Asian Cardiovasc Thorac Ann 2005;13:217-221
© 2005 Asia Publishing EXchange Ltd
Repair of Anomalous Pulmonary Artery with Interposition Graft: Midterm Results
Eli Levy, MD,
Eli Milgalter, MD,
Amiram Nir, MD1,
Azaria JJT Rein, MD1
Division of Cardiothoracic Surgery
1 Division of Pediatric Cardiology, Hadassah Medical Center, Jerusalem, Israel
For reprint information contact: Eli Levy, MD Tel: 972 2 677 6960/1 Fax: 972 2 643 8005 Email: elinava{at}netvision.net.il, Hadassah Medical Center, POB 12000, Jerusalem 91120, Israel.
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ABSTRACT
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Anomalous pulmonary artery arising from the aorta is a rare congenital anomaly. The midterm results of repair of this malformation by Gore-Tex graft interposition were examined in 5 patients: 3 with anomalous right pulmonary artery and 2 with anomalous left pulmonary artery from the ascending aorta. Echocardiography was adequate in 4 cases for diagnosis, planning the operation, and follow-up. Angiography was needed for diagnosis in one case where the echocardiographic findings were unclear. The mean follow-up period was 4 years. One patient with tracheoesophageal fistula and cardiac malformation died 2 months after the operation due to multi-organ failure. Three patients needed re-operation because of graft narrowing, and one was without problems 5.2 years postoperatively. In anomalous pulmonary artery from the ascending aorta, repair should be performed as early as possible to prevent pulmonary hypertensive changes. When the anomalous pulmonary artery cannot be anastomosed directly to the main pulmonary artery, an interposition graft can be placed safely without cardiopulmonary bypass. With appropriate follow-up, this can be a satisfactory solution, although it carries the risk of re-operation due to graft narrowing.
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INTRODUCTION
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Anomalous origin of the pulmonary artery (PA) from the ascending aorta is a rare congenital malformation. In such patients, the right pulmonary artery (RPA) or, much less commonly, the left pulmonary artery (LPA), arises from the ascending aorta with separate aortic and pulmonary valves, while the contralateral artery originates normally from the main pulmonary artery (MPA).1 The lung with the normally connected PA is thus subjected to the entire right ventricular output in addition to flow contributed by a ventricular septal defect (VSD), an aortopulmonary septal defect, or an atrial septal defect, which have been present in more than 60% of the previously reported cases.1–2 Without early surgical correction, the natural history of this condition is fatal with a mortality rate of 80% at 1 year of age.3 However, there has been a report of successful repair at an older age.4 Experience of 5 cases of repair of an anomalous PA arising from the ascending aorta is described.
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PATIENTS AND METHODS
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From November 1995 to May 2000, 5 patients with anomalous PA from the ascending aorta underwent surgical correction. There were 3 males and 2 females, their ages at operation ranged from 4 days to 13 months, with a mean of 3 months (Table 1
). All but patient number (no.) 2 had associated cardiac defects. Patient no. 1 had VACTER association (vertebral defects, anal atresia, cardiac anomalies, tracheoesophageal fistula with esophageal atresia, radial upper limb hypoplasia and renal defects), patient no. 3 had a tiny VSD and a right aortic arch, patient no. 4 had type A interrupted aortic arch and an aortopulmonary septal defect, and patient no. 5 had tetralogy of Fallot with absent pulmonary valve (Table 1). Three patients had anomalous origin of the RPA from the ascending aorta, two of them (patients nos. 1 and 2) had the RPA originating from the right lateral aspect of the aorta, and the other (patient no. 4) had the RPA originating from the posterior aspect of the aorta. The other two had anomalous LPA from the ascending aorta. The presenting symptoms were congestive heart failure, huge left-to-right shunt, pulmonary congestion, and edema.
All patients were diagnosed primarily by echocardiography prior to surgery, except patient no. 3 who had angiography because the LPA was not visualized well by echocardiography. Planning of the surgical approach was based solely on echocardiographic findings. All operations were performed through a median sternotomy. Cardiopulmonary bypass (CPB) was not used, except in patient no. 4. In the other cases, the aorta was dissected free where the main PA, anomalous PA, and contralateral PA branch were connected. Heparin (2 mg·kg–1) was given and a partial clamp was applied to the aorta on the side of the anomalous PA which was closed with a snare and disconnected from the aorta. The aorta was then closed primarily with two layers of 7/0 polypropylene suture. A Gore-Tex tube graft was connected end-to-end to the anomalous PA, the other end of the graft was connected to the main PA with 7/0 polypropylene sutures after a side-biting clamp was applied. A 6 mm Gore-Tex tube graft was used in 4 cases; patient no. 5 had a 5 mm graft. After removing the side-biting clamp, the pressures in the aorta and the MPA were measured.
Patient no. 1 was previously operated on at the age of 1 week to carry out repair of the tracheoesophageal fistula, gastrostomy, and colostomy, as a result of VACTER association. At the age of 21 days, a 6 mm interposition tube graft was interposed between the RPA and the MPA. Patient no. 3 had a tiny VSD and a collateral to the anomalous LPA, which was not closed during the operation. Patient no. 4 was admitted at the age of 2 weeks in cardiogenic shock. He was treated successfully with intravenous prostaglandin E1 and dopamine. An echocardiogram revealed type A interrupted aortic arch, aortopulmonary septal defect, and anomalous RPA from the aorta. Complete repair was carried out via a left thoracotomy and a median sternotomy. The chest was opened at the 3rd intercostal space, cross clamps were applied to the aortic arch, sparing the innominate artery, and to the descending aorta. End-to-end anastomosis was performed. Immediately afterwards, a median sternotomy was carried out. After snaring both pulmonary arteries, the patient was placed on CPB and cooled to 26°C, an aortic cross-clamp was applied, and cardioplegia was administered. The aortopulmonary septal defect was opened and inspected. The anomalous RPA was disconnected from the aorta, and the aorta was closed primarily. The aortopulmonary septal defect was closed with a Gore-Tex patch, de-airing was carried out, and the aortic cross-clamp was removed. On the beating heart, the RPA was connected to the MPA with a 6 mm Gore-Tex tube graft. The patient came off bypass without any difficulty. Patient no. 5 had a 5 mm Gore-Tex tube graft connecting the LPA to the MPA, and a PA band was applied because of tetralogy of Fallot and absent pulmonary valve.
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RESULTS
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There was one hospital death: patient no. 1 with VACTER association died 2 months after the operation because of multi-organ failure. The follow-up period of the survivors ranged from 2 years and 1 month to 5 years and 5 months, mean 4 years (Table 1
). Every patient was followed-up periodically by our pediatric cardiologists, and an echocardiogram was performed annually.
Patient no. 2 had no graft narrowing and only mildly elevated right ventricular pressure, according to echocardiography 5 years and 5 months after the operation. Patient no. 3 had stenosis of the graft to the LPA that was revealed by an echocardiogram 5.5 years after the first operation. A perfusion scan showed only 28% flow to the left lung. Catheterization demonstrated critical stenosis of the graft (Figure 1). The patient underwent re-operation, an incision was made through the Gore-Tex graft to the LPA, and a bovine pericardial patch was sutured to increase the tube diameter to 12 mm. A tiny perimembranous VSD partially obliterated by atrioventricular valve tissue was still present. Patient no. 4 suffered cyanosis (85% O2 saturation on room air), recurrent pulmonary infection, and pulmonary hypertension, 1.5 years after the operation. Echocardiography did not detect any flow in the graft on the right side, a perfusion scan demonstrated no perfusion to the right lung, and pulmonary angiography showed obstruction of the tube graft. At re-operation, the whole tube graft was found to be blocked by a thrombus. The graft was resected, and an 8 mm Gore-Tex tube was interposed between the MPA and the RPA. Three months later, the graft was patent with good flow through it, although a gradient of 24 mm Hg was measured across it by echocardiography. Since then, the patient has been asymptomatic with O2 saturation on room air of 98%. Patient no. 5 had transthoracic echocardiography 13 months after the operation, which showed reduced flow to the LPA in comparison to the RPA. The O2 saturation on room air was 98%, and the PA band was adequate with a gradient of 50 mm Hg across it. The shunt across the VSD was found to be mostly mild left-to-right with some right-to-left. Angiography demonstrated narrowing of the graft to the LPA. A second operation was carried out. The VSD was closed with a Dacron patch, debanding of the MPA was followed by an incision along the Gore-Tex graft, and an autologous pericardial patch was sutured to the MPA and to the Gore-Tex graft, increasing its diameter to 6 mm. It should be emphasized that the narrowing in the grafts was diagnosed first by echocardiography and confirmed by angiography in all 3 patients.
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Figure 1. Angiography of patient no. 3, at 4.5 years after the operation, showing narrowing of the interposition graft. IG = interposition graft, MPA = main pulmonary artery, RPA = right pulmonary artery.
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DISCUSSION
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Anomalous origin of the PA from the ascending aorta was first reported by Fraentzel5 in 1868, and since then, fewer than 180 cases have been documented in the English literature, each with interesting embryological, physiological and surgical features. Anomalous origin of the RPA from the ascending aorta occurs 4 to 8 times more often than anomalous origin of the LPA.6 Two types of anomalous RPA have been identified: proximal and distal.1,7 The proximal type (85% of cases) originates from the right posterior aspect of the ascending aorta close to the aortic valve.1 The distal type originates from the ascending aorta near the base of the innominate artery.8 Aortopulmonary septal defect (15%) and type A interruption of the aortic arch (9%) are associated anomalies with the proximal type, but are rare in the distal type.1,9 Patient no. 4 had type A interrupted aortic arch and aortopulmonary septal defect. Burke and Rosenfeld10 treated similar malformations by median sternotomy, CPB, and circulatory arrest to repair the interrupted aortic arch and connect the RPA directly to the MPA. In contrast, we used a left thoracotomy to repair the arch interruption by end-to-end anastomosis, and a median sternotomy with CPB for patch repair of the aortopulmonary septal defect and interposition of a graft between the RPA and the MPA, without circulatory arrest.
Echocardiography usually provided sufficient information and anatomical details to plan the surgery. Only patient no. 3, whose LPA was not visualized well by echocardiography, required angiography prior to surgery. Angiography might also be needed to rule out other malformations. Anomalous PA from the ascending aorta is an indication for complete surgical correction as soon as possible to prevent irreversible changes occurring in the pulmonary arterial vasculature, because microscopic features of pulmonary hypertension have been seen during the first month of life.3 Penkoske and colleagues2 found that mortality from palliative procedures, such as ligation of an anomalous RPA, ligation of a PDA, or PA banding, reached 82%, whereas in definitive correction, the mortality was only 26%.5,11–12 Nakamura and colleagues12 described a 22-day-old infant who was diagnosed by angiography as having an anomalous RPA from the ascending aorta with stenosis in the middle portion of the RPA. At operation 3 months later, complete obstruction of the RPA was found 10 mm distal to its origin, and dissection beyond the trifurcation of the RPA showed that the atrophic changes in the RPA extended into the intrapulmonary segments and prevented any surgical repair.
Surgical correction of anomalous PA from the ascending aorta was first accomplished with a polyester fiber tube graft.13 Primary repair was reported by Kirkpatrick and colleagues,14 whilst van Son and Hanley15 suggested a modification using autogenous aortic and main PA flaps to permit primary repair. The use of native tissue retains the growth potential of the repair during childhood and reduces the risk of thrombus formation or infection. If the anomalous RPA originates from the posterior aspect of the ascending aorta, primary repair is generally undertaken because of its close proximity to the MPA. When the anomalous RPA arises from the right lateral aspect of the aorta, an interposition graft is more suitable unless a modification is used that might allow primary repair.3,12,15 Distinct anatomic variations of LPA originating from the ascending aorta have not yet been defined. Primary repair should be attempted, although an interposition graft may be an alternative.6 We used a Gore-Tex tube graft in all our patients. The surgeons who used direct anastomosis, performed it with CPB,15–17 however, we believe that in these sick babies with pulmonary hypertension and often non-pliable tissues, avoiding CPB is advantageous unless other anomalies are present.
Long-term results of correction of anomalous PA are minimal. Nakamura and colleagues12 followed 2 patients after repair by direct anastomosis, for 1 year and 6 years, and one patient with anomalous RPA for 2 years after graft repair; no signs of stenosis were found. Johnson and colleagues18 operated on a 1.6 kg baby with anomalous RPA and CATCH 22 syndrome at 11 days; direct anastomosis between the RPA and MPA was undertaken. Four months later, stenosis at the RPA required arterioplasty with bovine pericardium; at 4-years old, balloon angioplasty was needed for restenosis of the right upper lobe branch. Dodo and colleagues6 carried out direct anastomosis between the LPA and MPA in 2 patients with anomalous LPA and DiGeorge syndrome; one had constricted LPA after the operation and needed reconstruction with a pericardial patch. Burke and Rosenfeld10 operated on an infant with aortopulmonary septal defect, type A interrupted aortic arch, and anomalous RPA. They completely repaired all lesions, including end-to-end anastomosis of the aorta, and direct anastomosis between the RPA and the MPA. Four months after the operation, they found bilateral pulmonary artery hypoplasia. A vascular stent was placed in the proximal RPA. Balloon dilation of the stenosis of the aortic arch was also performed successfully. Our patient with the same collection of malformations needed re-operation after 1.5 years to replace the obstructed interposition graft. Recent series with a mean follow-up of 3 years and 9 years after direct anastomosis showed substantial risk of stenosis in the anastomotic site.16–17 Three of the 4 survivors in our series needed re-operation at an average of 2.7 years after the initial repair.
In our opinion, interposition of a graft is safe and reproducible, especially in sick babies with pulmonary hypertension, when CPB carries significant risk, and with non-pliable tissues and the risk of stenosis at the anastomotic site, although it carries the risk of re-operation. Our experience shows that re-operation can be undertaken without mortality or morbidity. Others have also found interposition grafts satisfactory.3,13 Whilst primary repair has the theoretical advantage of maintaining the growth potential of the tissue during childhood and reducing the risk of thrombus formation and infection, long-term results of primary repair are few and not event-free.6,10,16–17 Moreover, when the anomalous RPA arises from the right lateral aspect of the aorta, an interposition graft is more suitable.3,12
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REFERENCES
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- Kutsche LM, Van Mierop LH. Anomalous origin of a pulmonary artery from the ascending aorta: associated anomalies and pathogenesis. Am J Cardiol 1988;61:850–6.[Medline]
- Penkoske PA, Castaneda AR, Fyler DC, Van Praagh R. Origin of pulmonary artery branch from ascending aorta. Primary surgical repair in infancy. J Thorac Cardiovasc Surg 1983;85:537–45.[Abstract]
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- 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 [Japanese]. Jpn J Thorac Cardiovasc Surg 1998;46:380–4.[Medline]
- Fraentzel O. Ein Fall von abnormer Communication der Aorta mit der Arteria pulmonalis. Virchows Arch [A] 1868;43:420–6.
- 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;75:1294–5.[Medline]
- Kauffman SL, Yao AC, Webber CB, Lynfield J. Origin of the right pulmonary artery from the aorta. A clinical-pathologic study of two types based on caliber of the pulmonary artery. Am J Cardiol 1967;19:741–8.[Medline]
- Odell JE, Smith JC 2nd. Right pulmonary artery arising from ascending aorta. Am J Dis Child 1963;105:53–62.[Abstract/Free Full Text]
- Berry TE, Bharati S, Muster AJ, Idriss FS, Santucci B, Lev M, 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]
- 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]
- Goldberg R, Motzkin B, Marion R, Scambler PJ, Shprintzen RJ. Velo-cardio-facial syndrome: a review of 120 patients. Am J Med Genet 1993;45:313–9.[Medline]
- Nakamura Y, Yasui H, Kado H, Yonenaga K, Shiokawa Y, Tokunaga S. Anomalous origin of the right pulmonary artery from the ascending aorta. Ann Thorac Surg 1991;52:1285–91.[Abstract]
- Armer RM, Shumacker HB, Klatte EC. Origin of the right pulmonary artery from the ascending aorta. Report of a surgically corrected case. Sogo Rinsho 1961;24:662–8.[Abstract/Free Full Text]
- Kirkpatrick SE, Girod DA, King H. Aortic origin of the right pulmonary artery. Surgical repair without a graft. Circulation 1967;36:777–82.[Abstract/Free Full Text]
- van Son JA, Hanley FL. Use of autogenous aortic and main pulmonary artery flaps for repair of anomalous origin of the right pulmonary artery from the ascending aorta. J Thorac Cardiovasc Surg 1996;111:675–6.[Free Full Text]
- Prifti E, Crucean A, Bonacchi M, Bernabei M, Leacche M, Murzi B, et al. Postoperative outcome in patients with anomalous origin of one artery branch from the aorta. Eur J Cardiothorac Surg 2003;24:21–7.[Abstract/Free Full Text]
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- Johnson MC, Watson MS, Strauss AW, Spray TL. Anomalous origin of the right pulmonary artery from the aorta and CATCH 22 syndrome. Ann Thorac Surg 1995;60:681–3.[Abstract/Free Full Text]
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ABSTRACT
INTRODUCTION
