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Tetralogy of FallotTetralogy of FallotJimmy WangJimmy Wang
Children’s Memorial HospitalChildren’s Memorial Hospital
November 2, 2007November 2, 2007
TetralogyTetralogy
Comes from Attic Theater in GreeceComes from Attic Theater in Greece A compound work made up of 4 distinct works, A compound work made up of 4 distinct works,
intended to be viewed in one sittingintended to be viewed in one sitting Duology, Trilogy, Pentalogy, HeptalogyDuology, Trilogy, Pentalogy, Heptalogy
Famous TetralogiesFamous Tetralogies
Literature: Shakespeare’s Literature: Shakespeare’s Richard II, Henry IV, Richard II, Henry IV, Henry V, Henry VIHenry V, Henry VI
Movies: Lethal Weapon, Movies: Lethal Weapon, Die Hard, Jaws, Austin Die Hard, Jaws, Austin Powers, Indiana Jones Powers, Indiana Jones (2008), Rambo (2008), (2008), Rambo (2008), Terminator (2009), Shrek Terminator (2009), Shrek (2010)(2010)
Medicine: Tetralogy of Medicine: Tetralogy of FallotFallot Shrek 4 in 2010
Dr. Etienne FallotDr. Etienne Fallot
Initially described in Initially described in 1672 by Danish 1672 by Danish anatomist, anatomist, paleontologist, and paleontologist, and geologist, Niels Stensengeologist, Niels Stensen
Named after French Named after French physician Etienne-Louis physician Etienne-Louis Arthur Fallot in 1888, Arthur Fallot in 1888, who accurately who accurately described the 4 described the 4 anatomic abnormalities anatomic abnormalities in TOFin TOF
Dr. Etienne Fallot, 1850-1911
Niels Stensen
4 Characteristics in TOF4 Characteristics in TOF
VSDVSD Right ventricular Right ventricular
outflow tract outflow tract obstructionobstruction
Overriding aortaOverriding aorta Right ventricular Right ventricular
hypertrophyhypertrophy
Radiographics 2007;27:1323-1334.
Tetralogy of FallotTetralogy of Fallot
http://commons.wikimedia.org/wiki/Image:Tetralogy_of_Fallot.svg
RV Outflow ObstructionRV Outflow Obstruction
Spectrum of outflow obstructionSpectrum of outflow obstruction 45% stenosis at RV infundibulum45% stenosis at RV infundibulum 10% stenosis at pulmonic valve 10% stenosis at pulmonic valve 30% combination of PV and infundibular 30% combination of PV and infundibular
stenosisstenosis 15% atresia of the pulmonic valve15% atresia of the pulmonic valve
Hypoplasia of the pulmonary annulus and Hypoplasia of the pulmonary annulus and main PAmain PA
Ventricular Septal DefectVentricular Septal Defect
Perimembranous defect (involving fibrous Perimembranous defect (involving fibrous base of the pulmonic valve)base of the pulmonic valve)
Extends to subpulmonic regionExtends to subpulmonic region Needs to be large enough to equalize Needs to be large enough to equalize
pressures in R and L heart pressures in R and L heart
Overriding Aorta & RVHOverriding Aorta & RVH
Overriding aorta can be variableOverriding aorta can be variable RVH develops secondarily to long RVH develops secondarily to long
standing elevated RV pressures and standing elevated RV pressures and increased stroke volume from RV outflow increased stroke volume from RV outflow obstructionobstruction
Etiology/PrevalenceEtiology/Prevalence
Caused by anterior malalignment of the Caused by anterior malalignment of the conal septum, with underdevelopment of conal septum, with underdevelopment of the infundibulumthe infundibulum
Most common cyanotic congenital heart Most common cyanotic congenital heart defect in children beyond infancy (10%)defect in children beyond infancy (10%)
4-8% of all congenital cardiac lesions4-8% of all congenital cardiac lesions 3-6 cases per 10,000 live births3-6 cases per 10,000 live births
AssociationsAssociations
Stenosis or obstruction at the origin of the Stenosis or obstruction at the origin of the L pulmonary artery in 40%L pulmonary artery in 40%
Right aortic arch in 25% Right aortic arch in 25% ASD in 5%ASD in 5% Abnormal coronary arteries in 5%Abnormal coronary arteries in 5%
Most common: aberrant origin of the anterior Most common: aberrant origin of the anterior descending artery from the right coronary descending artery from the right coronary artery, crosses RV outflow tract (potential artery, crosses RV outflow tract (potential surgical disaster)surgical disaster)
Clinical PresentationClinical Presentation
Spectrum ranges from acyanotic TOF/pink Fallot Spectrum ranges from acyanotic TOF/pink Fallot (with L to R shunt) to classic Fallot to severe (with L to R shunt) to classic Fallot to severe cyanosis in pulmonary atresia with VSD cyanosis in pulmonary atresia with VSD
Most patients p/w cyanosis at or shortly after Most patients p/w cyanosis at or shortly after birthbirth
Milder cases may present later with SOB on Milder cases may present later with SOB on exertion with relief in squatting positionexertion with relief in squatting position
““Tet spells” – paroxysm of hyperpnea, irritability, Tet spells” – paroxysm of hyperpnea, irritability, crying, & cyanosis, requires immediate medical crying, & cyanosis, requires immediate medical attention, may lead to convulsion, CVA, or deathattention, may lead to convulsion, CVA, or death
Physical ExamPhysical Exam
Cyanosis, tachypnea, clubbingCyanosis, tachypnea, clubbing Auscultation: heart murmur usually audible Auscultation: heart murmur usually audible
at birth - single S2, long cresendo-at birth - single S2, long cresendo-decresendo systolic murmur at mid & decresendo systolic murmur at mid & LUSB (usually grade 3-5/6), also with LUSB (usually grade 3-5/6), also with holosystolic regurgitant murmur of VSDholosystolic regurgitant murmur of VSD
Chest Radiograph in TOFChest Radiograph in TOF
Heart size ranges from Heart size ranges from slightly small to slightly slightly small to slightly largelarge
Decreased pulmonary Decreased pulmonary vascular markingsvascular markings
Superiorly turned cardiac Superiorly turned cardiac apex – boot shaped heartapex – boot shaped heart
R atrial enlargementR atrial enlargement Concavity of the PA Concavity of the PA
segmentsegment
Radiographics 2007;27:1323-1334.
Medical ManagementMedical Management
Treat hypoxic “Tet” spells (positioning, Treat hypoxic “Tet” spells (positioning, morphine, oxygen, sodium bicarbonate, morphine, oxygen, sodium bicarbonate, phenylephrine, ketamine, propranolol)phenylephrine, ketamine, propranolol)
Oral propranolol (prevent hypoxic spells)Oral propranolol (prevent hypoxic spells) Balloon dilatation of RV outflow tract & PV Balloon dilatation of RV outflow tract & PV
to delay surgical repairto delay surgical repair Antibiotic prophylaxis against SBEAntibiotic prophylaxis against SBE
Palliative Shunt PlacementPalliative Shunt Placement
Indications for shunt vs. Indications for shunt vs. surgical repair vary between surgical repair vary between institutions, usually done for institutions, usually done for more complicated cases (TOF more complicated cases (TOF with pulmonary atresia, with pulmonary atresia, severely cyanotic <3 months, severely cyanotic <3 months, severe hypoxic spells)severe hypoxic spells)
Goal: increase pulmonary blood Goal: increase pulmonary blood flowflow
Classic Blalock-Taussig shunt, Classic Blalock-Taussig shunt, Gore-Tex interposition shunt: Gore-Tex interposition shunt: anastomotic shunt between anastomotic shunt between subclavian artery & ipsilateral subclavian artery & ipsilateral PAPA
Correctional Surgical InterventionCorrectional Surgical Intervention
Patch closure of VSD & Patch closure of VSD & widening of RV outflow widening of RV outflow tracttract Symptomatic: variable Symptomatic: variable
between institutions, after between institutions, after 3 months of age 3 months of age preferred, increased preferred, increased mortality in pts <3 monthsmortality in pts <3 months
Mildly cyanotic: 3-24 Mildly cyanotic: 3-24 months of agemonths of age
Asymptomatic/Acyanotic: Asymptomatic/Acyanotic: 1-2 years of age1-2 years of age
www.inova.com
TOF with Absent Pulmonary ValvesTOF with Absent Pulmonary Valves
Occurs in 2% of patients with TOFOccurs in 2% of patients with TOF Absent PV or irregular rudimentary PV leafletsAbsent PV or irregular rudimentary PV leaflets Stenotic PV annulus less severe than classic Stenotic PV annulus less severe than classic
TOF – results in bidirectional shunting through TOF – results in bidirectional shunting through VSD (predominantly L to R, mild cyanosis VSD (predominantly L to R, mild cyanosis evolves into CHF after newborn period)evolves into CHF after newborn period)
Massive pulmonary artery aneurysmal dilatation Massive pulmonary artery aneurysmal dilatation from severe pulmonary regurgitationfrom severe pulmonary regurgitation
Massive PA compresses lower central airways Massive PA compresses lower central airways => hypoplasia, post-obstructive complications => hypoplasia, post-obstructive complications (PNA, atelectasis), usual cause of death(PNA, atelectasis), usual cause of death
Absent Pulmonary ValvesAbsent Pulmonary Valves
Dilated main PA Dilated main PA and hilar PA’sand hilar PA’s
Hyperinflated Hyperinflated lungs from central lungs from central airway obstructionairway obstruction
Slightly increased Slightly increased pulmonary pulmonary vascular markings vascular markings to diffuse bilateral to diffuse bilateral opacification of opacification of CHF from L to R CHF from L to R shuntingshunting
D.R. - 12 year old maleD.R. - 12 year old male
History of Tetrology of Fallot with absent History of Tetrology of Fallot with absent pulmonary valvespulmonary valves
Surgical history: surgical repair on 6Surgical history: surgical repair on 6thth day day of life in 1995, had RV to PA conduit of life in 1995, had RV to PA conduit revision in 1997revision in 1997
Now p/w increasing fatigue and dyspnea Now p/w increasing fatigue and dyspnea following strenuous exercise (basketball, following strenuous exercise (basketball, football)football)
Gated FIESTA Axial
Post-gad
RV FIESTA Short Axis
FIESTA 4 chamber view
ReferencesReferences 1. Boechat MI, Ratib O, Williams PL, et al. Cardiac MR Imaging and MR 1. Boechat MI, Ratib O, Williams PL, et al. Cardiac MR Imaging and MR
Angiography for Assessment of Complex Tetralogy of Fallot and Pulmonary Angiography for Assessment of Complex Tetralogy of Fallot and Pulmonary Atresia. Radiographics 2005; 25:1535-1546.Atresia. Radiographics 2005; 25:1535-1546.
2. Ferguson EC, Krishnamurthy R, Oldham SA. Classic Imaging Signs of 2. Ferguson EC, Krishnamurthy R, Oldham SA. Classic Imaging Signs of Congenital Cardiovascular Abnormalities. Radiographics 2007; 27:1323-Congenital Cardiovascular Abnormalities. Radiographics 2007; 27:1323-1334.1334.
3. Park MK. Pediatric Cardiology, 43. Park MK. Pediatric Cardiology, 4thth Edition. St. Louis, Mosby, Inc 2002, pp. Edition. St. Louis, Mosby, Inc 2002, pp. 189-200.189-200.
4. Westra, SJ. “Tetralogy of Fallot,” [Online] Available https://my.statdx.com. 4. Westra, SJ. “Tetralogy of Fallot,” [Online] Available https://my.statdx.com. StatDx 2007.StatDx 2007.
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