Download Tetralogy of Fallot

Tetralogy of Fallot
Tetralogy of Fallot
• I Have No
Disclosures
Tetralogy of Fallot
• Most Common cyanotic CHD
• 8-10% of CHD
• Male>Female
• 0.2-0.3 per 1000 live births
Tetralogy of Fallot
22q11.2 microdeletion
• 20% of TOF with pulmonary stenosis will
have microdeletion of 22q11.2
• 40% of TOF with pulmonary atresia will
have microdeletion of 22q11.2
• Most severe is DiGeorge Syndrome
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Conotruncal cardiac defects
Abnormal facies
Thymic hypoplasia
Cleft palate
hypocalcemia
Thrombocytopenia-Absent Radius
(TAR Syndrome)
K L Greenhalgh et al. J Med Genet 2002;39:876-881
• Bilateral absence of
the radii with
presence of thumbs
• Thrombocytopenia
• 22 -33% congenital
heart disease,
Tetralogy of Fallot
and Atrial Septal
Defects
Tetralogy of Fallot
Pathology
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Anterior and cephalad displacement of
the infundibular septum resulting in:
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Large malalignment VSD
RVOT obstruction
Aortic Override
Right Ventricular hypertrophy
Anatomy Tetralogy of Fallot
T of F Fetal
T of F Fetal
T of F
Neonatal Evaluation
• O2 saturation will depend on severity of
Pulmonary stenosis
• Usually no respiratory distress
• Systolic ejection murmur due to pulmonary
stenosis
T of F
Long Axis View
Tof F Short Axis
VSD: superior margin is formed by
the deviated infundibular Septum
VSD>
VSD: absence of the infundibular septum
Tetralogy of Fallot
Pulmonary Atresia
Malhotra,Sem Thorac Cardiovasc Surg Pediatr
Card Surg Ann 2009
T of F Pulmonary Atresia
Tetralogy of Fallot
Associated Lesions
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ASD 25%
Right aortic arch 20%
Persistent Left Superior Vena Cava (5%)
Complete AV canal (2% Down Syndrome)
Absent Pulmonary Valve
Coronary artery crossing the RVOT (5%)
T of F
AV canal
T of F
AV Canal
Absent Pulmonary Valve
Tetralogy of Fallot
T of F
Neonatal Management
• Depends of O2 saturation
• If O2 sats <90% at birth, Ductal
Dependent, begin PGE
• Cyanotic newborn infants will require BT
shunt or primary “repair”
• Newborn infants with O2 sats in the 90’s
should be observed until the PDA closes
Historical Perspective
•Blalock Taussig Shunt 1945
•Proposed by Helen Taussig and designed
by Alfred Blalock and Vivien Thomas
•Anastomosis of the subclavian artery to the
pulmonary artery
• Vivien Thomas perfected the procedure in
dogs and coached Blalock through the first
procedures.
November 29 1944
February 17 1947
The American Weekly
“Classic” and Modified BT Shunt
“Tet” Spells
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Peak incidence 2-4 months
Hyperpnea, crying, increasing cyanosis
Triggered by agitation, dehydration
Increased R>L shunt
Mechanisms:
• Increased pulmonary vascular resistance
• Decreased systemic vascular resistance
• ? Right ventricular outflow spasm
“Tet” Spells
• Increase RV preload by holding baby in
knee-chest position ( older children squat)
• Give O2 to reduce pulmonary vascular
resistance
• IV fluids
• Increase systemic resistance
(phenylephrine, Ketamine)
• ?propranolol
“Repair” of Tetralogy of Fallot
• The first repair of Tetralogy of Fallot was
done by Walton Lillehei at the University of
Minnesota in 1954
• Goal of “repair” is to close the VSD, relieve
pulmonary and subpulmonary stenosis
• Resection of subpulmonary tissue,
pulmonary valvuloplasty, transannular
patch, or homograft placement may be
required.
Tetralogy of Fallot Repair
Transannular patch.
"Atlas of Cardiothoracic Surgery" Sabiston 1995,
1994-2016 by WebMD LLC
T of F
LAD crossing the RVOT
Atlas of Cardiothoracic Surgery" Sabiston 1995,
Post Operative T of F
Left Anterior Descending coronary crossing the
RVOT
Native PA and Conduit
Tetralogy of Fallot Repair
Pulmonary Atresia
Conduit porcine-valved Dacron® conduit
Decellularized homograft:
2015 Besthearthospitalindia
mussenhealth.us
2016 © Brazilian Journal of Cardiovascular Surgery
Pulmonary Valve replacement
Pulmonary Valve Replacement
How Long do they Last?
• 11% re-operated after 5 years
• 42% Re-operated after 10 years
• 59% Re-operated after 15 years
• Pulmonary Valve replacements do not last
as long in younger (pediatric age) patients
Long Term Problems
• Pulmonary Insufficiency leading to RV
dilation and dysfunction
• Tricuspid Insufficiency leading to atrial
arrhythmias and symptoms of CHF
• Residual ASD or VSD
• Residual RVOT or Pulmonary artery stenosis
• RVOT aneurysm
• Atrial and Ventricular Arrhythmias
• Sudden Death
Mortality
• Early post operative mortality rate 1-2%
• > 20 y/o mortality rate 10%
• >30 y/o mortality rate triples
Factors associated with Sudden
Death
• QRS>180 msec
• “Repair” done at an older age
• Severe Pulmonary Insufficiency with RV
dilation and dysfunction
• Multiple cardiac operations
• Major aortopulmonary collateral vessels
• Abnormal LV diastolic/systolic function
Early Factors Limit Pulmonary
Valve Insufficiency
• Early after “repair” the RV is thick and noncompliant with elevated diastolic pressure
• Pulmonary Arteries are small with low
capacitance
• Faster heart rate with shorter diastolic time
Growth Increases Pulmonary Valve
Insufficiency
•With time and growth the RV becomes less
hypertrophied with fall in diastolic pressure
•Lower heart rates> longer diastolic time
•Increasing Pulmonary Artery Size
Post op T of F “Repair”
Severe Pulmonary Insufficiency
Resulting in RV dilation and
Tricuspid Valve Insufficiency
Post op T of F “Repair”
A Brief Word about the
Right Ventricle
•Systolic shortening more longitudinal than
than circumferential
•Single Conduction fascicle with long delay
from base to RVOT
•Systolic shortening is like peristalsis
•LV function accounts for 63% of RV
shortening
•Single Coronary Artery
RV Dilation due to Pulmonary
Valve Insufficiency
RV size and
function NOT well
seen by echo, use
Cardiac MRI
Severe Right Ventricular dilation/dysfunction
due to long term pulmonary stenosis and
insufficiency
Melody Valve
• Bovine jugular vein sewn into a stent of platinum
wire
• Prior 16-22 mm circumferential RVOT conduit
• body weight >20 kg.
• Severe Pulmonary insufficiency or stenosis
FDA Approval January 2015
The Melody® Valve is indicated for use as an adjunct to surgery in the
management of pediatric and adult patients with the following clinical conditions:
Existence of a full (circumferential) RVOT conduit that was equal to or greater than
16 mm in diameter when originally implanted
AND:
Dysfunctional Right Ventricular Outflow Tract (RVOT) conduits with a clinical
indication for intervention
AND EITHER:
Regurgitation: ≥ moderate
OR
Stenosis: mean RVOT gradient ≥ 35 mmHg
5-year freedom from re-intervention of 76 % and a
5-year freedom from explant of 92 %
Stent fracture and endocarditis have been the primary causes for
re-intervention
Pre-stenting before placement significantly
reduces stent fracture.
AHA Class IIa
1. It is reasonable to consider percutaneous pulmonary
valve replacement in a patient with an RV-to-pulmonary
artery conduit with associated moderate to severe
pulmonary regurgitation or stenosis
• Symptomatic patients with severe pulmonary regurgitation (PR) with
RV dysfunction and/or dilatation,
• Patients with symptomatic arrhythmias and severe PR with RV
dysfunction/dilatation
• Severe PR with RV dysfunction (on echo/MRI) and decreased
exercise tolerance in asymptomatic patients,
• Patients with moderate or severe PR and additional lesions
(residual VSD, branch PA stenosis, and tricuspid regurgitation)
needing intervention
• Asymptomatic patients with severe RVOT obstruction (RV
pressures 3/4 systemic
• 6) Symptomatic patients with RVOT obstruction (RV >1/2 systemic
pressure) with or without PR.
American Heart Association. Circulation. 2011;123:2607–2652
Sapien Valve
• Bovine Pericardial Tissue leaflets
• COMPASSION trial results: 4 year
freedom from surgical re-intervention=
91.8% and from repeat TPVR= 91.2%
TPVR= Transcather Pulmonary Valve Replacement
March 2016: FDA approved use of the
Sapien XT in percutaneous pulmonary
valve implantation
Conduits
Pre-stented
Transannular Patches
COMPASSION trial results
Valve-in-valve
Questions?