Close
New

Medscape is available in 5 Language Editions – Choose your Edition here.

 

Aortopulmonary Window Surgery Treatment & Management

  • Author: Mary C Mancini, MD, PhD, MMM; Chief Editor: Jonah Odim, MD, PhD, MBA  more...
 
Updated: Jan 05, 2016
 

Medical Therapy

Medical therapy is focused on preoperative stabilization. Surgical correction is the only effective treatment for aortopulmonary window (APW).

Intravenous prostaglandins (eg, alprostadil) may be required to maintain patency of the ductus arteriosus in patients with interrupted aortic arch in order to provide blood flow to the lower half of the body. The associated pulmonary arterial vasodilatation may further exacerbate the increased pulmonary blood flow.

Digoxin and furosemide are frequently administered to treat the heart failure and volume overload associated with this lesion.

Inotropic agents (eg, dopamine, dobutamine) may also be required for infants with significant heart failure and low cardiac output associated with myocardial dysfunction.

Next

Surgical Therapy

Surgery is the treatment for aortopulmonary window. After initial stabilization and correction of acidosis, surgery should be undertaken as soon as possible.

Surgery is performed with the use of cardiopulmonary bypass. An incision can be made into the anterior aspect of the aorta, the main pulmonary artery, or the aortopulmonary window itself.

Associated lesions are usually repaired during the same surgery. More complex repairs and myocardial protection strategies are required in patients with associated lesions, increasing the morbidity and mortality associated with the operation.

Previous
Next

Preoperative Details

Preoperative care is centered on correction of acidosis and stabilization of the child. Congestive heart failure symptoms are treated with digoxin, Lasix, and inotropes as necessary.

Elective intubation can also be performed and pulmonary blood flow regulated by altering the inspired fractions of oxygen and carbon dioxide.

Echocardiography is performed to define the anatomy and assess ventricular function. In complex lesions or in instances in which the coronary arteries cannot be clearly seen, cardiac catheterization may be required.

Patients presenting when older than 6 months need cardiac catheterization to rule out irreversible pulmonary hypertension.

Previous
Next

Intraoperative Details

Exposure is obtained through a median sternotomy. The aortopulmonary window should be directly visible. The aorta is cannulated as distally as possible. A single right atrial cannula or, if an atrial septal defect (ASD) or ventricular septal defect (VSD) is present, separate caval cannulae must be used.

Cardiopulmonary bypass is instituted, and the procedure is performed at moderate hypothermia. One of the pulmonary arteries can be snared early in the operation if pulmonary overcirculation remains a problem or has been exacerbated by the induction of general anesthesia. Deep hypothermic circulatory arrest (DHCA) may be necessary if the lesion is complex or extends distally into the arch of the aorta. This also applies to patients who require repair of an interrupted aortic arch.

The right and left pulmonary arteries should be snared before the administration of cardioplegia. The snares should be tightened to ensure good coronary flow and prevent runoff of cardioplegia into the pulmonary circulation. Consideration can be given to retrograde cardioplegia but is not mandatory. If DHCA is used for complex repairs, retrograde cardioplegia should not be necessary.

The defect is entered from the anterior aspect of the aorta, the main pulmonary artery, or the aortopulmonary window itself. The origins of the coronary arteries and branch pulmonary arteries are identified. A running nonabsorbable suture is then used to affix a patch of glutaraldehyde-treated pericardium or synthetic material to the posterior aspect of the defect. The remainder of the patch is then sewn to the superior and inferior aspects of the defects, with attention to the coronary arteries and branch pulmonary artery orifices. The anterior aspect of the patch is incorporated into the closure of the incision.

Associated anomalies require repair using the protocols for those lesions. Specifically, the interrupted aortic arch is reconstructed before closure of the aortopulmonary window. Because of the presence of the aortopulmonary window, a single aortic cannula can be used. The patient is then cooled to 18°C (64.4°F). The head vessels and branch pulmonary arteries are snared, and cardioplegia is delivered into the coronary arteries. The descending aorta can then be anastomosed to a separate aortotomy above the aortopulmonary window or incorporated into an extension of the incision used to open the aortopulmonary window. The aortopulmonary window is then closed using patch material. The frequent abnormal right pulmonary artery must be baffled to be continuous with the main pulmonary artery.

The patient is then warmed and weaned from cardiopulmonary bypass. The integrity of the repair is examined by means of transesophageal echocardiography. Protamine is administered to reverse the heparin, and the patient is decannulated and the incision closed.

Previous
Next

Postoperative Details

Inotropic support with milrinone, epinephrine, dopamine, or other agents can be anticipated in the initial postoperative period. A patient can usually be weaned off these over the next several hours and days, depending on his or her preoperative condition, length of time on cardiopulmonary bypass, and duration of hypothermic circulatory arrest.

Older patients may require treatment of postoperative pulmonary hypertension and pulmonary hypertensive crises. High levels of inspired oxygen remain one of the most effective pulmonary vasodilators. Deep sedation and paralysis are also effective in preventing hypertensive crises. If paralysis is not used, additional sedation should be used for endotracheal suctioning and other procedures. Inhaled nitric oxide may be effective for the treatment of pulmonary hypertension in intubated patients.

Patients may also require continued digitalis and Lasix, which may be discontinued in outpatient therapy.

Previous
Next

Follow-up

Patients require follow-up with their cardiac surgeon initially and a pediatric cardiologist indefinitely. The surgical repair can be monitored by means of serial echocardiography. Further operative intervention may be required for the development of pulmonary artery stenosis. Some element of heart failure may persist after surgery and require continued medical therapy.

Previous
Next

Complications

Pulmonary hypertensive crises may occur in the postoperative period. Patients at high risk should be sedated overnight, and paralysis should be considered. Acidosis should be avoided, and the pCO2 should be maintained at 30-35 mm Hg. Hypoxia should be avoided. Deep sedation should be confirmed before endotracheal suctioning. Finally, inhaled nitric oxide should be instituted for pulmonary artery pressures not managed by the above measures. Milrinone may also be used to lower pulmonary artery pressures and provide inotropic support. These measures can often be discontinued the next day.

Long-term follow-up is done with echocardiography. Recurrent coarctation and development of branch pulmonary artery stenosis are long-term risks.

Previous
Next

Outcome and Prognosis

Outcomes continue to improve with better management during the perioperative period. An example of this can be seen in Backer and Mavroudis' description of their 40-year experience at Northwestern University.[3] Early in their experience, repair primarily consisted of aortopulmonary window (APW) division and resulted in a 37% mortality rate (6 of 16 patients). However, no deaths occurred in their most recent series of 6 patients in which cardiopulmonary bypass and transaortic patch closure were used. Most series consistently report a mortality rate less than 10%. The mortality rate for simple aortopulmonary window without other associated anomalies should be near 0%.[6]

The prognosis of aortopulmonary window is excellent if repaired in infancy and preferably before the onset of significant pulmonary hypertension. In Backer and Mavroudis' series noted above, the average pulmonary vascular resistance was elevated at 5.4 U/m2, but only one patient died from complications of pulmonary hypertension.

Previous
Next

Future and Controversies

Little change has occurred in the diagnosis and management of aortopulmonary window (APW). Its frequent complexity and proximity to the aortic and pulmonary valves make catheter-based interventions unlikely in the future, although a catheter-based device has been used to close a residual defect following surgical repair. In addition, angioplasty with or without stenting may be effective in postoperative pulmonary artery stenoses.

Imaging modalities may advance and come to include MRI to better define the more complex lesions and avoid cardiac catheterization when the anatomy is unclear.

Previous
 
Contributor Information and Disclosures
Author

Mary C Mancini, MD, PhD, MMM Professor and Chief of Cardiothoracic Surgery, Department of Surgery, Louisiana State University School of Medicine in Shreveport

Mary C Mancini, MD, PhD, MMM is a member of the following medical societies: American Association for Thoracic Surgery, American College of Surgeons, American Surgical Association, Society of Thoracic Surgeons, Phi Beta Kappa

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Robert DB Jaquiss, MD Professor of Surgery, University of Arkansas for Medical Sciences; Chief, Pediatric Cardiothoracic Surgery, Arkansas Children's Hospital and Chief, Cardiothoracic Surgery, University of Arkansas for Medical Sciences

Robert DB Jaquiss, MD is a member of the following medical societies: American Academy of Pediatrics, American Association for Thoracic Surgery, American College of Cardiology, American College of Surgeons, American Heart Association, Congenital Heart Surgeons Society, International Society for Heart and Lung Transplantation, Society of Thoracic Surgeons

Disclosure: Nothing to disclose.

Chief Editor

Jonah Odim, MD, PhD, MBA Section Chief of Clinical Transplantation, Transplantation Branch, Division of Allergy, Immunology, and Transplantation, National Institute of Allergy and Infectious Diseases (NIAID), National Institutes of Health (NIH)

Jonah Odim, MD, PhD, MBA is a member of the following medical societies: American College of Cardiology, American College of Chest Physicians, American Association for Physician Leadership, American College of Surgeons, American Heart Association, American Society for Artificial Internal Organs, American Society of Transplant Surgeons, Association for Academic Surgery, Association for Surgical Education, International Society for Heart and Lung Transplantation, National Medical Association, New York Academy of Sciences, Royal College of Physicians and Surgeons of Canada, Society of Critical Care Medicine, Society of Thoracic Surgeons, Canadian Cardiovascular Society

Disclosure: Nothing to disclose.

Additional Contributors

Daniel S Schwartz, MD, FACS Medical Director of Thoracic Oncology, St Catherine of Siena Medical Center, Catholic Health Services

Daniel S Schwartz, MD, FACS is a member of the following medical societies: Society of Thoracic Surgeons, Western Thoracic Surgical Association, American College of Chest Physicians, American College of Surgeons

Disclosure: Nothing to disclose.

Acknowledgements

Hani A Hennein, MD, FACS, FAAP, FCCP Associate Professor of Surgery and Pediatrics, Case Western Reserve University School of Medicine; Chief, Section of Pediatric Cardiothoracic Surgery, Department of Surgery, University Hospitals of Cleveland, Rainbow Babies and Childrens Hospital

Disclosure: Nothing to disclose.

Jeff L Myers, MD, PhD Chief, Pediatric and Congenital Cardiac Surgery, Department of Surgery, Massachusetts General Hospital; Associate Professor of Surgery, Harvard Medical School

Jeff L Myers, MD, PhD is a member of the following medical societies: American College of Surgeons, American Heart Association, and International Society for Heart and Lung Transplantation

Disclosure: Nothing to disclose.

References
  1. Barnes ME, Mitchell ME, Tweddell JS. Aortopulmonary window. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2011. 14(1):67-74. [Medline].

  2. Gross RE. Surgical closure of an aortic septal defect. Circulation. 1952 Jun. 5(6):858-63. [Medline].

  3. Backer CL, Mavroudis C. Surgical management of aortopulmonary window: a 40-year experience. Eur J Cardiothorac Surg. 2002 May. 21(5):773-9. [Medline].

  4. Roubertie F, Kalfa D, Vergnat M, Ly M, Lambert V, Belli E. Aortopulmonary window and the interrupted aortic arch: midterm results with use of the single-patch technique. Ann Thorac Surg. 2015 Jan. 99 (1):186-91. [Medline].

  5. Yildirim A, Karabulut N, Dogan S, Herek D. Congenital thoracic arterial anomalies in adults: a CT overview. Diagn Interv Radiol. 2011 Oct 6. [Medline].

  6. Naimo PS, Yong MS, d'Udekem Y, et al. Outcomes of aortopulmonary window repair in children: 33 years of experience. Ann Thorac Surg. 2014 Nov. 98 (5):1674-9. [Medline].

  7. Berry TE, Bharati S, Muster AJ, et al. Distal aortopulmonary septal defect, aortic origin of the right pulmonary artery, intact ventricular septum, patent ductus arteriosus and hypoplasia of the aortic isthmus: a newly recognized syndrome. Am J Cardiol. 1982 Jan. 49(1):108-16. [Medline].

  8. Brown JW, Ruzmetov M, Okada Y, et al. Outcomes in patients with interrupted aortic arch and associated anomalies: a 20-year experience. Eur J Cardiothorac Surg. 2006 May. 29(5):666-73; discussion 673-4. [Medline].

  9. Chen MR, Wu SJ. Images in cardiovascular medicine. Unclassified type of aortopulmonary window. Circulation. 2006 Apr 18. 113(15):e703-4. [Medline].

  10. Daitoku K, Yamauchi S, Suzuki Y, Fukuda I. Right Pulmonary Artery Obstruction Is a Long-term Complication of Aortopulmonary Window Repair. Congenit Heart Dis. 2013 May 7. [Medline].

  11. Das BB, Pauliks LB, Chan KC. Anatomically corrected malposition of the great arteries in the setting of aortopulmonary window associated with holoprosencephaly. Pediatr Cardiol. 2006 Jan-Feb. 27(1):175-6. [Medline].

  12. Erez E, Dagan O, Georghiou GP, et al. Surgical management of aortopulmonary window and associated lesions. Ann Thorac Surg. 2004 Feb. 77(2):484-7. [Medline].

  13. Greenway SC, Bradley TJ, Caldarone CA, et al. Aortopulmonary window with anomalous origin of the right coronary artery from the pulmonary artery: Two cases highlighting the importance of complete pre-operative echocardiographic evaluation of the coronary arteries in all conotruncal anomalies. Eur J Echocardiogr. 2005 Dec 12. [Medline].

  14. Hew CC, Bacha EA, Zurakowski D, et al. Optimal surgical approach for repair of aortopulmonary window. Cardiol Young. 2001 Jul. 11(4):385-90. [Medline].

  15. Kirby ML. Pulmonary atresia or persistent truncus arteriosus: is it important to make the distinction and how do we do it?. Circ Res. 2008 Aug 15. 103(4):337-9. [Medline].

  16. Konstantinov IE, Karamlou T, Williams WG, et al. Surgical management of aortopulmonary window associated with interrupted aortic arch: a Congenital Heart Surgeons Society study. J Thorac Cardiovasc Surg. 2006 May. 131(5):1136-1141.e2. [Medline].

  17. Peer SM, Donofrio MT, Gaur L, Sinha P. Tricuspid atresia with aortopulmonary window: challenges in achieving a balanced circulation. Interact Cardiovasc Thorac Surg. 2013 Aug. 17(2):441-3. [Medline]. [Full Text].

  18. Prem Sekar R, Bhima Shankar PR, Cherian KM. Transcatheter closure of a residual aortopulmonary window through internal jugular vein access. Indian Heart J. 2012 Sep-Oct. 64(5):521-3. [Medline].

  19. Talwar S, Garg P, Kothari SS, Gulati GS, Anderson RH, Airan B. Aortopulmonary window with the absence of left pulmonary artery. World J Pediatr Congenit Heart Surg. 2012 Jul 1. 3(3):389-91. [Medline].

  20. Tkebuchava T, von Segesser LK, Vogt PR, et al. Congenital aortopulumonary window: diagnosis, surgical technique and long-term results. Eur J Cardiothorac Surg. 1997 Feb. 11(2):293-7. [Medline].

  21. Trowitzsch E, Schneider M, Urban A, Asfour B. Congenital pulmonary sling, aorto-pulmonary window and pulmonary vein obstruction as a diagnostic and therapeutic challenge in an infant with VACTERL association. Clin Res Cardiol. 2006 Jun. 95(6):338-43. [Medline].

  22. Valsangiacomo ER, Smallhorn JF. Images in cardiovascular medicine. Prenatal diagnosis of aortopulmonary window by fetal echocardiography. Circulation. 2002 Jun 18. 105(24):E192. [Medline]. [Full Text].

  23. Van Praagh R, Van Praagh S. The anatomy of common aorticopulmonary trunk (truncus arteriosus communis) and its embryologic implications. A study of 57 necropsy cases. Am J Cardiol. 1965 Sep. 16(3):406-25. [Medline].

  24. Wright JS, Freeman R, Johnston JB. Aorto-pulmonary fenestration. A technique of surgical management. J Thorac Cardiovasc Surg. 1968 Feb. 55(2):280-3. [Medline].

 
Previous
Next
 
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.