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Eur J Cardiothorac Surg 2004;26:762-766
© 2004 Elsevier Science NL

Ascending aortic origin of a branch pulmonary artery—surgical management and long-term outcome

Department of Cardiac Surgery, Royal Hospital for Sick Children, Dalnair Street, Glasgow G3 8SJ, Scotland, UK

Received 6 March 2004; received in revised form 25 June 2004; accepted 1 July 2004.

^* Corresponding author. Tel.: +44-141-201-0269. (E-mail: jim.pollock{at}yorkhill.scot.nhs.uk).

	Abstract

Top Abstract 1. Introduction 2. Patients and methods 3. Results 4. Discussion 5. Conclusions References

 
Objective: Ascending aortic origin of a branch pulmonary artery (AOPA, hemitruncus arteriosus) is a rare congenital malformation. While there have been isolated case reports, larger series, relating to long-term outcomes following surgery are few. This article analyses the surgical results of a series of nine patients, over a period of 29 years. Methods: Between 1974 and 2003, nine patients [neonates, 6; infants, 3; male, 5; female, 4] were operated on for AOPA. Median age at presentation was 14 days (range birth to 231 days). Seven patients (group 1) had associated simple lesions like patent ductus arteriosus or right aortic arch. Three patients (group 2) had complex lesions with right ventricular outflow tract obstruction. One patient (group 2) had DiGeorge syndrome. All patients except group 2 presented with congestive cardiac failure and, in addition one had pre-operative coronary ischemia. Diagnosis was established by angiocardiography in two patients and by echocardiography in eight. The median age at operation was 28 days (range 7–365). Follow-up period ranged from 7 months to 20.5 years (median 9 years). Results: All nine patients had an anomalous right pulmonary artery (RPA) arising from the proximal ascending aorta, while the left branch was of right ventricular origin. All had evidence of pulmonary hypertension or elevated right ventricular pressure pre-operatively. There was no operative mortality. Of eight patients who had direct anastomosis of the RPA to the main pulmonary artery, one required patch enlargement and another required stenting of an anastomotic stenosis. One patient had a RV-RPA conduit, which required replacement 8, 13, and 14 years later. At follow-up, all patients were alive. All patients in group 1 had normal haemodynamic function and were in NYHA class I. In group 2, all were in NYHA class II with evidence of right ventricular hypertrophy. Four patients had post-operative ventilation–perfusion scans which showed satisfactory perfusion to both lungs. Conclusions: Early surgery is indicated in this lesion and is compatible with good long-term outlook. Surgical repair should not be deferred for corrective procedures of associated cardiac anomalies.

	1. Introduction

Top Abstract 1. Introduction 2. Patients and methods 3. Results 4. Discussion 5. Conclusions References

 
Anomalous origin of a branch pulmonary artery from the ascending aorta (AOPA, hemitruncus arteriosus) is an unusual congenital heart malformation. Whilst there have been numerous reports since its first description in 1868 [1], larger studies, however, are lacking and only a few case series exist [2–8]. Surgical repair is associated with high mortality in the early years [4,5,9] and long-term result after surgery remains unclear. This study was undertaken to review our experience at the Royal Hospital for Sick Children, Glasgow over a time period of 29 years. This study represents one of the largest series reported in the literature [2,3,6] with emphasis on long-term result after surgical repair of this rare defect.

	2. Patients and methods

Top Abstract 1. Introduction 2. Patients and methods 3. Results 4. Discussion 5. Conclusions References

 
Between 1974 and 2003, nine patients [neonates, 6; infants, 3; male, 5; female, 4] were operated on for AOPA. The clinical records, echocardiographic and catheterisation data, operative findings, and follow-up records of these patients were analysed. Patients with truncus arteriosus were excluded from this study.

Seven patients (group 1) had simple associations like patent ductus arteriosus (PDA), right aortic arch (RAA), and patent foramen ovale (PFO) (Table 1, Fig. 1). All presented with congestive heart failure. In addition, one of them had low diastolic pressure and pre-operative evidence of coronary ischemia, requiring emergency surgical intervention.

View larger version (147K): [in this window] [in a new window]   Fig. 1. Echocardiogram showing the anomalous right pulmonary artery (RPA) arising from the ascending aorta (AA) and the normal left pulmonary branch (LPA) from the main pulmonary artery (MPA).

View larger version (111K): [in this window] [in a new window]   Fig. 2. Angiogram showing the anomalous right pulmonary branch (RPA), right aortic arch, anomalous left subclavian artery (LSCA) arising from descending aorta, and a PDA, which supplied the left pulmonary artery (LPA).

	3. Results

Top Abstract 1. Introduction 2. Patients and methods 3. Results 4. Discussion 5. Conclusions References

 
3.1. Morphology
The anomalous branch was the right pulmonary artery (RPA) in all instances, with the left branch of right ventricular origin. The anomalous branch arose from the proximal ascending aorta above the aortic sinus. None of the anomalous pulmonary branches had origin stenosis.

3.2. Surgical approach
The surgical approach in all cases was by median sternotomy. All the operations were done under cardiopulmonary bypass (CPB). The surgical procedures were: (1) direct reconnection by anastomosis of the anomalous branch to main pulmonary artery (MPA) in eight patients and (2) interposition of bio-prosthetic conduit in one patient.

3.2.1. Group 1
Six patients had direct reconnection of the anomalous right branch to the MPA. Following CPB, PDA closure was undertaken. The anomalous branch was then controlled with a sling prior to detachment from the ascending aorta and the aortotomy was oversewn. The anomalous RPA was mobilized to the pulmonary hilum, passed behind the aorta and anastomosed end to side to the MPA.

3.2.2. Group 2
The earliest patient in our series had RV—RPA continuity established using a 12mm Hancock porcine conduit with the MPA anastomosed to the conduit. A further two patients had direct reconnection of the anomalous branch to the MPA.

In addition, further procedures were required to correct or palliate the right ventricular outflow tract obstruction in this group: one patient (Tetralogy of Fallot, infundibular stenosis) required two central shunts (from the ascending aorta to the MPA and RPA, respectively,) after the AOPA repair to improve oxygenation and growth of pulmonary arteries. One patient (pulmonary atresia) had a modified Blalock–Taussig shunt from the innominate artery to the anomalous pulmonary branch after repair of the AOPA was accomplished.

3.3. Post-operative complications, cardiac intensive care and hospital stay
The median stay in intensive care was 11.5 days (range 6–17). The patients required post-operative mechanical ventilatory support for a median of 7 days (range 4–10). Two required nitric oxide post-operatively, but none needed extracorporeal membrane oxygenation. One patient had cardiac tamponade requiring re-exploration. Sternal closure was delayed for 1 day in two patients. There was no post-operative death. Median post-operative stay was 17.5 days (range 12–48).

3.4. Anastomotic stenosis and reintervention
Three of the nine patients needed reintervention after surgical repair of AOPA.

In group 1, one patient developed significant anastomotic stenosis 1 year after direct reimplantation of the AOPA and required pericardial patch enlargement of the proximal RPA. Cardiac catheterisation 7 years later showed a gradient of 10mmHg requiring no further intervention.

In group 2, the patient who had primary repair of AOPA and central shunts developed a significant anastomotic stenosis 2 years post-operatively. The RPA, however, remained well perfused via the central shunt. A left modified BT shunt was later required to allow further growth of the small LPA. Subsequently, a TOF repair was performed with patch closure of VSD, pulmonary annulus and MPA widening with homograft patch, resection of hypertrophic bands of the RVOT and ligation of central shunts and MBTS (age 4). The patient required balloon dilatation of MPA and stenting of the RPA 8 years after the primary AOPA repair. The pressure gradient fell from 21 to 10mmHg post-stenting.

The only patient with RV-RPA conduit outgrew his first conduit 8 years after the index operation and needed his conduit replaced (17mm Hancock). Five years later, another conduit replacement was required due to occlusion from pseudointimal hypertrophy (20mm Vascutek valved homograft). This conduit needed further replacement (22mm porcine Carpentier Edwards) a year later due to compression between the right ventricle and sternum.

Three other patients had immediate post-operative gradient measured of 21, 31 and 36mmHg. The gradients improved to 14, 16 and 5mmHg at 5, 3 and 3.5 years, respectively, requiring no further intervention.

3.5. Haemodynamic function
In group 1, all patients had evidence of elevated pulmonary pressure pre-operatively. Two had pre-operative angiocardiography, which confirmed a systemic pulmonary arterial pressure. Four had evidence of pulmonary hypertension on echocardiography (Table 1).

In group 2, the three patients with complex lesions had evidence of elevated right ventricular pressure pre-operatively. Cardiac catheterisation in two of these patients showed systemic RVP (Table 1).

On follow-up, all patients in group 1 had normal haemodynamic function. The mean MPAP in three patients who had follow-up angiocardiography was 12mmHg (range 11–15). Three patients did not have follow-up catheterisation, but echocardiography showed normal haemodynamic function without any evidence of elevated pulmonary or right ventricular pressure.

In group 2, all three patients had follow-up angiocardiography. All had evidence of right ventricular hypertrophy and elevated right ventricular pressure (range two-third to systemic level) (Table 1).

3.6. Lung perfusion
Four patients (all from group 1) had post-operative ventilation–perfusion (V/Q) scans which showed satisfactory perfusion to both lungs. In one patient, 35% of the pulmonary blood flow perfused the right lung (1 year post-operatively), and in another, 45% of the pulmonary blood blow perfused the right lung (4 years post-operatively). Two other patients had equal perfusion to both lungs.

3.7. Outcomes
All patients are alive on follow-up. The length of follow-up ranged from 7 months to 20.5 years (median 9 years). Four of these patients had been followed up for 12 years or more and were fit for discharge. One patient had partial epilepsy due to multiple cerebral infarcts after his fourth operation. All six patients in group 1 were in NYHA class 1 and the three patients in group 2 were in NYHA class 2.

	4. Discussion

Top Abstract 1. Introduction 2. Patients and methods 3. Results 4. Discussion 5. Conclusions References

 
From this study, we found two distinct groups of AOPA patients with separate clinical presentation and functional outcome after surgery. One group of patients with associated simple lesions without RVOTO had a more severe clinical presentation, but better functional outcome after surgery. On the other hand, another group of patients with complex lesions associated with RVOTO were well at first presentation, but poorer functional outcome after surgery due to associated co-morbidities.

Patients in the first group without RVOTO invariably presented with congestive cardiac failure. Unrestrictive pulmonary flow would lead to lung congestion and cardiac failure if the origin of the anomalous branch was not stenosed and right ventricular outflow tract obstruction was absent. One patient in this group had pre-operative evidence of coronary ischemia, which coincided with a drop in diastolic blood pressure. Low diastolic pressure occurred as a result of systemic blood flow to the anomalous pulmonary branch during the diastolic phase resembling a ‘steal syndrome’ leading to a compromise in the coronary perfusion. Whilst coronary ischemia has been reported in children with congenital coronary artery anomalies and other acquired disorder like Kawasaki disease, coronary ischemia is, in fact, has not been previously associated with AOPA. To the best of our knowledge, this represents the first reported case of coronary ischemia associated with AOPA in the literature. Hence, the possible complication of coronary ischemia in AOPA patients should always be borne in mind in the event of sudden deterioration of patients with known AOPA. In the second group, three patients who had complex lesions associated with various forms of right ventricular outflow tract obstruction did not have any lung congestion or cardiac failure. The reduced pulmonary blood flow from the right ventricle in these patients appears to be a protective factor in delaying the onset of pulmonary oedema.

While DiGeorge syndrome is frequently found with aortic arch and conotruncal anomalies especially in persistent truncus arteriosus; earlier studies did not find an association with AOPA [10–12]. However, the occurrence of DiGeorge Syndrome has been increasingly noted in several subsequent studies [2,5,13]. One patient (1/9) in our series was found to have DiGeorge syndrome with left kidney aplasia and right hydronephrosis. In our opinion, the occurrence of DiGeorge syndrome in AOPA patients is not negligible and screening for deletions of chromosome 22q11 in all AOPA patients is recommended. The infrequent association of AOPA with DiGeorge Syndrome in earlier studies is perhaps due to the rare occurrence of this lesion, an anomaly much more uncommon than persistent truncus arteriosus.

Patent arterial duct and aortopulmonary defect are common associations in anomalous origin of right pulmonary artery; other anomalies were relatively rare [11]. The most common associated anomaly found in our patients was patent arterial duct. On the other hand, we did not find any occurrence of aortopulmonary window, and other anomalies which were more commonly associated with AOLPA [2,11] were found in our patients with AORPA: Tetralogy of Fallot (two patients), right aortic arch (four patients) and anomalous origin of the left subclavian artery (two patients).

Surgical intervention is recommended as early in life as possible [2,5,7,9]. Abnormal pulmonary vasculature in the lung supplied by the anomalous pulmonary branch is a well-reported phenomenon [5,14]. Progressive pathological changes occur in the lung subjected to systemic pressures. In severe cases, medial and intimal proliferation with focal fibrinoid changes were found in necropsy specimens [5]. Corrective surgery is therefore important before these irreversible changes occur. Reconnection of this anomalous pulmonary artery should not be deferred for corrective procedures of other associated cardiac anomalies.

This once lethal condition has shown improved results since the first successful case described by Armer in 1961 using an interposition graft [15]. Six years later, Kirkpatrick et al. [16] reported the first successful anatomic repair without a graft in a 2-month-old infant. Since then, direct reimplantation is the commonest method used, and should be the method of choice if anastomosis can be achieved without tension [8,9]. Careful and adequate mobilisation of the anomalous branch is necessary. Where this is not feasible, other methods have been employed, including implantation using autologous tissue (pericardial patch, aortic ring) and graft interposition (prosthetic or homograft conduits) [2,6,8,17]. All the different techniques employed in appropriate circumstances appear to produce acceptable results and restenosis rates [6]. Nevertheless, prosthetic conduits will need replacement as the patient outgrows the conduit as demonstrated by the earliest patient in our series. Significant stenosis at the anastomotic site requiring further re-intervention is an important source of morbidity, and ranged from 12.5 to 36% in past studies [2,6]. In our studies, two patients (2/8) with direct reimplantation techniques had restenosis which required further intervention at 1 and 8 years, respectively, after the index operation. Little is known on whether this rate of the stenosis at the anastomotic site can be possibly reduced by enlargement with autologous tissue; an area remains to be evaluated.

Patients with simple lesions were shown to have normal functional outcome on long-term follow-up as demonstrated in our study. Unlike other congenital conditions associated with persistent elevation of pulmonary pressure even after surgical repair, e.g. congenital diaphragmatic hernia, return to normal haemodynamic function is expected in this group of patients after surgical correction. Although best possible surgical outcome is anticipated with early surgery, the presence of associated complex cardiac anomalies is an important factor in determining the long-term functional status of the patients. Associated complex cardiac anomalies necessitating further interventions, especially in those whom anatomical correction cannot be achieved, functional outcome tends to be poorer.

	5. Conclusions

Top Abstract 1. Introduction 2. Patients and methods 3. Results 4. Discussion 5. Conclusions References

 
We conclude that this rare but serious condition is amenable to surgical repair particularly if operated on early in life. Direct anastomosis is our surgical procedure of choice. In our opinion, reconnection of the anomalous branch is important to avoid irreversible pulmonary vasculature changes and should not be deferred for corrective procedures of associated cardiac anomalies. Whilst early surgical repair of AOPA is compatible with good long-term outlook, stenosis at the anastomotic site requiring further intervention is not uncommon.

	Acknowledgments

 
We would like to express our gratitude to Mr Eamon Murtagh and Mr Stuart Lilley of the Department of Cardiology, Royal Sick Children Hospital of Glasgow, for all their effort in providing us with technical support especially in preparation of the echocardiographic images to make this study possible.

	References

Top Abstract 1. Introduction 2. Patients and methods 3. Results 4. Discussion 5. Conclusions References

 

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13. Dodo H, Alejos JC, Perloff JK, Laks H, Drinkwater DC, Williams RG. Anomalous origin of the left main pulmonary artery from the ascending aorta associated with DiGeorge syndrome. Am J Cardiol 1995;75(17):1294-1295.[CrossRef][Medline]
14. Kuinose M, Tanemoto K, Murakami T, Kanaoka Y, Kobayashi G, Makabe M. Surgical treatment for a 16-year-old girl with anomalous origin of the right pulmonary artery from ascending aorta. Jpn J Thorac Cardiovasc Surg 1998;46(4):380-384.[Medline]
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This Article Abstract Full Text (PDF) Corrigendum (v32,p826) 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): Edward W.K. Peng Ganesh Shanmugam James C.S. Pollock Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Peng, E. W.K. Articles by Pollock, J. C.S. Search for Related Content PubMed PubMed Citation Articles by Peng, E. W.K. Articles by Pollock, J. C.S. Related Collections Congenital - acyanotic