Ann Thorac Surg 2008;85:1407-1411. doi:10.1016/j.athoracsur.2007.11.081
© 2008 The Society of Thoracic Surgeons
Original Articles: Pediatric Cardiac
Surgical Results of Anomalous Origin of the Right Pulmonary Artery From the Ascending Aorta Including Reoperation for Infrequent Complications
Noriyoshi Kajihara, MDa,*,
Yutaka Imoto, MDa,
Masato Sakamoto, MDa,
Yukie Ochiai, MDa,
Meikun Kan-o, MDa,
Kunitaka Joo, MDb,
Mamie Watanabe, MDb,
Tetsuji Yuge, MDb,
Toshihide Asou, MDc,
Yuko Takeda, MDc,
Akira Sese, MDa
a Department of Cardiovascular Surgery, Kyushu Koseinenkin Hospital, Kitakyushu, Japan
b Department of Pediatric Cardiology, Kyushu Koseinenkin Hospital, Kitakyushu, Japan
c Department of Cardiovascular Surgery, Kanagawa Children’s Medical Center, Yokohama, Japan
Accepted for publication November 28, 2007.
* Address correspondence to Dr Kajihara, Department of Cardiovascular Surgery, Kanagawa Children’s Medical Center, 2-138-4 Mutsukawa, Minami-ku, Yokohama, 232-8555, Japan (Email: n-c.kaji{at}f6.dion.ne.jp).
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Abstract
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Background: We evaluated the results of surgery for an anomalous origin of the right pulmonary artery from the ascending aorta.
Methods: From August 1986 to December 2005, 8 children (6 neonates) aged 7 to 180 days (mean, 35 ± 59 days) with anomalous origin of the right pulmonary artery from the ascending aorta underwent surgical repair at our institute. All except one child, who had the distal form, had the proximal form. Cardiac catheterization showed that the left pulmonary artery to systemic pressure ratio was 1.0 or more. Surgery was performed by direct anastomosis in 7 patients and by graft interposition in 1.
Results: There were no operative or late deaths. All patients postoperatively underwent cardiac catheterization that showed decreased left pulmonary artery to systemic pressure ratio ranging from 0.2 to 0.6. Follow-up periods ranged from 2 months to 13 years. We undertook reoperations for two infrequent postoperative causes. One patient exhibited significant supravalvar aortic stenosis and required patch enlargement of the ascending aorta 3 years after operation. The other patient (with the distal form) needed a reoperation after 1 month because of progressive stenosis at the anatomic site. Graft interposition was performed, and histopathologic examination showed that the tissue from the stenotic region looked like that of a ductus.
Conclusions: We undertook surgical repair for anomalous origin of the right pulmonary artery from the ascending aorta. Pulmonary hypertension was improved in all patients. Careful follow-up was necessary to detect supravalvar aortic and anastomotic stenosis early and late after operation.
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Introduction
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An anomalous origin of the right pulmonary artery from the ascending aorta (AORPA), a condition in which the right pulmonary artery arises from the ascending aorta in the presence of separate aortic and pulmonary valves [1, 2], is an unusual congenital cardiac anomaly. Patients characteristically present with early infantile respiratory distress and heart failure [3]. Pressure in the pulmonary artery arising from the aorta is systemic in almost all patients. As well, pressure in the pulmonary artery from the pulmonary trunk elevates until reaching a systemic or suprasystemic level. This phenomenon has not been fully explained, but several mechanisms have been postulated as the cause of bilateral pulmonary hypertension. These include circulating vasoconstrictor substances, neurogenic crossover from an unprotected lung, and development secondarily after left ventricular failure [4]. Because it has been shown that progressive pathologic changes occur in lung subjected to systemic pressures [5], surgical treatment is recommended as soon as a diagnosis is confirmed during the neonatal period. In this study, we aimed to review our surgical experience of AORPA, and to elucidate any problems arising after surgical treatment.
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Patients and Methods
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Patients
From August 1986 to December 2005, 8 children, 5 boys and 3 girls aged from 7 to 180 days (mean, 35 ± 59 days), including 6 neonates, with weights from 2.6 to 4.6 kg (mean, 3.2 ± 0.7 kg), with AORPA underwent surgical repair at Kyushu Koseinenkin Hospital. All patients are listed in Table 1. Common symptoms at presentation were tachypnea in 6 and cyanosis in 4. Cardiac defect, abnormal facies, thymic hypoplasia, cleft palate, and hypocalcemia syndrome (CATCH22) were diagnosed in patient 4. Patient 6 had preoperative cardiogenic shock and disseminated intravascular coagulopathy. All children were diagnosed by echocardiography or angiography (Fig 1) and cardiac catheterization. These examinations assessed cardiac function and associated anomalies. Clinical records were reviewed to document clinical features, operative procedures, and perioperative courses. The study was approved by the ethics committee of Kyushu Koseinenkin Hospital. Appropriate parental informed consent was obtained for each patient.
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Fig 1. Preoperative angiography. (A) The anomalous right pulmonary artery (RPA) arises proximally from the right posterior aspect of the ascending aorta (Ao) close to the aortic valve. (B) The left pulmonary artery (LPA) originates from the main pulmonary trunk. (LV = left ventricle; MPA = main pulmonary artery.)
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Except for patient 8, the right pulmonary artery (PA) originated from the right or posterior aspect of the ascending aorta within 1 to 3 cm of the aortic valve (proximal form; Fig 2A) in all children. In patient 8 the left PA originated from the main pulmonary trunk. All patients had simple associations like patent ductus arteriosus (PDA), patent foramen ovale, and aberrant right subclavian artery. In patient 8, the right PA originated from the distal portion of the ascending aorta just proximal to the origin of the innominate artery (distal form) [2]. This male infant had an associated atrial septal defect, PDA, and coarctation of the aorta.
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Fig 2. (A) The right pulmonary artery (RPA) originated from the right posterior aspect of the ascending aorta (Asc Ao). (B) The right pulmonary artery was cut from the ascending aorta at its origin. (RA = right atrium.)
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Preoperative cardiac catheterization was performed in 6 children. Pressure in the PA was elevated to systemic or suprasystemic levels, and the left PA to systemic pressure ratio was 1.0 or more in all patients (Table 1).
Operative Technique
Surgery was undertaken through median sternotomy. Cardiopulmonary bypass was used in all the patients except for patient 6. Ascending aortic and bicaval cannulation was implemented, and mild or moderate hypothermic cardiopulmonary bypass maintained. After aortic cross-clamping and occlusion of the right PA, cardiac arrest was obtained with the use of a cold crystalloid cardioplegic solution. The right PA was cut from the ascending aorta at its origin (Fig 2B) and anastomosed to the right lateral aspect of the main PA in end-to-side fashion (Fig 3). The defect in the aorta was directly sutured. Cardiopulmonary bypass time and aortic cross-clamping time were 100 ± 26 minutes and 43 ± 24 minutes, respectively.
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Fig 3. Schema of the right pulmonary artery (RPA) anastomosed to the main pulmonary artery (MPA). (Asc Ao = ascending aorta; LPA = left pulmonary artery.)
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Cardiopulmonary bypass was not used in patient 6, who had disseminated intravascular coagulopathy; here the right lateral aspect of the main PA was carefully clamped to keep the blood supply to the left PA. Graft interposition was performed using a 4-mm polytetrafluoroethylene graft. After anastomosis of the graft to the main PA, an anastomosis of the graft to the right PA was performed. The graft was reconstructed behind the ascending aorta.
Associated procedures undertaken included PDA ligation in 5 patients, coarctectomy and arch repair in 1 patient, and closure of the atrial septal defect in 1 patient (Table 2).
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Results
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Early Postoperative Course
There were no operative deaths. Early postoperative pulmonary hypertension crisis was identified in patient 5. This male infant required open chest cardiac massage and nitric oxide inhalation. He underwent sternal closure at postoperative day 3 and was weaned from the mechanical ventilator at postoperative day 5. In patient 6, who had preoperative cardiogenic shock and disseminated intravascular coagulopathy, the sternum was opened for 5 days and required peritoneal dialysis for 10 days.
Durations of postoperative mechanical ventilatory support time ranged from 1 to 18 days (mean, 6.3 ± 6.3 days). Three required postoperative nitric oxide inhalation. Other early postoperative complications were mediastinitis in 1, and complete atrioventricular block requiring permanent pacemaker implantation in another.
Postoperative cardiac catheterization (Fig 4) was undertaken in all patients, and showed decreased left PA to systemic pressure ratio ranging from 0.18 to 0.61 (mean, 0.35 ± 0.14).
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Fig 4. Postoperative angiography. In this patient, neither supravalvar aortic stenosis (A) nor pulmonary anastomotic stenosis of the right pulmonary artery (B) is present. (Ao = aorta; LV = left ventricle; RPA = right pulmonary artery; RV = right ventricle.)
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Follow-Up and Reintervention
There were no late deaths. Follow-up periods ranged from 2 months to 13 years (mean, 8.2 ± 4.6 years). We undertook reoperations for two infrequent postoperative courses as below.
Patient 3 exhibited significant supravalvar aortic stenosis with a pressure gradient of 44 mm Hg. This male infant underwent patch enlargement of the ascending aorta 3 years after the initial operation, and postrepair catheterization showed no residual pressure gradient. (Evaluation of the catheterization showed that the pressure gradient improved from 44 to 0 mm Hg [peak-to-peak]). Follow-up echocardiography demonstrated normal hemodynamic function after an interval of 9 years.
Patient 8 needed a reoperation 1 month after the initial operation because of progressive right PA stenosis at the anastomotic site. After exclusion of the stenotic region, graft interposition was performed using a 5-mm extruded polytetrafluoroethylene graft. Histopathologic examination showed that the tissue of the stenotic region looked like that of a duct. This graft needed further replacement (14-mm extruded polytetrafluoroethylene graft) 9 years later.
Three patients experienced mild to moderate pulmonary stenosis at the anastomotic site and required catheter balloon angioplasty. The duration between surgical treatment and balloon angioplasty ranged from 2 to 7 years.
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Comment
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Classification of Anomalous Origin of the Right Pulmonary Artery
Anomalous origin of the right PA from the ascending aorta, AORPA, is classified into two subgroups according to associated cardiac anomalies [6]. One group of patients has simple associated anomalies such as PDA or patent foramen ovale. Patients in this group have been demonstrated to have severe clinical symptoms, but better functional outcomes after surgical treatment. The other group has major associated cardiac anomalies such as tetralogy of Fallot, aortopulmonary septal defects, interruption of the aortic arch, or ventricular septal defects. Previous reports have shown that major associated cardiac anomalies are identified in 28.7% of patients, and that outcomes in this group depend on these various associated anomalies [7]. In our patients, all except 1 had simple associated lesions, whereas the most commonly associated anomaly was PDA. Only 1 patient had aortic coarctation.
There is another classification of AORPA, this taken from aspects of the morphologic features [2, 8]. In the proximal form, the anomalous PA arises proximally from the posterior or left posterior aspect of the ascending aorta close to the aortic valve. Only in patient 8 did the left PA originate from the main pulmonary trunk. In the literature, it appears that most patients have been regarded as this type. Kutsche and Van Mierop [2] classified 85% (76 of their 89 AORPA patients) as the proximal form in their pathologic and anatomic study. In the distal form, the anomalous PA originated from the ascending aorta just proximal to the innominate artery or from the base of the artery itself. In several cases of the distal form, the anomalous right PA had a narrowed site that was usually distant from its origin [8]. The distal form is considered to be pathogenetically distinct from the proximal form [1, 2]. In our patients, 1 patient was classified as distal form, but this male infant did not have preoperative narrowing of the PA.
Supravalvar Aortic Stenosis
One patient in our study showed significant postoperative supravalvar aortic stenosis, and patch enlargement of the ascending aorta was required 3 years after the initial operation. Postoperative supravalvar aortic stenosis has not, as far as we can ascertain, been reported before as a complication of postoperative AORPA. However, there is a potential risk from this complication because excision of the right PA needs to be rather large to get a sufficiently wide anastomosis. We thus suggest that an aortic defect should be treated by autologous pericardial patch or prosthetic patch if even a slight stenosis is anticipated.
Anastomotic Stenosis
Patient 8 needed to undergo reoperation because of progressive anastomotic stenosis. Histopathologic examination of the stenotic region showed very similar findings for the arterial duct. This male infant had the distal form of AORPA with atrial septal defect, PDA, and coarctation of the aorta. The literature shows that patients with the distal form of AORPA frequently have narrow PAs [2, 8]. Although we did not use prostaglandins preoperatively for this patient, the anomalous right PA was not narrowed. We considered that precautions against progressive anastomotic stenosis are especially needed in patients of the distal form of AORPA, even if there is not preoperative pulmonary stenosis.
Significant stenosis at the anastomotic site requiring further reintervention is an important cause of morbidity; and from past studies ranges from 12.5% to 40% for an anomalous origin of one PA from the ascending aorta [6, 9]. However, these reports show that many postoperative significantly stenotic patients presented with AORPA, and there were only a few patients of an anomalous origin of the left PA from the ascending aorta. Hence, we inferred that in our study, the rate of stenosis at the anastomotic site of AORPA was not large. Although several techniques have been reported [7, 10, 11], long-term results remain to be clarified.
Conclusions
We evaluated the surgical results for anomalous origins of the right PA from the ascending aorta (AORPA). All patients but 1 had the proximal form. There were no hospital or late deaths. Two patients required reoperations; 1 had significant supravalvar aortic stenosis, and patch enlargement of the ascending aorta was required 3 years later; the other had progressive anastomotic stenosis that required graft interposition to repair. Careful follow-up is clearly needed for supravalvar aortic and anastomotic stenoses in early and late postoperative courses for patients diagnosed with AORPA.
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References
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- 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;85:537-545.[Abstract]
- Kutsche LM, Van Mierop LH. Anomalous origin of a pulmonary artery from the ascending aorta: associated anomalies and pathogenesis Am J Cardiol 1988;61:850-856.[Medline]
- Fontana GP, Spach MS, Effmann EL, Sabiston Jr DC. Origin of the right pulmonary artery from the ascending aorta Ann Surg 1987;206:102-113.[Medline]
- Keane JF, Maltz D, Bernhard WF, Corwin RD, Nadas AS. Anomalous origin of one pulmonary artery from the ascending aorta: diagnostic, physiological and surgical considerations Circulation 1974;50:588-594.[Abstract/Free Full Text]
- Yamaki S, Suzuki Y, Ishizawa E, et al. Isolated aortic origin of right pulmonary artery: report of a case with special reference to pulmonary vascular disease in the left and right lungs Chest 1983;83:575-578.[Medline]
- Peng EW, Shanmugam G, Macarthur KJ, Pollock JC. Ascending aortic origin of a branch pulmonary artery—surgical management and long-term outcome Eur J Cardiothorac Surg 2004;26:762-766.[Abstract/Free Full Text]
- Prifti E, Crucean A, Bonacchi M, et al. Postoperative outcome in patients with anomalous origin of one pulmonary artery branch from the aorta Eur J Cardiothorac Surg 2003;24:21-27.[Abstract/Free Full Text]
- Nakamura Y, Yasui H, Kado H, et al. Anomalous origin of the right pulmonary artery from the ascending aorta Ann Thorac Surg 1991;52:1285-1291.[Abstract]
- Abu-Sulaiman RM, Hashmi A, McCrindle BW, Williams WG, Freedom RM. Anomalous origin of one pulmonary artery from the ascending aorta: 36 years’ experience from one centre Cardiol Young 1998;8:449-454.[Medline]
- McMurtry K, Srivastava S, Nguyen KH. Description of a new technique for reimplanting the anomalous right pulmonary artery Ann Thorac Surg 2003;76:628-630.[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;111:675-676.[Free Full Text]
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Abstract
Introduction
