Down syndrome and congenital heart disease: perioperative planning and management

Abstract

Approximately 50% of newborns with Down syndrome have congenital heart disease. Non-cardiac comorbidities may also be present. Many of the principles and strategies of perioperative evaluation and management for patients with congenital heart disease apply to those with Down syndrome. Nevertheless, careful planning for cardiac surgery is required, evaluating for both cardiac and noncardiac disease, with careful consideration of the risk for pulmonary hypertension. In this manuscript, for children with Down syndrome and hemodynamically significant congenital heart disease, we will summarize the epidemiology of heart defects that warrant intervention. We will review perioperative planning for this unique population, including anesthetic considerations, common postoperative issues, nutritional strategies, and discharge planning. Special considerations for single ventricle palliation and heart transplantation evaluation will also be discussed. Overall, the risk of mortality with cardiac surgery in pediatric patients with Down syndrome is no more than the general population, except for those with functional single ventricle heart defects. Underlying comorbidities may contribute to postoperative complications and increased length of stay. A strong understanding of cardiac and non-cardiac considerations in children with Down syndrome will help clinicians optimize perioperative care and long-term outcomes.

Congenital Heart Disease and Its Association in Children With Down Syndrome

“…It is suggested by studies that the most common lesion observed in children with DS having CHD is variable according to different geographic regions in the world [ 9 , 10 ]. Therefore, it is important to know the profile and association of CHD in DS for a given geographic area for the planning of management like intervention, surgical management and medical follow-up keeping in view the high risk of pulmonary vascular disease in these patients [ 11 , 13 ].…”

Unplanned Return to Cardiac Intensive Care Unit of Children After Congenital Heart Surgery: Incidence, Outcomes, and Risk Factors

“…In addition to cardiac problems, chromosomal abnormalities also may cause developmental delays and slow postoperative recovery. Dennis noted that underlying comorbidities with Down syndrome might increase postoperative complications and length of stay [Delany 2021]. Longer ventilator use may reflect worse postoperative lung conditions in these patients, requiring more extended hospital stays.…”

Down Syndrome: A Review of Key Perioperative Implications

“…Even if the assessment reveals no changes or symptoms of cervical instability, the anesthesia professional should consider avoiding neck flexion or extension during laryngoscopy and using an in‐line neck stabilizer or collar. Also, the anesthesia professional should consider the use of fiberoptic 9 or video‐assisted 10 laryngoscopy. Perioperative nurses should be prepared to assist with airway management if needed.…”

“…The nurse should ensure that the severity of the abnormality, the status of repair, and the presence of any residual lesions have been thoroughly assessed and documented before surgery. Because children with DS have an increased risk of developing bradycardia during anesthesia induction, anticholinergic agents (eg, atropine) should be readily available during the procedure 9 …”

“…Infection prevention is integral to perioperative nursing. Individuals with DS are not only more likely than individuals without DS to develop an infection, but also may experience prolonged recovery 9,50 . The perioperative nurse should determine if the patient with DS has been medically optimized for surgery.…”