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Aortopulmonary window

Provided by "Pediatric Heart Surgery — a reference for professionals"

 

Pathophysiology

Aortopulmonary Window illustration

Aortopulmonary window is a rare congenital heart defect in which there is communication between the ascending aorta and the main pulmonary artery above two distinct semilunar valves. This defect may vary in size, but it usually results in a large left-to-right shunt. The amount of shunting that occurs in patients with aortopulmonary window depends on the size of the aortopulmonary window and the relative vascular resistances in the systemic and pulmonary circulations. This left-to-right shunt results in increased pulmonary artery blood flow, left atrial and left ventricular volume overload, and right ventricular pressure overload. Clinical presentation is similar to that of a patient with a large ventricular septal defect or patent ductus arteriosus. Patients present with congestive heart failure and decreased cardiac output. If pulmonary vascular resistance is high, patients are at risk for early development of pulmonary vascular obstructive disease. Repair should be performed as early as possible once the diagnosis is made to avoid these complications.

Surgical technique

Repair of aortopulmonary window requires cardiopulmonary bypass and aortic cross-clamping. The aorta or main pulmonary artery is opened, the aortopulmonary window defect is visualized, and a patch of synthetic material or pericardium is sutured in place to close the defect. Cardiopulmonary bypass time and aortic cross-clamp time required to repair this defect are short to moderate.

Postoperative considerations

Preoperative pulmonary overcirculation and congestive heart failure make these patients susceptible to pulmonary hypertensive episodes. Invasive monitors used include arterial and central venous catheters. An LA line is used when needed for hemodynamic management. LA pressure acts as a surrogate for left ventricular end diastolic pressure, an excellent indicator of left ventricular performance. Non-invasive monitors include NIRS (near-infrared spectroscopy) probes to assess adequacy of regional and global perfusion and traditional pulse oximetry. Vasoactive agents might include epinephrine and/or milrinone. Numerous other agents are available and are tailored to the specific needs of the patient, targeting adequate oxygen delivery to the tissues of the body and optimization of cardiac output. Postoperative arterial oxygen saturation and intracardiac pressures should be normal. Excessive bleeding and atrioventricular conduction abnormalities are uncommon complications. Hospital stay following this repair averages 7 to 10 days.