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Pediatric Tuberous Sclerosis Patients Undergoing ELECTIVE General Anesthesia
LESS THAN (<) 3 Years Old
1. At least one ECG
a. If history of abnormal ECG – ECG within 12 months.
2. Pediatric Cardiology evaluation within 12 months
3. At least one post-natal ECHO
a. NO Cardiac Rhabdomyoma OR Regressing Rhabodmyoma (including prenatal echo) = ECHO OR Pediatric Cardiology evaluation within 12 months that includes recommendations for continued surveillance (Ex: ECHO at two years old, etc).
b. Non-regressing Cardiac Rhabdomyoma (including prenatal echo) = ECHO within 12 months
3-12 Years Old
1. At least one ECG within 3 years
a. If history of abnormal ECG – ECG AND Pediatric Cardiology evaluation within 12 months
2. At least one post-natal ECHO
a. NO Cardiac Rhabdomyoma OR Regressing Rhabodmyoma (including prenatal echo) = normal ECHO at/after age of two OR Pediatric Cardiology evaluation that includes recommendations for continued surveillance (Ex: no need for f/u ECHO, etc.)
b. Non-regressing Cardiac Rhabdomyoma (including prenatal echo) =
i. If most recent ECHO shows a non-obstructive rhabdomyoma, patient requires an ECHO OR a Pediatric Cardiology evaluation within 12 months that includes recommendations for continued surveillance (Ex: echo q2 years).
ii. If most recent ECHO shows an obstructive rhabdomyoma, patient requires an ECHO AND a Pediatric Cardiology evaluation within 12 months
12-15 Years Old
1. At least one ECG within 3 years
a. If history of abnormal ECG – ECG AND Pediatric Cardiology evaluation within 12 months
2. Pediatric Cardiology Evaluation within 3 years (to assess for re-emergence of pathology with puberty)
3. At least one post-natal ECHO - Repeat Echo per Cardiology Recommendation (see echo requirements for 3-12 years old for more specific information)
15-18 Years Old
1. At least one ECG within 3 years
a. If history of abnormal ECG – ECG AND Pediatric Cardiology evaluation within 12 months
2. Prior Pediatric Cardiology Evaluation during Adolescence
3. At least one post-natal ECHO - Repeat Echo per Cardiology Recommendations (see echo requirements for 3-12 years old for more specific information)
4. Renal Function Panel with GFR within 12 months
Tuberous Sclerosis is a genetic syndrome affecting many different organ systems. Patients manifest widespread harmatomas including cardiac rhabdomyomas. AHA has issued a special report regarding these patients and states that “There is an increasing appreciation for latent cardiovascular phenotypes, indicating a need for continued surveillance of these patients.1” The AHA also states “80% to 85% of children with confirmed TSC have identifiable rhabdomyomas when younger than 2 years.1” To better serve these patients and to avoid unnecessary studies, the OU Children’s Hospital Pediatric Anesthesiology, Cardiology, and Hematololgy/Oncology divisions have collaborated to devise the above guidelines. We are placing a heavier emphasis on continued pediatric cardiology assessment and less emphasis on yearly echocardiograms (especially in those patients > 3 years old).
References:
1. Hinton RB, Prakash A, Romp RL, Krueger DA, Knilans TK. Cardiovascular Manifestations of Tuberous Sclerosis Complex and Summary of the Revised Diagnostic Criteria and Surveillance and Management Recommendations From the International Tuberous Sclerosis Consensus Group. Journal of the American Heart Association. 2014
2. Thatte, N., Guleserian, K., & Veeram Reddy, S. (2016). New-onset cardiac rhabdomyoma beyond infancy in a patient with tuberous sclerosis complex. Cardiology in the Young, 26(2), 396-399. doi:10.1017/S1047951115001183
3. Shenkman Z, Rockoff MA, Eldredge EA, Korf BR, Black PL, Soriano SG. Anaesthetic management of children with tuberoussclerosis. Paediatric Anaesthesia. 2002 12: 700–704
Updated: 5/06/2020
Kent Ward, MD – Cardiology
Rene McNall, MD – Hematology/Oncology
Abhishek Bavle, MD – Hematology/Oncology
Cassandra Duncan-Azadi, MD - Anesthesiology
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