Pediatric Cardiac Anesthesia 2: Complex Shunts
Transcript of Pediatric Cardiac Anesthesia 2: Complex Shunts
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Intensive Review of Pediatric Anesthesia 2015
Pediatric Cardiac Anesthesia 2: Complex Shunts
Susan R Staudt, MD, MSEd With co-authorship attribution to
Dean Andropoulos, MD & Emad B Mossad, MD
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Intensive Review of Pediatric Anesthesia 2015
Learning Objectives
• Follows/builds on simple shunts (Dr. Mossad) • Review complex shunts and mixing lesions: Truncus
Arteriosus, d-TGA, TAPVR • Discuss Single ventricle lesion types • Refresh knowledge of anatomy and physiology of the
3 stages of single ventricle palliation • Examine remaining miscellaneous cardiac and
extracardiac congenital heart defects • NOTE: NO DISCLOSURES or COI
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Intensive Review of Pediatric Anesthesia 2015
Incidence/Prevalence of Congenital Heart Disease
• 8 per 1000 infants in the U.S. are born with CHD – Most common birth defect requiring treatment – 32,000 neonates annually; 8-9000 surgeries
• Survival now >95% for CHD surgery – ≈30,000 CHD surgeries annually in US: 25,000 <18yrs; 5,000 >18 yrs
• ≈ 1 million children with CHD • > 1 million adults with CHD—1 in 150
– 55% simple lesions: ASD, VSD most common – 30% moderately complex: TOF most common – 15% complex lesions: TGA, single ventricle lesions
Circulation 2013;127:e153 4
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Intensive Review of Pediatric Anesthesia 2015
Incidence of Common CHDz Lesions in Children • Ventricular septal defect: 20.1% • Atrial septal defect: 16.8% • Valvar pulmonic stenosis: 12.6% • Patent ductus arteriosus: 12.4% • Tetralogy of fallot: 7% • Coarctation of aorta: 6.8% • Valvar aortic stenosis: 5.5% • Atrioventricular septal defects: 3.9% • Transposition of the great vessels: 3.6% • Other (11.3% )
Circulation 2012;125:e97 5
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Intensive Review of Pediatric Anesthesia 2015
VSD 15.4 15.7 31 5
AoCoA 10.5 7.5 5.6 11
Transposition (TGA) 10.4 9.9 4.5 2
Tetralogy of Fallot 9.9 8.9 5.5 3
Patent Ductus Arteriosus 6.7 6.1 7.1 -
AV canal 3.9 5.0 4.4 17.5
ASD 0.5 3.0 7.5 -
Hypoplastic Left Heart (HLHS) 3.7 7.4 3.1 16
Single ventricle, other >10% 4.3 2.4 1.5 2
Tricuspid atresia (HRHS) 4.7 2.6 2.4 4
Pulmonary Stenosis 3.0 3.3 7.0 5
Truncus Arteriosus 2.1 1.4 1.4 1.5
Pulmonary Atresia 1.9 3.1 1 -
TAPVC (total anomalous pulm vein connection) 3.6 2.6 1.4 1
Double outlet Rt Venticle 3.0 1.5 1.2 3
Ebstein’s Anomaly 0 1 1 7
4 Recent Reports Relative Incidence CHDz lesions presenting for surgical repair…
Adapted from Table 16-3 COTE et al.
Conclusions: Most common Surgically repaired lesions: VSD+CoA+TGA+TOF+ HLHS/SV +PDA = > 63% lesions (include asd and av canal =75%)
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Intensive Review of Pediatric Anesthesia 2015
Truncus Arteriosus • Major types are I,II,III and depend on
location/degree of PA branching • Common arterial trunk results in systemic,
pulmonary, and coronary circulations in parallel
• Lowering PVR with excessive FiO2 and hyperventilation creates systemic/coronary steal and myocardial ischemia
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Intensive Review of Pediatric Anesthesia 2015
Andropoulos and Gottlieb; Congenital Heart Disease,
Anesthesia and Uncommon Diseases, 6th Ed., Fleisher L., (ed.) 2012, p. 101
Truncus Arteriosus
8
Type I Type II
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Intensive Review of Pediatric Anesthesia 2015
Truncus Arteriosus
9 Andropoulos and Gottlieb; Congenital Heart Disease,
Anesthesia and Uncommon Diseases, 6th Ed., Fleisher L., (ed.) 2012, p. 101
Type III Type IV
REPAIR: VSD Closure-- place RV-PA valved conduit and if needed build PA confluence and PA-plasty (type 4 worst outcome)
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Intensive Review of Pediatric Anesthesia 2015
Anomalous Pulmonary Venous Return • Major types are partial (PAPVR) and total (TAPVR) • PAPVR usually has mild symptoms of small left-to-
right shunt and sinus venosus ASD • TAPVR symptomatology depends on degree of
obstruction to pulmonary venous return • Infradiaphragmatic TAPVR prone to severe
obstruction, hypoxia, respiratory failure – Increasing PBF with high FiO2 and iNO may
paradoxically worsen obstruction before repair 10
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Intensive Review of Pediatric Anesthesia 2015
Total Anomalous Pulmonary Venous Return
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Supracardiac Cardiac Andropoulos and Gottlieb; Congenital Heart Disease,
Anesthesia and Uncommon Diseases, 6th Ed., Fleisher L.,
(ed.) 2012, p. 103
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Intensive Review of Pediatric Anesthesia 2015
Total Anomalous Pulmonary Venous Return
12 Andropoulos and Gottlieb; Congenital Heart Disease,
Anesthesia and Uncommon Diseases, 6th Ed., Fleisher L.,
(ed.) 2012, p. 103
Infracardiac Mixed
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Intensive Review of Pediatric Anesthesia 2015
Left-to-Right Shunt Lesions Left-Sided Obstructive Lesions
Right Sided Obstructive Lesions Transposition of the Great Arteries
Single Ventricle Lesions Miscellaneous
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Intensive Review of Pediatric Anesthesia 2015
D-Transposition of the Great Arteries
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Andropoulos and Gottlieb; Congenital Heart Disease,
Anesthesia and Uncommon Diseases, 6th Ed., Fleisher L., (ed.) 2012, p. 116
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Intensive Review of Pediatric Anesthesia 2015
D-Transposition of the Great Arteries
• Aorta arises from right ventricle, pulmonary artery from left ventricle , resulting in parallel circulation with “transposition physiology”
• Oxygenation depends on mixing at the atrial level (most important), PDA, or VSD (15-25%)
• Balloon atrial septostomy is often necessary in the neonatal period
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Intensive Review of Pediatric Anesthesia 2015
Arterial Switch for D-TGA • Procedure of choice since mid 1980’s • Performed as neonate; CPB without DHCA • 15-25% also have VSD • Beware coronary problems after repair
– Global myocardial dysfunction – ST segment changes – Segmental wall motion abnormalities
• LV can be deconditioned – Intolerant of high preload or afterload – LAP 4-6, systolic BP 60s often desirable
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Intensive Review of Pediatric Anesthesia 2015
Arterial Switch Operation
17 Andropoulos and Gottlieb; Congenital Heart Disease,
Anesthesia and Uncommon Diseases, 6th Ed., Fleisher L., (ed.) 2012, p. 116
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Intensive Review of Pediatric Anesthesia 2015
Congenitally Corrected Transposition of the Great Arteries (L-TGA)
• Ventricles are inverted, aorta arises from LV and PA from LV
• RA empties to LV, and LA to RV • Other associated anomalies include VSD and
pulmonic stenosis • Complete atrioventricular block is common in
cc-TGA (2% per year) • Right (systemic) ventricle usually fails over time
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Intensive Review of Pediatric Anesthesia 2015
Congenitally Corrected Transposition of the Great Arteries (L-TGA)
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Andropoulos and Gottlieb; Congenital Heart Disease,
Anesthesia and Uncommon Diseases, 6th Ed., Fleisher L.,
(ed.) 2012, p. 117
Mitral valve
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Intensive Review of Pediatric Anesthesia 2015
Double Outlet Right Ventricle
• VSD with both aorta and pulmonary artery arising completely or partially from RV
• Pathophysiology depends on degree of RVOTO • Cyanosis and TOF physiology are seen with
significant RVOTO • Acyanosis with CHF can be seen with no
RVOTO; in this case a VSD patch may be only needed surgery
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Intensive Review of Pediatric Anesthesia 2015
Spectrum of DORV – Taussig Bing
REPAIR: Rastelli procedure-valved RV to PA homogaft, as patching of VSD precludes native PA from use (Also this is used in TGA with PS)
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Intensive Review of Pediatric Anesthesia 2015
Left-to-Right Shunt Lesions Left-Sided Obstructive Lesions
Right Sided Obstructive Lesions Transposition of the Great Arteries
Single Ventricle Lesions Miscellaneous
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Intensive Review of Pediatric Anesthesia 2015
Notes on “single ventricle lesions” • Broad Spectrum of pathology- hence a not uncommon
“type”-40%-50% of all cyanotic congenital heart disease is treated via this path!
• Classic HRHS: (tricuspid atresia, stenosis) • Classic HLHS :(mitral and/or aortic atresia, stenosis) • In addition are other lesions precluding a 2 ventricle repair:
PA/IVS, severe Ebsteins anomaly, unbalanced AV canals, double inlet LV, various “mixed lesions”
• Prognosis is better with: HRHS, more than 1 mass of ventricle, full term, no associated anomalies, absence of lung or airways disease
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Intensive Review of Pediatric Anesthesia 2015
Single Ventricle Notes -2
Norwood /Sano/ stage 1
palliation
Hemifontan /BDG Fontan
HLHS HRHS
Ventricle-PA conduit Create Aorta from PA Ligate PDA NO V WORK REDUCTION PT CYANOTIC
Connect SVC to PA (remove V-PA conduit) UNLOADS V WORK (partially) PT CYANOTIC
Connect IVC to PA RETURNS V to NL Workload PT CAN BECOME ACYANOTIC
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Intensive Review of Pediatric Anesthesia 2015
Tricuspid Atresia • Plate-like obstruction instead of tricuspid valve • Neonatal presentation depends on degree of
obstruction to pulmonary blood flow: – Severe: profound cyanosis when PDA closes – Mild/moderate: mild/moderate cyanosis, no CHF – None: mild or no cyanosis, CHF
• Systemic ventricle is the left ventricle – Good long term outcomes despite single ventricle
physiology
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Intensive Review of Pediatric Anesthesia 2015
Tricuspid Atresia
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Andropoulos and Gottlieb; Congenital Heart Disease,
Anesthesia and Uncommon Diseases, 6th Ed., Fleisher L., (ed.) 2012, p. 123
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Intensive Review of Pediatric Anesthesia 2015
Hypoplastic Left Heart Syndrome • 0.7% of CHD but one of most common neonatal
operations • Severe mitral and aortic stenosis or atresia • Very small or non-existent LV • Depends on PDA for systemic and coronary
circulation • Diagnosis: fetal echo; cyanosis at birth; acidosis,
murmur, poor peripheral perfusion • Formerly 100% fatal in first month; now 70-75%
survive long term 27
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Intensive Review of Pediatric Anesthesia 2015
Hypoplastic Left Heart Syndrome
Andropoulos and Gottlieb; Congenital Heart Disease,
Anesthesia and Uncommon Diseases, 6th Ed., Fleisher L.,
(ed.) 2012, p. 120 28
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Intensive Review of Pediatric Anesthesia 2015
Pathophysiology: Mixing Lesions
30
Andropoulos and Gottlieb; Congenital Heart Disease,
Anesthesia and Uncommon Diseases, 6th
Ed., Fleisher L., (ed.) 2012, p. 78
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Intensive Review of Pediatric Anesthesia 2015
Surgical Repairs: Norwood Stage I Palliation
• Done as neonate: PDA dependent on PGE1
• Delicate balance between systemic and pulmonary circulations – Often FiO2 0.21, PaCO2 40-50 mm Hg
• CPB with DHCA or RCP for aortic arch reconstruction
• Can also be done a hybrid procedure in cath lab – Sternotomy, PDA stent, PA banding, ASD stent
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Intensive Review of Pediatric Anesthesia 2015
Classic Norwood repair or stage 1 palliation
Aortic arch reconstructed from PA, pulm valve and homograft; 3.5 mm B-T shunt placed for pulm blood flow
Shunt can over or undercirculate; can thrombose or kink; runoff aorta to pa lowers diastolic pressure; least risk of distorting PA’s and no ventricular incision
SANO modification
Same aortic arch repair; larger 5.0 mm RV-PA conduit instead of BT shunt
Requires a ventriculotomy (scars ventricle); higher diastolic pressure; ? Less risk of thrombosis, kinking and early complications?
Hybrid procedure
Off CPB- PA band placed via sternotomy and pda is stented (cath)
No CPB required; arch reconstruction occurs at 2nd stage of repair-infant left with retrograde coronary perfusion down tiny aorta until 2nd stage
HLHS-Three Different Approaches to Stage One Palliation
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Intensive Review of Pediatric Anesthesia 2015
Surgical Repairs: Norwood Stage I Palliation
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“Classic” Norwood Sano Modification
NEJM 2010;362:1980
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Intensive Review of Pediatric Anesthesia 2015
Hybrid Approach for Stage I Palliation
34
Andropoulos and Gottlieb; Congenital Heart Disease,
Anesthesia and Uncommon Diseases, 6th Ed., Fleisher
L., (ed.) 2012, p. 121
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Intensive Review of Pediatric Anesthesia 2015
Bidirectional Cavopulmonary Connection (Glenn, Hemifontan)
• Stage II or first palliation for essentially all single ventricle patients
• Performed at age 2-6 months, following cath, echo possibly MRI to risk stratify
• CPB, often no crossclamp unless intracardiac or aortic repair
• After repair desire transpulmonary pressure gradient of ≈ 10 mm Hg with low atrial pressure
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Intensive Review of Pediatric Anesthesia 2015
Bidirectional Cavopulmonary Connection (Glenn)
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Intensive Review of Pediatric Anesthesia 2015
Cardio-Cerebro-Pulmonary Circulation & Glenn SVC anastomosis
37
Andropoulos and Chang. Chapter 20: Pediatric Cardiac Intensive Care. In, Allen H. et al (eds.) Moss & Adams’ Heart
Disease…, 8th ed. p. 514.
êPaCO2èêCBFè êSVC flowèêPBFè êSaO2, CO, and DO2
Systemic ventricle
(both PVR and cerebral autoregulation of SVC flow can impact Qp)
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Intensive Review of Pediatric Anesthesia 2015
Fontan Completion • Total cavopulmonary connection • Final palliation for any single ventricle
– Repair at 2-4 years, as the 2nd(HRHS) or 3rd (HLHS) stage • CPB with or without crossclamping • Fenestration allows “pop-off” for R-L shunting, preserving
C.O. with some arterial desaturation • CVP is driving force for C.O.: no right sided pulmonary
ventricle • Spontaneous Ventilation/Early tracheal extubation highly
desirable 38
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Intensive Review of Pediatric Anesthesia 2015
Fontan Completion
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Lateral Tunnel Extracardiac Andropoulos and Gottlieb;
Congenital Heart Disease, Anesthesia and Uncommon
Diseases, 6th Ed., Fleisher L., (ed.) 2012, p. 119
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Intensive Review of Pediatric Anesthesia 2015
Fontan Ten Commandments • Age > 4 years • Sinus Rhythm • Normal systemic venous return • Normal Right Atrial volume • Mean PAP <15 mmHg • PVR <4 Wood units/m2 • PA/Aorta ratio > 0.75 • LVEF (or SVEF)>0.6 • Competent AVV • Absent PA distortion
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Intensive Review of Pediatric Anesthesia 2015
Left-to-Right Shunt Lesions Left-Sided Obstructive Lesions
Right Sided Obstructive Lesions Transposition of the Great Arteries
Single Ventricle Lesions Miscellaneous Cardiac Lesions
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Intensive Review of Pediatric Anesthesia 2015
Anomalous Left Coronary Artery from Pulmonary Artery
• ALCAPA results in myocardial ischemia and infarction in infants as PVR declines and coronary flow to the LV reverses
• Dilated cardiomyopathy ensues; ALCAPA must be ruled out in infants with DCM
• Maintain elevated PVR with low FiO2 and hypercapnea in unrepaired ALCAPA – Elevate PA pressure, increase L coronary pressure,
minimize steal into PA 42
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Intensive Review of Pediatric Anesthesia 2015
Anomalous Left Coronary Artery from Pulmonary Artery
43
Andropoulos and Gottlieb; Congenital Heart Disease,
Anesthesia and Uncommon Diseases, 6th Ed., Fleisher L.,
(ed.) 2012, p. 124
Ao
PA
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Intensive Review of Pediatric Anesthesia 2015
Valvular Regurgitation • Aortic, mitral, pulmonic, or tricuspid regurgitation
can be isolated or component of more complex lesion
• Severe AI most problematic: low diastolic pressure causing coronary ischemia
• MR can lead to pulmonary hypertension if severe • PR and TR usually well tolerated • Hemodynamic goals: reduce afterload, maintain
preload, high-normal heart rate 44
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Intensive Review of Pediatric Anesthesia 2015
Valvular Regurgitation
45
Severe MR From Failure of Coaptation of MV Leaflets
Andropoulos and Gottlieb; Congenital Heart Disease, Anesthesia and Uncommon Diseases, 6th Ed., Fleisher L., (ed.) 2012, p. 126
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Intensive Review of Pediatric Anesthesia 2015
Pathophysiology: Regurgitant Lesions
46
Andropoulos and Gottlieb; Congenital Heart Disease,
Anesthesia and Uncommon Diseases, 6th Ed., Fleisher L.,
(ed.) 2012, p. 78
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Intensive Review of Pediatric Anesthesia 2015
Vascular Rings • Many variations; result from abnormal
regression of the developing aortic arches • Respiratory and/or GI symptoms
– Stridor, “wheezing”, dysphagia
• Double aortic arch (60%), right aortic arch with aberrant L subclavian artery are most common
• CT angio (preferred) or MRI for diagnosis 47
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Intensive Review of Pediatric Anesthesia 2015
Symptoms-Imaging-Monitoring
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Intensive Review of Pediatric Anesthesia 2015
Pericardial Effusion/Tamponade • Increases in intrapericardial pressure cause equalization
of diastolic pressures in all 4 cardiac chambers, resulting in tamponade physiology
• Distended neck veins, pulsus paradoxus, tachycardia, and low cardiac output result
• Induction of anesthesia with PPV can compromise ventricular filling and cause CV collapse
• Sedation, local anesthesia, needle pericardiocentesis; if GA keep SVR up; PPV destabilizing (? Avoid or ino/pressor “bridge”) -team ready to go ie tap then window
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Intensive Review of Pediatric Anesthesia 2015
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Intensive Review of Pediatric Anesthesia 2015
Echo imaging – Pulsus Paradoxus