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Ann Thorac Surg 2004;77:484-487
© 2004 The Society of Thoracic Surgeons

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Original article: cardiovascular

Surgical management of aortopulmonary window and associated lesions

^a Department of Cardiothoracic Surgery, Anesthesiology and Heart Institute, Schneider Children's Medical Center of Israel, Rabin Medical Center, affiliated with the Sackler Faculty of Medicine, Tel-Aviv University, Petach Tikva, Israel

Accepted for publication August 6, 2003.

^* Address reprint requests to Dr Erez, Department of Cardiothoracic Surgery, Rabin Medical Center (Beilinson Campus), Petach Tikva 49100, Israel.
e-mail: eldade{at}clalit.org.il

	Abstract

Top Abstract Introduction Patients and methods Results Comment References

 
BACKGROUND: Aortopulmonary window is a rare congenital heart defect commonly associated with other cardiac anomalies. Although single institutional experience is low, several surgical techniques have been reported. The purpose of this study is to describe our approach to the management of aortopulmonary window and its associated lesions.

METHODS: Between February 1996 and November 2002, 13 patients underwent repair of aortopulmonary window. The age range went from 4 days to 5.5 months (mean 42 ± 52 days), with 9 patients younger than 1 month old. The weight range was from 1.9 to 6.7 kg (mean 3.5 ± 1.2 kg). Concomitant cardiac anomalies were present in 11 patients. The major additional anomalies were interruption of aortic arch in 4 patients and tracheal stenosis in 1 patient. Initial diagnoses were made using two-dimensional echocardiography only.

RESULTS: There was one postoperative death. In general, patients with aortopulmonary window and additional major defects had a prolonged intensive care unit and hospital stay when compared with the other patients. Follow-up time ranged from 2 months to 6.8 years (mean of 2.5 ± 2.2 years). There were no reoperations and no late deaths. Transcatheter balloon dilatation of the repaired aortic arch was required in 1 patient and of the right pulmonary artery in another. All other patients had good flow to both pulmonary arteries. No residual shunts were detected at the aortopulmonary window site, and pulmonary pressures were normal.

CONCLUSIONS: Aortopulmonary window may be effectively diagnosed with echocardiography. Early surgical treatment (neonatal period, if possible) is safe and associated with the best long-term results, even in the presence of other cardiac anomalies. Complete separation and reconstruction of both aorta and pulmonary arteries under direct vision may prevent recurrence and distortion of adjacent structures.

	Introduction

Top Abstract Introduction Patients and methods Results Comment References

 
Aortopulmonary window (APW) is an abnormal communication between the ascending aorta and the main pulmonary artery, just above two normally formed semilunar valves. It may occur alone, although it is more commonly associated with other cardiovascular anomalies. The wide variations in defect size and location and in the relationship between the ascending aorta and pulmonary arteries have led to the development of several different classifications [1–4].

Aortopulmonary window causes a significant arterial level systemic to pulmonary artery shunt, similar to a large patent ductus arteriosus (PDA), leading to congestive heart failure, failure to thrive, and the development of pulmonary hypertension. Surgical management is indicated at the time of diagnosis to prevent the development of irreversible pulmonary vascular disease. However, owing to the rarity of this entity, reports on treatment and outcome are limited to relatively small groups of patients [5–7]. Several surgical techniques have been described for the management of isolated APW [8–12] or combined with other complex lesions, mainly interrupted aortic arch (IAA) [13, 14]. The purpose of this report was to describe our experience with different surgical techniques in patients with APW and associated lesions.

	Patients and methods

Top Abstract Introduction Patients and methods Results Comment References

 
Between February 1996 and November 2002, 13 patients were diagnosed with APW and underwent repair at the Schneider Children's Medical Center of Israel.

There were 10 males and 3 females, ages ranged from 4 days to 5.5 months old (median 14.5 days old; mean 42 ± 52 days); 9 patients were less than 1 month old. The weight range was from 1.9 to 6.7 kg (mean weight 3.5 ± 1.2 kg). Two patients had noncardiac anomalies: hypothyroidism, and cleft lip and palate in 1 patient; and tracheal and bronchial stenosis in 1 patient.

Associated anomalies are presented in Table 1. Only 2 patients had isolated APW. Initial diagnoses were made with two-dimensional echocardiography. None of the patients underwent diagnostic cardiac catheterization, and all patients except 1 patient (weight < 2 kg) were assessed with intraoperative transesophageal echocardiography.

View larger version (33K): [in this window] [in a new window]   Fig 1. Aortopulmonary window defect.

View larger version (42K): [in this window] [in a new window]   Fig 2. The defect is approached directly through an incision made along the middle of the aortopulmonary window itself. Both aorta and pulmonary artery defects are closed under direct vision of adjacent structures.

View larger version (34K): [in this window] [in a new window]   Fig 3. The aorta and pulmonary arteries are completely separated. A different pericardial patch is used to close the defect in each vessel.

A modified surgical approach was used in 4 patients who underwent simultaneous repair of an IAA. The CPB was initiated with two arterial cannulas, one positioned in the aorta distal to the defect and the second placed through the PDA into the descending aorta. The patients were cooled to 18°C. In 3 patients, total circulatory arrest was used for the arch repair (mean time 23 ± 7.4 minutes). The pulmonary artery was detached from the aorta, the PDA was divided, and all PDA tissue was 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. In the fourth patient, total circulatory arrest was avoided. One arterial cannula was positioned at the base of the innominate artery, and after the patient was cooled to 25°C, the second cannula from the PDA was repositioned in the descending aorta just above the diaphragm. This allowed for continuous CPB during arch repair.

Cardiopulmonary bypass time ranged from 39 to 250 minutes, with a mean of 101 ± 58 minutes; aortic cross-clamping time ranged from 26 to 158 minutes, with a mean of 60 ± 39 minutes. The following associated procedures were performed combined with the APW repair: in 6 patients atrial septal defect (ASD) closure; in 4 patients PDA division and IAA repair; in 1 patient ventricular septal defect (VSD) patch closure, tracheal and bronchial stenosis repair using autologous resected trachea, and a glutaraldehyde preserved autologous pericardial patch.

	Results

Top Abstract Introduction Patients and methods Results Comment References

 
The sole perioperative death occurred in a 3.3-kg, 17-day-old patient with severe congestive heart failure. Acute liver and renal failure developed in the patient before the diagnosis was made. He had a large APW combined with a patent foramen ovale (PFO). The operation was done as an emergency salvage procedure, but liver function failed to recover and the patient died 32 days after surgery.

Of the remaining patients, those without IAA (n = 7) had a good recovery; intensive care unit stay ranged from 3 to 6 days (mean 4 ± 1.1 days), and hospital stay ranged 6 to 34 days, (mean 12 ± 9 days). The patients with APW and IAA had a longer recovery period, with an intensive care unit stay of 8 to 75 days (median 22 days), and a hospital stay of 17 to 105 days (median 51 days). Two patients had acute renal failure postoperatively and were treated with temporary peritoneal dialysis, and one of the two required prolonged ventilatory support due to left phrenic nerve paralysis and chylothorax. Both recovered without surgical intervention 4 weeks postoperatively.

Duration of follow-up ranged from 2 months to 6.8 years (mean of 2.5 ± 2.2 years). Follow-up data were available for the entire group from our Heart Institute database. Each patient was examined by echocardiography during follow-up on more than one occasion. There were no reoperations and no late deaths. One patient had successful balloon dilatation of an aortic arch narrowing that developed 4 months after APW and IAA repair (peak gradient reduced from 40 to 15 mm Hg). Another patient had successful balloon dilatation of the right pulmonary artery 7 months after combined APW/IAA repair (peak gradient reduced from 50 to 10 mm Hg) with postoperative mild stenosis of the left main bronchus. The remaining patients had good flow to both pulmonary arteries, no residual shunts at the APW site, and normal pulmonary pressures. At the latest follow-up visit none of the patients had evidence of aneurysm formation in the ascending aorta and there was no aortic or pulmonary valve insufficiency.

	Comment

Top Abstract Introduction Patients and methods Results Comment References

 
Abnormal partitioning of the aortic sac by the aorticopulmonary septum during embryologic development is thought to be the underlying cause of APW [3]. The size and location of the defect may vary, but it is limited to the area between the two semilunar valves and the branch pulmonary arteries. The exact location of the defect and the relationship it creates between the ascending aorta and the pulmonary arteries define its morphology. The variety of morphologic forms has led to the development of several different classifications of APW [1–4]. In Richardson's classification [4], which may be used as a guide for the appropriate surgical approach, APW is divided into three types: type I, simple defect located exclusively between the ascending aorta and main pulmonary artery; type II, defect located more distally between ascending aorta and pulmonary trunk with extension into the origin of the branch pulmonary artery (usually the right pulmonary artery); and type III, anomalous origin of the pulmonary artery from the ascending aorta with no defect between the aorta and pulmonary artery.

Aortopulmonary window may also be defined as simple or complex, which has prognostic importance. Simple APW is associated with no or only minor additional lesions (PFO, ASD, and PDA), and its surgical repair carries low morbidity and mortality. Complex APW is associated with major lesions (IAA, transposition of the great arteries, and tetralogy of Fallot), prognosis after surgical repair is worse, and is usually defined by the morphology of the associated lesion [6, 7]. Complex APW accounts for about 25% of all patients.

In the last 10 years echocardiography has served as the principal diagnostic tool in patients with congenital heart disease. Based on our experience, and that of others [6, 15], echocardiography has proven reliable for the diagnosis of APW. Multiplanar imaging is mandatory in order to determine the detailed morphology of the defect; the most useful plane is the parasternal short axis with cranial angulation. However, if the operator is not alert to the possibility of APW [12] the findings may be misinterpreted. The high pulmonary pressure and pulmonary vascular resistance results in a low-velocity flow across the defect, which is difficult to detect with color Doppler mapping. Exposing the patient to high fractional concentration of oxygen in a hood for several minutes may decrease pulmonary vascular resistance and thereby increase left-to-right shunting, which leads to a better Doppler signal. Some centers recommend additional diagnostic confirmation by cardiac catheterization. We believe cardiac catheterization should be performed only if the morphology cannot otherwise be defined or, as McElhinney and associates [6] suggested, in patients older than 6 months old to evaluate the presence of irreversible pulmonary vascular disease.

Today most centers agree that surgical treatment for APW should be performed as early as possible after birth. If the diagnosis is made later in life, operability is determined by assessment of pulmonary arterial resistance during cardiac catheterization [5–8]; at an older age, even in the presence of reversible pulmonary vascular resistance, repair of APW may not alter the development of severe pulmonary vascular disease later in life [7].

Since the first successful repair of APW by Gross in 1952, several surgical techniques have been used, from less invasive simple ligation or division without CPB to patch closure of the defect through the great vessels under direct vision using CPB and arresting the heart [8–14]. Our review of the literature clearly revealed that surgical experience with APW is limited, probably because the lesion is rare, and each institute treats only 1 or 2 patients annually. In the past, when the use of echocardiography was limited, simple ligation or division of APW carried a relatively high complication rate (recannalization, bleeding, pulmonary artery narrowing) due to the inadequate preoperative definition of the defect morphology, combined with the necessary dissection around the APW that led to a high risk of entering the defect and bleeding [7]. Today, division of the APW should be reserved for simple, type I relatively small size APW located at a safe distance from the branch pulmonary arteries and semilunar valves. Transcatheter closure of this type of APW has also been reported [16].

In the majority of our patients, we completely separated the aorta and pulmonary artery and closed the defect in each artery with a different pericardial patch. In this manner repair could be carried out under direct vision of the nearby structures in each artery. This approach is safe. It precludes recurrence of the defect and minimizes the chance of distorting adjacent structures. Long-term follow-up is necessary regarding the use of pericardial patch for repair of both aorta and pulmonary artery defects. However, the position of the defects in between the great arteries makes the likelihood of aneurysm formation small.

In 2 patients we used a transaortic approach to close the defect with a single pericardial patch. In most recent reports, this is the preferred technique for APW repair [6, 7, 17]. It allows good visualization of the origins of the coronary arteries and both semilunar valves. However it carries the potential for residual/recurrence of the defect because there is no definite separation of the two arteries.

Complex APW, according to most recent reports, is associated with the highest mortality and morbidity (in the form of late reinterventions) [5–7], related mainly to the associated lesion and not to the APW itself. Four of our patients underwent combined APW/IAA repair. None died. One patient had stenosis of the right pulmonary artery and left main bronchus probably because of increased tension and compression by the repaired arch. This may occur when patch augmentation is not used in primary repair of the arch. There is no evidence to suggest that recurrent arch stenosis is more common in patients with combined APW/IAA than those with VSD/IAA [18]. Our recent advancement in the repair of APW/IAA is the avoidance of circulatory arrest during arch repair. This strategy may allow the operation to be performed under moderate hypothermia, reducing the complications associated with hypothermic circulatory arrest [19].

In conclusion, considerable progress has been made in recent years in the diagnosis and surgical treatment of all forms of APW, with a significant decrease in morbidity and mortality. This report summarizes our 7-year experience with echocardiographic diagnosis and early surgical treatment (neonatal period if possible) with complete separation, and reconstruction of both aorta and pulmonary arteries under direct vision. The procedure was found to be technically successful, with no late deaths and no need for reoperation.

	References

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