HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS		QUICK SEARCH:   [advanced] Author: Keyword(s): Year:  Vol:  Page:

This Article Abstract Full Text (PDF) 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): Kotturathu M. Cherian Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Murthy, K. S. Articles by Cherian, K. M. Search for Related Content PubMed PubMed Citation Articles by Murthy, K. S. Articles by Cherian, K. M.

Ann Thorac Surg 1999;67:760-764
© 1999 The Society of Thoracic Surgeons

---

Original Articles

Evolving surgical management for ventricular septal defect, pulmonary atresia, and major aortopulmonary collateral arteries

^a Institute of Cardiovascular Diseases, Madras Medical Mission, Chennai, India

Accepted for publication August 31, 1998.

Address reprint requests to Dr Murthy, Institute of Cardiovascular Diseases, Madras Medical Mission, 4A, Dr J J Nagar, Mogappair, Chennai-600 050, India

	Abstract

Top Abstract Introduction Material and methods Results Comment Acknowledgments References

 
Background. The purpose of this study was to evaluate the results of various surgical modalities that have been evolving for the treatment of ventricular septal defect, pulmonary atresia, and major aortopulmonary collateral arteries.

Methods. From 1993 to May 1997, 14 patients (group 1) were treated with staged unifocalization through thoracotomies and final repair by midsternotomy. From June 1997 to February 1998, 10 patients (group 2) were treated with midsternotomy, single-stage complete unifocalization, and repair.

Results. In group 1, 14 patients had 21 procedures (1.5 procedures per patient), of which 3 patients (21%) had final correction. There were two deaths (14%). One patient died of blocked shunt. Another patient who had aneurysmal dilation of homograft tubes that were used for unifocalization died after final repair because of low cardiac output. In group 2, 10 patients had ten surgical procedures for complete unifocalization and 9 of 10 (90%) of them achieved final correction. One patient with low cardiac output in whom we did not close the ventricular septal defect died (10%) of suprasystemic right ventricular pressure.

Conclusion. In single-stage complete unifocalization, more patients had final correction. It reduces the number of operations and hospitalization and hence is more cost effective than multistaged procedures.

	Introduction

Top Abstract Introduction Material and methods Results Comment Acknowledgments References

 
One of the most challenging groups of diseases to treat surgically is pulmonary atresia, ventricular septal defect (VSD), and major aortopulmonary collateral arteries (MAPCAs). In the initial study period patients were treated with conventional multistage unifocalization of pulmonary blood supply through thoracotomies, followed by final repair through sternotomy if anatomy is suitable [1–3]. Recently an aggressive approach of single-stage unifocalization and complete repair through median sternotomy has been described [4, 5]. We report our experience with evolving surgical treatment of this anomaly.

	Material and methods

Top Abstract Introduction Material and methods Results Comment Acknowledgments References

 
From 1993 to February 1998, 24 patients with VSD, pulmonary atresia, and MAPCAs were treated surgically in our institute. They were divided into two groups. Group 1 (from 1993 to May 1997) comprised 14 patients who were treated with multistaged unifocalization, and group 2 (from June 1997 to February 1998) comprised 10 patients treated with median sternotomy and single-stage unifocalization and repair. The patient demographics of the two groups are given in Table 1. The diagnosis was made by echocardiography and confirmed by car-diac catheterization and angiocardiography with selective delineation of MAPCAs.

Surgical technique
In both groups our aim was to establish a single source of blood supply to the lungs from pulmonary arteries and the MAPCAs and promote their growth. Even though lung has dual supply, if the MAPCAs were large in size (more than 2 mm) they were unifocalized to bring as many arteries as possible for pulmonary blood supply. Smaller MAPCAs (less than 2 mm) were ligated.

Multistage unifocalization and repair
In group 1, the initial operation was performed through posterolateral thoracotomy, and a conduit was established from the subclavian artery to its native PA and MAPCAs. The following methods were used to unifocalize the MAPCAs. End-to-side anastomosis of the polytetrafluoroethylene tube to native pulmonary artery and side-to-side or end to side anastomosis of the MAPCAs were done as described by Iyer and Mee [1] in 11 patients (Fig 1). End-to-end or end-to-side anastomosis of the polytetrafluoroethylene tube to the unifocalized pericardial tube was done as described by Marelli and coworkers [2] in 2 patients (Fig 2) or to the antibiotically sterilized homograft as described by us [6] in one patient (Fig 3). The same procedure was repeated on the other side via posterolateral thoracotomy, after 3 months to 1 year of initial procedure. The definitive repair was done after 1 or 2 years of complete unifocalization. The final correction was done using cardiopulmonary bypass with ligation of shunts, closure of VSD, and RV to PA homograft conduit.

View larger version (90K): [in this window] [in a new window]   Fig 1. Staged unifocalization and modified Blalock-Taussig shunt with Gore-Tex (polytetrafluroethylene) graft. (A) Preoperative angiogram. (B) Postoperative angiogram. (C and D) Diagram of both procedures.

View larger version (25K): [in this window] [in a new window]   Fig 2. Preoperative (A) and postoperative (B) diagrams of staged unifocalization with a pericardial tube and subclavian artery to pericardial tube shunt with a polytetrafluroethyelene graft.

View larger version (106K): [in this window] [in a new window]   Fig 3. Staged bilateral unifocalization with tailored homograft conduit and modified Blalock-Taussig shunt with polytetrafluroethyelene graft. (A) Preoperative angiogram. (B) Postoperative angiogram. (C and D) Diagram of both procedures.

View larger version (105K): [in this window] [in a new window]   Fig 4. Single-stage unifocalization. (A) Preoperative angiogram. (B) Postoperative angiogram. (C and D) Diagram of preoperative and postoperative conditions.

	Results

Top Abstract Introduction Material and methods Results Comment Acknowledgments References

 
In group 1, 14 patients had 21 operations (1.5 procedures per patient) in which only 3 patients had complete repair. There were two deaths, one was from blocked shunt in which the pericardial tube was used to unifocalize MAPCAs, and the other death was in a patient in whom bilateral unifocalization was done using tailored homograft conduits [6]. She developed aneurysmal dilatation of homograft tubes (Fig 3). She had complete repair after 1 year of bilateral complete unifocalization. Right ventricular pressure was suprasystemic after completion of repair, so the VSD patch was removed. Even after stiff inotropic supports and supportive bypass, the patient could not be weaned from cardiopulmonary bypass and finally succumbed. The two patients who survived complete repair had echocardiogram, cardiac catheterization, and angiocardiogram; RV-to-LV pressure ratio was 0.54 and 0.92 in these patients.

In group 2, each patient had one operation. All patients had complete unifocalization and repair, except 1 patient in whom the VSD was left open because of suprasystemic RV pressure. This patient died on the seventh postoperative day because of low cardiac output and multiorgan failure. Echocardiography, cardiac catheterization, and angiocardiography were done in all patients; all showed good placement of conduits with pulmonary arteries. The RV-to-LV pressure ratios were from 0.3 to 0.9 (mean, 0.63) (Table 2).

	Comment

Top Abstract Introduction Material and methods Results Comment Acknowledgments References

 
From 1993 to May 1997 (4.5 years), 14 patients (group 1) were treated with multistage procedures. Ten patients had first-stage palliative unifocalization, 4 had bilateral unifocalization, and only 3 had complete repair. Whereas from June 1997 to February 1998 (8 months), 10 patients (group 2) had single-stage complete unifocalization and repair, of which the VSD was left open in 1 patient. With either approach our aim was to intervene as early in life as possible to establish normal cardiovascular physiology. The reason that there are fewer cases in group 1 is that most parents of these children were not willing to agree to multiple-staged procedures with uncertain final outcome. It is very expensive to have multiple operations, and some of them did not return for the second unifocalization and final correction. We observed that antibiotic-treated homografts that were used for staged unifocalization of MAPCAs eventually developed aneurysmal dilatation (Fig 3). Other problems of staged procedures include the following: patients remain symptomatic and saturations do not improve after unifocalization until the final correction is done. At the time of final repair, the posterior mediastinum and hilar regions were significantly scarred, with development of secondary collaterals, which increases the risk of bleeding and the surgical risk. These reasons led us to choose single-stage unifocalization and repair. Although our study is not a prospective randomized double-blind study, nonetheless, it directly compares different surgical approaches to this complex lesion.

In group 1, 14 patients had 21 operations (1.5 operations per patient) with 3 of 14 (21%) obtaining final correction. Two patients (14.3%) who had staged unifocalization died. Previously published reports showed that a median of three surgical procedures (range, two to six) were required before complete repair was attained. Multiple-staged procedures resulted in complete repair in 11.5% to 60.5% of patients. The overall mortality was from 10.2% to 19.2% [1–3]. Although this approach has not been universally successful, it has offered some hope in an otherwise hopeless situation.

In group 2, 10 patients had complete unifocalization and repair in 10 procedures. In one patient, VSD was left open because of suprasystemic RV pressure. This patient died on the seventh postoperative day as a result of low cardiac output. Our results are comparable to the results obtained by Reddy and associates [5], who achieved complete repair in 90% of cases. None of our patients had other complications, such as phrenic palsy or bronchospasm, which might result from our strict policy of limited dissection along the aorta and of obtaining a sufficient length of MAPCAs to bring them to the transverse sinus.

In conclusion, we believe that in single-stage unifocalization and repair, more patients will have complete repair at an earlier age. It reduces the number of operations and hospitalization and thereby is less expensive than multistage unifocalization. Approaching the MAPCAs through midline sternotomy is safe and reproducible. We currently prefer single-stage unifocalization and repair through sternotomy rather than multistage procedures.

	Acknowledgments

Top Abstract Introduction Material and methods Results Comment Acknowledgments References

 
We thank Mr Dhinakar Seetharaman, MVS, for help in preparation of the manuscript.

	References

Top Abstract Introduction Material and methods Results Comment Acknowledgments References

 

1. Iyer K.S., Mee R.B.B. Staged repair of pulmonary atresia with ventricular septal defect and major systemic to pulmonary artery collaterals. Ann Thorac Surg 1991;51:65-72.[Abstract/Free Full Text]
2. Marelli A.J., Perloff J.K., Child J.S., Laks H. Pulmonary atresia with ventricular septal defects in adults. Circulation 1994;89:243-251.[Abstract/Free Full Text]
3. Puga F.J., Leoni F.E., Julsrud P.R., Mau D.D. Complete repair of pulmonary atresia, ventricular septal defect and severe peripheral arborization abnormalities of central pulmonary arteries. J Thorac Cardiovasc Surg 1989;98:1018-1029.[Abstract]
4. Shanley C.J., Lupinetti F.M., Shah N.L., Beckman R.H., Crowley D.C., Bove E.L. Primary unifocalization for the absence of intrapericardial pulmonary arteries in the neonate. J Thorac Cardiovasc Surg 1993;106:237-247.[Abstract]
5. Reddy V.M., Liddicoat J.R., Hanley F.L. Midline one-stage complete unifocalization and repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals. J Thorac Cardiovasc Surg 1995;109:832-845.[Abstract]
6. Hamid I.A., Rao G.S., Bharati S.A., Cherian K.M. Unifocalization of major aortopulmonary collateral arteries in pulmonary atresia and ventricular septal defect using antibiotic sterilized pulmonary homograft: a case report. J Cardiac Surg 1994;9:115-119.[Medline]

This article has been cited by other articles:

				Endorsed by the Association for European Paediatri, Authors/Task Force Members, H. Baumgartner, P. Bonhoeffer, N. M. S. De Groot, F. de Haan, J. E. Deanfield, N. Galie, M. A. Gatzoulis, C. Gohlke-Baerwolf, et al. ESC Guidelines for the management of grown-up congenital heart disease (new version 2010): The Task Force on the Management of Grown-up Congenital Heart Disease of the European Society of Cardiology (ESC) Eur. Heart J., December 1, 2010; 31(23): 2915 - 2957. [Full Text] [PDF]

				K. M. Amark, T. Karamlou, A. O'Carroll, C. MacDonald, R. M. Freedom, S.-J. Yoo, W. G. Williams, G. S. Van Arsdell, C. A. Caldarone, and B. W. McCrindle Independent Factors Associated With Mortality, Reintervention, and Achievement of Complete Repair in Children With Pulmonary Atresia With Ventricular Septal Defect J. Am. Coll. Cardiol., April 4, 2006; 47(7): 1448 - 1456. [Abstract] [Full Text] [PDF]

				K. J. Roche, R. Rivera, M. Argilla, N. R. Fefferman, L. P. Pinkney, H. Rusinek, and N. B. Genieser Assessment of Vasculature Using Combined MRI and MR Angiography Am. J. Roentgenol., April 1, 2004; 182(4): 861 - 866. [Abstract] [Full Text] [PDF]

				M. Griselli, S. P. McGuirk, D. S. Winlaw, O. Stumper, J. V. de Giovanni, P. Miller, R. Dhillon, J. G. Wright, D. J. Barron, and W. J. Brawn The influence of pulmonary artery morphology on the results of operations for major aortopulmonary collateral arteries and complex congenital heart defects J. Thorac. Cardiovasc. Surg., January 1, 2004; 127(1): 251 - 258. [Abstract] [Full Text] [PDF]

				B. W. Duncan, R. B. B. Mee, L. R. Prieto, G. L. Rosenthal, C. I. Mesia, A. Qureshi, O. P. Tucker, J. F. Rhodes, and L. A. Latson Staged repair of tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries J. Thorac. Cardiovasc. Surg., September 1, 2003; 126(3): 694 - 702. [Abstract] [Full Text] [PDF]

				J. M. Cho, F. J. Puga, G. K. Danielson, J. A. Dearani, D. D. Mair, D. J. Hagler, P. R. Julsrud, and D. M. Ilstrup Early and long-term results of the surgical treatment of tetralogy of Fallot with pulmonary atresia, with or without major aortopulmonary collateral arteries J. Thorac. Cardiovasc. Surg., July 1, 2002; 124(1): 70 - 81. [Abstract] [Full Text] [PDF]

				K. S. Murthy, S. Krishnanaik, R. Coelho, A. Punnoose, S. B. Arumugam, and K. M. Cherian Median sternotomy single stage complete unifocalization for pulmonary atresia, major aorto-pulmonary collateral arteries and VSD-early experience Eur J Cardiothorac Surg, July 1, 1999; 16(1): 21 - 25. [Abstract] [Full Text] [PDF]

This Article Abstract Full Text (PDF) 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): Kotturathu M. Cherian Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Murthy, K. S. Articles by Cherian, K. M. Search for Related Content PubMed PubMed Citation Articles by Murthy, K. S. Articles by Cherian, K. M.