HLHS & NORWOOD PROCEDURE

1. NORWOOD
PROCEDURE- A REVIEW
MURTAZA KAMAL
16/AUGUST/2018
murtaza.vmmc@gmail.com
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2. DR.WILLIAM IMON NORWOOD(1941-)
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3. CONTENT OFTODAY’S SEMINAR
• UNDERSTANDING HLH PHYSIOLOGY: IT’S DISSECTION
• GOALS OF PALLIATIVE MEASURES
• STAGED PALLIATIVE MEASURES
• UNDERSTANDING DIFFERENT METHORDS OF STAGE 1 PALLIATION
• PROS + CONS OF STAGE 1 PALLIATIVE MEASURES
• INTERSTAGE ISSUES
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4. HLH PHYSIOLOGY: DEFINITION
•INABILITY OF LT. HEARTTO SUSTAIN
ADEQUATE CO FOLLOWING BIRTH BECAUSE
OF UNDERDEVELOPMENT OF ≥1 LEFT
HEART STRUCTURES DESPITE MEDICAL OR
SURGICAL MANAGEMENT
• Kirklin/Barratt-Boyes Cardiac Surgery, 4th Edition
4

5. 1. LEFT HEART
• MORPHOLOGIC COMPOSITE OR UNIT
• EACH PLAYS IMPORTANT ROLE IN DETERMINING LH FUNCTION
• LT. ATRIUM
• MITRALVALVE
• LT.VENTRICLE
• AORTICVALVE
• AORTA
5

6. 2. PHYSIOLOGIC DEFINITION:WHY?
• DEFINITION IS PHYSIOLOGIC, NOT MORPHOLOGIC
• MORPHOLOGIC DEFINITION NOT POSSIBLE: AS UNDERDEVELOPMENT
OF AVARIABLE NUMBER OF SPECIFIC LT. HEART STRUCTURES, EITHER
ALONE OR INVARIOUS COMBINATION: RESPONSIBLE FOR
PHYSIOLOGIC INADEQUACY
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7. 3. HLH PHYSIOLOGY
• HLH PHYSIOLOGY: PRESENT WITHIN A RELATIVELY NARROW BAND OF
BROAD CONTINUUM OF HYPOPLASTIC LESIONS OF LT. HEART
• RANGE OF CONTINUUM: ISOLATED SIMPLE LESIONSTO COMPLEX
MULTILEVEL LESIONS
• POINT ALONG THIS GRADUALLY INCREASING CONTINUUM OF HLH
ABNORMALITIES WHERE HLHP IS ENCOUNTERED: IMPOSSIBLETO PINPOINT
MORPHOLOGICALLY
• CAN BE A RESULT OF SEVERE ABNORMALITY OF A SINGLE LT. HEART
STRUCTURE OR A COMBINATION OF SEVERAL MINOR ABNORMALITIES
7

8. 4.WHAT DO WE MEAN BY “ DESPITE
MEDICAL OR SURGICAL INTERVENTION?”
• THIS PHRASE IMPORTANT:AS ABNORMALITIES SUCHAS CRITICALAS OR ISOLATED
SEVERECOA MAY MEET CRITERIAOF PHYSIOLOGIC DEFINITION BEFORE BUT NOT
AFTER INTERVENTIONTOCORRECTABNORMALITY
• THE LT. HEART IS INCAPABLEOF SUSTAINING SYSTEMICCARDIAC OUTPUT,THERE BY
LIMITINGTHERAPEUTICOPTIONSTO:
• RECONSTRUCTIONSTHAT USEA SINGLE (RT.)VENTRICULAR PUMPING
CHAMBER-
• NORWOOD PROCEDURE, BDG, FONTAN
• HEARTTRANSPLANT
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9. CONCEPT OF BORDERLINE HLHP
• IMPOSSIBLETO DEFINE A SPECIFIC POINT ALONGTHE MORPHOLOGIC
AND PHYSIOLOGIC CONTINUUM WHERE LT. HEART BECOMES
UNQUESTIONABLY UNRESOLVABLE
• A ZONE ALONGTHIS CONTINUUM INWHICH ITS CURRENTLY NOT
POSSIBLETO PREDICT WITH CERTAINITY WHETHER LT. HEART CAN BE
SALVAGED WITH SURGICAL RECONSTRUCTIVE METHORDS
• IMPORTANCE: CLINICAL DILEMMA OF SURGEONS: MAKING
DICHOTOMOUS DECISION
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10. MILESTONESOFTHE CONDITION
• 1850: AORTIC ATRESIA: CANTON (Canton M. Congenital obliteration of origin of the aorta.
Trans Pathol Soc (Lond) 1850;2:38.)
• 1950: IN 50% CASES OF MITRAL ATRESIATHERE WAS CO EXISTANCE OF
AORTIC ATRESIA WITH SEVERE UNDERDEVELOPMENT OF LEFT SIDE OF
HEART: BROCKMAN (Brockman JL. Congenital mitral atresia. Am Heart 1950;40:301.)
• 1952: HYPOPLASIA OFTHE AORTICTRACT COMPLEXES: LEV(Lev M.
Pathologic anatomy and interrelationship of hypoplasia of the aortic tract complexes. Lab Invest 1952;1:61.)
• 1958: HLHS: NOONAN & NADAS (Noonan JA, Nadas AS.The hypoplastic left heart syndrome:
an analysis of 101 cases. Pediatr Clin North Am 1958;5:1029.)
• 1976: EMSPHASIZEDTHAT PRESENGE OF LARGEVSD, AORTIC ATRESIA
CAN COEXISTWITH NORMAL DEVELOPMENT OF LV & MITRALVALVE:
ROBERTS (Roberts WC, Perry LW, Chandra RS, Myers GE, Shapiro SR, Scott LP. Aortic valve atresia. A new
classification based on necropsy study of 73 cases. Am J Cardiol 1976;37:753.)
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11. MANAGEMENT MILESTONES
• 1970: CAYLER: ANASTOMOSIS OF RPA+ ASCENDING AORTA + B/L PA
BANDING (Cayler GG, Smeloff EA, Miller GE Jr. Surgical palliation of hypoplastic left side of the heart. N
Engl J Med 1970;282:780.)
• OTHERS: DOTY, LEVITSKY, BEHRENDT
• 1983: NORWOOD, LANG, HANSEN (NorwoodWI, Lang P, Hansen DD. Physiologic repair of
aortic atresia–hypoplastic left heart syndrome. N Engl J Med 1983;308:23.)
• 1985: ALLOGRAFT HEARTTRANSPLANT: BAILEY (Bailey LL, Nehlsen-Cannarella SL,
Doroshow RW, Jacobson JG, Martin RD, Allard MW, et al. Cardiac allotransplantation in newborns as therapy for
hypoplastic left heart syndrome. N Engl J Med 1986;315:949.)
• 1993: HYBRID PROCEDURE: B/L BRANCH PA BANDING SURGICALLY+
PDA STENTING+ ATRIAL SEPTOSTOMY CATHETER BASED: AVOIDING
CPB (Gibbs JL, Wren C, Watterson KG, Hunter S, Hamilton JR. Stenting of the arterial duct combined with
banding of the pulmonary arteries and atrial septectomy or septostomy: a new approach to palliation for the
hypoplastic left heart syndrome. Br Heart J 1993;69:551-5.)
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12. NORWOODADDRESSED ISSUESWHICH
THE PREVIOUS DIDN’T
• HAD LONGTERM AORTIC GROWTH POTENTIAL
• MINIMUM PULMONARYVALVE DISTORTION
• ADDESSED DISTAL ARCH OBSTRUCTION
• PRESERVATION OF PA PATENCY
• BALANCED PULMONARY AND SYSTEMIC BLOOD FLOWS
• ENSURED ADEQUATE ATRIAL LEVEL MIXING
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13. MORPHOLOGYAND MORPHOGENESIS
• HEART ENLARGED TO ABOUT
TWICE NORMAL WEIGHT FOR AGE
• SHAPE DETERMINED BY LARGE
RIGHT AND SMALL LEFT HEART
CHAMBERS
• van der Horst RL, Hastreiter AR, DuBrow IW,
Eckner FA. Pathologic measurements in aortic
atresia. Am Heart J 1983;106:1411
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14. MAJOR MORPHOLOGIC SUBTYPES
• AORTIC AND MITRAL ATRESIA: MC (2/3RD)
• AORTIC ATRESIA WITH MITRAL STENOSIS
• AORTIC STENOSIS WITH MITRAL ATRESIA: LC(5%)
• AORTIC AND MITAL STENOSIS
WITHINTHESE SUBTYPESTHERE IS IMPORTANCE OF
ATRIAL SEPTUM
LV CAVITY SIZE AND LV MUSCLE SIZE
ASCENDING AORTA AND ARCH
DUCTUS ARTERISUS
14

15. 1. AORTICVALVE & ASCENDINGAORTA
• AORTIC ATRESIA: ABSCENT AORTICVALVE
• DIMINITIVE AORTIC SINUSES OFVALSALVA
• NORMAL RCA+ LCA DISTRIBUTION AND
POSITION
• NARROW ASCENDING AORTA (<2MM)
• PORTION OF AORTA B/WATRETICVALVE
AND BRACHIOCEPHALICTRUNK: SERVES
ONLY AS A CONDUIT FOR CORONARY
BLOOD FLOW
• Elzenga NJ, Gittenbergerde GrootAC. Coarctation and related aortic arch
anomalies in hypoplastic left heart syndrome. Int J Cardiol 1985;8:379
• Milo S, Ho SY, Anderson RH. Hypoplastic left heart syndrome: can this
malformation be treated surgically?Thorax 1980;35:351 15

16. 1. AORTICVALVE & ASCENDINGAORTA
• 80%: LOCALISED COA: JUXTRA DUCTAL MOSTLY ASSOCIATED WITHTHOSE
WITH MORE SEVERE HYPOPLASIA
• IF PATENT BUT HYPOPLASTIC AORTICVALVE:
• REDUCED AMOUNT OF FORWARD FLOW
• DIAMETER OF ASCENDING AORTA: 2-6 MM
• COA: COMMON
• Elzenga NJ, Gittenbergerde Groot AC. Coarctation and related aortic arch anomalies in hypoplastic left heart
syndrome. Int J Cardiol 1985;8:379
• Milo S, Ho SY, Anderson RH. Hypoplastic left heart syndrome: can this malformation be treated surgically?
Thorax 1980;35:351
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17. 2. LV AND MITRALVALVE
• 95% CASES OF AORTIC ATRESIA:
• LV SEVERLY HYPOPLASTIC
• INTACT IVS
• MV: ATRETIC( 1/3RD) OR SEVERLY HYPOPLASTIC(2/3RD)
• IF MV PATENT IN ASSOCIATION WITH AORTIC ATRESIA: LV- CORONARY
CONNECTION MAY BE PRESENT (TO DECOMPRESS LV)
• 5% CASES OF AORTIC ATRESIA:
• NORMAL LV CAVITY SIZEWITH LARGEVSD
• MITRALVALVE ATRESIA OR NORMAL MV
• Sinha SN, Rusnak SL, Sommers HM, Cole RB, Muster AJ, Paul MH. Hypoplastic left ventricle syndrome. Analysis
of thirty autopsy cases in infants with surgical considerations. Am J Cardiol 1968;21:166.
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18. 2. LV AND MITRALVALVE
• IN AORTIC STENOSIS:
• LVTENDSTO BE LARGER
• MV ALMOST ALWAYS HYPOPLASTIC, MAY BE ATRETIC WHEN
SEVERE AS PRESENT
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19. 3. RV
• ENLARGED WITH UNIFORM HYPERTROPHY
• MARKED INCREASE IN CAVITY SIZE (3X)
• TV+ PV LARGERTHAN NORMAL
• TR:VARIABLE DEGREE +NT
• Hastreiter AR,Van der Horst RL, Dubrow IW, Eckner FO.Quantitative angiographic and morphologic aspects of
aortic valve atresia. Am J Cardiol 1983;51:1705.
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20. 4. PAs
• LARGE PULMONARYTRUNK
• CONTINUES DIRECTLY INTO LARGE PDA
• RT & LT BRANCHES: ARISE RELATIVELY POSTERIORLY AND AT RT
ANGLES
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21. 5. ATRIA & ATRIAL SEPTUM
• LA: SMALLTHICKWALLED
• ATRIAL SEPTUM:THICK BAS UNSATISFACTORY
• STRETCHED PFO: ATRIAL COMMUNICATION : FOR LT RT SHUNTING
• IF INTACT ATRIAL SEPTUM/ SEVERLY RESTRICTIVE+ MITRAL OR AORTIC
ATRESIA OR BOTH PV HT
• PV HT: BEGINS IN FETAL LIFE: IMPLICATIONS FOR FETAL LUNG
DEVELOPMENT
• DILATED PULMONARY LYMPHATIC CHANNELS: EFFECT ON POSTNATAL
LUNG PHYSIOLOGY AND SURGICAL OUTCOME
• Glatz JA,Tabbutt S, Gaynor JW, Rome JJ, Montenegro L, SprayTL, et al. Hypoplastic left heart syndrome with atrial level
restriction in the era of prenatal diagnosis. AnnThorac Surg 2007;84:1633-8.
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22. 6. OTHERASSOCIATED CARDIAC
ANOMALIES
• UNCOMMON
• BICUSPID PV: 4%
• CLEFTTV,TV DYSPLASIA, DOTV
• CORONARY ARTERY ANOMALY UNCOMMON: EXCEPTION AORTIC ATRESIA
AND MS: 50% CASES
• Mahowald JM, Lucas RV Jr, Edwards JE.Aortic valvular atresia. Associated cardiovascular anomalies. Pediatr Cardiol 1982;2:99.
• Baffa JM, Chen SL, Guttenberg ME, Norwood WI,Weinberg PM.Coronary artery abnormalities and right ventricular histology in
hypoplastic left heart syndrome. J Am Coll Cardiol 1992;20:350.
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23. 07. ASSOCIATED NON CARDIAC
ABNORMALITIES
• FREQUENT
• 28-40%
• CHROMOSOMAL AND GENETIC DEFECTS
• CNS ABNORMALITIES
• Natowicz M, Chatten J, Clancy R, Conard K, GlauserT, Huff D, et al. Genetic disorders and major extracardiac anomalies
associated with hypoplastic left heart syndrome. Pediatrics 1988;82:698.
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24. CLINICAL FEATURES: PRESENTATION
• NEONATAL PERIOD PRESNTATION
• MILD CYANOSIS, RD, HR↑
• RAPID DETERIORATION, HEART FAILURE, DEATH (PULMONARY
OVERCIRCULATION AND SYSTEMIC OBSTRUCTION DUCT CLOSURE)
• DUCT CLOSURE:TIMINGVARIES: HOURSTOWEEKS RAPID
CIRCULATORY COLLAPSE
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25. CLINICAL EXAMINATION
• PERIPHERAL PULSES AND PERFUSION: POOR
• BP: LOW
• PRECORDIAL IMPULSE: HYPERACTIVE RVTYPE
• S2: ACCENTUATED AND SINGLE
• MODERATE INTENSITY MID SYSTOLIC MURMUR
• HEART FAILURE: RALES+ HEPATOMEGALY
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26. CXR
• MODERATE CARDIOMEGALY
• PULMONARY PLETHORA
• DUETO ↑ 𝑃𝐵𝐹
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27. ECG
• RAD
• RVH
• NO LV FORCES
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28. ECHOCARDIOGRAPHY
• DIAGNOSTIC+ DEFINITIVE
• LARGE RV,TV, PDA+ SMALL/
ABSCENT LV, AORTC/ MV &
ASCENDING AORTA
• ATRIAL SEPTUM STATUS
• DOPPLER: RETROGRADE
FLOW IN AORTIC ARCH &
ASCENDING AORTA IF
AORTIC ATRESIA
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29. ECHO
29

30. SCRATCHYOUR HEADS
• WHEN CANTHERE BE
ANTEGRADE FLOW IN
ASCENDING AORTA IN SETTING
OF AORTIC ATRESIA??
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This Photo by Unknown Author is licensed under CC BY-SA

31. LV CA FISTULA
31
• LV BLOOD
• CORONARY ARTERY
• ASCENDING AORTA

32. CARDIAC CATHETERISATION
• RARELY NEEDED
• BORDERLINE HLHP:TO CHARACTERISE PHYSIOLOGY, MV GRADIENT AND
LVEDP
• TO DECIDE IF 2VENTRICULAR REPAIR CAN BE DONE
• SEVERLY RESTRICTIVE/ INTACT IAS RESULTING IN PV HT
• BAS, BLADE SEPTOSTOMY, ATRIAL SEPTUM PUNCTURE WITH DILATATION
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33. CT/ MRI
• LIMITED ROLE
• CT ANGIO: IMPORTANT IN FOLLOW UP: ANATOMICAL DETAILS OF
AORTIC ARCH AND PA GROWTH & DEVELOPMENT
• MRI: QUANTITATIVE ANALYSIS OF NEOAORTIC REGURGITAION,TR, LV
SIZE
• Dillman JR, Dorfman AL, Attili AK, Agarwal PP, Bell A, Mueller GC, et al. Cardiovascular magnetic resonance
imaging of hypoplastic left heart syndrome in children. Pediatr Radiol 2010;40:261-74.
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34. NATURAL HISTORY
• NEW ENGLAND REGIONAL INFANT CARDIAC RROGRAMME: 4TH MC CHD (7.5%)
• Fyler DC. Report of the New England Regional Infant Cardiac Program. Pediatrics 1980;65:375.
• 70%: BOYS
• Barber G, Chin AJ, Murphy JD, Pigott JD, Norwood WI. Hypoplastic left heart syndrome: lack of correlation between preoperative demographic and laboratory findings and survival following
palliative surgery. Pediatr Cardiol 1989;10:129.
• SEVERE HT FAILURE: 1ST WOL
• MANY DIEWITHIN 1-2WEEKS
• 40%: SURVIVE NEONATAL PERIOD
• >6WEEKS: SURVIVAL UNCOMMON
• Brockman JL. Congenital mitral atresia. Am Heart 1950;40:301.
• Lambert EC, Canent RV, Hohn AR. Congenital cardiac anomaliesin the newborn. A review of conditions causing death or severe distress in the first month of life. Pediatrics 1966;37:343.
• Redo SF, Engle MA, Ehlers KH, Farnsworth PB. Palliative surgery for mitral atresia. Arch Surg 1967;95:717. 34

35. NATURAL HISTORY
• 25% CARDIAC DEATHS DURING 1ST
WEEK
• 15% CARDIAC DEATHS IN 1ST MONTH
• DUCTAL CLOSURE:
• RESTRICTION OF SYSTEMIC
PERFUSION
• METABOLIC ACIDOSIS
• CIRCULATORY COLLAPSE
• DEATH
• IF DUCTUS PATENT:
• PROGRESSIVE INCREASE IN PBF
AND SUBSEQUENT DECREASE IN
SYSTEMIC CIRCULATION
• PULMONARY EDEMA
• CORONARY HYPOPERFUSION
• SYSTEMIC HYPOTENSION
• DEATH
• RARELY: LONGTERM SURVIVAL: PDA
PATENT+ PVR FAILSTO FALL IN
NEONATAL PERIOD
35

36. SURVIVAL OF NEONATES
NOTREATMENT OPTIMAL MEDICALTREATMENT
36

37. TECHNIQUESOF OPERATION
• RECONSTRUCTION
• NORWOOD AND ITSVARIANTS
• HYBRID PROCEDURE
• CARDIACTRANSPLANTATION
• RECENT SURVEY (2007): 56 INSTITUTES
• 86% : RECOMMEND NORWOOD/VARIENTS
• 14%: NO RECOMMENDATION: LEFTTO PARENTS
• Wernovsky G, Ghanayem N, Ohye RG, Bacha EA, Jacobs JP, Gaynor JW, et al. Hypoplastic left heart syndrome: consensus and controversies in 2007. Cardiol Young 2007;17 Suppl
2:75-86.
37

38. RECONSTRUCTIVE SURGERY
• ALL DEFINITIVE REPAIR ARE PALLIATIVE
• PRINCIPALS OF INITIAL SURGICAL MANAGEMENT:
1. ESTABLISHMENT OF A COMPLETELY UNOBSTRUCTED
SYSTEMIC ARTERIAL PATHWAY FROM RVTO ALL ORGANS
2. A RESTRICTIVE CONNECTION B/W SYSTEMIC & PULMONARY
CIRCULATIONS SOTHAT Qp & Qs ARE ADEQUATELY BALANCED
3. UNOBSTRUCTED FLOW OF PV RETURN ACROSS ATRIAL SEPUM
TO RA
38

39. LETS HEAR DR.
NORWOOD’S STORY
39

40. THE ORIGINAL NORWOOD’S STORY
• FTNVD, 3.7 KG, 24HOL:TACHYPNEA+ MURMUR
• ECHO: LV NOTVISUALISED, PROSTIN STARTED,
TRANSFERRED TO BOSTON MEDICAL CENTER
• MILD CYANOSIS, MODERATE RD, 37.7 DEG C, HR-132,
RR-88/MIN, BP: 74/40 (UL) 80/35 (LL)
• HYPERACTIVE RV IMPULSE, S1-N,S2- SINGLE+
ACCENTUATED, GR 3 LONG MID SYSTOLIC MURMUR
AT LSB, LIVER-4CM
• CXR: MODERATED CARDIOMEGALY, INCREASED PBF
• ECG: 150, SINUS RHYTHM, RAD (150DEG), RVH, NO LV
FORCES
• NORWOOD ET AL: PHYSIOLOGIC REPAIR OF AORTIC ATRESIA: HLHS: MEDICAL INTELLIGENCE: 1983 40

41. THE ORIGINAL NORWOOD’S STORY
• ECHO: AO ATRESIA, HYPOPLASTIC ASCENDING AORTA, MA,LV ABSENCE, OP-ASD
• CARDIAC CATH: HYPOPLASTIC ASC AORTA (≤2 MM), NONRESTRICTIVE IA
COMMUNICATUION, NON RESTRICTIVE PDA
• D3 OL: OPERATED
• CPB: PDA FOR ARTERIAL INFUSION & RA APPENDAGE FORVENOUS RETURN
• BODY COOLEDTO 20 DEG C
• BRANCHVESSELS AORTA OCCLUDED
• CIRCULATION ARRESTED
• NORWOOD ET AL: PHYSIOLOGIC REPAIR OF AORTIC ATRESIA: HLHS: MEDICAL INTELLIGENCE: 1983
41

42. THE NORWOOD PROCEDURE
42
NORWOOD ET AL: PHYSIOLOGIC REPAIR OF AORTIC ATRESIA: HLHS:
MEDICAL INTELLIGENCE: 1983

43. THE ORIGINAL NORWOOD STORY
• SATS IMPROVED: 85%, D/S-18TH POD
• 7 MONTHS OGF AGE: CARDIAC CATH: NORMAL PVWP, NO GRADIENT FROM RV DES
AORTA, SATS: 75%
• 16MONTHSOF AGE: CYANOSIS INCREASED+ DEC EXERCISETOLERANCE
• CARDIAC CATH: SAME FINDINGS
• 2ND STAGE REPARATIVE SX: PULMONARY + SYSTEMIC SIRCULATIONS SEPARATED BY
PARTIONINGATRIA, SO LA ASSOCIATEDWITHTV, RA ANASTOMOSEDWITH BPAs
+SHUNT REMOVED
• EXTUBATED: 36 HOURS
• D14: CARDIAC CATH: RESULTSCOMPARABLEWITH PATIENTSWITH GOOD LONGTERM
PROGNOSISWITH FONTAN
• D/S: D21 OF SX
• NORWOOD ET AL: PHYSIOLOGIC REPAIR OF AORTIC ATRESIA: HLHS: MEDICAL INTELLIGENCE: 1983
43

44. 2ND STAGE SURGERY: NORWOOD
44
NORWOOD ET AL: PHYSIOLOGIC REPAIR OF AORTIC ATRESIA: HLHS: MEDICAL INTELLIGENCE: 1983

45. NORWOOD: FINDINGSAT CARDIAC
CATHETERISATION
45
NORWOOD ET AL: PHYSIOLOGIC REPAIR OF AORTIC ATRESIA: HLHS: MEDICAL INTELLIGENCE: 1983

46. PRE OPERATIVE
MANAGEMENT
46

47. PRE OP MANAGEMENT
• 1. PGE1
• 2. RESPIRATORY SUPPORT & INSPIRATORY GASES
• 3.VASOACTIVE MEDICATIONS
• 4. OTHER ADJUNCTIVETHERAPIES
47

48. PGE1
• PHYSIOLOGICAL DUCTALCLOSURE:
• 20%: D1OL
• >80%:D2OL
• NEARLYALL: D4OL
• MAINTAINING PATENCYVITAL HERE
• INITIATE PROSTIN IMMEDIATELYON SUSPITION/ DIAGNOSIS
• BABIESWITH SHOCK/ SUSPECTED PDA CLOSURE/ RESTRICTIVE DUCT: 5-100NG/KG/MIN
• S/Es: HYPOTENSION, RESPIRATORY DEPRESSION
• CAFFINE/ AMINOPHYLLIN: DEC INCIDENCEOF APNEA
• PATIENTSWITH AMINOPHYLLIN INFUSION: DIDN’T REQUIRE INTUBATION IN COMPARITIONOF
PLACEBO (35%)
• Lim DS, Kulik TJ, Kim DW, Charpie JR, Crowley DC, Maher KO. Aminophylline for the prevention of apnea during prostaglandin E1 infusion. Pediatrics. 2003;112(1 Pt 1):e27–e29.
48

49. RESPIRATORY SUPPORT & INSPIRATORYGASES
• PRINCIPAL 1: EXCESS O2 PULMONARY VASODILATOR:TO BE AVOIDED
• PRINCIPAL 2:TO INCREASE PVR + REDUCE Qp/Qs
• CONTROLLED PPV, AVOID HYPERVENTILATION, USE PEEP
• INSPIRED CO2: LOWERS Ph+ INCREASES PVR INCREASES SYSTEMIC BLOOD FLOW
• INDUCTION OF HYPOXIATO INCREASES Qp/Qs: N2+O2 (FiO2: 14-20%)
49

50. RESPIRATORY SUPPORT &
INSPIRATORYGASES
• Preoperative neonates with HLHS who were anesthetized and under
neuromuscular blockade were ventilated with a hypoxic gas mixture (fiO2 of
0.17) and with supplemental CO2(fiCO2 of 0.03). Although both strategies
were successful in acutely reducing SaO2 and Qp/Qs, only hypercarbia
improved systemic oxygen delivery. Furthermore, whereas hypercarbia
improved cerebral oxygenation, hypoxia provided no benefit to cerebral
saturation.
• TabbuttS, Ramamoorthy C, Montenegro LM, et al. Impact of inspired gas mixtures on preoperative infants with hypoplastic left heart syndrome during controlled
ventilation. Circulation. 2001;104(12 Suppl 1):159–164.
• Ramamoorthy C,TabbuttS, KurthCD, et al. Effects of inspired hypoxic and hypercapnic gas mixtures on cerebral oxygen saturation in neonates with univentricular heart
defects. Anesthesiology. 2002;96(2):283–288.
50

51. VASOACTIVE MEDICATIONS
• INDICATION IN CARDIOGENIC SHOCK, REDUCED RV FUNCTION
• HIGHER DOSES: INCREASES SVR, INCREASED Qp/Qs
• THOSEWITH HIGH Qp/Qs + COMPROMISED SYSTEMIC PERFUSION
INODILATORTHERAPY WITH MILRINONE: USEWITH CAUTION: MAY REDUCE
PVR INCREASE IN Qp/Qs, EXCESSIVE HYPOTENSION
51

52. OTHERADJUNCTIVETHERAPIES
• BENEFITS FROM INCREASED O2 CARRYING CAPACITY INCREASE PCV
• PARENTRAL NUTRITION AND FLUID MANAGEMENT
• DIURETICS: RESPIRATORY INSUFFICIENCY/ INTERSITIAL OEDEMA
52

53. PRE OP MANAGEMENT IN A NUTSHELL
• BALANCE PULMONARY TO SYSTEMIC FLOW
• OPTIMISE SYSTEMIC PERFUSION
• PRESERVR ORGAN FUNCTION
53

54. STAGED PALLIATION
54

55. STAGED PALLIATION
• STAGE 1: 3 APPROACHES
• STAGE 2: SUPERIOR CAVO-PULMONARY CONNECTION
• STAGE 3: FONTAN COMPLETION
55

56. STAGE 1 PALLIATION
• GOALS:
• 1. RELIEF OF DUCTAL DEPENDENT
SYSTEMIC FLOW
• 2. PROVISION OF UNRESTRICTED
CORONARY ARTERY FLOW
• 3. NON RESTRICTIVE ASD TO
PREVENT PULVENOUS HT
• 4. PROVISION OF RELIABLE BUT
RESTRICTED SOURCE OF PBF
•NORWOOD’SWITH
MODIFIED BTT SHUNT
•NORWOOD’SWITH
SANO MODIFICATION
•HYBRID PROCEDURE
56

57. STAGE 1 PALLIATION
57

58. STAGE 1 PALLIATION
• CPB, DEEP HYPOTHERMIA,ALTERED PERFUSION(CIRCULATORYARREST/ REGIONAL
PERFUSION)
• AIM: CREATIONOF A STABLEANATOMYTHAT PERMITSGROWTH+ MATURATION OF
PULMONARYVASCULATURE , SOTHAT SUBSEQUENT SV PALLIATION CAN BE
ACCOMODATED
• NEEDED: LOW INCIDENCEOF RECURRENT/ RESIDUAL LESIONSA SOURCEOF
INTERSTAGE MORTALITY
• SMALLERASCENDINGAORTIC SIZE+AORTIC ATRESIA: RISK FACTOR FOR
MORTALITY INDICATESTHAT CORONARY INSUFFICIENCY ISA CAUSE OF DEATH
FOLLOWING ST 1 PALLIATION
• Burkhart HM,Ashburn DA, Konstantinov IE, et al. Interdigitating arch reconstruction eliminates recurrent coarctation after the norwood
procedure. JThorac Cardiovasc Surg. 2005;130(1):61–65.
• Bartram U, Grunenfelder J,VanPraagh R. Causes of death after the modified norwood procedure: a study of 122 postmortem cases. Ann
Thorac Surg. 1997;64(6):1795–1802.
58

59. STAGE 1 PALLIATION
• HENCE, STRATEGIESTARGETING CREATION OF A LARGE ASCENDING AORTA
TO PULMONARY ROOT ANASTOMOSIS LIKELYTO RESULT IN IMPROVED
OUTCOMES
• ARCH RECONSTRUCTION STRATEGIESTHAT INCLUDE COARCTECTOMY
LOWER INCIDENCE OF LATE ARCH OBSTRUCTION
• Burkhart HM, Ashburn DA, Konstantinov IE, et al. Interdigitating arch reconstruction eliminates recurrent coarctation after the norwood procedure. J
Thorac Cardiovasc Surg. 2005;130(1):61–65.
• Bartram U,Grunenfelder J,VanPraagh R. Causes of death after the modified norwood procedure: a study of 122 postmortem cases. AnnThorac Surg.
1997;64(6):1795–1802.
• MahleWT, SprayTL, Gaynor JW, Clark BJ 3rd. Unexpected death after reconstructive surgery for hypoplastic left heart syndrome. AnnThorac Surg.
2001;71(1):61–65.
• Forbess JM, Cook N, Roth SJ, SerrafA, Mayer JE Jr, Jonas RA.Ten-year institutional experience with palliative surgery for hypoplastic left heart
syndrome. Risk factors related to stage I mortality. Circulation. 1995;92(9 Suppl):262–266.
59

60. STAGE 1 PALLATION: MODIFIED BTT SHUNT
LIMITATIONS
• Qp/Qs MISMATCH
• DIASTOLIC AORTIC RUNOFFTO PULMONARY CIRCULATION RISK AORTO-
CORONARY FLOW IMPAIRMENT
• COMPETITION B/W CEREBRAL + PULMONARY CIRCULATION IF FROM
INNOMINATE ARTERY
• SUSCEPTIBLETO OCCLUTIONTHROMBOSIS/THROMBOEMBOLISM
60

61. ST 1 PALLIATION: RV PA CONDUIT
• ADVANTAGES:
• ELIMINATION OF DIASTOLIC
RUNOFF
• INCREASED DIASTOLIC PRESSURE+
IMPROVED CORONARY PERFUSION
• Mair R,Tulzer G, Sames E, et al. Right ventricular to pulmonary artery
conduit instead of modified blalock-taussig shunt improves postoperative
hemodynamics in newborns after the norwood operation. JThorac
Cardiovasc Surg. 2003;126(5):1378–1384.
• Pizarro C, Malec E, Maher KO, et al. Right ventricle to pulmonary artery
conduit improves outcome after stage I norwood for hypoplastic left heart
syndrome. Circulation. 2003;108(Suppl 1):155–160.
• Sano S, Ishino K, Kado H, et al. Outcome of right ventricle-to-pulmonary
artery shunt in first-stage palliation of hypoplastic left heart syndrome: a
multi-institutional study. AnnThorac Surg. 2004;78(6):1951–1958.
• Ohye RG, Ludomirsky A, Devaney EJ, Bove EL.Comparison of right
ventricle to pulmonary artery conduit and modified blalock-taussig shunt
hemodynamics after the norwood operation. AnnThorac Surg.
2004;78(3):1090–1093.
• DISADVANTAGES:
• NEGATIVE EFFECTON RV FUNCTION
• VENTRICULARARRTHYMIAS
• IMPAIRED PAGROWTH
• NEED FOR AN EARLIER ST 2 PROCEDURE
• Mahle WT, Cuadrado AR, Tam VK. Early experience with a modified norwood procedure
using right ventricle to pulmonary artery conduit. Ann Thorac Surg. 2003;76(4):1084–1088.
• Pizarro C, Mroczek T, Malec E, Norwood WI. Right ventricle to pulmonary artery conduit
reduces interim mortality after stage 1 norwood for hypoplastic left heart syndrome. Ann
Thorac Surg. 2004;78(6):1959–1963.
• Ghanayem NS, Jaquiss RD, Cava JR, et al. Right ventricle–to–pulmonary artery conduit
versus Blalock-Taussig shunt: a hemodynamic comparison. Ann Thorac Surg.
2006;82(5):1603–1610.
61

62. ST 1 PALLIATION: RV PA CONDUIT
• PEDIATRIC HEART NETWORK- SINGLEVENTRICLE
RECONSTRUCTION TRIAL:
• RCT, 549 PATIENTS
• TRANSPLANTATION FREE SURVIVAL 12 MONTHS AFTER
RANDOMISATION WAS HIGHER WITH RVPA CONDUITTHAN MBT
SHUNT (74%VS 64%, P=0.01)
• AFTER 12 MONTHS: NO SURVIVAL ADVANTAGE TO RVPA CONDUIT,
RVPA SHUNT GROUP HAD MORE UNINTENDED INTERVENTIONS
(P=0.003) AND COMPLICATIONS (P=0.002)
62

63. PHN-SVRTRIAL
• BENEFIT OF RVPA CONDUIT ENTIRELY AMONG TERM NEONATES WITH
AORTIC ATRESIA HIGHER DIASTOLIC PRESSUREWITH IMPROVED
CORONARY BLOOD FLOW  IS OF BENIFIT AMONG PATIENTS WITH
SMALLEST ASCENDING AORTA
• FOLLOW UP SHOWS NO LONGTERM BENEFITS OVER MBT SHUNT
• Tweddell JS, Sleeper LA, Ohye RG, et al.. Intermediate-term mortality and cardiac transplantation in infants with single-ventricle lesions: risk factors and their
interaction with shunt type. J Thorac Cardiovasc Surg. 2012;144(1):152–159.e2.
• Newburger JW, Sleeper LA, Frommelt PC, et al.. Transplant-free survival and interventions at 3 years in the single ventricle reconstruction trial. Circulation.
2014;129(20):2013–2020.
63

64. ST 1 PALLIATION: HYBRID PROCEDURE
• SURGICAL BPA BANDING+ DUCT STENTING+ BALLOON SEPTOSTOMY
• AVOIDED: CBP+ DEEP HYPOTHERMIA
• ST 2 AORTIC ARCH RECONSTRUCTION+ BDG
• SHORTCOMING: RETROGRADE ARCH OBSTRUCTION CEREBRAL+
CORONARY ISCHEMIA (HIGHEST RISK ATRETIC AORTICVALVE)
• Bacha EA, Daves S, Hardin J, et al. Single-ventricle palliation for high-risk neonates: the emergence of an alternative hybrid stage I
strategy. JThorac Cardiovasc Surg. 2006;131(1):163–171.
• Akintuerk H, Michel-Behnke I,Valeske K, et al. Stenting of the arterial duct and banding of the pulmonary arteries: basis for combined
norwood stage I and II repair in hypoplastic left heart. Circulation. 2002;105(9):1099–1103.
• Galantowicz M, Cheatham JP. Lessons learned from the development of a new hybrid strategy for the management of hypoplastic left heart
syndrome. Pediatr Cardiol. 2005;26(3):190–199.
64

65. INTER STAGE
MANAGEMENT
65

66. GOALS OF INTERSTAGE MANAGEMENT
• PHARMACOLOGICAL THERAPYTARGETED AT OPTIMISING INEFFICIENT
PARALLEL CIRCULATION INHERENTTO HLHS AFTER ST 1 PALLIATION
• VIGILANT MONITORING OF PHYSIOLOGICVARIANCES TO IDENTIFY
DESTABILISING PATHOLOGY
• ADEQUETE NUTRITION+ SOMATIC GROWTH
66

67. MEDICALTHERAPY
• 1. CHRONIC AFTERLOAD REDUCTION:
• ACE –s: BEFIFICIAL IF:
• Qp/Qs>2,
• MODERATETO SEVERE AVVR,
• CHF,
• INCREASED SVR
• IF SVR SUBOPTIMALLY CONTROLLED: CLONIDINE
• AFTERLOAD REDUCTIONTITRATED WITH CAUTIONTO AVOID
WORSENING HYPOXIA FROM EXCESSIVE LOWERING OF Qp/QS
+DIASTOLIC HYPOTENSION IMPARED CORONARY FLOW
67

68. MEDICALTHERAPY
• 2. CHRONIC DIURETICTHERAPY:
• PULMONARY OVERCIRCULATION
• CHF
• AVOID IVVOLUME DEPLITION DEC CO, INCREASE RISK OF SHUNT
THROMBOSIS
• 3. ANTIPLATELETTHERAPY:
• ASPIRIN: 5MG/KG/DAY
• 4. O2:TO MAINTAIN SATS> 78%
68

69. RISK FACTORS FOR INTERSTAGE DEATH
• LIMITED CIRCULATORY RESERVE INHERENT IN AVOLUME LOADED SVWITH
PARALLEL CIRCULATION+ CYANOSIS PLACES AT RISK OF LATE MORBIDITY +
MORTALITY
• PHN-SVRTRIAL:TIME B/W ST1+ST 2 12% MORTALITY (MBT-18%, RVPA
SHUNT-6%)
• Ohye RG, Sleeper LA, Mahony L, et al. Comparison of shunt types in the norwood procedure for single-ventricle lesions. N Engl J Med.
2010;362(21):1980–1992.
• Newburger JW, Sleeper LA, Frommelt PC, et al..Transplant-free survival and interventions at 3 years in the single ventricle reconstruction
trial. Circulation. 2014;129(20):2013–2020.
• Ghanayem NS,Allen KR,TabbuttS, et al. Interstage mortality after the norwood procedure: results of the multicenter single ventricle reconstruction
trial. JThorac Cardiovasc Surg. 2012;144(4):896–906.
69

70. RISK FACTORS FOR INTERSTAGE DEATH
• 1. ANATOMIC DIAGNOSIS+ RESIDUAL/
RECURRENT LESIONS:
• AORTIC ATRESIA + DIMINUTIVE
ASCENDING AORTA: LOWEST
PHYSIOLOGIC RESERVE, INCREASED RISK
OF LATE DEATH
• PHN-SVRTRIAL: AATHE ONLY
ANATOMIC FINDING TO BE ASSOCIATED
WITH INTERSTAGE DEATH
• Simsic JM, Bradley SM, Stroud MR, Atz AM. Risk factors for interstage death
after the norwood procedure. Pediatr Cardiol. 2005;26(4):400–403.
• Hehir DA, DominguezTE, Ballweg JA, et al. Risk factors for interstage death
after stage 1 reconstruction of hypoplastic left heart syndrome and
variants. JThorac Cardiovasc Surg. 2008;136(1):94–99; 99.e1–e3.
• 2. RESTRICTIVE ASD
• 3. ARCH OBSTRUCTION
• 4. OBSTRUCTED SHUNT FLOW
• 5. PA DISTORTION
• 6. AVV INSUFFICIENCY
• 7. ARRTHYMIAS
• 8. COMMONLY ACQUIRED CHILDHOOD GI+
RESPIRATORY ILLNESSES
70

71. CARDIAC
TRANSPLANTATION
71

72. PRIMARYTRANSPLANTATION
• BAILEY : NEONATAL TRANSPLANTATIONS
• 1985-1996: 176 INFANTS LISTED 19% DIED PRIORTO DONOR
IDENTIFICATION
• 142 PATIENTS:TRANSPLANTED 1.5HRS- 6 MTS (MEDIAN: 29 DAYS)
• ACTURIAL SURVIVAL:
72
1 MONTH 91%
1YR 84%
5YRS 76%
7YRS 70%

73. PRIMARYTRANSPLANTATION
• FOLLOW UP:
• GOODGROWTH+ DEVELOPMENT
• NEURODEVELOPMENTAL DELAY: 11%
• NORMAL PSYCHOMOTOR DEVEKOPMENT: 91%
• NORMAL DEVELOPMENTAL INDEX: 96%
• DONOR SHORTAGE: 25-30% MORTALITY
• WEST: ABO INCOMPATIBLE TRANSPLANTS: DECREASE IN MORTALITY
• West LJ, Pollock-Barziv SM, Dipchand AI, et al. ABO-incompatible heart transplantation in infants. N Engl J Med. 2001;344(11):793–800.
• West LJ, Pollock-Barziv SM, Lee KJ, Dipchand AI, Coles JG, Ruiz P. Graft accommodation in infant recipients of ABO-incompatible heart
transplants: donor ABH antigen expression in graft biopsies. J Heart LungTransplant. 2001;20(2):222.
73

74. TAKE HOME MESSAGE
• Challenges remaining:
• Options for failing circulation
• Optimizing long-term neurodevelopmental outcome
• Justifying allocation of increasingly scarce health care resources to a complex group of
patient
• . Short-term goals:
• Identification of causes of HLHS to decrease incidence
• Improvements in fetal intervention to improve outcome for those born with HLHS
• Improved medical, mechanical, and transplant strategies for treatment of failing
circulation to improve survival and QOL of affected individuals
74

75. 75