adequate.

We believe the double-trapdoor technique is simple, is easily reproducible, and has the benefits previously discussed that are valuable when operating on such small patients.


   References
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 Abstract
 Introduction
 Technique
 Comment
 References
 

  1. Fontana G.P., Spach M.S., Effman E.L., Sabiston D.C. Origin of the right pulmonary artery from the ascending aorta. Ann Surg 1987;206:102-113.[Medline]
  2. Armer R.M., Shumacker H.B., Klatte E.C. Origin of the right pulmonary artery from the ascending aorta. Report of a surgically corrected case. Circulation 1961;24:662-668.[Abstract/Free Full Text]
  3. Kirkpatrick S.E., Girod D.A., King H. Aortic origin of the right pulmonary artery. Circulation 1967;36:777-782.[Abstract/Free Full Text]
  4. Fucci C., di Carlo D.C., Di Donato R., Marino B., Calcaterra G., Marcelletti C. Anomalous origin of the right pulmonary artery from the ascending aorta: repair without cardiopulmonary bypass. Int J Cardiol 1989;23:309-313.[Medline]
  5. Sibley Y.D., Roberts K.D., Silove E.D. Surgical correction of anomalous origin of right pulmonary artery from aorta in a four day old neonate. Br Heart J 1986;56:98-100.[Abstract/Free Full Text]
  6. Matsuda H., Zavanella C., Lee P., Subramanian S. Aortic origin of the right pulmonary artery. Ann Thorac Surg 1977;24:374-378.[Abstract]
  7. Di Eusanio G., Mazzola A., Gregorini R., et al. Anomalous origin of right pulmonary artery from the ascending aorta. J Cardiovasc Surg (Torino) 1989;30:709-712.[Medline]



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