Table 2.

Classes of risks and suggested peri-operative precautions/actions for specific syndromes.

Class	Syndromes	Actions
Cardiac rhythm	HTX (SND, AV block, tachyarrhythmia), WS (LQT,   ventricular ectopy), TS (LQT), Costello (atrial   tachycardia), Holt–Oram (AV block)	Maintenance of normal electrolyte levels, routine   placement of temporary pacing wires
Vascular (systemic)	TS, WS, LDS, PHACES	Pre-operative vascular imaging studies, documentation   of pre-operative BP, patient-specific BP goals,   ultrasound-guided arterial access
Vascular (pulmonary)	DS, HTX, EVC	Pre-operative cardiac catheterisation, post-operative   manoeuvers to minimise PVR
Myocardial	HTX (non-compaction cardiomyopathy), trisomy 13   (non-compaction cardiomyopathy)	Intra-operative myocardial protection, anticipatory post-   CPB management of ventricular dysfunction
Respiratory	Upper airway anomalies: DS, 22q11 deletion,   CHARGE, PHACES, Cri du chat, Cornelia de Lange Lower airway disease: DS, EVC, MFS/LDS	Pre-operative anatomic upper airway evaluation,   extubation protocols, post-operative evaluation of   airway protection mechanisms, otolaryngology/   pulmonary consultation
Immunologic/infectious	DS, 22q11 deletion, HTX, Kabuki, Smith–Magenis,   Wolf–Hirschhorn, Cornelia de Lange	Immunology consultation, broad-spectrum   antimicrobial prophylaxis, minimise invasive   monitoring
Haematologic	22q11 deletion, NS, AGS, Jacobsen, Cornelia de Lange	Haematology consultation, post-CPB antifibrinolytics,   BP control, rapid access to blood products, liberal blood   product administration
Neurologic	Seizure: DS, 22q11 deletion, Kabuki, Smith–Magenis,   Wolf–Hirschhorn	Seizure: neuroprotection, peri-operative EEG evaluation,   normocalcaemia (22q11 deletion)
	Cerebrovascular: AGS, PHACES, LDS, WS, NS Cervical instability: DS, LDS	Cerebrovascular: pre-operative cerebrovascular imaging,   cerebral perfusion pressure monitoring, urgent imaging   for neurological changes Cervical instability: appropriate positioning/support
Endocrine	Hypothyroidism: DS, TS, WS, PHACES, Jacobsen,   Smith–Magenis Pituitary dysfunction: CHARGE	Pre-operative thyroid function testing, endocrinology   consultation as needed, steroid replacement
Lymphatic	DS, TS, NS	Monitoring for chylothorax and sequelae if present, early   transition to low and medium chain triglyceride diet/   formula, minimise central venous pressure

AGS = Alagille syndrome; AV = atrioventricular; BP = blood pressure; CPB = cardiopulmonary bypass; DS = Down syndrome; EEG = electroencephalogram; EVC = Ellis–van Creveld; HTX = heterotaxy syndrome; LDS = Loeys–Dietz syndrome; LQT = prolonged QT interval; MFS = Marfan syndrome; NS = Noonan syndrome; PVR = pulmonary vascular resistance; SND = sinus node dysfunction; TS = Turner syndrome; and WS = Williams syndrome