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Ann Thorac Surg 2006;81:950-957
© 2006 The Society of Thoracic Surgeons

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Original article: Cardiovascular

Outcomes in 45 Children With Ductal Origin of the Distal Pulmonary Artery

^a Division of Cardiology, Department of Pediatrics, University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada
^b Department of Diagnostic Imaging, University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada
^c Department of Cardiovascular Surgery, University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada

Accepted for publication August 29, 2005.

^_* Address correspondence to Dr McCrindle, The Hospital for Sick Children, 555 University Ave, Toronto, ON M5G 1X8, Canada (Email: brian.mccrindle{at}sickkids.ca).

Pediatric cardiac surgery: To participate in The Annals of Thoracic Surgery CME Program, please visit http://cme.ctsnetjournals.org.

 

	Abstract

Top Abstract Introduction Patients and Methods Results Comment Footnotes References

 
BACKGROUND: There are no large series describing the morphologic spectrum and the clinical outcomes of children with ductal origin of the distal pulmonary artery (PA).

METHODS: Medical records were reviewed for all children presenting between 1970 and 2001. Angiograms were reviewed at presentation and at last available follow-up.

RESULTS: Forty-five patients were identified with median presenting age of 14 days (range, birth to 6.5 years). Ductal origin of the distal PA occurred as an isolated finding in 16 patients (36%), with tetralogy of Fallot in 12 (27%), with pulmonary atresia–ventricular septal defect in 13 (29%), and with heterotaxy in 4 (9%). Diagnostic pulmonary venous wedge angiography was performed in 21 patients (47%). Surgical procedures were undertaken in 31 patients, and were initial systemic-ductal PA shunt in 13 patients, interposition graft in 6, direct anastomosis to the main PA in 2, ductal PA banding in 2, unifocalization of the ductal PA with complete or staged pulmonary atresia-ventricular septal defect repair in 7, and heart transplantation in 1 patient. Surgical revision was required in 3 patients and catheter interventions in 12 patients. Overall 20-year survival was 70% and was improved among patients without congestive heart failure at presentation (p = 0.08, hazard ratio: 2.81). Reconstruction of the ductal PA decreased the prevalence of pulmonary parenchymal hypoplasia (p < 0.001) and scoliosis at last available follow-up.

CONCLUSIONS: Ductal origin of the distal PA is associated with important multisystem morbidity and mortality. Early diagnosis and repair of the ductal PA, especially in children presenting with pulmonary overcirculation, may improve outcomes.

Ductal origin of the distal pulmonary artery (PA) is a congenital condition in which the right or left PA supplying all the bronchopulmonary segments of the lung is in continuity with the distal end of the arterial duct (AD), without continuity to the main PA [1]. Closure of the AD results in progressive loss of blood flow to the dependent PA with perfusion of the affected lung dependent on either the bronchial or anomalous systemic collateral arteries, and is associated with multisystem morbidity. Pulmonary hypertension, lung and hemithorax hypoplasia, hemoptysis, scoliosis, high altitude pulmonary edema, and respiratory failure can occur [1–11]. Appreciation of the pathophysiology, improved diagnostic imaging, and early intervention may prevent these sequelae [6, 7, 12–20]. That such a PA is not "absent" but undergoes progressive diminution upon closure of AD has been recognized [1, 7, 8, 12–17]. We sought to determine the morphologic spectrum, impact of interventions, residual disease burden, and survival of patients with ductal origin of the distal PA.

	Patients and Methods

Top Abstract Introduction Patients and Methods Results Comment Footnotes References

 
Patients
The databases of the Divisions of Cardiology and Pathology at the Hospital for Sick Children, Toronto, were reviewed to identify patients younger than 10 years presenting between January 1970 and April 2001.

Imaging Data
Angiograms were reviewed. A PA was deemed to have ductal origin when not confluent to the main PA with evidence of an ipsilateral AD. A ligamental AD was identified from a remnant diverticulum at typical sites on the aortic arch (Fig 1). Bilateral ductal origin of the distal PAs was identified by the presence of bilateral ADs with absence of continuity between one of the distal PAs and the main PA. Patients with pulmonary atresia were excluded from the study if aortopulmonary collateral arteries were a sole supply to bronchopulmonary segments of the lung without branches from the vessel at the distal end of AD. Pulmonary artery dimension was estimated by measuring the PA proximal to the upper lobe branch on the initial and the most recent angiogram with correction for magnification. The estimated dimension was indexed to the body surface area to normalize for age. The AD was described as follows: patent with opacification of the pulmonary artery, nonpatent diverticular, and nonpatent in the absence of angiographic findings of patency or diverticulum when there were operative findings of an AD. Plain radiographic films of the chest were reviewed for lung hypoplasia, which was classified as grade 1, 25%; grade 2, 25% to 50%; grade 3, more than 50%; and grade 0, no reduction of lung volume as compared with the contralateral lung.

View larger version (172K): [in this window] [in a new window]   Fig 1. (A) Selective angiogram showing arterial duct from the base of innominate artery with right pulmonary artery at its distal end. The triad of imaging findings with ligamental origin of the pulmonary artery is as follows: (B) diverticulum on the aortic arch on the same side as (C) nonopacification of the pulmonary artery and (D) demonstration of its presence by a pulmonary venous wedge angiogram.

Data Analysis
Results are presented as number (percent) of patients, median with range or mean ± SD as appropriate. Where there are missing data, the number of nonmissing values is given. Time-related survival was determined by Kaplan-Meier estimates. The independent effect of the following variables on time-related survival was sought using Cox's proportionate hazard modeling: age at presentation, age at diagnosis, presenting features, associated cardiac and non-cardiac lesions, medical interventions, surgical interventions, transcatheter interventions, size of the ductal PA and the contralateral PA at diagnosis and at the latest angiographic follow-up, and the patency of AD at presentation. Changes in the PA dimensions from presentation to latest follow-up were compared using paired t tests. All analyses were performed using SAS statistical software Version 8 (SAS Institute, Cary, North Carolina) using default settings.

	Results

Top Abstract Introduction Patients and Methods Results Comment Footnotes References

 
Demographics and Presentation
Forty-five eligible patients were identified. Mean birth weight (n = 27) was 3.17 kg (± 0.57 kg). Median age at presentation was 14 days (range, birth to 6.5 years). Patients with an isolated ductal PA without associated cardiac lesions presented significantly later (median, 3 months; range, birth to 8 years) than patients with associated complex cardiac lesions (median, 1 day; range, birth to 4 days; p = 0.002). Presenting features were cyanosis (n = 30), heart murmur (n = 23), congestive heart failure (n = 12), frequent pulmonary infections (n = 9), pulmonary hypertension (n = 7), failure to thrive (n = 6), dextrocardia on chest radiography (n = 4), electrocardiographic right ventricular hypertrophy (n = 3), chest pain (n = 2), small hemithorax on chest radiography (n = 2), and feeding difficulties (n = 1). Of patients with cyanosis, 13 had duct-dependent pulmonary circulation.

Diagnosis
Median age at diagnosis was 69 days (range, birth to 7.9 years). Patients with isolated ductal PA were diagnosed later (median, 98 days; range, 19 days to 6.8 years) than those with complex cardiac lesions (median, 16 days; range, birth to 7.9 years; p = 0.10). Pulmonary venous wedge angiograms (n = 27) were performed in 21 patients (47%) to demonstrate the presence of, or further evaluate a known ductal PA (n = 22), identify the pulmonary veins (n = 1), estimate PA pressure (n = 1), and estimate PA size (n = 1).

Morphologic Spectrum
Ductal PA occurred in isolation (ie, without a major intracardiac malformation) in 16 patients (36%), with tetralogy of Fallot in 12 (27%), with pulmonary atresia–ventricular septal defect in 13 (29%), and with heterotaxy in 4 (9%). Six of the 14 patients (13%) with bilateral AD had bilateral ductal PAs. The AD giving origin to the ductal PA was patent in 21 patients and nonpatent diverticular in 18 patients. In those with unilateral ductal PA, the ductal PA was contralateral to the aortic arch in all patients without a major intracardiac malformation, in 5 (42%) with tetralogy of Fallot, and in 2 (15%) with pulmonary atresia–ventricular septal defect. Cardiac lesions grouped according to diagnostic type are listed in Table 1.

Direct end-to-end anastamosis to the main PA was performed in 2 patients as an initial surgical procedure and in 2 after initial systemic-ductal PA shunt operation. Reoperation (n = 7) defined as surgical intervention after achievement of complete repair was undertaken in 6 patients. After direct anastamosis of the ductal PA to the main PA, anastomotic revision was required in 3 patients. Of the 13 patients with pulmonary atresia–ventricular septal defect, 6 underwent unifocalization procedure of the ductal PA as a single-stage repair and 1 had unifocalization as part of a staged repair. Initial banding of the ductal PA was performed in 2 patients to control heart failure. Cardiopulmonary bypass time was significantly shorter (mean, 87 ± 36 minutes) in patients with isolated ductal PA than it was in patients with complex cardiac lesions (mean, 154 ± 57 minutes; p = 0.061). Aortic cross-clamping, performed in 18 procedures (median time, 43 minutes; range, 23 to 137) was not required in patients undergoing repair for isolated ductal PA.

Transcatheter Strategies
Catheter interventions (34 procedures), undertaken in 16 patients (35%), included procedures after surgical intervention in 12 (26%) and presurgical intervention in 7, including stent placement (n = 3) and balloon angioplasty (n = 1) of the AD, balloon atrial septostomy (n = 1), and transeptal puncture (n = 2). Median time to postsurgical catheter intervention was 6 months (range, 0 to 3.6 years). Procedures on the ductal PA were performed in 7 patients; these were balloon angioplasty (n = 11), stent implantation (n = 6), and stent dilation (n = 2). Procedures on the nonductal PA included angioplasty (n = 4), stent implantation (n = 2), and stent dilation (n = 1). Transcatheter reintervention (12 procedures) for augmentation of PA dimension was undertaken in 5 patients. Stent implantation in Blalock-Taussig shunt (n = 1), conduit dilation (n = 1), stent implantation in right ventricular outflow tract (n = 2), balloon dilation of right ventricular outflow tract (n = 1), intraoperative balloon angioplasty of the PA (n = 1), and coil embolization of major aortopulmonary collateral artery (n = 1) were other catheter interventions.

Results of Intervention
Figure 2 is a flow diagram outlining events after presentation in all 45 patients. Interventions were performed more often (p = 0.005) in patients with complex defects (86%) versus isolated ductal PA (44%). Intracardiac repair with confluent PAs was achieved in 8 patients (67%) with tetralogy of Fallot and in 10 (77%) with pulmonary atresia–ventricular septal defect. Cardiac transplantation resulted in full repair with confluent PAs in 1 patient with heterotaxy. Complete repair of the ductal PA was achieved in 22 patients, of whom 17 survived. Complete repair was attempted but not achieved in 10, and was not attempted in 13 patients. Of these 23 patients, 16 survived. Complete repair of the ductal PA was more likely (p = 0.005) in patients with complex lesions (66%) than in patients without such lesions (19%). Late presenting patients with diminutive ductal PA occurring in isolation were more likely to be routed to nonintervention. Causes of death are listed in Table 2. Important cardiac morbidity occurred in 6 patients (13%) and included infective endocarditis (n = 2), inferior vena cava thrombus (n = 1), femoral vein stenosis (n = 1), chronic atrial flutter with venous thrombosis (n = 1), and reentrant tachycardia (n = 1).

View larger version (24K): [in this window] [in a new window]   Fig 2. Flow chart showing outcomes in 45 patients with a ductal pulmonary artery after presentation to the Hospital for Sick Children.

View larger version (14K): [in this window] [in a new window]   Fig 3. Kaplan-Meier survival curve after presentation for the entire group with ductal pulmonary artery. Dotted lines represent the 95th percentile.

View larger version (27K): [in this window] [in a new window]   Fig 4. Initial and latest angiographic dimension of (top) the ductal pulmonary artery (PA) and (bottom) the nonductal pulmonary artery. (DDO = ductal origin of the distal pulmonary artery; non-DDO = non-ductal origin of the distal pulmonary artery.)

	Comment

Top Abstract Introduction Patients and Methods Results Comment Footnotes References

 
Ductal origin of the PA occurs in 1.5% of patients with discontinuous PAs [22]. The terms previously used to describe this condition, "unilateral absent pulmonary artery" and "isolated unilateral pulmonary artery agenesis," are misleading because an intrapulmonary vessel with relatively normal distribution is present in these patients [14]. More recently, the term "isolated unilateral absence of the proximal pulmonary artery" has been applied [12]. The term "ductal origin of the distal PA" is preferable because it gives insight into the embryology of this rare lesion and most correctly captures those anatomic features with important pathophysiologic impact [6, 14, 17, 19–22].

The principal embryologic explanation for ductal origin of the PA is involution of the proximal sixth aortic arch, which results in absence of the proximal pulmonary artery and the persistent connection of the hilar pulmonary artery to the distal sixth aortic arch (destined to become the ductus arteriosus) [12, 14]. All patients in our study had a ductus arteriousus or ligamentum ipsilateral to the absent proximal PA, and complete literature review revealed only 1 case [20] in which an ipsilateral ductus arteriosus was not identified [1, 8, 12, 14, 19, 22–26].

Presentation and Diagnosis
Cyanosis is a common presenting feature in those with associated cardiac lesions. When ductal origin of the distal PA is in isolation, persistent pulmonary hypertension is common, occurring in 19% to 88% of infants [7, 10, 25]. Frequent respiratory infections, an abnormal chest radiogram, or hemoptysis are later presenting features [2, 10, 11, 27, 28]. Accurate diagnosis requires several modalities [12, 14]. Echocardiography is a useful screening modality, but is insufficient to make a definitive diagnosis [12, 14, 18, 20]. One study found that echocardiography depicted the absent PA in older patients, but findings were inconclusive in infants [14]. Demonstration of the hilar blind end of the PA with branches to all the bronchopulmonary segments of the lung by pulmonary venous wedge angiography is diagnostic [2, 29]. Angiography is particularly helpful in planning surgical intervention as it can provide hemodynamic data and depict hypoplastic intrapulmonary vessels that may require rehabilitation with a systemic-ductal PA shunt before single-stage repair [14]. Noninvasive techniques such as contrast-enhanced arterial and venous phase computed tomography and magnetic resonance imaging are potential alternatives to angiography [12, 14, 30]. These modalities have consistently produced quality anatomic definition of the pulmonary arteries in previous studies [12–14, 20] and can demonstrate the presence of additional pathology, such as aortopulmonary collaterals. Ventilation-perfusion scans are useful to follow PA perfusion equivalence after surgical repair [12, 14].

Treatment Strategies
Treatment varied extensively in our study population, reflecting the historical nature of this series. Initial palliation with a systemic-ductal PA shunt for those without pulmonary overcirculation was favored early in our experience. This strategy continues to be used in our center as a first-stage rehabilitative procedure for those with hypoplastic ductal PA followed by later complete reconstruction, similar to the management paradigm for patients with small PAs and a diagnosis of pulmonary atresia and ventricular septal defect.

Although ductal PA banding was undertaken in 2 patients, we would not recommend this in the present era, as patients with overcirculation generally have adequate size pulmonary arteries, and the contemporary results with primary complete repair (ie, primary unifocalization of the ductal PA) at our institution have been favorable in this population. Furthermore, patients presenting with overcirculation in this series generally had the worst outcomes and may derive magnified benefit from early definitive correction.

Direct end-to-end anastomosis without patch augmentation cannot be recommended, as all patients in our series managed with this approach required either surgical revision or catheter-based reintervention for stenosis. Newer strategies using autologous tissue reconstruction with anterior patch augmention may potentially obviate these problems and provide attractive alternatives to other repair methods [12, 13]. Welch and colleagues [12] described a technique incorporating a filleted ligamentum to bridge the gap between the ductal PA and the main PA with anterior pulmonary homograft patch augmentation. The 2 patients managed with this technique at 30 months of follow-up had equivalent blood-flow distribution to both lungs on ventilation-perfusion scan, no evidence of pulmonary stenosis, and normal PA pressures. Kosaka and coworkers [13] similarly described a completely autologous tissue reconstruction in which a longitudinally incised ductal PA is anastomosed to a reverse U-shaped pedicled flap developed from the left PA. The anterior surface is then covered with an autologous pericardial patch. At 6 years postoperatively, angiography demonstrated normal PA pressures without evidence of PA stenoses.

Interposition grafts were used in 10 patients in our series, either as a single-stage reconstruction or as the second stage in patients requiring initial rehabilitation with a systemic-ductal PA shunt. The small number of patients managed with a single strategy precluded definitive identification of either the optimal method of reconstruction of the ductal PA, or the optimum graft material. However, the important disadvantages of using an interposition graft, including somatic outgrowth and need for anticoagulation, make this a theoretically less attractive option for many children [12, 13], especially given the recent introduction of autologous tissue reconstruction.

Transcatheter therapy has an important role in management of patients both preoperatively and during follow-up. In the short term, stent implantation in the AD can maintain blood flow to the ductal PA. Balloon atrial septostomy supports cardiac output in those with severe pulmonary hypertension. Stenosis after surgical PA reconstitution can be managed by balloon angioplasty and stent implantation. Patients with recurrent hemoptysis benefit from coil embolization of collateral arteries [11].

Early diagnosis and intervention have been advocated in other case reports [12, 13, 18–20, 25, 28, 31, 32]. The benefit of early intervention may be magnified in patients with pulmonary overcirculation. In our study, the presence of congestive heart failure and larger ductal PA dimensions were associated with increased mortality. Delayed therapy may result in diminutive PA size or pulmonary hypertension, precluding later reconstruction [12–14, 31, 32]. Furthermore, the likelihood of life-threatening hemorrhage from acquired indirect aortopulmonary collaterals may complicate later surgical repair [25, 28]. Such patients may require pneumonectomy for intractable infection or unremitting hemoptysis [25, 28].

Detection of a benefit to complete repair of the ductal PA is complicated by several factors: (1) those not undergoing repair had favorable morphology and no associated cardiac malformations; (2) the morbidity in unrepaired patients occurs mainly late, and thus is likely underreported with only midterm follow-up data available in this series; and (3) incorporation of older repair methods may "dilute" potential benefits gained by more recent surgical and catheter-based techniques.

Our data on PA dimensions suggest suboptimal growth of the ductal PA irrespective of the type of repair chosen. The evidence of considerable residual disease burden in our patients at last follow-up supports this contention. However, patients who underwent complete repair had less lung hypoplasia and scoliosis than did patients who remained unrepaired.

Inferences and Recommendations
Our experience suggests that the critical factor that determines treatment strategy is ductal PA size at diagnosis. For patients without pulmonary overcirculation, early staged repair with systemic-ductal PA shunt to rehabilitate the hypoplastic ductal PA followed by later autologous tissue reconstruction with patch augmentation is advisable. Similarly, early single-stage repair with autologous tissue reconstruction and patch augmentation for patients with adequately sized PAs or with pulmonary overcirculation is preferred. Close surveillance by noninvasive imaging with judicious reintervention is indicated to reduce residual disease. Patient education and surveillance is indicated for the late presenting patient who is deemed unsuitable for intervention.

To conclude, we have described the largest series of patients with ductal origin of the distal PA and have shown that there is important multisystem morbidity and mortality associated with this condition. Early diagnosis and repair of the ductal PA, especially in patients presenting with congestive heart failure or a large ductal PA, may improve outcomes.

	Footnotes

Top Abstract Introduction Patients and Methods Results Comment Footnotes References

 
^_* Dr Freedom died on May 7, 2005.

	References

Top Abstract Introduction Patients and Methods Results Comment Footnotes References

 

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This Article Abstract Full Text (PDF) 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): William G. Williams Brian W. McCrindle Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Trivedi, K. R. Articles by McCrindle, B. W. Search for Related Content PubMed PubMed Citation Articles by Trivedi, K. R. Articles by McCrindle, B. W. Related Collections Congenital - acyanotic