Interact CardioVasc Thorac Surg 2007;6:200-203. doi:10.1510/icvts.2006.138503
© 2007 European Association of Cardio-Thoracic Surgery
Institutional report - Congenital |
Surgical experience of aortopulmonary window repair in infants
Anil Bhan,
Manju Gupta*,
Smartin Abraham,
Rajesh Sharma,
Shyam Sunder Kothari and
Rajnish Juneja
Departments of Cardiothoracic and Vascular Surgery and Cardiology, Cardiothoracic Center, All India Institute of Medical Sciences, New Delhi, India
Received 12 July 2006;
received in revised form 18 November 2006;
accepted 21 November 2006
*Corresponding author. Tel.: +91-9868301060.
E-mail address: drmgupta{at}hotmail.com (M. Gupta).
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Abstract
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The aim of this study was to retrospectively analyze our results of both simple and complex aortopulmonary window (APW) repair in infants. From September 1994 to May 2003, 21 infants which included 15 with simple APW (weight 3.9±0.8 kg and age 5.1±3.7 months) and six with complex APW (weight 4.03±1.1 kg and age 5±3.8 months) underwent APW repair at the All India Institute of Medical Sciences, New Delhi, India. The approach for APW repair was ligation without CPB in four patients, division and suturing using CPB in one patient, trans-aortic with Goretex patch closure in 11 patients, trans-pulmonary in one patient and trans-window in four patients. The hospital mortality was 13% and 33% for simple and complex APW, respectively. On mean follow-up of 39 months there was no re-operation or late death. An early repair of APW is mandatory to achieve a good surgical result. Trans-aortic repair of APW is the procedure of choice for all APWs, except in the case of large defects where anterior sandwich patch technique (trans-window repair) may be done. In our view, simple ligation without CPB should be avoided due to the possibility of residual APW and distortion of pulmonary artery.
Key Words: Congenital heart disease; Conotruncal abnormalities; Pulmonary artery hypertension
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1. Introduction
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Aortopulmonary window (APW) accounts for 0.1% of all cardiac anomalies [1]. As the natural history of APW is similar to that of a large VSD or PDA, early recognition is necessary for a successful surgical result before the onset of irreversible pulmonary vascular disease. A review of literature revealed limited surgical experience of APW repair, with each institute treating only one or two patients annually [2]. The aim of this study was to report our experience of 21 patients with APW who presented for surgical treatment and to retrospectively analyze our results of both simple and complex aortopulmonary window repair in infants.
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2. Patients and methods
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From September 1994 to May 2003, 21 infants underwent APW repair at the All India Institute of Medical Sciences, New Delhi, India. The medical records were reviewed with regards the clinical presentation, pathophysiologic findings, surgical treatment and hospital mortality. The study group included nine boys and six girls with simple APW weighing 2.8–5.5 kg (mean 3.88±0.82) and aged 1–12 months (mean 5.1±3.7) and five boys and one girl with complex APW weighing 2.7–5.8 kg (mean 4.03±1.13) and aged 0.1–11 months (mean 5.0±3.8) at the time of repair. Clinical presentation included varying degrees of cardiac failure, recurrent lower respiratory tract infection and/or failure to thrive. Two cases of complex APW with interrupted aortic arch required preoperative stabilization with prostaglandin infusion and ionotropic support. Associated cardiac anomalies seen in both groups are listed in Tables 1 and 2. Chest radiography revealed cardiomegaly and pulmonary plethora in all patients. Electrocardiographic findings included biventricular hypertrophy in 16 patients, left ventricular hypertrophy in two patients and right ventricular hypertrophy in three patients. Diagnosis of APW was made on two-dimensional echocardiography in all cases, but a correct anatomical type according to Richardson classification could not be diagnosed in the case of type II and III defects [5]. Cardiac catheterization was done in infants  6 months of age for evaluating the presence of irreversible pulmonary vascular disease and in case of complex APW for delineating the exact morphology. Catheterization study revealed a large left to right shunt (mean pulmonary/systemic flow ratio 2.8±1.5), elevated pulmonary artery pressure (mean 86.4±15.5) and increased pulmonary vascular resistance (mean 14.52±7.44) in all 11 patients.
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3. Operative technique
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Median sternotomy approach was used. Preoperative findings revealed Richardsons' type I APW in 15 patients, type II in three patients and type III in three patients [3]. Cardiopulmonary bypass was instituted after occluding pulmonary arteries with moderate hypothermia using two venous cannulas and a single arterial cannula positioned in the aorta distal to the defect in 17 patients. Aorta cross-clamped. Antegrade cold blood cardioplegia injected. The approach for APW repair was ligation without CPB in four patients, division and suturing using CPB in one patient, trans-aortic with Goretex patch closure in 11 patients, trans-pulmonary in one patient and trans-window in four patients. The interrupted aortic arch was repaired concomitantly under deep hypothermic circulatory arrest in two patients. The pulmonary artery was detached from the aorta, the PDA divided and the ductal tissue resected from the descending aorta. The mobilized descending aorta was directly anastomosed to the aortic window and the pulmonary artery repaired with an autologous pericardial patch.
The associated procedures performed, combined with APW repair, were ASD closure in three patients, VSD closure in two patients, PDA ligation in seven patients, total correction in two patients and PDA division with IAA repair in two patients.
The cardiopulmonary bypass time ranged from 25–165 min (mean 80.9+38.9 min), aortic cross-clamping time ranged from 29–119 min (mean 47.2+22.88 min). Operative data are summarized in Table 3.
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4. Results
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The hospital mortality was 13% (2/15) and 33% (2/6) for simple and complex APW, respectively. One patient with preoperative respiratory infection died due to septicaemia on the 10th postoperative day. A two-month-old child, after APW repair with total correction for Tetrology of Fallot, died suddenly in the ICU on postoperative day 0. The cause of death could not be identified. One patient, following type III APW and type B IAA repair, developed renal failure with multiorgan dysfunction syndrome and expired on POD eight. One patient died on the first POD due to post-CPB pulmonary hemorrhage. The operative results are summarized in Table 4.
Among the 17 survivors, the mean ICU stay was 3.53 days (range 1–15 days) and the mean hospital stay was 11.3 days (range 6–30 days). Re-exploration was required in one patient for bleeding. One patient recovered following prolonged ventilatory support for repeated lung collapse.
Duration of follow-up ranged from 2 months to 6.8 years (mean 39 months). There were no re-operation or late death. All the patients were subjected to chest radiography and echocardiography at one and three months following discharge and thereafter based on clinical findings. Chest X-ray done at one month revealed a decrease in cardiac size with absence of pulmonary plethora. Echocardiography showed no significant distortion of the great vessels in 13 patients, a 2-mm residual APW in two patients following ligation, a gradient of 16 mmHg at the origin of right pulmonary artery following ligation in one patient and following trans-aortic repair in one patient. As all these four patients were doing well clinically and radiographically, they are being kept under frequent regular follow-up. None of the patients had evidence of ascending aortic aneurysm formation and aortic and pulmonary valve insufficiency.
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5. Discussion
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An aortopulmonary window (APW) is a communication between the pulmonary artery (PA) and the ascending aorta in the presence of two separate semilunar valves. Mori et al. classified the APW as proximal, distal and total, depending on the extent of lesion [5]. Richardson classified APW as simple defects between the ascending aorta and pulmonary trunk (type I), defects extending distally to include the origin of the right main pulmonary artery (type II), and anomalous origin of the right main pulmonary artery from the ascending aorta with no other aortopulmonary communication (type III) [3]. Ho et al. further modified it with the advent of trans-catheter device closure of APW [5]. Recently, Jacobs and the Congenital Heart Surgery Database Committee have advocated the use of hierarchical levels for APW [5].
Simple APW may be associated with PDA, right aortic arch (RAA) and patent foramen ovale (PFO). Complex APW is associated with VSD, IAA, TGA, TOF or coronary artery anomalies.
Echocardiography has proved itself as a reliable diagnostic tool [2], though type II and III lesions are difficult to differentiate from PDA [4]. Even in our series, echocardiography failed to make correct morphological diagnosis in patients with type II and III defects.
Cardiac catheterization and cineangiography with retrograde aortography is done in infants 6 months of age for evaluating the presence of irreversible pulmonary vascular disease and in case of complex APW for delineating the exact morphology.
The spectrum of the surgical techniques which evolved with time include simple ligation [6], division and suturing without CPB [7], division and suturing with CPB [8], trans-pulmonary artery closure using CPB [9], trans-window closure (anterior sandwich patch closure) [10], trans-aortic direct closure [11] to trans-aortic patch closure of the defect using CPB and arresting the heart [12].
As the trans-aortic repair using CPB allows accurate visualization of coronaries arteries and right pulmonary artery reconstruction using a dacron patch without distorting either the aorta or the pulmonary artery, it is the recommended approach for most patients with an APW [5], except in the case of large APW where there is an additional hazard of catching the left coronary artery in a transluminal stitch [4]. In such cases, alternative techniques like the anterior sandwich patch closure technique (trans-window technique) that allows better visualization of the defect, coronary ostia and the aortic cusps, has been used with success [5,10]. The approach through pulmonary arteriotomy should be avoided as it has inadequate exposure with the risk of damaging the unseen aortic cusps and coronary ostia [4] and results in a higher need for re-intervention postoperatively [13]. Though ligation seems a simpler option for repair of simple small size APW located at a safe distance from the branch pulmonary arteries and semilunar valves, however, it may lead to an incomplete closure of the defect, coronary artery injuries and distortion of the pulmonary artery. In our series following ligation the incidence of residual APW and distortion of pulmonary artery leading to minor trans-pulmonary gradient was 2/4 (50%) and 1/4 (25%), respectively. As the distortion of the pulmonary artery involves only a part of the circumference of the artery, it should improve with the growth of the pulmonary arteries with age. Therefore, ligation of APW may be considered with caution only in very sick infants who would not be able to withstand cardiopulmonary bypass, keeping in mind the possibility of residual APW and distortion of pulmonary artery. Recently, isolated successful attempts of trans-catheter closure of APW have been reported [14]. In our opinion, as the APW is usually an oblong defect and is rarely circular, therefore, it is not suitable for device closure without vessel distortion. In the present study, the approach for APW repair was ligation without CPB in four patients, division and suturing using CPB in one patient, trans-aortic with Goretex patch closure in 11 patients, trans-pulmonary in one patient and trans-window in four patients with no re-operation.
Postoperative mortality depends on the age of the patient at operation, status of preoperative pulmonary vascular disease and presence of associated intra-cardiac defects [5]. Early diagnosis and repair is an essential pre-requisite for a successful surgical result before the pulmonary vascular disease becomes irreversible. The hospital mortality is 8–25% with a higher mortality in case of APW associated with interrupted aortic arch [5,13]. Better pediatric cardiopulmonary bypass, myocardial protection, deep hypothermic circulatory arrest and surgical techniques have led to performance of single-stage repair of APW and the associated intra-cardiac defect, including interrupted aortic arch in neonates with improved outcome, though re-intervention for aortic arch obstruction is common [15]. In our series, complex APW was present in six cases, which was the reason for a hospital mortality of 19% (4/21). The long-term survival was good, with no re-operation or late death in our series. As the occurrence of postoperative pulmonary hypertensive crisis is well documented, routine placement of a pulmonary arterial line has been advocated for postoperative pulmonary artery pressure monitoring.
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6. Conclusions
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APW is a rare cardiac anomaly with each cardiac surgical institute having limited surgical experience of treating only one or two patients annually. As the natural history of APW is similar to that of a large VSD or PDA, early recognition is necessary for a successful surgical result before the onset of irreversible pulmonary vascular disease in both simple and complex APW. Patients with complex APW are sicker at the time of presentation to the hospital with a poorer prognosis after surgical repair. Diagnosis of APW can be made in neonates with reasonable accuracy using echocardiography, though cardiac catheterization with cine-angiography may be required for clear anatomic delineation of type II and III defects. We advocate trans-aortic repair for all APW except in large defects where anterior sandwich patch technique (trans-window repair) may be done. In our view, simple ligation without CPB should be avoided due to the possibility of residual APW and distortion of pulmonary artery. The possible occurrence of pulmonary hypertensive crisis should be kept in mind while managing these children in the postoperative period.
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Abstract
1. Introduction