Surgical Treatment of Congenital Heart Disease

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Surgical Treatment of Congenital Heart Disease
Alistair Phillips, MD Attending Surgeon, Columbus
Childrens Hospital, Heart Center September 6,
2007
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Congenital Heart Disease

• 35,000 babies born with heart defects
• 8 in a 1000 births have a structural defect in
  the heart
• 3000 babies born with tetralogy of Fallot
• 1,000,000 Americans have a congenital heart
  defect
• Surgery improved from 1970s to now
• Mortality 30 1970, 5 now
• Changing therapies and interventions, improving
  outcomes

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Broad Classification

• Cyanotic Heart Disease
• Acyanotic Heart Disease
• Shunts L ? R
• Obstructive lesions
• Valvular lesion
• Stenosis
• Regurgitation

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Cyanotic Lesions

• Hypoplastic Left Heart Syndrome
• Pulmonary Atresia
• Ebsteins Anomaly
• Terrible Ts

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Terrible Ts

• Transposition of the Great Arteries
• Tetralogy of Fallot
• Truncus Arteriosus
• Total Anomalous Pulmonary Venous Return
• Tricuspid Atresia

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Acyanotic Lesions

• Left to right shunts Excessive pulmonary blood
  flow
• ASD
• VSD
• PDA
• Coronary artery fistula
• Obstructive lesions
• Coarcation of the Aorta
• LVOT obstruction subaortic membrane,
  supravalvar obstruction
• Valvular heart disease

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Diagnosis

• Physical exam - type of murmur
• CXR
• Echo
• Cardiac catheterization

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TAPVR
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• Four chamber view
• Showing perimembranous VSD

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Surgical Goals

• Balance QpQs
• Unobstructed flow to pulmonary and systemic beds
• Try to have two ventricular repair
• Remove all shunts

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Cardiopulmonary Bypass

• Maintain circulatory support while the heart is
  arrested
• Cool patient decrease metabolic needs
• May use circulatory arrest, profound hypothermia
  to 18 C
• Can stop circulation for up to 60 minutes
• Arrest the heart with cardioplegia
• Hyperkalemic solution
• Blood
• Other additives

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CPB

• Collect Blue blood
• Oxygenate the blood
• Return the blood bypassing the heart and lungs
• Collect blood lost in the surgical field

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Cardiopulmonary Circuit
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Specific Diseases

• Patent Ductus Arteriousus
• Atrial Septal Defect
• Ventricular Septal Defect
• Atrioventricular Canal Defect Atrioventricular
  septal defect
• Transposition of the Great Arteries
• Tetralogy of Fallot
• Single Ventricle
• Valvular heart disease

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Ductus Arteriousus

• Patent Ductus Arteriousus
• One of three fetal shunts
• Closes usually w/i first few days, tissue
  responsive to oxygen tension

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Patent Ductus Arteriousus

• Patency increased with decreased gestational age
  and birth weight
• 30 overall gt 30 weeks
• 80, 28-30 weeks
• Ductus not as sensitive to O2
• More sensitive to prostaglandins
• Surgical intervention
• Respiratory distress
• Large PDA
• Failure of indomethacin x 2
• NEC
• Intracranial hemorrhage

Recurrent Laryngeal nerve
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Obstructive Lesions

• Coarctation of the Aorta
• Location Juxta ductal
• Bimodal presentation
• Infants
• Ductal dependent circulation
• Surgery
• Late Presentation
• UE hypertension
• Stent

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When to Operate for Acyanotic Shunts?

• QpQs gt 2, unrestrictive
• With continued shunt ultimately will develop
  pulmonary vascular disease
• ASD shunt dependent of Ventricluar compliance,
  operate 3-5 years of age
• VSD shunt dependent on pressure gradient, operate
  6 to 9 months, or earlier if symptomatic, failure
  to thrive

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ASD
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ASD- Anatomy/ Prevalence

• Secundum 75
• Primum 15
• Sinus Venosus 10
• Cor Sinus (rare)

Braunwaulds Heart Disease, 6th ed
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ASD - Clinical

• Majority repaired in childhood, but may present
  in adolescence/adulthood,
• Now closed by device, no surgery, ? LOS
• Asymptomatic
• murmur, abnormal ECG/CXR
• Symptomatic
• dyspnea/CHF
• CVA/emboli
• Atrial Fibrillation

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Secundum ASD
Septal Leaflet TV
Triangle of Koch
Tendon of Todaro
CS
ASD
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Sinus Venosus
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Ventricular Septal Defects

• Types
• Perimembranous
• Inlet
• Outlet (Subpulmonic, Doubly Committed,
  Subarterial)
• Muscular
• Pathophysiology Similar L to R shunt
• Differences Important
• Most spontaneously close
• Perimembranous close to conduction system
• Surgical Approach Perimembranous via TV, Apical
  via ventricularotmy, Subpulmonic via PA
• Outlet associated with insufficiency
• Interventional techniques being developed

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VSD

• Outlet
• Perimembranous
• Inlet
• Muscular

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Perimembranous VSD
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VSD
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Outlet VSD
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Atrioventricular Canal Defect

• Absence of endocardial cushion derivatives
• Lower atrial septum
• Inlet ventricular septum
• Medial mitral and tricuspid valve
• Primum ASD
• Inlet VSD
• Common AV Valve

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AV Canal
Rastelli classification (anatomy of anterior
bridging leaflet) A Common leaflet but cordae
attached to crest of septum B Papillary muscle
attached to left part of leaflet from right of
septum, very rare C Free floating common
anterior leaflet without cordal attachment to
ventricular crest All Tet- AV canals
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Atrioventricular Canal Defect
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AV Canal
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Repaired L AVV
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AV Canal

• 98 1-yr, 96 20-yr survival (Mayo)
• Reoperation in 18 (9) for L AV valve regurg.
• Hanley - 7 re-operation for Left A-V valve
  problems
• Downs syndrome no increased risk
• Age - controversial
• Date of operation - earlier worse

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Timing of Surgery

• Complete Concern pulmonary HTN
• i) Complete repair by 3-6 months
• ii) By 1yr for asymptomatic pts. (Boston 6
  months)
• TET-AV canal PAs small and protected, BT shunt
  allow PAs to grow and makes repair easer with
  larger heart, elective repair 1-2 years of age
• Partial (pulmonary HTN rare)
• i) Elective repair at 3-5 yrs (Boston by 1yr)
• ii) Significant Left A-V valve regurgitation
  occurs earlier

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Post-op Considerations

• 20 with AV regurgition post-op
• Benefit from ACE inhibition
• Early extubation, remove positive pressure
  ventilation
• Diuresis

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Management Cyanotic Lesions

• Ductal dependent lesion
• Start Prostaglandin E1 (PGE1)
• Initial dose 0.1 mg/kg/min, can titrate down
• Side-effects apnea, hypotension, edema, low
  grade fever
• Clinical deterioration if pulmonary venous
  obstruction present
• HLHS with restrictive/intact atrial septum
• TGA with intact ventricular septum and a
  restrictive/intact atrial septum
• TAPVR with obstruction

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Management

• Intervention to improve mixing or pulmonary blood
  flow
• Balloon atrial septostomy in D-TGA
• Pulmonary valvotomy
• RF to connect RVOT to PAs in pulmonary atresia

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Spiral Forming Great Arteries
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Transposition of the Great Arteries
Sinus 1 if observer sitting in non-facing
sinus, first sinus going counter-clockwise Sinus
2 second sinus going counter-clockwise
R
2
1
A
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D- TGA

• Survival
• In a multi-institutional study of 513 patients
  with TGA/- VSD the 1 month and 1 year survival
  was 84 and 82 respectively
• As of the mid-1990s the risk of post-operative
  death is less than 5
• Risk Factors
• Presence of an intramural coronary artery
• Older age of patients with simple TGA
• Longer periods of circulatory arrest
• Multiple VSDs
• Augmentation of the aortic arch
• Older patients PA band to train LV

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Post-op Considerations

• Systemic ventricle not conditioned to work
• Afterload reduce LV, dont allow elevated
  pressure
• Judicious use of fluid
• Diuresis
• Early extubation

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Tetralogy of Fallot

• 4 features
• Ventricular septal defect
• Dextraposition of the Aorta, overriding
• Pulmonic Stenosis
• Right ventricular hypertrophy

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Hypercyanotic Spell

• Caused by severe R to L shunting
• Infundibular spasm
• Dehydration
• Increased intrathoracic pressure

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Squatting Tet SpellHypercyanotic Episode

• Increases systemic vascular resistance, increase
  venous return
• Increases pulmonary blood flow
• Increases oxygenation, decreasing cyanosis

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Tetralogy of Fallot

• Pallalitive procedures Shunts
• Blalock-Taussig Shunt Classic or modified
• Waterston (RPA) and Potts (LPA)
• Indications
• Small birth weight, other defect (i.e. AV canal),
  LAD from RCA, requiring conduit, do later to
  place larger conduit, small PAs, especially seen
  Tet/PA
• Complete Repair, within 1st year of live
• Close VSD, gortex patch
• Resection of subpulmonic obstruction
• Transannular patch vs. suprannular
• Pulmonary valvotomy

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Modified Blalock-Taussig Shunt

• Instead of native vessel use gortex tube to
  divert blood to lungs
• Very common operation in 2005 for palliation for
  other complex lesions
• Does not grow
• Can become occluded, especially if patient
  becomes dehydrated

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Post-op Considerations

• RV thick, non-compliant
• Milirone beneficial
• Beta-Blockers may be beneficial
• Maintain CVP initial post-op period
• Early extubation, remove positive pressure
  ventilation
• RBBB block common especially if have extensive
  RVOT muscle resection with transannular patch,
  elevation of Tropinin common

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TAPVR

• Supracardiac
• 50 of cases usually does not become obstructed
• Connect confluence to LA
• Into dome of LA
• Through the RA
• Divide vertical vein
• Intracardiac 25
• Infradiaphagmatic 20

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Post-op Considerations

• Most will be volume overloaded, especially if
  obstructed, need lasix
• With obstuction, can develop reactive pulmonary
  vascular bed low threshold to use Nitric Oxide
  post-op (never use pre-op)
• 5 risk of developing recurrent PV stenosis, need
  to re-op, using a sutureless technique
• Sew LA to pericardium around PV confluence

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Single Ventricle Physiology - Goals

• Reliable pulmonary artery blood flow
• Unobstructed outflow from the heart
• Maintain coronary perfusion
• Eliminate AV valve regurgitation
• Allow for mixing at atrial level
• Three stages
• 1st Shunt
• 2nd Bidirectional Glenn
• 3rd Fontan

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Tricuspid Atresia

• As with all single ventricle physiology
• Balance QpQs
• Unobstructed atrial mixing
• Ensure unobstructed systemic CO
• Most patients with reduced Qp or ductal dependent
  Qp will require BT shunt
• Some with high Qp, nonrestrictive VSD will
  require PA band, ultimately may have 1 ½ repair
  (Bi-directional Glenn with PA band)

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Pulmonary Atresia

• With intact ventricular septum, 1 to 1.5 of all
  congenital heart disease
• Sinusoids RV dependent coronary blood flow,
  suggested on Echo require catheterization to
  define
• If none, in cath lab may proceed with PDA stent
  and RF ablation of RVOT into the MPA
• If significant muscular PA, or RV dependent
  coronary blood proceed with BT shunt
• If no RV dependent coronary blood flow, central
  shunt with transannular patch
• 60 alive after 1 year

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Hypoplastic Left Heart Syndrome

• Several varieties all with small, diminutive LVs
• Mitral atresia
• Aortic atresia
• Unbalanced AV canal
• Surgical intervention
• Norwood
• Hybrid procedure Bands and Stents

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Expanding Role

• Earlier diagnosis
• Adult patients with previous repair
• Largest growth
• Patients previously palliated needing repair
• Failed repairs
• Hybrid approach

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Conclusions

• Improved outcomes
• Better understanding of disease process
• Complete repair
• Better operative techniques
• Better perfusion
• Improved cardiopulmonary bypass
• Improved cardiac protection
• Better post-operative care - ICU and Wards
• Team work