vasculature Such dissection may result in lung parenchymal injury and contusion It may also disrupt the blood supply to the airways, resulting in necrosis and inflammation with an accumulation of airway secretions.64 Excessive airway secretions may cause airflow obstruction and impaired gas exchange Children are also at risk for reperfusion pulmonary edema, which is common after this procedure The risk of reperfusion edema is related to the degree of preoperative stenosis of the reconstructed vessels65 but is usually selflimited and does not affect the duration of postoperative mechanical ventilation.66 Diuretic therapy and ventilator strategies optimizing mean airway pressure to maintain functional reserve capacity are often helpful to hasten improvement of presumptive reperfusion edema Patients who undergo unilateral unifocalization revision surgery or who require more extensive reconstruction on one side are at risk for significant ventilation/perfusion deficits Elevated dead space ventilation is associated with longer ventilatory dysfunction.67 Attention to lung recruitment, airway clearance, and ventilation/perfusion mismatch are important in the postoperative management of unifocalization/pulmonary artery reconstruction patients Other potential respiratory complications are airway obstruction from surgical rearrangement of MAPCAs, phrenic nerve injury, pulmonary hemorrhage, upper airway obstruction, and bronchomalacia Children with a 22q11 deletion are at higher risk for preoperative airway abnormalities and malacia, which may worsen after surgery and can necessitate prolonged mechanical ventilation.68 Early detection and intervention for respiratory complications is important and may reduce the duration of stay in the intensive care unit and hospitalization Diagnostic modalities that are sometimes indicated include chest ultrasound, fluoroscopy to evaluate diaphragmatic movement, computed tomography, bronchoscopy, cardiac catheterization, and lung perfusion scintigraphy, depending on the particular scenario Postoperatively, children may demonstrate diaphragmatic weakness or paralysis due to phrenic nerve injury In such circumstances, our practice is to perform surgical diaphragmatic plication after paradoxical diaphragmatic movement is documented by fluoroscopy and dependence on positive pressure ventilation is demonstrated In cases of diaphragmatic weakness, muscle strength generally returns as phrenic nerve injury recovers In patients with tracheobronchial compression or malacia, tracheostomy is sometimes necessary in children with prolonged postoperative respiratory failure and/or dependence on noninvasive positive pressure ventilation and can aid in developmental and pulmonary rehabilitation Nutritional and physical rehabilitation are initiated early after surgery and continued throughout the postoperative stay, as these children require significant accessory muscle strength in order to prevent atelectasis and lung collapse Enteral feeds are initiated once patients are hemodynamically stable, and total parenteral nutrition is given to supplement nutrition After extubation, patients may require noninvasive positive pressure ventilation and are weaned to supplemental oxygen via nasal cannula Nasal cannula oxygen supplementation may be continued after discharge from the hospital Children with prolonged exposure to benzodiazepines and narcotics are weaned slowly and sometimes discharged while still on oral medications All children without a contraindication receive anticoagulation with aspirin for a minimum of 1 year after unifocalization surgery Nursing Considerations The main nursing considerations following surgery for TOF/PA/MAPCAs are focused on close hemodynamic monitoring, airway clearance, and recognition of complications Upon arrival from the operating room, normothermia is maintained Hyperthermia increases metabolic demand and predisposes to tachyarrhythmias, whereas hypothermia can worsen postoperative bleeding—a significant risk in patients who have undergone extensive dissection and pulmonary artery reconstruction with prolonged cardiopulmonary bypass Chest tube output and blood product replacement are important indicators of the potential need for surgical reexploration and require close monitoring Fluid shifts are common, as is acute kidney injury, requiring close monitoring of hemodynamic parameters and urine output Airway clearance begins in the immediate postoperative period with frequent suctioning of the endotracheal tube and the institution of mucolytic therapy with nebulized N-acetylcysteine and albuterol on the first postoperative day The bedside nurse works closely with the respiratory therapist to optimize airway clearance and lung mechanics It is important to continue pulmonary clearance after extubation, and the bedside nurse plays a key role in early mobilization, assistance with coughing, and oropharyngeal suctioning The importance of this effort cannot be overemphasized; it can make the difference between a patient being reintubated and leaving the intensive care unit Appropriate pain control is also crucial to facilitate mobilization and the clearance of secretions Bedside nurses are also key advocates for patients and families, who have often come from long distances and have experienced frequent hospitalizations and surgeries, which is the case at our program Follow-up Evaluation and Reintervention Following complete repair, whether single stage or after palliation, we perform an echocardiogram and quantitative lung perfusion scintigraphy at hospital discharge and recommend repeating these studies several times over the first 12 months as a means of screening for early adverse remodeling of the reconstructed pulmonary circulation We also recommend a routine cardiac catheterization 1 year after repair Findings indicative of increasing right ventricular pressure or progressive changes in the distribution of lung perfusion on noninvasive evaluation may merit earlier catheterization During catheterization, even modest pulmonary artery stenosis is typically treated with balloon angioplasty in an effort to optimize the uniformity of lung perfusion and pulmonary artery pressure (Figs 36.21 and 36.22, Videos 36.5 through 36.7), even if central pulmonary artery pressure is normal or low Stent implantation is avoided If there is more extensive or severe obstruction along with elevated central pulmonary artery pressure and balloon angioplasty does not provide sufficient relief, surgical revision is performed