Williams–Beuren syndrome: a complete guide for oral healthcare | Journal of Oral Medicine and Oral Surgery

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Table V

Risk stratification for WBS undergoing procedures under general anesthesia.

Low risk	>20 Years of age, cardiac involvement limited to mild supravalvar or branch pulmonary artery stenosis, normal ECG, lacking renal artery involvement.
Moderate risk	Hypertension, supravalvar or branch pulmonary artery stenosis of moderately involvement, mild bilateral outflow tract obstruction, renal artery stenosis, renal dysfunction, ECG findings of QTc between 450 and 500 ms, lung/airway abnormalities, severe gastroesophageal reflux.
High risk	<3 Years of age, history of adverse cardiovascular event, presence of preprocedural arrhythmia, bilateral outflow tract obstruction of ≥ moderate severity, SVAS gradient of ≥40 mm Hg with concomitant left ventricular hypertrophy, involvement of coronary artery, thoracic aorta diffuse stenosis, ≥ moderate left/right ventricular hypertrophy, cardiac ischemic symptoms/ECG evidence,ECG findings of QTc ≥500 ms

Low risk

>20 Years of age, cardiac involvement limited to mild supravalvar or branch pulmonary artery stenosis, normal ECG, lacking renal artery involvement.

Moderate risk

Hypertension, supravalvar or branch pulmonary artery stenosis of moderately involvement, mild bilateral outflow tract obstruction, renal artery stenosis, renal dysfunction, ECG findings of QTc between 450 and 500 ms, lung/airway abnormalities, severe gastroesophageal reflux.

High risk

<3 Years of age, history of adverse cardiovascular event, presence of preprocedural arrhythmia, bilateral outflow tract obstruction of ≥ moderate severity, SVAS gradient of ≥40 mm Hg with concomitant left ventricular hypertrophy, involvement of coronary artery, thoracic aorta diffuse stenosis, ≥ moderate left/right ventricular hypertrophy, cardiac ischemic symptoms/ECG evidence,ECG findings of QTc ≥500 ms

ECG, Electrocardiogram; QTc, QT interval on ECG (corrected); SVAS, supravalvar aortic stenosis (Adapted from Collins et al. [26]).

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