cyanotic diseases - congenital heart disease core...
TRANSCRIPT
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Cyanotic Diseases Congenital Heart Disease, Teaching Course
EUROECHO 2010
M. Serdar Küçükoğlu M.D., FESCIstanbul University Institute of Istanbul University Institute of
Cardiology
EUROECHO CONGRESS - COPENHAGEN -TEACHING COURSE 2010
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Cyanotic Congenital Heart Diseasey g
Classification
Cyanosis with lowl fl
Cyanosis with highpulm Flowpulm. flow
- TOFEb t i A l
pulm. Flow- TGA
Double outlet V- Ebstein Anomaly- Tricuspid Atresia
P l At i
- Double outlet V- Double inlet V
TAPVD- Pulm. Atresia - TAPVD
EUROECHO CONGRESS - COPENHAGEN -TEACHING COURSE 2010
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Tetralogy of FallotTetralogy of FallotEtienne Louis ArthurEtienne- Louis Arthur Fallot made the firstpublished bedsidepublished bedsidediagnosis that wasproven at post mortemproven at post-mortemin 1888 and called thecondition “ maladie bleue”condition maladie bleue
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Epidemiology
Prevalence varies from 0.26- 0.48/ 1000Prevalence varies from 0.26 0.48/ 1000 Incidence: 3/10000 live birthsMost common cyanotic CHDMost common cyanotic CHD6.8 of all CHD10 % f ll GUCH ti t10 % of all GUCH patients
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Morphology Ventricular Septal DefectMorphology- Ventricular Septal Defect
80% of cases fibrous continuation between the mitral, ,tricuspid, and aortic valves
20% cases there is a muscular rim a round the defect.
The pulmonary valve annulusd b h l i itends to be hypoplastic or atretic.
EUROECHO CONGRESS - COPENHAGEN -TEACHING COURSE 2010
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Morphologyp gyOverriding AortaDegree of override can vary from an exclusive connection of the right ventricle to an exclusive connection to the leftan exclusive connection to the left ventricle
P l St iPulmonary StenosisInfundibular / valvular
Concentric RV Hypertrophy Secondary to RV outflow obstruction
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Associated LesionsAssociated LesionsPulmonary valvar stenosisy
Pulmonary atresia
Absence of pulmonary valve leaflets
Right Aortic Arch (25 %)
PFO or ASD (Pentalogy of Fallot)
A l i i f LAD f RCA (3%)Anomalous origin of LAD from RCA (3%)
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EUROECHO CONGRESS - COPENHAGEN -TEACHING COURSE 2010
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SurgerySurgery
P lli ti d t iPalliative procedures to increase pulmonary blood flow
- Blalock Tausing shunt: L Sub CA to PA- Waterston shunt: Asc Ao to RPA- Potts shunt: Desc Ao to LPA
RepairRepair
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Surgical Repairg pClosure of VSDR li i f RVOTRelieving of RVOT
- Pulmonary valvotomyR ti f i fi d bil l- Resection of infindubiler muscle
- RVOT or subannular patchT l t h if PV l t i ti- Transannular patch- if PV annulus restrictive
- Pulmanary valve implantation E t di d it b t th RV d PA- Extracardiac conduit between the RV and PA
- Closure of PFO or ASD
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Survival Following Complete Repair of TOFTOF
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Follow- Up of Repaired ToFp pSignificant PR- Following transannular patchResidual RVOT obstResidual RVOT obst.RV Dilatation , TR, RV FailureResidual VSDResidual VSDProgressive AI with or without root dilationLV DysfunctionLV DysfunctionInfective EndocarditisElectrical Complications: RBBB BifasicularElectrical Complications: RBBB, Bifasicular
block, 3° AV block, A Fl. , AF, NSVT, VTSudden Cardiac DeathSudden Cardiac Death
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Cyanotic Congenital Heart Diseasey g
Classification
Cyanosis with lowl fl
Cyanosis with highpulm Flowpulm. flow
- TOFEb t i A l
pulm. Flow- TGA
Double outlet V- Ebstein Anomaly- Tricuspid Atresia
P l At i
- Double outlet V- Double inlet V
TAPVD- Pulm. Atresia - TAPVD
EUROECHO CONGRESS - COPENHAGEN -TEACHING COURSE 2010
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Ebstein’s AnomalyEbstein s Anomaly• Apical displacement of the septal and postero-Apical displacement of the septal and postero
lateral leaflets of the TV• Atrialization of RV inflow- smaller “functional” RV• Atrialization of RV inflow- smaller functional RV• Tricuspid regurgitation (occasionaly stenotic)• Right Atrial enlargement• PFO or ASD (50%)• Accessory pathways-risk of atrial tachycardias
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Ebstein’s AnomalyMay be Associated with
PFOASDASDVSD with or without Pul.
atresiaatresiaPulmonary Out Flow Obstr.PDAPDAAo CoarctationLV CardiomyopathyLV Cardiomyopathy
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Ebstein Anomaly
Prevalance: 1:50-100000 live birthsClinical Features:- Depends on the severity of the pathology and the p y p gy
magnitude of L-R shunt- In milder forms only murmur and arrhytmiaIn milder forms only murmur and arrhytmia- In severe forms cyanosis related to the extent of
R-L shuntingR-L shunting- WPW Syndrome
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Ebstein Anomaly-Echocardiography
Increase in RV volume
Paradoxical septal motionParadoxical septal motion
Increase in TV excursion
Delay in TV closurecompared to MV closure
Decreased TV EF slope
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EUROECHO CONGRESS - COPENHAGEN -TEACHING COURSE 2010
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EUROECHO CONGRESS - COPENHAGEN -TEACHING COURSE 2010
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Surgical Management• Indications for intervention- Deteriorating FC (NYHA≥Class III)Progressive cyanosis- Progressive cyanosis
- Right heart failure- Paradoxical embolism - Recurrent supraventricular arrhythmiap y- Asymptomatic progressive cardiomegaly
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Indications for Intervention in Ebstein’s Anomaly
ESC GUCHESC GUCH Guidelines2010
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Surgery
Tricuspid valve repairp pTricuspid valve replacementPlication of the atrialized RVPlication of the atrialized RVFor high risk patients a bidirectional cavo-pulmonary connection (to reduce RV preload)pulmonary connection (to reduce RV preload)Ablation of the accessory pathwaysClosure of PFO/ASD if present
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Late ComplicationsLate Complications
• Reoperation of TV if TR persist• Valve replacement may be necessary ifValve replacement may be necessary if
bioprosthesis fails or mechanic valvethrombosisthrombosis
• Late arrhytmias may occur• Complete heart block may occur
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EUROECHO CONGRESS - COPENHAGEN -TEACHING COURSE 2010
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Prognosis and Managementg gSurvival at 1 year 67% and 10 year 59 %.
Survival after Tricuspid bioprosthesis: 10 yr93% and 15 yr 81%. 94% in NYHA I/II.
Prognosis is worse if : - NHYA FC III-IV NHYA FC III IV, - CTR>65 %- atriyal fibrilation,y- severe cyanosis, - severe TR,
f ti l < %35 - functional rv area < %35
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Cyanotic Congenital Heart Diseasey g
Classification
Cyanosis with lowl fl
Cyanosis with highpulm Flowpulm. flow
- TOFEb t i A l
pulm. Flow- TGA
Double outlet V- Ebstein Anomaly- Tricuspid Atresia
P l At i
- Double outlet V- Double inlet V
TAPVD- Pulm. Atresia - TAPVD
EUROECHO CONGRESS - COPENHAGEN -TEACHING COURSE 2010
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Transposition of the Great Arteries (TGA)
• D loop• L LoopL Loop• RV follow the loop
D loop RV on the rightL loop RV on the leftL loop RV on the left
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L TGA Congenitally corrected TGAL- TGA -Congenitally corrected TGA
Atria- ventricular discordanceVentriculo- arterialdiscordancediscordance
Associated Anomalies: (98%)-VSD (75%)-P or subP stenosis (75%)-L sided ( TV- Ebstein like)-L sided ( TV- Ebstein like) valvular anomalies (75%) -Complete AV block (2%/y)
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EUROECHO CONGRESS - COPENHAGEN -TEACHING COURSE 2010
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D- TGA- Complete TGApIncidence 20-30/1000,000 live births.Without treatment 30% die within 1st week, 50% within 1st month, 70% in 6 months and 90% in first year.With current medical and surgical interventions 90% early and midterm survival. I l t T iti th ti b t t iIn complete Transposition the connection between atria and ventricles are normal.The connections between the ventricles and the greatThe connections between the ventricles and the great arteries are discordant.
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D-TGA, Complete TGA, p
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SurgerySurgeryMost commonly done proceduresMost commonly done procedureswere atrial switch operations:Blood redirected at atrial level with abaffle made of dacron or pericardium(Mustard)(Mustard)With atrial flaps (Senning) to achievep ( g)physiological correction
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Atrial Switch Surgery(Mustard, Senning procedures)
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EUROECHO CONGRESS - COPENHAGEN -TEACHING COURSE 2010
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Consequences of MustardConsequences of Mustard
Most patients reaching adulthood have NYHA I/II symptoms over the next 25 years50% d l d t t li d f ti f th RV50% develop moderate systolic dysfunction of the RVbut only few present with CHF.1/3rd have severe systemic TR1/3rd have severe systemic TR. Atrial flutter arises in 20% by age 20. 50% patients have sinus node dysfunction by age 2050% patients have sinus node dysfunction by age 20. Baffle leak or obstruction can also occur.
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Baffle leakBaffle leak
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Indications for Catheter Interventions Indications for Catheter Interventions in TGA patients after Atrial Switch
ESC GUCH Guidelines 2010
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Indications for Surgical Interventions in TGA patients after Atrial Switch
ESC GUCH Guidelines2010
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Rastelli ProcedureRastelli Procedure
• Procedure for TGA with VSD and pulmonary/subpulmonary stenosisp y/ p y
• Blood is redirected at ventricular levellevel
• LV tunneled to Ao via VSD• RV- PA via a valved conduit
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Complications of the Rastelli Procedure
• RV- PA conduit stenosis causing exercise intolerance or RV anginag
• Subaortic obstruction (LV-Ao tunnel)i d causing dyspnea or syncope
• Residuel VSDResiduel VSD
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SurgerySurgery
C t i l it hCurrent practice is the arterial switchoperation developed in 1980’s.Blood is redirected at the level of the great arterythe morphological left ventricle becomes the subaortic ventricle the morphological p gright ventricle becomes the subpulmonicventricleventricle
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Arterial Switch OperationArterial Switch Operation
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Consequences of Arterial SwitchConsequences of Arterial Switch
• supra neopulmonary artery stenosissupra neopulmonary artery stenosis• ostial coronary artery disease• progressive neoaortic valve
regurgitation regurgitation
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Indication for Interventions in TGA patients after Arterial Switch
ESC GUCH GuidelinesGuidelines2010
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Cyanotic Congenital Heart Diseasey g
Classification
Cyanosis with lowl fl
Cyanosis with highpulm Flowpulm. flow
- TOFEb t i A l
pulm. Flow- TGA
Double outlet V- Ebstein Anomaly- Tricuspid Atresia
P l At i
- Double outlet V- Double inlet V
TAPVD- Pulm. Atresia - TAPVD
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Single Ventricle Physiology andFontan Circulation
Biventricular repair can not be doneBiventricular repair can not be done
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EUROECHO CONGRESS - COPENHAGEN -TEACHING COURSE 2010
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Tricuspid Atresia Single Ventricle
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EUROECHO CONGRESS - COPENHAGEN -TEACHING COURSE 2010
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Indication for Intervention in Single Ventricles
ESC GUCHGuidelines2010
EUROECHO CONGRESS - COPENHAGEN -TEACHING COURSE 2010
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Glenn ProcedureGlenn ProcedurePalliative procedure for cyanotic patients or as a p y pstep to Fontan procedure or corrective surgery (e.gsingle ventricle)Goal: to improve pulmonary blood flow • Establishes a direct connection between SVC and right PA (directs half of the blood volume directly to the lung without the assistance of the ventricle)D t t l l dDoes not create volume overload The venous return is under low pressure low risk for pulmonary vascular obstructive disease • Performedpulmonary vascular obstructive disease • Performed at 3-8 months of age
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Glenn ShuntGlenn Shunt
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Fontan CirculationFontan CirculationUsed when biventricular connections are not possible, th t f ti i t i l t b ff ti lthe two functioning ventricles cannot be effectively established (e.g. double-inlet single ventricle, tricuspid atresia)atresia)Directs the blood directly to the lungs without assistance of the ventricleof the ventricle
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Fontan Total Cavo-Pulmoner Anastomosis
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Fontan PrognosisFontan Prognosis
• 10 year survival is 60-70%• Late term complications includeLate term complications include
- Atrial flutter or fibrillation - Right atrial thrombus- Obstruction of the Fontan circutObstruction of the Fontan circut- Ventricular dysfunction- Protein loosing enteropathy
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Cyanotic Congenital Heart Diseasey g
Classification
Cyanosis with lowl fl
Cyanosis with highpulm Flowpulm. flow
- TOFEb t i A l
pulm. Flow- TGA
Double outlet V- Ebstein Anomaly- Tricuspid Atresia
P l At i
- Double outlet V- Double inlet V
TAPVD- Pulm. Atresia - TAPVD
Contributions Omaç Tüfekçioğlu M DContributions Omaç Tüfekçioğlu M.D.
EUROECHO CONGRESS - COPENHAGEN -TEACHING COURSE 2010
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EUROECHO CONGRESS - COPENHAGEN -TEACHING COURSE 2010