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Ann Thorac Surg 1996;61:646-650
© 1996 The Society of Thoracic Surgeons

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Original Article: Cardiovascular

Complete Repair of PA-VSD With Diminutive or Discontinuous Pulmonary Arteries by Transverse Thoracosternotomy

Departments of Cardiothoracic Surgery, and Pediatric Cardiology, University of Vienna, Vienna, Austria

Accepted for publication October 5, 1995.

	Abstract

Top Footnotes Abstract Introduction Patients and Methods Results Comment References

 
Background. Optimal treatment and the optimal sequence of surgical and interventional steps to correct pulmonary atresia with ventricular septal defect and hypoplastic or discontinuous intrapericardial pulmonary arteries is still under discussion. Collateral arteries may be hardly accessable through median sternotomy at total correction. Bilateral transsternal thoracotomy gives wide access to the heart, both pleural spaces and hilar structures.

Methods. We used this incision for total correction of pulmonary atresia with ventricular septal defect in 6 patients. Three had Blalock-Taussig shunts placed previously, and intrapericardial pulmonary arteries were absent in all patients but 1, in whom they were hypoplastic. Central pulmonary arteries were enlarged with pericardial patches or replaced with tube grafts; the number of unifocalized collateral arteries varied between two and eight.

Results. One patient died of respiratory failure and sepsis (16.7%). Oxygen saturation increased from 76% (range, 65% to 88%) preoperatively to 96% (range 91% to 99%) postoperatively. Mean postoperative pulmonary artery pressure was 30 mm Hg (range, 28 to 34 mm Hg). One patient had to be reoperated on through the same incision due to scarring and shrinkage of the peripheral anastomoses. Six months after operation 2 patients are in New York Heart Association functional class I and 2 are in class II.

Conclusions. Transverse thoracosternotomy gives excellent access to the anatomical structures necessary to correct complex cases of pulmonary atresia with ventricular septal defect and may reduce the number of surgical procedures.

	Introduction

Top Footnotes Abstract Introduction Patients and Methods Results Comment References

 
Optimal treatment of pulmonary atresia (PA) with ventricular septum defect (VSD) with hypoplastic or discontinouos pulmonary arteries and multifocal pulmonary blood supply by major aortopulmonary collateral arteries (MAPCAs) is still controversial. The wide variation of collateral blood supply, with either segmental stenosis and hypoperfusion or widely patent vessels with pulmonary overflow and the various degree of available pulmonary arteries as well as surgically placed aortopulmonary shunts, creates a unique situation in each patient and thus makes analysis of data difficult. As usual in such complex situations many techniques have been described for correction. Current options are pericardial tube substitute of pulmonary arteries and anastomoses to MAPCAs [1], primary unifocalization and systemic shunt in the case of discontinuous pulmonary arteries [2], right ventricular outflow tract patch enlargement [3], or placement of a valved [4] or nonvalved [5] conduit to hypoplastic pulmonary arteries. Others suggest a primary central shunt to enhance pulmonary artery growth [6], and various steps of unifocalization and correction with or without fenestrated VSD patch have been described. In general several surgical or interventional procedures are necessary to achieve complete repair, and sometimes preliminary thoracotomies are advised to control hardly accessible collaterals. With the introduction of sequential bilateral lung transplantation we started to use transverse bilateral thoracotomies and saw the excellent exposure of both hilar structures, the heart, and the aorta provided by this incision. It seemed logical to make use of this access for the repair of complex situations in PA-VSD with multifocal collateral pulmonary blood supply where exposure of both pleural spaces and the heart, frequently after previous thoracotomies and consecutive development of pleural adhesions, is necessary (Fig 1). This incision also may be used for total correction in a two-step regimen where newborns or infants with hypoplastic pulmonary arteries are prepared with shunting and possibly unifocalization to encourage pulmonary artery growth.

View larger version (132K): [in this window] [in a new window]   Fig 1. . Incision and situation of pulmonary blood supply in patient 4.

	Patients and Methods

Top Footnotes Abstract Introduction Patients and Methods Results Comment References

PATIENT 2.
The right lung was perfused by a widely patent vessel originating from the right descending aorta. After ligation a 13-mm PTFE graft was anastomosed to this collateral artery. The stenotic collateral feeding the left lung was mobilized and enlarged with a pericardial patch.

PATIENT 3.
Patient 3 was the only patient with a hypoplastic intrapericardial pulmonary artery. Two MAPCAs supplying the right lung, originating from the right descending aorta and entering the right hilum dorsally, were transected (Fig 2). Then they were mobilized so that they could be anastomosed to the hypoplastic right pulmonary artery (Fig 3). One MAPCA was ligated, mobilized, and anastomosed to the left pulmonary artery. The unifocalization anastomoses and the left and right pulmonary arteries were enlarged with a glutaraldehyde-tanned autologous pericardial patch.

View larger version (115K): [in this window] [in a new window]   Fig 2. . Schematic (A) and intraoperative (B) view of the collateral situation to the right hilum in patient 3. The major aortopulmonary collateral arteries have been divided at their origin from the right descending aorta.

View larger version (97K): [in this window] [in a new window]   Fig 3. . Schematic (A) and intraoperative view (B) of the technique used to achieve unifocalization in patient 3. Both collateral arteries have been mobilized and turned around hilar structures to achieve direct anastomoses to the hypoplastic right pulmonary artery. Inserts depict the stages of correction.

View larger version (363K): [in this window] [in a new window]   Fig 4. . Preoperative angiography of patient 4. The formerly translocated collateral artery was occluded and the branch pulmonary arteries were stenotic at the graft insertion.

View larger version (115K): [in this window] [in a new window]   Fig 5. . Control angiogram after total correction in patient 4. Pulmonary artery mean pressure was 34 mm Hg at that time.

PATIENT 6.
A patent shunt supplied the right pulmonary artery and a stenotic collateral to the left pulmonary artery, in which significant poststenotic dilatation developed. The left vessel was mobilized into the pericardium and the right pulmonary vessels were connected to the right ventricular outflow using a tube constructed from tanned autologous pericardium.

Postoperative Evaluation
All patients underwent a postoperative cardiac catheterization 4 weeks to 3 months postoperatively. They were seen on a regular basis in the outpatient department, where clinical examinations and echocardiograms were done.

	Results

Top Footnotes Abstract Introduction Patients and Methods Results Comment References

 
One patient died perioperatively (operative mortality, 16.7%). This patient (patient 5) with complex pulmonary blood supply had undergone bilateral shunting procedures before. She had systolic pulmonary artery pressure at the systemic level after weaning from cardiopulmonary bypass. As a consequence focal pulmonary edema developed in the following days, and extracorporeal membrane oxygenation was necessary to treat the ensuing respiratory failure. She could be weaned from extracorporeal support but died of septic pericarditis 2 weeks after operation. The postoperative pulmonary artery mean pressure of the survivors measured at control angiography was 30 mm Hg (range, 28 to 34 mm Hg). Arterial oxygen saturation increased from 76% (range, 65% to 88%) preoperatively to 96% (range 91% to 99%) postoperatively. In patient 4 progressive stenoses developed at the patched anastomoses. Suprasystemic right ventricular pressures caused right heart decompensation, and she was reoperated on 12 months after total correction. Again transverse thoracosternotomy was used. To avoid complete dissection of lungs and heart, thoracotomy was performed one intercostal space higher at reoperation. Anastomoses to the right pulmonary branches, to the left pulmonary artery and to the dorsally routing vessel supplying the right lower lobe were enlarged with bovine pericardium. Two patients are in New York Heart Association functional class I, 2 are in class II, and the patient after reoperation is in class III 2 months after operation.

	Comment

Top Footnotes Abstract Introduction Patients and Methods Results Comment References

 
Correction of PA-VSD with multifocal pulmonary blood supply usually requires several steps for surgical correction. Cumulative mortality ranges from 16% to 45% [4–7], and of course multiple surgical procedures are expensive and cause additional morbidity and inconvenience for the patient. The standard access via a median sternotomy gives only limited exposure of dorsally routing collateral arteries. Thus in certain cases preliminary thoracotomies for control of collateral arteries are advised [4]. Palliative unifocalizations are performed through thoracotomies in such a way that the newly constructed pulmonary arteries will be accessable through median sternotomy at total correction [1]. In contrast, surgical access gained by the transverse thoracotomy allows dissection and transposition of MAPCAs in both sides of the chest and the mediastinum and provides exposure for intracardiac repair. In cases of failed previous shunting pleural adhesions can be lysed under optimal conditions. Due to the good exposure and the possibility of applying bulldog clamps or occluding catheters to the pulmonary vessels, circulatory arrest can be avoided. We think that this approach is suitable to reduce the number of procedures for correction of complex cases, mainly when mediastinal pulmonary arteries are absent or occluded after previous procedures. Preparing infants or newborns for total repair, however, we prefer a two-step regimen with unifocalization and shunting on one side to encourage growth of the pulmonary arteries. If possible, total correction should be postponed until adult-sized conduits can be implanted.

Manipulation and reperfusion of previously hypoperfused lung segments usually causes focal pulmonary edema and reduced ventilatory function [1]; this is seen also in patients after pulmonary endarterectomy [8]. In cases of borderline pulmonary artery size or complicated collateral supply, unilateral unifocalization may be the primary procedure of choice to avoid severe respiratory failure as we observed in patient 5.

Glutaraldehyde-fixed autologous pericardium is a well-accepted patch material in the pulmonary vasculature and is technically easier to handle than PTFE patches. In 1 patient (patient 5) we observed a progressive narrowing of the patch-enlarged anastomoses by scarring of the implanted pericardium. The ideal substitute for replacement of pulmonary arteries in growing individuals remains to be determined.

We could not find a disadvantage of the clam-shell incision. The theoretic concern of reduced pulmonary function caused by this more painful thoracotomy incision compared with midline sternotomy is outweighed by the better surgical exposure and the option of a reduced number of surgical procedures necessary. Generally patients after correction of this malformation have to be on mechanical ventilation for a few days due to pulmonary reasons, so they awaken when incisional pain has faded away already. We did not observe any healing problems in our patients and could not find an increased rate of such complications in the immunosuppressed group of lung transplant recipients either.

Bilateral transsternal thoracosternotomy provides a very good exposure for correction of complicated PA-VSD. In our series we could not find any disadvantage of this incision, and besides facilitating operation by the bilateral access, the number of surgical steps necessary for correction may be reduced.

	Footnotes

Top Footnotes Abstract Introduction Patients and Methods Results Comment References

	References

Top Footnotes Abstract Introduction Patients and Methods Results Comment References

 

1. Permut LC, Laks H. Surgical management of pulmonary atresia with ventricular septal defect and multiple aortopulmonary collaterals. Adv Cardiac Surg 1994;5:75–95.[Medline]
2. Shanley CJ, Lupinetti FM, Shah NL, Beekman RH, Crowley DC, Bove EL. Primary unifocalization for the absence of intrapericardial pulmonary arteries in the neonate. J Thorac Cardiovasc Surg 1993;106:237–47.
3. Haas GS, Laks H, Milgalter E. Pulmonary atresia with ventricular septal defect. Cardiac Surg 1989;3:425–43.
4. Castañeda AR, Mayer JE, Lock JE. Tetralogy of Fallot, pulmo-nary atresia, and diminutive pulmonary arteries. Prog Pediatr Cardiol 1992;1:50–60.
5. Puga FJ. Surgical treatment of pulmonary atresia and ventricular septal defect. Prog Pediatr Cardiol 1992;1:37–49.
6. Iyer KS, Mee RBB. Staged repair of pulmonary atresia with ventricular septal defect and major systemic to pulmonary artery collaterals. Ann Thorac Surg 1991;51:65–72.[Abstract]
7. Ziemer G, Kaulitz R, Karck M, Luhmer I. Offene Herzchirurgie bei Fallot Tetralogie mit Pulmonalatresie: Palliation und Korrektur. Acta Chir Austriaca 1993;25:91–5.
8. Levinson RM, Shure D, Moser KM. Reperfusion pulmonary edema after pulmonary artery thrombendarterectomy. Am Rev Respir Dis 1986;134:1241–5.[Medline]

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This Article Abstract Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Anton Moritz Gregor Wollenek Ernst Wolner Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Moritz, A. Articles by Wolner, E. Search for Related Content PubMed PubMed Citation Articles by Moritz, A. Articles by Wolner, E.