Congenital Heart Disease

1. Congenital Heart Disease GregGordon MD American Society of Dentist Anesthesiologists Baltimore, MD, May 3, 2012

2. Training for Career in Pediatric Cardiac Anesthesia Specific Fellowship: Rare Suggested training (US & UK): • Pediatric Anesthesia: 12 months • Adult Cardiac Anesthesia: 6 months • Pediatric Cardiac Anesthesia: 6 months • Pediatric Critical Care: 6 months Baum V & De Souza DG. Pediatric Anesthesia 17:407, 2007 White MC & Murphy TWG. Pediatric Anesthesia 17:421, 2007

3. ? Children & adults scheduled for dental or oral surgery and known to have CHD Preop heart murmur: Is it CHD?

4. Adults with CHD in US today 1,500,000 Growing 2% per year Cahalan MK. Anesthetic Management of Patients with Heart Disease. IARS 2003 Review Course Lectures Andropolous, D. Anesthesia for the Patient with Congenital Heart Disease For Noncardiac Surgery. ASA Refresher Course Lectures 2011

5. 3 y/o with TOF s/p right BTS For dental restorations • Turns blue with crying • Scheduled to undergo cardiac repair • in 3 months • SpO2 93 • Systolic ejection murmur • Slight clubbing of fingers • Hct 52 Tammy

6. 5 year-old for dental work Systolic murmur Victor VSD Needs surgical closure Cardiologist recommended dental restorations first

7. 11 y/o with tricuspid atresia s/p Fontan procedure For lengthy oral surgery with possible large blood loss • Temporary BTS at age 3 weeks • Modified Fontan at age 3 years • Meds: digoxin, captopril • SpO2 88 on RA, 98 in O2 • P 67, BP 99/42 • First degree AV block Fran

8. 26 y/o with D-TGA s/p Mustard in infancy Dental restorations Developmental delay Pacemaker Travis

9. 4 y/o D-TGA s/p Jatene in infancy Dental restorations Very active Keeps up with peers Never any cyanosis Tracy

10. Objectives Participants will be able to more intelligently discuss: • Newborn and infant heart and lungs • Initial evaluation the child’s heart • Pathophysiology of selected CHDs • Anesthetic implications of CHD

11. Pediatric Anesthesia Congenital Heart Disease Lesson Presentation Quiz greggordon.org

12. Fetal Circulation Placenta (oxygenation) -> Umbilical vein -> Ductus venosus (liver bypass) –> IVC -> Foramen ovale (RV bypass) -> Left atruim -> Left ventricle –> Ascending aorta (brain) -> SVC -> Right atrium -> Right ventricle -> Main pulmonary artery -> Ductus arteriosus (lung bypass) -> Descending aorta -> Placenta

13. The Newborn Heart Foramen Ovale Functional closure first hours as LAP > RAP Probe-patent 50% of 5-year-olds 25% of 20-year-olds Paradoxical embolus

14. The Newborn Heart Ventricular tissue • Fewer myocytes • Greater proportion of connective tissue • Relative RVH So: • Decreased compliance • More sensitive to preload

15. The Newborn Heart • Near peak of Starling curve • Stroke volume relatively fixed • C.O. relatively heart rate dependent Normally near peak of Starling curve Stroke volume relatively fixed C.O. relatively heart rate dependent

16. The Newborn Heart Ca++ Newborn myocardium derives relatively high fraction of activator Ca from the extracellular pool, so Beware Ca channel blockers

17. The Preterm Infant Heart More sensitive to depressant effects of inhaled agents Decreased response to catecholamines Relatively high PVR persists Pulmonary vasculature more sensitive to vasoconstriction by: Hypoxia Acidosis Hypercarbia

18. CHD Pearl murmur in newborn = benign disease

19. Initial evaluation of child’s heart History: To determine Level of function 1.Well compensated with no limitations 2. Some limitations 3. Poorly compensated with severe limitations CHF and/or cyanosis

20. Initial evaluation of child’s heart History - cyanosis • Turn blue? • At rest? • When crying? • Passes out? • Stops playing and squats

21. Initial evaluation of child’s heart History - CHF Run around like crazy? Like sibs? Or tends to be quiet, slow? Infant – feeding behavior: Slow to finish bottle? Sweats when nursing? Eyes puffy in the morning?

22. Initial evaluation of child’s heart Physical exam • Listen to heart first when/if infant quiet • (warm stethoscope) • First concentrate on S1 and especially S2 • Louder than normal? • Split normally? • Systolic murmur: • Starts after or obscures S1? • Diastolic murmur? • Widely radiating murmur? • Palpate liver • BP in arm and leg • Tongue - cyanosis

23. CHD Pearl Sudden CHF in ‘healthy’ 10-day-old = complicated coarct

24. General Approach to CHD Patient • Define cardiovascular pathology • Predict pathophysiology • Determine hemodynamic goals • Anticipate emergency treatments Cahalan MK. Anesthetic Management of Patients with Heart Disease. IARS 2003 Review Course Lectures

25. Recent Cardiologist Evaluation Needed? Completely corrected, Well compensated and stable: Probably not Complex and/or poorly compensated; Cyanotic and/or single ventricle: YES: Evaluation & ECHO within 3-6 mos

26. Don’t worry

27. Almost any anesthetic technic may be used in any CHD patient if • the anesthesiologist understands • the pathophysiology of the lesion and • the pharmacology of the drugs employed.

28. Normal Neonate 1 week SVC PV 60 99 LA RA m=2 m=4 65 RV LV 30/3 65 80/5 99 MPA 65 Ao 99 30/12 m=18 80/50

29. Some basic definitions physiologic L to R shunt = lungs to lungs shunt Blood that is returning to the heart from the lungs is recirculated back to the lungs without going out to the rest of the body.

30. Some basic definitions physiologic R to L shunt = body to body shunt Blood that is returning to the heart from the body is recirculated directly back to the body without going to the lungs to be oxygenated.

31. Some basic definitions effective pulmonary blood flow= body to lungs flow Blood that is returning to the heart from the body that is actually directed to the lungs to be oxygenated.

32. Some basic definitions Nonrestrictive VSD VSD large enough that pressure equalizes in the two ventricles (no pressure gradient can be maintained) LV pressure = RV pressure

33. Premature 1 week old PV SVC 28 weeks EGA RA LA 96 65 RV LV 65/10 65/12 65 96 Ao MPA PDA 65/30 65/25 80 92

34. to R arm & head To L arm MHMC PDA ligation

35. CHD Pearl blue newborn + no airway or breathing problem + quiet heart = decreased PBF lesion (TOF)

36. Tetralogy Of Fallot Most common cyanotic lesion NB: cyanosis plus quiet heart Diminished pulmonary blood flow Ao ejection click Hypercyanotic “tet” spells tachypnea, pallor, LOC, less murmur Tammy

39. 3 y/o with TOF s/pright BTS • Define cardiovascular pathology • Predict pathophysiology • Determine hemodynamic goals • Anticipate emergency treatments Tammy

40. Tetralogy Of Fallot • Essentially a duality: • severe RVOT obstruction plus • nonrestrictive VSD • With anatomic consequences: • RVH • Overriding aorta Tammy • And physiologic consequences • R to L shunt • Diminished pulmonary blood flow

42. Tetralogy of Fallot SVC 40 96 RA LA m=5 m=4 RV LV 85/6 85/5 40 85 MPA 50 Ao 40 15/10 85/45

43. Tetralogy Of Fallot s/pright BTS? Blalock-Taussig Shunt Tammy

44. Thomas-Blalock-Taussig Shunt Vivien Thomas Alfred Blalock Helen Taussig Vivien Thomas, Partners of the Heart, 1998 and Something the Lord Made - Best Made-for-TV Movie, 2004

45. Thomas-Blalock-Tuassig

46. Dr. Blalock does the Blalock (Johns Hopkins)

47. Systemic to Pulmonary Shunts

49. Tetralogy Of Fallot - Goals Maintain adequate tissue oxygenation • Avoid increasing O2 demand • Maintain SVR, systemic BP • Minimize PVR Maintain good hydration, especially if polycythemic Tammy Oral premed/induction midazolam + ketamine (0.6 mg/kg + 6 mg/kg)

50. Tetralogy Of Fallot - Goals Minimize PVR Oxygen to FIO2 = 1 Mild hyperventilation PaCO2 low 30’s pH 7.45 Adequate anesthesia Adequate analgesia Normothermia, warm Nitric oxide Tammy