Prevalence and Anatomy of Retroesophageal Major Aortopulmonary Collateral Arteries

doi:10.1016/j.athoracsur.2016.03.011

The Annals of Thoracic Surgery

Volume 102, Issue 3, September 2016, Pages 877-882

https://doi.org/10.1016/j.athoracsur.2016.03.011 Get rights and content

Background

Major aortopulmonary collateral arteries (MAPCAs) are the sole source of pulmonary blood flow in patients with pulmonary atresia and absent ductus arteriosus. The anatomy of MAPCAs can be highly variable, both in the number of MAPCAs supplying each lung and the anatomic origin and course of the MAPCAs. This study evaluated the prevalence and anatomy of retroesophageal MAPCAs in patients undergoing repair of pulmonary atresia/ventricular septal defect/MAPCAs.

Methods

This was a concurrent analysis of 68 consecutive patients (March 2013 through October 2015) undergoing a primary surgical procedure for pulmonary atresia/ventricular septal defect/MAPCAs. A detailed analysis of the MAPCA anatomy was made intraoperatively for each patient, including the total number of MAPCAs to each lung and the presence or absence of a retroesophageal course. These data were correlated with the preoperative cardiac catheterization images.

Results

A retroesophageal MAPCA was identified during the operation in 45 of the 68 patients (67%), all of which were located on the side opposite the arch. For the 36 patients with a left aortic arch, 77% had a retroesophageal MAPCA compared with 53% of patients with a right arch. Forty-six percent of retroesophageal MAPCAs coursed within the muscular fibers of the esophagus (intraesophageal) and were more common to the left lung than the right (72% vs 32%). A midsegment stenosis was present in 84% of the retroesophageal MAPCAs, and this was more severe when the MAPCAs were intraesophageal than when they were not (80% vs 42%).

Conclusions

These data demonstrate that two-thirds of patients had a retroesophageal MAPCA and that there were significant differences in prevalence and anatomy depending on the side of the aortic arch. These data provide important insights into the origin and course of retroesophageal MAPCAs.

Section snippets

Material and Methods

The Stanford University Institutional Review Board approved this study. Patients eligible for inclusion in the study were those undergoing a primary surgical procedure for PA/VSD/MAPCAs. The study summarizes our experience with 68 consecutive patients undergoing PA/VSD/MAPCA operations from March 2013 through October 2015.

We perform the entire MAPCA dissection before cardiopulmonary bypass is initiated. This dissection is performed between the ascending aorta and superior vena cava and inferior

Results

A retroesophageal MAPCA was diagnosed in 45 of the 68 patients (67%) intraoperatively. However, the prevalence of retroesophageal MAPCAs differed significantly depending on whether there was a left or right aortic arch (Fig 4). The prevalence of retroesophageal MAPCAs was 77% for the 36 patients who had a left aortic arch vs 53% in the 32 patients who had a right aortic arch (p < 0.05).

Of the 45 patients with retroesophageal MAPCAs, 41 (91%) had 1 retroesophageal MAPCA, and 4 had 2

Comment

This study was performed to evaluate the prevalence and anatomy of retroesophageal MAPCAs in patients with PA/VSD/MAPCAs. These data demonstrate that a retroesophageal MAPCA was identified during the operation in 45 of 68 patients (67%). Of the 45 patients with a retroesophageal MAPCA, 41 had 1 retroesophageal MAPCA, and 3 had 2 retroesophageal MAPCAs. Thus, 49 retroesophageal MAPCAs were identified during the operation, of which 23 were intraesophageal. A significant difference was noted in

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Cited by (23)

Midterm fate of unifocalized major aortopulmonary collateral arteries in patients with retroesophageal major aortopulmonary collateral arteries
2022, Journal of Thoracic and Cardiovascular Surgery
Citation Excerpt :
The 37 patients included in this study were a highly select cohort based on the requirement to have at least 1 retroesophageal MAPCA. This subset of patients has been intensely scrutinized with detailed mapping of their MAPCA and pulmonary artery anatomy.12,13 The majority of these patients have now been followed for an extensive period of time (median, 69 months), with comprehensive follow-up obtained, including the cardiac catheterization and surgical history.
Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (MAPCAs) is a relatively rare and complex form of congenital heart disease. Unifocalization of MAPCAs has been advocated by some groups for the treatment of this condition. The purpose of this study was to assess the midterm fate of unifocalized MAPCAs in a cohort of patients with retroesophageal MAPCAs.
This was a retrospective review of 37 patients who underwent a unifocalization procedure. All patients in this study had 1 or more retroesophageal MAPCAs, and detailed mapping of the MAPCAs was made based on a combination of the cardiac catheterization and surgical findings.
The 37 patients had a total of 166 MAPCAs, or 4.5 MAPCAs per patient. One hundred twenty-nine (78%) MAPCAs were unifocalized, whereas 37 (22%) were ligated because they were dual supply. Median follow-up was 69 months. At follow-up cardiac catheterization, evaluation of the 129 unifocalized MAPCAs demonstrated that 123 (95%) had antegrade flow, whereas 6 were occluded. For the 123 MAPCAs with antegrade flow, 97 (80%) were widely patent, whereas 26 were stenotic. Thirteen of the 37 patients have subsequently undergone reintervention on MAPCAs that were determined to be stenotic following unifocalization. Seven of these patients had mild disease and had complete resolution with balloon (n = 5) or surgical revision (n = 2). Six patients with moderate or severe disease underwent surgical revision with confirmed resolution in 4 of 6.
The data demonstrate that the majority of unifocalized MAPCAs remain widely patent following unifocalization. However, one-quarter of unifocalized MAPCAs develop stenoses or occlusion. These results suggest the fate for most unifocalized MAPCAs is favorable but highlight the need for close vigilance.
To Unifocalize or Not to Unifocalize?: A Comparison of Retroesophageal Versus Anterior Collaterals
2022, Annals of Thoracic Surgery
Citation Excerpt :
While most MAPCAs are located anteriorly, there is a smaller subset of MAPCAs that course behind the esophagus (REMs). We previously evaluated the prevalence and anatomy of REMs in a paper published several years ago.12 The current study sought to evaluate the physiologic and surgical implications of REMs vs anteriorly located MAPCAs.
The anatomy of major aortopulmonary collateral arteries (MAPCAs) can be highly variable with regard to number, anatomic origin, course, and relationship to the native pulmonary arteries. Some MAPCAs travel behind the esophagus (retroesophageal) and bronchus before entering the lung parenchyma. This study compared the physiologic and surgical characteristics of retroesophageal vs anterior located MAPCAs.
This was a retrospective review of 42 patients who had 1 (n = 36) or 2 (n = 6) retroesophageal MAPCAs. These MAPCAs were then characterized as (1) single supply, meaning no connection to the pulmonary arteries; (2) dual supply, but inadequate connection to the distal pulmonary vascular bed; and (3) dual supply with adequate connection.
The 42 patients presented with 187 MAPCAs, or 4.5 MAPCAs per patient. Of these, 48 MAPCAs were retroesophageal, including 40 that were single supply, 6 were dual supply with inadequate connection, and 2 had dual supply with adequate connection. On the basis of this anatomy and physiology, 96% of retroesophageal MAPCAs were unifocalized. For the 139 anterior MAPCAs, 89 were single supply, 15 were dual supply with inadequate connection, and 35 were dual supply with adequate connection; thus, 75% of anterior MAPCAs were unifocalized (P < .01 compared with retroesophageal MAPCAs).
The data demonstrate that retroesophageal MAPCAs had very different anatomy and physiology compared with anterior MAPCAs. These results suggest that nearly every retroesophageal MAPCA should be unifocalized to incorporate the lung segments supplied.
Measurement of Residual Collateral Flow in Pulmonary Atresia With Major Aortopulmonary Collaterals
2019, Annals of Thoracic Surgery
Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (MAPCAs) is a rare form of congenital heart disease characterized by the entirety of pulmonary blood flow originating from systemic vessels. This study measured the residual collateral flow after harvesting of the MAPCAs for surgical repair.
The study enrolled 32 patients with pulmonary atresia with ventricular septal defect and MAPCAs who were undergoing their first surgical procedure. The median age was 6.8 months, and median weight was 5.7 kg. The patients had a mean of 4.2 ± 0.7 MAPCAs. The cardiopulmonary bypass circuit was modified to contain a diversion loop in the left ventricular vent system to accurately measure residual collateral flow. During the period of aortic cross-clamp (for ventricular septal defect repair), the diversion loop was opened for 1-minute intervals, and the residual collateral flow collected. The systemic perfusion temperature was 25° and flow rate was 100 mL · kg⁻¹ · min⁻¹.
The mean residual collateral flow was 5.5 mL · kg⁻¹ · min⁻¹ (range, 0.8 to 15.2 mL · kg⁻¹ · min⁻¹). The corresponding calculated pulmonary blood flow–to–systemic blood flow ratio values ranged from 1.01 to 1.36. There was a significant correlation between residual collateral flow and preoperative saturation (p < 0.05).
The data demonstrate a wide range of residual collateral flow values after harvesting of the MAPCAs. The amount of residual collateral flow was correlated with preoperative saturation. These results suggest that some patients at the higher end of this spectrum may require adjustments in pump flow to assure adequate systemic perfusion.
Surgical algorithm and results for repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals
2018, Journal of Thoracic and Cardiovascular Surgery
Citation Excerpt :
The most common origin of MAPCAs is off the anterior surface of the descending aorta. However, two thirds of patients will have a retro-esophageal MAPCA, which originates from the lateral side of the aorta.30 Retro-esophageal MAPCAs are frequently posterior to the airway and have midsegment stenoses, and therefore require a different surgical approach compared with MAPCAs originating from the anterior aorta.
Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries is a complex and heterogeneous form of congenital heart disease. There is a controversy regarding the optimal treatment of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. The purpose of this study was to summarize our algorithm and surgical results for pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries.
This was a retrospective review of 307 patients undergoing primary surgical treatment of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. Excluded from this analysis were patients who had undergone prior surgical treatment at another institution and patients with single ventricle and major aortopulmonary collateral arteries. There were 3 surgical pathways, including midline unifocalization (n = 241), creation of an aortopulmonary window (n = 46), and other (n = 20).
For the 241 patients who underwent midline unifocalization, 204 (85.4%) had a single-stage complete repair. There were 37 patients who underwent a midline unifocalization and central shunt, and 24 have subsequently undergone complete repair. Forty-six patients underwent an aortopulmonary window, of whom 36 have subsequently had a complete repair. There were 20 patients who had complex anatomy and underwent procedures other than described, and14 have subsequently undergone complete repair. Thus, for the patients currently eligible, 280 (93.0%) have achieved complete repair. For the 204 patients who had a single-stage complete repair, the mean right ventricle to aortic pressure ratio was 0.36 ± 0.09. Seventy-six patients underwent a staged repair, and the mean right ventricle to aortic pressure ratio was 0.40 ± 0.09 (P < .05 compared with single-stage repair). There were 3 (1.5%) early and 8 (4.0%) late deaths for the single-stage complete repair cohort versus 4 (4.0%) early and 15 (14.9%) late deaths for all other procedures (P < .01).
The data demonstrate that more than 90% of patients with pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries achieved complete repair. The overall mortality was significantly lower in the subgroup of patients who underwent single-stage complete repair.
Repair of Ductus or Hemi-Truncus to One Lung and Major Aortopulmonary Collaterals to the Other Lung
2018, Annals of Thoracic Surgery
There are patients born with pulmonary atresia and a ductus arteriosus or hemi-truncus to one lung and major aortopulmonary collateral arteries (MAPCAs) to the contralateral lung. The purpose of this study was to review our surgical results for this relatively rare subset of patients.
This was a retrospective review of 35 patients with ductus/hemi-truncus in association with pulmonary atresia with ventricular septal defect and MAPCAs. Our surgical algorithm is bifurcated into two strategies: (1) patients with “favorable” MAPCAs are candidates for single stage complete repair, and (2) patients with “unfavorable” MAPCAs undergo a staged approach. The median age at surgery was 3 months (range, 1 to 7 months), and the mean number of MAPCAs was 3.1 ± 1.3.
Twenty patients underwent a single stage complete repair. All 20 of these patients are alive with a right ventricle to aortic pressure ratio of 0.34 ± 0.07. The distribution of pulmonary blood flow by lung perfusion scan was 44% to the right and 56% to the left lung. Fifteen patients underwent a staged approach, including unifocalization of the MAPCAs to a central shunt. There were 2 interim deaths. Twelve patients have undergone complete repair with a right ventricle to aortic pressure ratio of 0.38 ± 0.07. There was 1 late death after complete repair. The distribution of pulmonary blood flow was 39% to the right and 61% to the left lung.
The data demonstrate that 32 of 35 patients with ductus/hemi-truncus and MAPCAs ultimately achieved complete repair with relatively low pulmonary artery pressures.
Comprehensive Management of Major Aortopulmonary Collaterals in the Repair of Tetralogy of Fallot
2018, Seminars in Thoracic and Cardiovascular Surgery: Pediatric Cardiac Surgery Annual
The heterogenous anatomy of Tetralogy of Fallot with major aortopulmonary collateral arteries has engendered a similar degree of diversity in its management and, ultimately, outcome. We summarize our comprehensive treatment paradigm for this lesion evolved over 15 years of experience through 458 patients and the results obtained. An updated analysis of 307 patients treated primarily at our institution is included. A review of recent literature, comparison of management strategies, and discussion of ongoing controversies are provided.

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Original articleCongenital heart surgeryPrevalence and Anatomy of Retroesophageal Major Aortopulmonary Collateral Arteries

Background

Methods

Results

Conclusions

Section snippets

Material and Methods

Results

Comment

J Thorac Cardiovasc Surg

J Thorac Cardiovasc Surg

J Thorac Cardiovasc Surg

J Am Coll Cardiol

Ann Thorac Surg

Ann Thorac Surg

Ann Thorac Surg

Chest

Ann Thorac Surg

Systemic arterial supply to lungs in pulmonary atresia and its relation to pulmonary artery development

Br Heart J

Growth and development of pulmonary circulation in pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries

Br Heart J

Unifocalization of major aortopulmonary collateral arteries in pulmonary atresia with ventricular septal defect is essential to achieve excellent outcomes irrespective of native pulmonary artery morphology

J Thorac Cardiovasc Surg

Early and intermediate outcomes after repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries: Experience with 85 patients

Circulation

Original article
Congenital heart surgery
Prevalence and Anatomy of Retroesophageal Major Aortopulmonary Collateral Arteries