Recommendations for Long-Term Follow-up Investigations and Frequency All patients should have regular follow-up by an appropriately trained cardiologist at least once a year These investigations should include, at a minimum, a thorough clinical assessment, electrocardiogram, echo-Doppler examination to detect and quantify residual pulmonary stenosis and regurgitation, any residual ventricular septal defect, right and left ventricular size and function, aortic regurgitation, and the size of the aortic root Investigations are directed toward the known postoperative sequelae and will vary according to the type of operation performed To document and detect acute changes in right ventricular size and function as well as to assess pulmonary regurgitant fraction, follow-up should include magnetic resonance imaging at least every 2 or 3 years.86 Rarely, in some patients, catheterization may be required for intervention on the pulmonary arteries or for coronary angiography in those at risk of coronary atherosclerosis undergoing surgery in later life The role of arrhythmic surveillance is more controversial There is minimal evidence showing the value of either Holter monitoring or electrophysiologic studies for the stratification of risk in those free of clinical symptoms, but they may be useful in defining the type and severity of the arrhythmia in patients who complain of undocumented palpitations Pregnancy All patients with tetralogy should have cardiologic counseling prior to conception as well as follow-up by an appropriately trained cardiologist during pregnancy.87 Genetic counseling is also recommended Men and women with tetralogy have reduced reproductive fitness Their offspring are at significantly elevated risk for severe coronary heart disease (CHD) Recurrence risk for CHD in offspring is 4.8%, with no significant difference between men and women with TOF The risk of severe CHD in offspring (2.3%) far exceeds population expectations.88 Assessment of the risk of 22q11 deletion using fluorescent in situ hybridization is also recommended prior to embarking on a planned pregnancy The risk of transmission is 50% if one of the parents has such a deletion The risk of pregnancy in patients with TOF after corrective surgery depends on the hemodynamic state The risk is low, approaching that of the general population, in patients with good underlying hemodynamics In patients with significant residual obstruction across the right ventricular outflow tract, severe pulmonary regurgitation, tricuspid regurgitation, and right ventricular dysfunction as well as left ventricular dysfunction, the increased volume load of pregnancy may lead to right heart failure and arrhythmias In the rare case of right ventricular failure during pregnancy, delivery prior to term should be considered.87 Peripartal antibiotic prophylaxis is recommended Nursing Considerations Nursing staff involved in the care of pediatric cardiac patients should have a basic understanding of cardiovascular physiology and anatomy along with lesion-specific pathophysiology With the ability to recognize signs of low cardiac output syndrome, diastolic dysfunction, or rhythm disturbances and to anticipate any potential complications based on age, prior clinical condition and comorbidities as well as earlier surgical intervention, the nurse can anticipate and observe for signs of complications in order to intervene promptly and prevent harmful consequences Preoperative Nursing Considerations Nursing management for the neonate with TOF varies depending on the degree of right ventricular outflow tract obstruction, with a focus on minimizing metabolic demands, supporting respiratory function and oxygenation, and providing for nutritional needs to ensure appropriate growth Once an individual has been diagnosed with TOF, immediate management is determined by the child's oxygen levels Children with mild obstruction require close monitoring for worsening of hypoxia, with plans for surgical repair to be completed at 3 to 9 months of age Before surgery, clinic visits for these children should occur every 3 to 4 weeks to monitor saturation, work of breathing, feeding tolerance, and weight gain These children should have routine wellchild checks, immunizations to include palivizumab (Synagis), and iron supplementation to prevent anemia.89 Prevention of agitation, dehydration, and iron deficiency are key to avoiding hypercyanotic spells, thereby giving the child time to grow and mature prior to surgical intervention Children with TOF presenting with moderate to severe obstruction have a higher risk for increased work of breathing, hypercyanotic spells, and metabolic acidosis.90 Medical management of symptomatic newborns presenting with significant cyanosis may require a prostaglandin infusion to maintain ductal patency and improve pulmonary blood flow to increase oxygen levels These infants should be monitored for complications of a prostaglandin infusion, such as apnea, fever, irritability, flushing, bradycardia, hypotension, and hypoglycemia.91 Surgical intervention will be necessary in the neonatal period For neonates or older infants admitted to the hospital with worsening cyanosis, ... considered.87 Peripartal antibiotic prophylaxis is recommended Nursing Considerations Nursing staff involved in the care of pediatric cardiac patients should have a basic understanding of cardiovascular physiology and anatomy along with