Ventricular Septal Defect (VSD)
Aortopulmonary Window
What is aortopulmonary Window?
Aortopulmonary window (APW) is a rare congenital heart defect that results from an abnormal separation of the two main arteries leading from the heart, the aorta and the pulmonary artery. An aortopulmonary window allows the ‘red’ oxygenated blood to pass from the aorta back into the pulmonary artery and mix with the unoxygenated or ‘blue’ blood.
There are three types of APW defects:
Type I: a proximal defect in the wall between the aorta and the pulmonary artery usually located right above the sinus of valsalva.
Type II: a distal defect in the wall between the aorta and the pulmonary artery.
Type III: a total defect that usually extends the length of the wall between the aorta and the pulmonary artery
When an APW occurs, the aortic and the pulmonary valves are usually normally formed. Commonly the defect is a single hole located just above the aortic and pulmonary valves, and is limited in size.
What are some of the other heart defects associated with aortopulmonary window?
APW can be associated with a patent ductus arteriosus, interrupted aortic arch, aortic valve atresia, and in some cases it has been associated with tetralogy of Fallot.
What happens when an aortopulmonary window occurs?
When an APW occurs, a certain amount of (red) or oxygenated blood is shunted or passed to the pulmonary artery and mixes with the (blue) or unoxygenated blood. The amount of blood that is shunted or pushed from the aorta to the pulmonary artery is directly related to the size of the defect and the amount of resistance that is present in the patient’s lungs.
How is aortopulmonary window diagnosed?
If the shunt is small, few or no symptoms may occur. Usually, however, most APW defects are large and have some degree of shunting from the aorta to the pulmonary artery.
Most APW’s are found as a result of a routine check-up. An abnormal heart sound or murmur may be heard, and a more in depth cardiac study may be ordered. The child may have a history of poor feeding, delayed growth, and a history of multiple respiratory infections. Upon radiological exam the heart is usually enlarged and the pulses in the legs and the arms are usually bounding or very strong.
How is an aortopulmonary window treated?
Aortopulmonary windows are normally treated with a surgical open heart procedure. The timing of the repair is dependent upon the amount of blood that is being shunted to the lungs and how many symptoms the child might be having due to excess pulmonary blood flow. The surgery usually is performed within the newborn period.
The aorta is entered after the patient is placed on the heart lung machine. A patch made out of an aortic artery homograft is sewn into place along the communication between the aorta and pulmonary artery. Careful consideration is taken during the suturing of this patch because of the normal proximity of the defect to the entrance into the left coronary artery. The aorta is then closed and the child is taken off the heart lung machine.
Will my child’s heart be normal after this corrective surgery for APW?
The surgical procedure to correct APW is a fairly successful approach to a 100% cure for this defect. However, parents and patients must remember that APW itself can cause irreversible damage to the lungs if not diagnosed and treated in a timely manner. Once the diagnosis has been made surgery should not be delayed.
If treated successfully a child will have a normal circulation and should have no lasting effects from the aortopulmonary window itself.
What are some complications that may occur with the surgical repair of APW?
Complications that can be associated with cardio-pulmonary bypass and heart surgery in general include:
• Infection
• Bleeding
• Blood transfusions
• Stroke
• Death
Complications that are particular to APW include:
• Persistent pulmonary hypertension
• Injury to the coronary arteries or pulmonary arteries
• Injury to the aortic or pulmonary valve.
Contact Information:
John Mark Morales MD, FACS, FAAP
Chief of Cardiothoracic, Director of Perfusion Services
Certified by the American Board of Surgery, American Board of Thoracic SurgeryMark Bielefeld, MD
Driscoll Children's Hospital Chief of Staff
Certified by the American Board of Surgery, American Board of Thoracic SurgeryThoracic surgeons are available for questions and consultations: (361) 854-0201. For appointments, assistance, and physician references in Corpus Christi call:
(361) 854-0201 or (800) DCH-LOVE
Fax : 361-855-7572
E-MAILS : Mark Morales, MD
Mark Bielefeld, MD
For further information on any surgical procedures you can contact Carol Kaplan, RN, Surgical Nurse Liaison at (361) 694-5150. Consultation and surgery for inpatients is provided in concert with neonatology and pediatric cardiology departments. Complete evaluation and management for infants, children, adolescents and adults with congenital or acquired cardiac, vascular or thoracic anomalies.
Cardiothoracic Associates
3533 S. Alameda, Suite 202
Corpus Christi, Texas 78411
Office: (361) 854-0201 or (361) 694-5150
Fax: (361) 855-7572
Hours: 9am to 6pm
Fri 9am to 5pm
Voted Best Place to Work in 2006 and 2007 | Ways to Give | Contact Us
Copyright © Driscoll Children's Hospital 2008
Corpus Christi, Texas
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