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): Gary K. Lofland Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Lofland, G. K. Search for Related Content PubMed PubMed Citation Articles by Lofland, G. K.

Eur J Cardiothorac Surg 2000;18:480-486
© 2000 Elsevier Science NL

The management of pulmonary atresia, ventricular septal defect, and multiple aorta pulmonary collateral arteries by definitive single stage repair in early infancy

The Children's Mercy Hospital, University of Missouri Kansas City, 2401 Gillham Road, Kansas City, MO 64108, USA

Received 6 September 1999; received in revised form 26 May 2000; accepted 26 July 2000.

Tel.: +1-816-234-3580; fax: +1-816-460-1012

	Abstract

Top Abstract 1. Introduction 2. Methods 3. Results 4. Discussion Appendix A. Conference... References

 
Objective: The management of infants and children with pulmonary atresia, ventricular septal defect, and multiple aorta pulmonary collateral arteries (PA/VSD/MAPCA) has proven to be challenging. Therapeutic approaches have included staged unifocalization, shunting, coiling of collateral vessels, and heart/lung transplantation. Results have been variable and frustrating. Hoping to take advantage of growth potential in pulmonary segments supplied by MAPCA, a more radical approach was adopted in March of 1997. This consists of single stage complete unifocalization with closure of the ventricular septal defect and establishment of right ventricular to pulmonary arterial continuity with a cryopreserved pulmonary allograft (Rastelli type correction) through a midline sternal incision. Methods: During an 18-month period, eleven consecutive infants with PA/VSD/MAPCA underwent complete surgical correction. The ages ranged from 5 days to 5 months. Weights ranged from 2.2 to 5.6 kg. Through a standard median sternotomy incision, the pericardium and both pleural spaces were opened. Normothermic cardiopulmonary bypass was instituted. Section of all collaterals was accomplished without hypoxemia, and all collaterals were ligated at their origin from the aorta. They were then brought through posterior mediastinum to construct a pulmonary artery confluence. The ventricular septal defect was closed, and continuity was established between the right ventricle and the newly created pulmonary artery confluence with cryopreserved allografts. Results: Ten of 11 patients survived operation, with postoperative courses that were uncomplicated. Length of stay ranged from 7–16 days, with a median length of stay of 11 days. One perioperative death occurred in a patient with preoperative co-morbidities of necrotizing enterocolitis, with no functioning gastrointestinal tract, intraventricular hemorrhage, and ventilator dependency since birth. At angiography, this patient has no demonstrable central pulmonary arteries and multiple diminutive aorta pulmonary collaterals. Autopsy revealed no demonstrable pulmonary arteries within the pulmonary parenchyma. All patients have been followed closely, and have grown normally. Two patients undergone repeat cardiac catheterization because of the echocardiographic demonstration of right ventricle pressures that had exceeded 50% of systemic. Both patients were treated with balloon angioplasty and one of these patients has had stenting of stenotic pulmonary arterial segments. No other patients have required additional hospitalization. Right ventricular pressures have remained less than fifty percent of systemic by echocardiographic assessment in all other patients. Conclusions: We feel that a single stage correction of pulmonary atresia, ventricular septal defect, and multiple aorta pulmonary collateral arteries can be accomplished in early infancy with acceptable morbidity and mortality. The initiation of normothermic cardio-pulmonary bypass greatly facilitates dissection of collaterals and prevents hypoxemia. Interventional cardiology with balloon angioplasty and stenting of abnormal pulmonary arterial segments is both an important essential adjunct in the management of these patients. We remain optimistic that a single stage approach to this complex lesion coupled with cardiac catheterization, balloon angioplasty, and stenting, will provide long-term results superior to staged approaches.

Key Words: Pulmonary atresia • Pulmonary atresia with ventricular septal defect • Major aorta pulmonary collaterals • Unifocalization

	1. Introduction

Top Abstract 1. Introduction 2. Methods 3. Results 4. Discussion Appendix A. Conference... References

 
Pulmonary atresia (PA) with ventricular septal defect (VSD) and major aorta pulmonary collaterals (MAPCA) is a complex and rare lesion in which considerable morphologic variability exists regarding the sources of pulmonary blood flow. The true central pulmonary arteries range from a size approaching normal to complete absence. Major aorta pulmonary collaterals, probably derived embryologically from the splanchnic vascular plexus [1], are also highly variable in their size, number, course, origin, arborization, and histopathologic makeup [2–5]. A segment of lung may be supplied solely from the true pulmonary arteries, solely from the aorta pulmonary collaterals, or from both with connections between the two sources occurring at central or peripheral points and at single or multiple sites [4]. By contrast the intracardiac component of this defect is usually relatively straightforward, usually with a large anteriorly malaligned ventricular septal defect, a conotruncal type defect, well developed right and left ventricles with appropriate atrioventricular valves, and normal atrioventricular and ventriculoarterial connections.

The ultimate goal of surgical therapy is a biventricular correction. Definitive correction should include closure of the ventricular septal defect, closure of any interatrial communications, and establishment of continuity between a constructed pulmonary artery confluence and a right ventricle [6–9]. This has necessitated a series of staged unifocalizations in an attempt to build pulmonary arteries, followed by establishment of right ventricular to pulmonary artery continuity, with or without closure of the ventricular septal defect. This approach has generally required on average, approximately three separate operations [6–8]. Other individuals have taken a more aggressive approach in an attempt to achieve a single stage correction [10].

An important physiologic component of a favorable outcome is the post repair peak right ventricular pressure [6,11]. While obviously this should be low, it may depend greatly upon the pulmonary arterial and MAPCA morphology. The natural history of major aorta pulmonary collaterals includes progressive stenosis and occlusion, sometimes making any segment of lung supplied by these collaterals inaccessible or unusable if incorporated into definitive correction. Manipulation of these aorta pulmonary collaterals through attempts of unifocalization can produce iatrogenic occlusion in the form of scarring or anastomotic stenoses. Major aorta pulmonary collaterals without obstruction can lead to pulmonary vascular obstructive disease in the segment supplied by these collaterals. A single stage correction in early infancy before stenoses can occur, and before irreversible pulmonary vascular obstructive disease can occur, would logically be an ideal strategy for managing this otherwise frustrating condition. The earlier in the life that the greatest number of healthy lung segments can be incorporated into the unifocalized pulmonary circulation, the more likely one is to capitalize upon the tremendous potential for additional growth and development of the pulmonary circulation and parenchyma that exists in early infancy.

	2. Methods

Top Abstract 1. Introduction 2. Methods 3. Results 4. Discussion Appendix A. Conference... References

 
Beginning in March of 1997 eleven consecutive infants with pulmonary atresia, ventricular septal defect and multiple pulmonary collateral underwent a single staged definitive correction. All diagnoses were established at birth or shortly thereafter, and all patients underwent cardiac catheterization at the time of initial diagnosis. Ages ranged from 5 days to 5.5 months with a mean and median age of three months. Weights ranged from 2.2 to 5.6 kg with a mean and median weight of 3 kg. None of the patients had undergone previous cardiac surgical procedures. The diagnosis was suggested by echocardiography and established definitively by cardiac catheterization with selective angiocardiography. All collaterals were identified by selective angiography (Figs. 1 and 2) .

View larger version (38K): [in this window] [in a new window]   Fig. 1. Angiogram and schematic illustration of collateral vessels.

View larger version (17K): [in this window] [in a new window]   Fig. 2. Schematic illustration of collateral vessels in two patients demonstrating anatomic and morphologic variability.

If the diagnosis was made in the newborn period, the patient was discharged shortly after birth and the repair was done electively in the first 2–3 months of life. One patient in whom major collaterals existed was rapidly developing severe unrelenting congestive heart failure and underwent complete correction at 5 days of age. A second patient was scheduled for repair at 2–3 months of age, but postponement was necessary because of respiratory syncytial viral infections. If patients had diminutive collaterals and central pulmonary arterial segments on initial cardiac catheterization, a second cardiac catheterization was performed at 2–3 months of age to assess growth of both collaterals and central pulmonary arterial segments. All patients had a single ventricular septal defect all of the malalignment type. All patients had atrial septal defects.

2.1. Technique
Through a standard midline incision, a median sternotomy and a subtotal thymectomy were performed. Both pleural spaces were opened widely just beneath the sternum and well anterior to the phrenic nerve. An attempt was made to dissect the collaterals prior to establishing cardio-pulmonary bypass in all patients but was not successful in identifying all of the collaterals in any of the patients. Consequently, the strategy rapidly evolved of establishing normothermic cardio-pulmonary bypass with dual caval cannulation to allow for complete cardio-pulmonary decompression. This simple maneuver allowed for a much easier identification and dissection of collaterals, as both the heart and lungs could be manipulated to achieve better exposure of the posterior mediastinum. The posterior pericardium was incised between the superior vena cava and the aorta and the floor of the pericardial reflection and the transverse sinus was opened and the posterior mediastinal soft tissues dissected to expose the aortic segments and the collaterals in this region. The presence of a nasogastric tube or a transesophageal echocardiographic probe also facilitated identification of posterior mediastinal structures. Opening the posterior mediastinal space also allowed for an avenue for rerouting collaterals so that the collaterals could be dissected along their entire length and all anastomoses accomplished under no tension. As the collaterals were very tortuous and extremely friable with consistently poor tissue quality, the dissection along their length was accomplished very carefully, to avoid disruption of the adventitia. The tortuousity of the collaterals, however, contributed to the ability to perform an anastomosis of the collaterals to either native pulmonary artery or pulmonary allograft without any tension on the length of the collaterals or the suture line. All collaterals were then permanently ligated at their origin, from the aorta usually using a single vascular clip. Since all collaterals were ligated while on cardio-pulmonary bypass, oxygenation was never in question. Following ligation, all collaterals were transected and unifocalization was accomplished with tissue to tissue anastomoses. Collaterals could be anastomosed to other collaterals or to the native pulmonary artery. Throughout the unifocalization process emphasis was on avoiding any synthetic or allograft materials in the periphery, and none were used in these patients. All anastomoses were accomplished using 7-0 Prolene suture on a fine needle (Ethicon, Somerset, NJ). Collaterals were anastomosed in the following manner:

1. Side to side anastomosis of the collateral to the central pulmonary arteries thereby augmenting the hypoplastic pulmonary arteries.
   
2. Side to side anastomosis of collateral to peripheral native pulmonary artery.
   
3. End to side anstomosis of collateral to collateral or collateral to native pulmonary artery.
   

An attempt was always made to bring as many collaterals as possible into as central a location as possible in an attempt to reconstruct a pulmonary artery confluence. Of note is that upon transection of the collateral at its origin, very little back bleeding was encountered. Clamping of the collateral itself was rarely if ever necessary.

Upon completion of unifocalization, the patient was then cooled to 22°C. The aorta was then cross-clamped and cardioplegic solution was given. A longitudinal ventriculotomy was then made in the right ventricular infundibulum and resection of hypertrophic muscle bundles was accomplished. The VSD was then closed with a Dacron sauvage patch with a continuous 5-0 Prolene suture. The right atrium was opened to close the atrial septal defect, leaving a small patent foramen ovale. It was elected to leave a patent foramen to function as a ‘pop-off’ valve for systemic venous blood in the event of right ventricular dysfunction or elevated right-sided pressures. At this stage rewarming commenced and the aortic cross-clamp was released. A cryopreserved pulmonary allograft was used in all cases to connect the right ventricle to the reconstructed neopulmonary arterial system. The distal conduit was anastomosed to the reconstructed pulmonary arterial confluence using 6-0 Prolene. The proximal anastomosis was accomplished with 5-0 Prolene and if necessary, a proximal extension utilizing expanded polytetrafluoroethylene (PTFE). The right atrium was then closed. Upon completion of rewarming, the patient was weaned from cardio-pulmonary bypass. The duration of cardio-pulmonary bypass ranged from 119 to 160 min with a mean of 138 min. The duration of the aortic cross clamping was from 25 to 58 min with a mean of 32 min. Modified ultrafiltration was performed in all patients. Transesophageal echocardiography was performed in all patients to assess the integrity of the intracardiac repair, and to obtain an echocardiographic assessment of right-sided pressures. Transthoracic pressure monitoring lines were not utilized in any patient. Bilateral pleural and mediastinal tubes were placed and the sternum was closed. Nitric oxide was available for all patients in the event right-sided pressures were unacceptably high, but was utilized in only one.

2.2. Follow-up
At discharge, all patients had echocardiography to assess right ventricular performance and to assess right-sided pressures. Additional follow-up was obtained within 1–2 weeks of discharge and at monthly intervals for the next 6 months. Follow-up evaluation has been achieved in all patients by direct patient contact.

	3. Results

Top Abstract 1. Introduction 2. Methods 3. Results 4. Discussion Appendix A. Conference... References

 
3.1. Early results
Ten of 11 patients survived the initial operation. The length of stay postoperatively ranged from 7–16 days, with a median length of stay of approximately 11 days. Duration of mechanical ventilation postoperatively was 14–36 h, with a median duration of mechanical ventilation of approximately 18 h.

Minimal inotropic support (dopamine 5 µg/kg per min) was required for a median duration of 2 postoperative days. Intravenous nitroglycerine and nitroprusside were used in doses of 0.3 µg/kg per min. No patients required delayed sternal closure. Nitric oxide was available on standby for all patients, but was utilized for only one patient. The patient in whom Nitric oxide was utilized was the one perioperative death that occurred in the series.

The one patient who expired had surgery performed at 2.5 months of age. He had been intubated and mechanically ventilated since birth. He had experienced comorbidity of an intraventricular hemorrhage and severe necrotizing enterocolitis. He had been maintained on total parental nutrition since birth. At cardiac catheterization his aorta pulmonary collateral segments were multiple and diminutive. No central pulmonary arteries were demonstrable by cardiac catheterization. At autopsy, he had no demonstrable pulmonary arteries within the pulmonary parenchyma.

No patients experienced phrenic nerve paralysis and no chylothoraces occurred. At discharge, all patients underwent echocardiography. All patients had right ventricular pressures less than 50% of systemic. No patients had residual ventricular septal defects, and there was no more than trivial tricuspid regurgitation in all patients. At discharge, the arterial oxygen saturation in all patients was greater than 95% on room air. No patients required supplemental oxygen following discharge.

3.2. Late results
All patients have been followed at regular intervals. Two patients have gradually evolved right ventricular pressures of greater that 50% of systemic, and have undergone repeat cardiac catheterization. Both of these patients had stenotic pulmonary arterial segments treated with balloon angioplasty with good results (Fig. 3) . One patient required stenting. All patients have continued to experience normal growth and development. Of note is that no patients have required rehospitalization for any cardiac or non-cardiac causes, despite a particularly virulent season with respiratory syncytial virus. No patients in this series have subsequently developed bronchopulmonary dysplasia.

View larger version (87K): [in this window] [in a new window]   Fig. 3. AP and lateral angiograms at 14 months post repair. The patient underwent balloon angioplasty of the right pulmonary artery with reduction in right ventricular pressure to 40% systemic.

	4. Discussion

Top Abstract 1. Introduction 2. Methods 3. Results 4. Discussion Appendix A. Conference... References

Pulmonary atresia with ventricular septal defect and major aorta pulmonary collaterals is uncommon however comprising only about 25% of all cases of pulmonary atresia and ventricular septal defect [6]. In this defect there is an absolute deficiency of central pulmonary artery, with most of the pulmonary parenchyma being supplied by major aorta pulmonary collaterals. The systemic hemodynamic and morphologic studies of the collateral supply in this lesion have been accomplished by McCartney and Associates [19], Haworth [20], Haworth and colleagues [21], Thiene and co-workers [22], and Rabinovitch and associates [5]. The concept of unifocalization of multiple sources of vascular supply to the lungs in an attempt to establish a pulmonary arterial confluence was suggested by Haworth and McCartney [4].

The results with the staged approach of unifocalization have been extremely variable [6–9,23]. In all series reviewed, total repair was accomplished in 12–60% of patients. These series also do not take in to account attrition occurring prior to surgical intervention, or exclusion of patients who are deemed unsuitable for any intervention. It is felt that only 20–30% of a cohort of newborn infants with this combination of anomalies will have complete repair with acceptable right ventricular hemodynamics if a delayed stage approach is taken [10].

In 1995, Reddy, Liddicoat and Hanley demonstrated that by using a one-stage approach through a mid-line sternal incision, it was possible to achieve a complete correction [10]. In their series of ten patients ranging in age from 1.43 months to 37.34 years, complete correction was achieved in all patients. Although delayed closure of the ventricular septal defect was felt to be occasionally necessary, it was felt by these investigators that definitive correction could be accomplished at an early age. These investigators speculated that ideally, correction should be accomplished between 3 and 6 months of age. In the present series, both the mean and median age of correction was 3 months as opposed to a median age of correction of 2.08 years and a mean age of correction of 5.96 years.

Although Nakata index has been discussed extensively [24], we did not employ Nakata index in this series of patients. It was our feeling the definitive repair could be accomplished at quite an early age; consequently, no patients who where referred were excluded.

Some authors have advocated bilateral thoracotomies with a transsternal (clamshell) approach [25]. We did not find it necessary to utilize this approach. We also did not find it necessary to modify in any way our standard sternotomy incision.

In conclusion, complete unifocalization of all sources of pulmonary blood supply, an intracardiac repair of pulmonary with ventricular septal defect in major aorta pulmonary collaterals, can be accomplished in a single stage through a standard median sternotomy incision. The repair can be accomplished with acceptable morbidity and mortality, with a length of hospital stay that is not excessive. Mid-term follow-up results reveal excellent hemodynamics and functional status. Balloon angioplasty has been accomplished with good results, and stenting of stenotic segments remains and important therapeutic adjunct.

We do, however, feel that patient selection will ultimately prove to very important. Although this was a consecutive patient series, one patient in this series experienced no growth whatsoever of his aorta pulmonary collaterals between birth and the time of operation, and had no central pulmonary arteries demonstrable anatomically. Only through a multi-institutional study would we be able to accumulate the numbers of patients that might allow for risk stratification based upon anatomy.

We feel that early definitive correction with incorporation of these clearly abnormal segments into the normal pulmonary vascular bed provides the best opportunity for pulmonary arterial and pulmonary parenchymal growth. It is hoped that with combination of balloon angioplasty and stenting, future operations would be limited to central conduit changes only. While the long term fate of collaterals remains uncertain, early and mid-term results remain encouraging.

	Footnotes

 
Presented at the 13th Annual Meeting of the European Association for Cardio-thoracic Surgery in Glasgow, Scotland, UK, September 5–8, 1999.

	Appendix A. Conference discussion

Top Abstract 1. Introduction 2. Methods 3. Results 4. Discussion Appendix A. Conference... References

 
Mr M. Pozzi (Liverpool, UK): I have two questions for you: (1) do you think there is any difference between the patients with MAPCAs only? My impression is that patients with pulmonary arteries on which MAPCAs can be anastomosed on have a much better prognosis after this type of repair. (2) Although I fundamentally agree with your approach, at the moment I would recommend a complete, single stage repair at 2–3 months of age only in patients with unrestricted flow through the MAPCAs, while I would delay to between 12 and 24 months of age when the MAPCAs present several stenosis. I would appreciate your comments on this approach.

Dr Lofland: In answer to your first question, there is no question that the operation is technically easier if there are some sort of pulmonary arteries or even some sort of pulmonary arterial confluence to which all of the collateral vessels can subsequently be anastomosed. We haven't noticed any difference in longterm outcome in these patients, however. I think it is naive to assume that these collateral vessels are going to be completely normal pulmonary arteries, however. They seem to have a bit of adventitia and an intima with very little in the way of media. And we embarked upon this series with a bit of trepidation, expecting to find multiple stenotic areas that you have alluded to, and have been pleasantly surprised to find that those haven't developed in the patients who have undergone an early correction, at least not to date, but we are prepared certainly to treat those with balloon angioplasty.

I think regarding the age of correction, we are still in a little bit of uncharted territory, since most of these patients historically have been corrected at older ages.

Dr A. Corno (Lausanne, Switzerland): At the poster presentation of this morning, the Japanese group of Osaka presented very good results with a pericardial roll to reconstruct the pulmonary arteries continuity. They left the pericardial roll in an unusual position, anterior to the aorta and the vena cava, and they claim no compression of this conduit. Since from the angio it seems that you leave your reconstruction of the pulmonary artery posterior to the aorta, do you believe that the anterior position could reduce the need for postoperative dilatation or stent because of obstruction of the pulmonary arteries?

And a second question, since you leave everything behind the aorta usually, how can you prevent or reduce the incidence of bronchial compression with the new reconstructed pulmonary artery?

Dr Lofland: In answer to the first question, I don't know if bringing the conduit in a different position will reduce the incidence of either branch stenosis or a conduit obstruction. I don't have an answer to that. We simply chose to put ours in a more anatomic location, and we had no technical difficulty in achieving that. I also expected these patients to manifest some degree of tracheobronchial malacia at about 5–6 months of age, and we have been pleasantly surprised in that none of these patients who have undergone an early correction have demonstrated any degree of tracheobronchial malacia. I have always associated that with pulmonary atresia also. We had not experienced that in this series, though.

Dr G. Stellin (Padova, Italy): We all enjoyed your presentation, obviously. Frank Hanley in his original presentation at the AATS in Boston in 1995 showed us that he approaches the collateral vessels through the transverse sinus. You said to us you are approaching the collaterals through the right or left pleural space. Can you tell us what are the advantages to do that?

Dr Lofland: In the paper that came out of that presentation, Dr Hanley did mention that he opened both pleural spaces to facilitate direct dissection, and that is very necessary. We also open up the posterior pericardium, the transverse sinus posteriorly, and that really does facilitate the bringing up of these collaterals through that space to perform the pulmonary artery confluence reconstruction.

Dr Stellin: So, your approach is both through the transverse sinus and left pleural space?

Dr Lofland: Yes. I could not reach all of the collaterals to the posterior leaf through the transverse sinus, and I found early on in this series that I simply had to open the pleural spaces, decompress the lungs completely, and that way the lungs could be actually moved out of the operative field, which greatly facilitates dissection.

Dr H. Uemura (Osaka, Japan): Recently, our preference is a one-stage repair, as you say, in patients with MAPCAS, but in our series a third of such patients could undergo a onestage repair and the remaining two thirds of our patients could not undergo a onestage repair because of complete absence or very vestigial nature of the central pulmonary arteries. Do you think in all patients you can establish a onestage repair?

Dr Lofland: No, I don't, and I think we demonstrated that with the one perioperative death in our series. That patient had collaterals that were minuscule and no pulmonary arteries that had developed over the two and a half months of that patient's life. I think that the prognosis for that patient was fairly dismal. In embarking upon this series, though, we did not deny this kind of approach to any patient who presented.

	References

Top Abstract 1. Introduction 2. Methods 3. Results 4. Discussion Appendix A. Conference... References

 

1. DeRuiter M.C., Gittenberger-de Groot A.C., Poelmann R.E., Vanlperen L., Mentink M.M.T. Development of the pharyngeal arch system related to the pulmonary and bronchial vessels in the avian embryo: with a concept on systemic-pulmonary collateral artery formation. Circulation 1993;87:1306-1319.[Abstract/Free Full Text]
2. Liao P., Edwards W.D., Julsrud P.R., Puga F.J., Danielson G.K., Feldt R.H. Pulmonary blood supply in patients with pulmonary atresia and ventricular septal defect. J Am Coll Cardiol 1985;6:1343-1350.[Abstract]
3. Jefferson K., Rees S., Somerville J. Systemic arterial supply to the lungs in pulmonary atresia and its relation to pulmonary artery development. Br Heart J 1972;34:418-427.[Free Full Text]
4. Haworth S.G., Macartney F.J. Growth and development of pulmonary circulation in pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. Br Heart J 1980;44:14-24.[Free Full Text]
5. Rabinovitch M., Herrera-DeLeon V., Castaneda A.R., Reid L. Growth and development of the pulmonary vascular bed in patients with tetralogy of Fallot with or without pulmonary atresia. Circulation 1981;64:1234-1249.[Free Full Text]
6. Puga F.J., Leoni F.E., Julsrud P.R., Mair D.D. Complete repair of pulmonary atresia, ventricular septal defect, and severe peripheral arborization abnormalities of the central pulmonary arteries. J Thorac Cardiovasc Surg 1989;98:1018-1029.[Abstract]
7. 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]
8. Sawatari K., Imai Y., Kurosawa H., Isomatsu Y., Momma K. Staged operation for pulmonary atresia and ventricular septal defect with major aortopulmonary collateral arteries. J Thorac Cardiovasc Surg 1989;98:738-750.[Abstract]
9. Sullivan I.D., Wren C., Stark J., de Leval M.R., Macartney F.J., Deanfield J.E. Surgical unifocalization in pulmonary atresia and ventricular septal defect. A realistic goal?. Circulation 1988;78(suppl):1113-1115.
10. Reddy V.M., Liddicoat J.R., Hanley F.L. Midline one-stage unifocalization and repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals. J Thorac Cardiovasc Surg 1995;109:832-844.[Abstract]
11. Kirklin J.W., Blackstone E.H., Shimazaki Y., Maehara T., Pacifico A.D., Kirklin J.K., Bargeron L.M. Survival, functional status, and reoperations after repair of tetralogy of Fallot with pulmonary atresia. J Thorac Cardiovasc Surg 1988;96:102-116.[Abstract]
12. Hofbeck M., Rauch A., Buheitel G., Leipold G., von der Emde J., Pfeiffer R., Singer H. Monosomy 22q11 in patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries. Heart 1998;79:180-185.[Abstract/Free Full Text]
13. Digilio M.C., Marion B., Grazioli S., Agostino D., Giannotti A., Dallapiccola B. Comparison of occurrence of genetic syndromes in ventricular septal defect with pulmonic stenosis (classic tetralogy of Fallot) versus ventricular septal defect with pulmonic atresia. Am J Cardiol 1996;77:1375-1376.[Medline]
14. Momma K., Kondo C., Matsuoka R., Takao A. Cardiac anomalies associated with a chromosome 22q11 deletion in patients with conotruncal anomaly face syndrome. Am J Cardiol 1996;78:591-594.[Medline]
15. Matsuoka R., Takao A., Kimura M., Imamura S., Kondo C., Joh-o K., Ikeda K., Nishibatake M., Ando M., Momma K. Confirmation that the conotruncal anomaly face syndrome is associated with a deletion within 22q11. Am J Med Gen 1994;53:285-289.[Medline]
16. Kelly D., Goldberg R., Wilson D., Lindsay E., Carey A., Goodship J., Burn J., Cross I., Shprintzen R., Scambler P. Confirmation that the velo-cardio-facial syndrome is associated with haplo-insufficiency of genes at chromosome 22q11. Am J Med Genet 1993;45:308-312.[Medline]
17. Wilson D.I., Scambler P.J. The chromosome 22q11 deletion syndromes. Appl Cytogenet 1995;21:33-39.
18. Chessa M., Butera G., Bonhoeffer P., Iserin L., Kachaner J., Lyonnet S., Munnich A., Sidi D., Bonnet D. Relation of genotype 22q11 deletion to phenotype of pulmonary vessels in tetralogy of Fallot and pulmonary atresia-ventricular septal defect. Heart 1998;79:186-190.[Abstract/Free Full Text]
19. Macartney F., Deverall P., Scott O. Haemodynamic characteristics of systemic arterial blood supply to the lungs. Br Heart J 1973;35:28-37.[Free Full Text]
20. Haworth S.G. Collateral arteries in pulmonary atresia with ventricular septal defect: a precarious blood supply. Br Heart J 1980;44:5-13.[Free Full Text]
21. Haworth S.G., Rees P.G., Taylor J.F.N., Macartney F.J., de Leval M., Stark J. Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries: effect of systemic pulmonary anastomosis. Br Heart J 1981;45:133-141.[Abstract/Free Full Text]
22. Thiene G., Bortolotti U., Gallucci V., Valente M.L., Volta S.D. Pulmonary atresia with ventricular septal defect. Br Heart J 1977;39:1223-1233.[Free Full Text]
23. Marelli A.J., Perloff J.K., Child J.S., Laks H. Pulmonary atresia with ventricular septal defect in adults. Circulation 1994;89:243-251.[Abstract/Free Full Text]
24. Nakata S., Imai Y., Takanashi Y., Kurosawa H., Tezuka K., Nakazawa M., Ando M., Takao A. A new method for the quantitative standardization of cross-sectional areas of the pulmonary arteries in congenital heart diseases with decreased pulmonary blood flow. J Thorac Cardiovasc Surg 1984;88:610-615.[Abstract]
25. Moritz A., Marx M., Wollenek G., Domanig E., Wolner E. Complete repair of PA-VSD with diminutive or discontinuous pulmonary arteries by transverse thoracosternotomy. Ann Thorac Surg 1996;61:646-650.[Abstract/Free Full Text]

This article has been cited by other articles:

				K. Januszewska, E. Malec, G. Juchem, I. Kaczmarek, R. Sodian, P. Uberfuhr, and B. Reichart Heart-lung transplantation in patients with pulmonary atresia and ventricular septal defect J. Thorac. Cardiovasc. Surg., September 1, 2009; 138(3): 738 - 743. [Abstract] [Full Text] [PDF]

				N. Ishibashi, T. Shin'oka, M. Ishiyama, T. Sakamoto, and H. Kurosawa Clinical results of staged repair with complete unifocalization for pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries Eur. J. Cardiothorac. Surg., August 1, 2007; 32(2): 202 - 208. [Abstract] [Full Text] [PDF]

				O. Reinhartz, V. M. Reddy, E. Petrossian, S. Suleman, R. D. Mainwaring, D. N. Rosenthal, J. A. Feinstein, R. Gulati, and F. L. Hanley Unifocalization of major aortopulmonary collaterals in single-ventricle patients. Ann. Thorac. Surg., September 1, 2006; 82(3): 934 - 938. [Abstract] [Full Text] [PDF]

				E. M. Ngan, I. M. Rebeyka, D. B. Ross, M. Hirji, J. F. Wolfaardt, R. Seelaus, A. Grosvenor, and M. L. Noga The rapid prototyping of anatomic models in pulmonary atresia J. Thorac. Cardiovasc. Surg., August 1, 2006; 132(2): 264 - 269. [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]

				A. Gupta, J. Odim, D. Levi, R.-K. Chang, and H. Laks Staged repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries: Experience with 104 patients J. Thorac. Cardiovasc. Surg., December 1, 2003; 126(6): 1746 - 1752. [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]

				D. Metras, P. Chetaille, B. Kreitmann, A. Fraisse, O. Ghez, and A. Riberi Pulmonary atresia with ventricular septal defect, extremely hypoplastic pulmonary arteries, major aorto-pulmonary collaterals Eur. J. Cardiothorac. Surg., September 1, 2001; 20(3): 590 - 597. [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): Gary K. Lofland Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Lofland, G. K. Search for Related Content PubMed PubMed Citation Articles by Lofland, G. K.