The population of adult patients with both Down syndrome and congenital heart disease
is increasing due to better medical and surgical care. This cohort presents with multiple
unique comorbidities, secondary to both progressions of health conditions associated
with the aging of patients with Down syndrome, as well as due to pathophysiologic
effects of uncorrected, corrected, or palliated congenital heart disease. These patients
need frequent medical care and interventions requiring multiple anesthetics. This
review focuses on the various factors relevant to the perioperative medical care of
adult patients with both Down syndrome and congenital heart disease, founded on existing
literature. Various anesthetic considerations for the different patterns of noncardiac
and cardiac comorbidities are reviewed, and a systematic approach for the perioperative
anesthetic management of these patients is presented.
Key Words
To read this article in full you will need to make a payment
Purchase one-time access:
Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online accessOne-time access price info
- For academic or personal research use, select 'Academic and Personal'
- For corporate R&D use, select 'Corporate R&D Professionals'
Subscribe:
Subscribe to Journal of Cardiothoracic and Vascular AnesthesiaAlready a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
References
- Co-occurring medical conditions in adults with Down syndrome: A systematic review toward the development of health care guidelines.Am J Med Genet. 2018; 176: 116-133
- Estimation of live birth and population prevalence of Down syndrome in nine U.S. states.Am J Med Genet. 2017; 173: 2710-2719
- Cause of death in adults with Down syndrome in the United States.Disabil Health J. 2020; 13100947
- Associated congenital anomalies among cases with Down syndrome.Eur J Med Genet. 2015; 58: 674-680
- Health conditions associated with aging and end of life of adults with Down syndrome.Int Rev Res Ment Retard. 2010; 39C: 107-126
- Congenital heart disease in Down syndrome – A review of temporal changes.J Congenit Heart Dis. 2021; 5: 1
- Unoperated tetralogy of Fallot: Case report of a natural survivor who died in his 73rd year; is it ever too late to operate?.Postgrad Med J. 2005; 81: 133-134
- Transposition of great arteries with intact ventricular septum in an adult: A successful outcome.Ann Thorac Surg. 2022; 113: e343-e346
- The natural history of congenitally corrected transposition of the great arteries.World J Pediatr Congenit Heart Surg. 2011; 2: 59-63
- Mystery still unresolved: Untouched ‘Blue heart’ presenting at 40yrs of age.Ann Pediatr Cardiol. 2020; 13: 72-74
- Anatomic observations on complete form of persistent common atrioventricular canal with special reference to atrioventricular valves.Mayo Clin Proc. 1966; 41: 296-308
- Tetralogy of Fallot.Circulation. 2014; 130: e26-e29
- Differences in morbidity and mortality in Down syndrome are related to the type of congenital heart defect.Am J Med Genet. 2020; 182 (1342-50)
- Univentricular heart.Circulation. 2007; 115: 800-812
- Congenital heart disease and cardiac procedural outcomes in patients with trisomy 21 and Turner syndrome.Congenit Heart Dis. 2017; 12: 820-827
- Cardiovascular disease in Down syndrome.Curr Opin Pediatr. 2018; 30: 616-622
- Cardiovascular and general health status of adults with Trisomy 21.Int J Cardiol. 2017; 241: 173-176
- Managing the adult patient with congenital heart disease.Anesthesiol Clin. 2020; 38: 643-662
- Pulmonary hypertension in the population with Down syndrome.Cardiol Ther. 2022; 11: 33-47
- Cane RJ. The implications and management of acute odontogenic infection in association with Down and Eisenmenger syndromes and schizophrenia in a rural setting.Aust Dent J. 2007; 52: 61-66
- Congenital heart surgery outcomes in Down syndrome: Analysis of a national clinical database.Pediatrics. 2010; 126: 315-322
- Eisenmenger syndrome and long-term survival in patients with Down syndrome and congenital heart disease.Heart. 2016; 102: 1552-1557
- Recent advances in targeting the prostacyclin pathway in pulmonary arterial hypertension.Eur Respir Rev. 2015; 24: 630-641
- Heart failure in adult congenital heart disease: Tetralogy of Fallot.Heart Fail Rev. 2020; 25: 583-598
- The adult with Fontan physiology: Systematic approach to perioperative management for noncardiac surgery.J Cardiothorac Vasc Anesth. 2011; 25: 320-334
- Outcome after a ‘perfect’ Fontan operation.Circulation. 1990; 81: 1520-1536
- Fontan palliation for single-ventricle physiology: Perioperative management for noncardiac surgery and analysis of outcomes.J Cardiothorac Vasc Anesth. 2017; 31: 2296-2303
- Tricuspid atresia: Where are we now?.J Card Surg. 2020; 35: 1609-1617
- Evaluation and management of the child and adult with Fontan circulation: A scientific statement from the American Heart Association.Circulation. 2019; 140: E234-E284
- Perioperative management of the Fontan patient for cardiac and noncardiac surgery.J Cardiothorac Vasc Anesth. 2022; 36: 275-285
- Long-term outcome after the arterial switch operation: 43 years of experience.Eur J Cardiothorac Surg. 2021; 59: 968-977
- Down syndrome: Cognitive and behavioral functioning across the lifespan.Am J Med Genet. 2015; 169: 135-149
- Epilepsy in Down syndrome: A highly prevalent comorbidity.J Clin Med. 2021; 10: 2776
- A general review of the otolaryngologic manifestations of Down Syndrome.Int J Pediatr Otorhinolaryngol. 2014; 78: 899-904
- Down syndrome and Alzheimer's disease: Common molecular traits beyond the amyloid precursor protein.Aging. 2020; 12: 1011-1033
- Epilepsy and other comorbidities in Down syndrome.Z Epileptol. 2022; 35: 235-241
- Otolaryngologic management of Down syndrome patients: What is new?.Curr Opin Otolaryngol Head Neck Surg. 2017; 25: 493-497
- High frequency of mitral valve prolapse and aortic regurgitation among asymptomatic adults with Down's syndrome.JAMA. 1987; 258: 1793-1795
- Chromosomal abnormalities and atrial fibrillation and ischemic stroke incidence: A nationwide population-based study.Sci Rep. 2020; 10: 15872
- Risk of major cardiovascular events in people with Down syndrome.PLoS One. 2015; 10e0137093
- Heart transplantation in children with Down syndrome.J Am Heart Assoc. 2022; 11e024883
- Pneumonia and respiratory infections in Down syndrome: A scoping review of the literature.Am J Med Genet. 2021; 185: 286-299
- Down syndrome and craniovertebral instability. Topic review and treatment recommendations.Pediatr Neurosurg. 1999; 31: 71-77
- Prevalence of atlanto-occipital and atlantoaxial instability in adults with Down syndrome.World Neurosurg. 2014; 82: 215-218
- Musculoskeletal anomalies in children with Down syndrome: An observational study.Arch Dis Child. 2019; 104: 482-487
- An observational study of adults with down syndrome eating independently.Dysphagia. 2014; 29: 52-60
- Gastrointestinal disorders in Down syndrome.Gastroenterol Hepatol Bed Bench. 2014; 7: 6-8
- Endocrine manifestations of Down syndrome.Curr Opin Endocrinol Diabetes Obes. 2018; 25: 61-66
- Origins of leukaemia in children with Down syndrome.Nat Rev Cancer. 2005; 5: 11-20
- Down syndrome as an independent risk factor for thrombosis in children.Blood. 2006; 108: 1489
- Low bone turnover and low bone density in a cohort of adults with Down syndrome.Osteoporos Int. 2013; 24: 1333-1338
- Managing the care of adults with Down's syndrome.BMJ. 2014; 30: g5596
- An excess of testicular germ cell tumors in Down's syndrome: Three case reports and a review of the literature.Cancer. 1997; 80: 929-935
- Perioperative care of adults with Down syndrome: A narrative review.Can J Anesth. 2021; 68: 1549-1561
- Enhanced assessment of perioperative mortality risk in adults with congenital heart disease.J Am Coll Cardiol. 2021; 78: 234-242
- 2018 AHA/ACC guideline for the management of adults with congenital heart disease: Executive summary: A report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines.J Am Coll Cardiol. 2019; 73: 1494-1563
- 2020 ESC Guidelines for the management of adult congenital heart disease.Eur Heart J. 2021; 42: 563-645
- Health care management of adults with Down syndrome.Am Fam Physician. 2001; 64: 1031-1038
- Differences in mortality characteristics in neonates with Down's syndrome.J Perinatol. 2017; 37: 427-431
- Arrhythmias in adult patients with congenital heart disease and pulmonary arterial hypertension.Heart. 2018; 104: 1963-1969
- STOP-Bang questionnaire should be used in all adults with Down syndrome to screen for moderate to severe obstructive sleep apnea.PLoS One. 2020; 15e0232596
- Clinically relevant drug interactions with anti-Alzheimer's drugs.CNS Neurol Disord Drug Targets. 2017; 16: 501-513
- Alzheimer's disease, anesthesia, and surgery: A clinically focused review.J Cardiothorac Vasc Anesth. 2014; 28: 1609-1623
- Anesthesia in adults with congenital heart disease.Curr Opin Anaesthesiol. 2017; 30: 418-425
- A clinical approach to neuraxial morphine for the treatment of postoperative pain.Pain Res Treat. 2012; 2012612145
- Dexmedetomidine as an additive to spinal anaesthesia in orthopaedic patients undergoing lower limb surgeries: A randomized clinical trial comparing two different doses of dexmedetomidine.J Clin Diagn Res. 2017; 11: UC09-UC12
- Spinal anesthesia in a patient with Down's syndrome.Can J Anesth. 2004; 51: 638
- Increased risk of dementia in people with previous exposure to general anesthesia: A nationwide population-based case-control study.Alzheimers Dement. 2014; 10: 196-204
- Asleep versus awake: Does it matter?: Pediatric regional block complications by patient state: A report from the Pediatric Regional Anesthesia Network.Reg Anesth Pain Med. 2014; 39: 279-283
- Airway management in cervical spine injury.Int J Crit Illn Inj Sci. 2014; 4: 50-56
- Hemodynamic changes in children with Down syndrome during and following inhalation induction of anesthesia with sevoflurane.J Clin Anesth. 2010; 22: 592-597
- Alzheimer's disease and suxamethonium.Anaesthesia. 1997; 52: 1018
- Potential drug–drug interactions in alzheimer patients with behavioral symptoms.Clin Interv Aging. 2015; 10: 1457-1466
- Exposure-response relationship of tranexamic acid in cardiac surgery: A model-based meta-analysis.Anesthesiology. 2020; 134: 165-178
- The lymphatic system in the Fontan patient-pathophysiology, imaging, and interventions: What the anesthesiologist should know.J Cardiothorac Vasc Anesth. 2022; 36: 2669-2678
- Xenon anaesthesia for laparoscopic cholecystectomy in a patient with Eisenmenger's syndrome.Br J Anaesth. 2001; 86: 882-886
- Spectrum of current management of pediatric pulmonary hypertensive crisis.Crit Care Explor. 2019; 1: e0037
- Prevention of infective endocarditis: Guidelines from the American Heart Association: A guideline from the American Heart Association Rheumatic Fever, Endocarditis and Kawasaki Disease Committee, Council on Cardiovascular Disease in the Young, and the Council on Clinical Cardiology, Council on Cardiovascular Surgery and Anesthesia, and the Quality of Care and Outcomes Research Interdisciplinary Working Group.J Am Dent Assoc. 2008; 139 (Suppl): 3S-24S
- Opioids for acute pain management in patients with obstructive sleep apnea: A systematic review.Anesth Analg. 2018; 127: 988-1001
- Pain assessment for people with dementia: A systematic review of systematic reviews of pain assessment tools.BMC Geriatr. 2014; 14: 138
Article info
Publication history
Published online: December 09, 2022
Identification
Copyright
© 2022 Elsevier Inc. All rights reserved.
