Ann Thorac Surg 1997;64:1167-1169
© 1997 The Society of Thoracic Surgeons
Case Report
Berry Syndrome, a Complex Aortopulmonary Malformation: One-Stage Repair in a Neonate
Pietro A. Abbruzzese, MD,
Maurizio Merlo, MD,
Enrico Chiappa, MD,
Renzo Bianco, MD,
Federica Ferrero, MD,
Carlo M. Cappone, MD
Dipartimento di Cardiologia e Cardiochirurgia and Servizio di Anestesia e Rianimazione, Ospedale Infantile Regina Margherita, Torino, Italy
Accepted for publication May 3, 1997.
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Abstract
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Successful one-stage repair of a Berry syndrome (interrupted aortic arch, distal aortopulmonary septal defect, right pulmonary artery branch originating from the ascending aorta, and intact ventricular septum) in the neonatal period has been reported in only 2 cases. We report the case of a newborn operated on with deep hypothermic arrest and isolated myocardial perfusion in whom the interrupted aortic arch was corrected by direct anastomosis between the ascending and descending aorta and the aortopulmonary septal defect was treated with reconstruction of the pulmonary trunk and right pulmonary artery, using a flap of aortic tissue. A native pericardial patch was used to reconstruct the ascending aorta.
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Introduction
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Since Berry and associates' original description [1] of the rare association of distal aortopulmonary septal defect (APSD), aortic origin of the right pulmonary artery (RPA), intact ventricular septum, patent ductus arteriosus, and interrupted or hypoplastic aortic arch, scattered reports in the literature have confirmed the possibility of correcting surgically this complex malformation [2]. However, most repairs were performed in infants and older children using a staged approach. We describe a successful case of one-stage surgical correction in a neonate in whom the only prosthetic material employed was a pericardial patch.
A newborn male infant weighing 3.800 kg was referred to our hospital with severe congestive heart failure, acidosis, and anuria. The patient was immediately resuscitated, and stable hemodynamics and normal renal function were obtained over the following 8 days. Echocardiography revealed a type B interrupted aortic arch, a confluent APSD, an intact ventricular septum, and a patent ductus arteriosus; it also suggested the anomalous origin of the RPA from the right side of the ascending aorta.
Surgical correction was performed on day 8 through a median sternotomy. The external anatomy confirmed a type B interrupted aortic arch and a large APSD. Extensive dissection of the arch vessels, ascending and descending aorta, and patent ductus arteriosus was started, and it was continued after institution of cardiopulmonary bypass with a single venous cannula. The arterial cannula was precisely placed on the right side of the ascending aorta, halfway between the interrupted aortic arch and the superior border of the APSD to leave space enough above the APSD and below the interrupted aortic arch to place the aortic cross-clamp. At the start of cardiopulmonary bypass the branch pulmonary arteries were snugged. Slow descent of the temperature allowed preservation of a sinus rhythm at 15°C. The aorta was then cross-clamped distal to the aortic cannula, the arch vessels were snared, and the pump flow was lowered to 10% to perfuse only the myocardium [3], which remained slowly beating during arch repair.
The repair was performed by anastomosing end-to-side the fully mobilized descending aorta to the left posterolateral aspect of the ascending aorta, after removing all ductal tissue. After removal of the aortic cross-clamp and of the snares in the head vessels, full flow bypass was resumed and the anastomosis was checked for bleeding and undue tension. The aortic cross-clamp was reapplied proximal to the aortic cannula, cardioplegia was infused, and the APSD repair was started. The ascending aorta was transversely incised distal to the APSD, which appeared to be very large. There was a ridge between the semilunar valves only a few millimeters wide. The RPA arose from the right posterolateral aspect of the aorta, far from the APSD. The coronary arteries were normally positioned. The aortic incision was bifurcated on its right side, the upper extension straightly transecting the ascending aorta at the level of the superior aspect of the APSD. The lower extension, encircling anteriorly the orifice of the RPA, was carried posteriorly and caudally toward the aortic valve to leave a large flap of tissue surrounding the origin of the RPA. The incision was then continued on the left side of the aorta, anteriorly along the border of the APSD, finally joining the upper extension (Fig 1A
). Right pulmonary artery–main pulmonary artery continuity was then obtained using the large posterior aortic flap as the anterior wall of the RPA by baffling it to the anterior border of the APSD (Fig 1B). The large posterior defect on the proximal ascending aorta was then filled in with an autologous glutaraldehyde-treated pericardial patch. An end-to-end tension-free anastomosis was performed between the aortic stumps.
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Fig 1. . Repair of the aortopulmonary septal defect (the already accomplished ascending–descending aortic anastomosis is visible on the upper part of the drawing). (A) A large posterior flap of tissue has been obtained by carrying the posterior incision of the aorta proximally (inferiorly). (B) The aortic flap is employed to reconstruct the main pulmonary artery–right pulmonary artery continuity. The aortopulmonary septal defect represents now the origin of the right pulmonary artery.
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After rewarming, the patient was easily weaned from cardiopulmonary bypass on moderate inotropic support. Pressure measurements showed no gradients across the aortic arch or between the main pulmonary artery and the RPA. Electively delayed sternal closure was performed 4 days later. The subsequent hospitalization was uneventful, and the baby was discharged in good clinical condition on the 19th postoperative day. One year later echocardiography confirmed a good arch reconstruction without any gradient and an unobstructed pulmonary continuity (Fig 2).
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Fig 2. . (A) Parasternal short-axis view of the great vessels showing aortopulmonary septal defect reconstruction (white arrowhead) with confluent pulmonary vessels. (B) Coronal suprasternal view of ascending aorta (AAO), aortic arch, and descending aorta (DAO). Color-flow mapping shows laminar blood flow with regular profile of the vessels. (LCC = left common carotid artery; LPA = left pulmonary artery; LSA = left subclavian artery; MPA = main pulmonary artery; RPA = right pulmonary artery.)
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Comment
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The 2 other survivors of one-stage neonatal repair of a Berry syndrome underwent reestablishment of the pulmonary continuity respectively by anterior reimplantation of the RPA on the main pulmonary artery [4] and by plication of the posterior aortic wall to create a tunnel for rerouting of the pulmonary blood flow [5]. Both methods were associated with suboptimal results, as stated by the authors of those reports. We thought that reconstruction of the pulmonary bifurcation was the most critical step in the procedure and that the geometry of it would be best preserved by leaving the origin of the RPA where it was. At the same time we were less concerned about the reconstruction of the ascending aorta, which was a less demanding procedure. We therefore elected to use a method similar to that described by Kitagawa and colleagues [6], using native aortic tissue for an "in situ" reconstruction of the pulmonary continuity. We also augmented the ascending aorta with an autologous pericardial patch to allow a tension-free anastomosis. In our patient an isolated myocardial perfusion technique was used during arch repair to minimize the subsequent ischemic time, which was not predictable ahead of time. An echocardiographically based diagnosis, an adequate preoperative stabilization, and an operation directed to ward minimization of ischemic time and complete repair without residual gradients at the aortic and pulmonary levels were the keys to a successful correction of a rare and potentially lethal malformation.
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Footnotes
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Address reprint requests to Dr Abbruzzese, Divisione di Cardiochirurgia, Ospedale Infantile Regina Margherita, Piazza Polonia 96, 10126 Torino, Italy.
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References
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- Berry TE, Bharati S, Muster AJ, et al. Distal aortopulmonary septal defect, aortic origin of the right pulmonary artery, intact ventricular septum, patent ductus arteriosus and hypoplasia of the aortic isthmus: a newly recognized syndrome. Am J Cardiol 1982;49:108–16.[Medline]
- Chiu I, Wang J, Wang M, Wang C. One-stage repair of aortopulmonary septal defect and interrupted aortic arch. Ann Thorac Surg 1994;58:1529–32.[Abstract/Free Full Text]
- Sano S, Mee RBB. Isolated myocardial perfusion during arch repair. Ann Thorac Surg 1990;49:970–2.[Abstract/Free Full Text]
- 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;58:543–5.[Abstract/Free Full Text]
- Boonstra PW, Talsma M, Ebels T. Interruption of the aortic arch, distal aortopulmonary window, arterial duct and aortic origin of the right pulmonary artery in a neonate: report of a case successfully repaired in a one-stage operation. Int J Cardiol 1992;34:108–10.[Medline]
- Kitagawa T, Katoh I, Taki H, et al. New operative method for distal aortopulmonary septal defect. Ann Thorac Surg 1991;51:680–2.[Abstract/Free Full Text]
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Abstract
Introduction
