UCSF Congenital Cardiac Anesthesia Tutorials Tetralogy of Fallot

UCSF Congenital Cardiac Anesthesia Tutorials Tetralogy of Fallot for Cardiac Anaesthesia 1 William C K Ng

acknowledgements 2

To. F: Intro ❖ 10% of CHD ❖ Tetrad ❖ • Over-riding Aorta • RVOTO (sub-PS) • Unrestrictive VSD • RVHT TOF/PA, TOF/Absent PV • ❖ MAPCA Other lesions and syndromes 3

History, Examination, Investigations ❖ Prenatal diagnosis vs. Postnatal diagnosis - more obvious when PDA is closed. • Cyanosis • Syndromes • Chest ❖ ECG: RAD ❖ CXR: “clog-shaped” heart Cœur en sabot. 4

Consequences and associations of To. F ❖ Biventricular origin of Aorta ❖ HT RVOT ❖ PA hypoplasia or PV absence, and consequences of each ❖ Other ❖ • PV dysplasia • RAA with mirror branching • Coronary pathways: LAD from RCA Syndromes: VACTERL, CHARGE, Alagille, 22 q 11 deletion, Trisomy 21 5

❖ Absent PV -> PR -> RV volume overload > dilated PAs. 6 ❖ APCs in pulmonary atresia: ❖ “No Flow, No Grow. ” said a wise man from Alabama.

• • • RVOTO and dynamic changes VSD: number, location, size (unrestricted) Coronaries Other abnormalities Function and dynamic changes 7 Echocardiograp hy

Pathophysiology ❖ ❖ R-L shunt, cyanotic “Tet” spells, dynamic RVOTO (infundibular spasms). “Pink Tets” • ❖ ❖ CHF, pulmonary volume overload RV HT -> diastolic dysfunction, risk for ischaemia, poor protection on CPB, post-operative problems. SVR and PVR balance • Avoid increased contractility, tachycardia and decreased preload 8

Repair: Pre-operative Considerations ❖ Surgical Planning ❖ Premedication ❖ Symptoms? ❖ IV access ❖ Palliative Shunt prior to Repair? ❖ Hemodynamic goals • ❖ <1 month • <6 months, <4 kg • Alternative: balloon dilation Definite Repair • contractility • preload • afterload • rate ❖ PDA, APC, Interventions? ❖ >6 months ❖ Medications ❖ >4 months, >4 kg ❖ Induction 9

Repair: Intraoperative Considerations ❖ ❖ PVR • ❖ ❖ • O 2, CO 2, p. H Perfusion Pressure • ❖ RV, LV Surgical Goals: • Relief of RVOTO • VSD repair • Preservation of RV function ❖ Morales et al. on Right Infundibular sparing 10 IM/IV: Ketamine, Fentanyl, Rocuronium, low-dose Sevoflurane Monitoring • NIRS • Invasive monitoring and CVC Rescue drugs, fluid, maneouvres ❖ ❖ ❖ Induction dangers, access and plan B alpha, IVF, beta-blocker, sevoflurane, abdominal pressure, position Maintenance ❖ Fentanyl ❖ Fast-track

Repair of To. F ❖ ❖ ❖ RVOT • TAP • Ventricular incision vs. infundibular sparing • Valve sparing, repair • RV-PA conduit in coronaries over RVOT • Homograft VSD ASD 11

Post-operative and Late considerations ❖ RV function ❖ ❖ ❖ ❖ Inotropes, filling & RAp, PVR RVp: LVp (<0. 75) Disposition Conduction system (VSD); JET (10%) Cardiac Catherization • Pulmonary rehabilitation • BDs, Stents, APCs ❖ Redo: RV-PA conduit, PVR ❖ Mortality ❖ Shunt: physiological, post-op ventilation; thrombosis, stenosis ❖ Ventricular incision, TA patch and complications Predictors on CMR (LV CS, RV LS) Morbidity ❖ Ventricular function, ET ❖ Atrial Tachyarrhythmias (atrial dilation), VT ❖ Residual VSDs (>3 mm) ❖ QRS >180 s and PVR ❖ PBF: too much/little ❖ Aortic Dilation 12

To. F and non-Cardiac Surgery ❖ Pre-repair? Hemodynamic goals: C. R. A. P. ❖ Un-repaired with cyanosis? ❖ Earlier repairs with Transannuloplasty: PR and RV dilation ❖ Post-repair: • Function on history • Echo assessment, RVOT, PV, RV • PA: anatomy and haemodynamics • EPS: atrial tachy-arrhythmias (AF) • Role of CMR (see. Ezzat et al. ) 13

To. F: Key Points ❖ Most common form of cyanotic heart disease ❖ Hemodynamic Management Goals ❖ Unrepaired: PVR vs. SVR ❖ Repaired: ❖ ❖ Induction ❖ Post-op. complications ❖ Cath-lab pulmonary rehabilitation ❖ Late outcomes BTS: another time 14