Aortopulmonary window

Aortopulmonary window

Overview

Aortopulmonary (AP) window refers to a rare defect, a hole between the wall of the aorta (the major artery that pumps blood from the heart to the rest of the body) and the pulmonary artery that pumps blood to the lungs. Instead of normal circulation taking place, the hole allows blood from the aorta to leak into the pulmonary artery, causing too much blood to flow into the lungs. This causes high blood pressure in the lungs (pulmonary hypertension) and eventually heart failure. The bigger the hole, the more blood flows into the pulmonary artery. Coronary artery anomalies are often seen with aortopulmonary windows.

The defect develops in the womb when the fetus’ aorta and pulmonary artery do not divide normally.

Symptoms

Symptoms in infants can include:

  • Heart failure with tachypnea (abnormally rapid breathing)
  • Sweating during feeding  
  • Poor weight gain and delayed growth
  • Irritability
  • Rapid breathing or heartbeat
  • Respiratory infections

Left undiagnosed and uncorrected, these lesions may cause pulmonary arterial hypertension (high blood pressure in the lungs) with resulting cyanosis (bluish discoloration of the skin caused by lack of oxygen in the bloodstream) in older age groups.

Diagnosis

A pediatrician can diagnosis an abnormal heart sound (murmur) with a stethoscope, and may order such tests as:

  • Cardiac catheterization — a thin tube is inserted into the coronary arteries to view the heart and blood vessels and directly measure pressure in the heart and lungs
  • Chest X-ray
  • Echocardiogram
  • MRI of the heart

Treatment

Congenital heart disease surgery is required to correct an aortopulmonary window. Treatment for AP window includes closing the hole between the aorta and the pulmonary artery with a patch. Usually, this surgery is done as soon as possible after the diagnosis is made, when the child is a newborn. Surgery to fix an aortopulmonary window is successful in most cases, if the defect is treated quickly. Delayed treatment may result in pulmonary hypertension, congestive heart failure or even death.

In adult patients with AP window, a cardiac catheterization may be warranted to document the size of the defect and the relative pressure in the pulmonary artery. When possible, an operation to close the defect is performed. The patient may require ongoing treatment for pulmonary hypertension.

 

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