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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
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
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
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
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
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
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%)
EUROECHO CONGRESS - COPENHAGEN -TEACHING COURSE 2010
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
EUROECHO CONGRESS - COPENHAGEN -TEACHING COURSE 2010
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
Survival Following Complete Repair of TOFTOF
EUROECHO CONGRESS - COPENHAGEN -TEACHING COURSE 2010
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
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
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
Ebstein’s AnomalyMay be Associated with
PFOASDASDVSD with or without Pul.
atresiaatresiaPulmonary Out Flow Obstr.PDAPDAAo CoarctationLV CardiomyopathyLV Cardiomyopathy
EUROECHO CONGRESS - COPENHAGEN -TEACHING COURSE 2010
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
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
EUROECHO CONGRESS - COPENHAGEN -TEACHING COURSE 2010
EUROECHO CONGRESS - COPENHAGEN -TEACHING COURSE 2010
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
Indications for Intervention in Ebstein’s Anomaly
ESC GUCHESC GUCH Guidelines2010
EUROECHO CONGRESS - COPENHAGEN -TEACHING COURSE 2010
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
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
EUROECHO CONGRESS - COPENHAGEN -TEACHING COURSE 2010
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
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
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
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)
EUROECHO CONGRESS - COPENHAGEN -TEACHING COURSE 2010
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.
D-TGA, Complete TGA, p
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
Atrial Switch Surgery(Mustard, Senning procedures)
EUROECHO CONGRESS - COPENHAGEN -TEACHING COURSE 2010
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.
Baffle leakBaffle leak
Indications for Catheter Interventions Indications for Catheter Interventions in TGA patients after Atrial Switch
ESC GUCH Guidelines 2010
EUROECHO CONGRESS - COPENHAGEN -TEACHING COURSE 2010
Indications for Surgical Interventions in TGA patients after Atrial Switch
ESC GUCH Guidelines2010
EUROECHO CONGRESS - COPENHAGEN -TEACHING COURSE 2010
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
EUROECHO CONGRESS - COPENHAGEN -TEACHING COURSE 2010
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
EUROECHO CONGRESS - COPENHAGEN -TEACHING COURSE 2010
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
Arterial Switch OperationArterial Switch Operation
Consequences of Arterial SwitchConsequences of Arterial Switch
• supra neopulmonary artery stenosissupra neopulmonary artery stenosis• ostial coronary artery disease• progressive neoaortic valve
regurgitation regurgitation
Indication for Interventions in TGA patients after Arterial Switch
ESC GUCH GuidelinesGuidelines2010
EUROECHO CONGRESS - COPENHAGEN -TEACHING COURSE 2010
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
Single Ventricle Physiology andFontan Circulation
Biventricular repair can not be doneBiventricular repair can not be done
EUROECHO CONGRESS - COPENHAGEN -TEACHING COURSE 2010
EUROECHO CONGRESS - COPENHAGEN -TEACHING COURSE 2010
Tricuspid Atresia Single Ventricle
EUROECHO CONGRESS - COPENHAGEN -TEACHING COURSE 2010
EUROECHO CONGRESS - COPENHAGEN -TEACHING COURSE 2010
EUROECHO CONGRESS - COPENHAGEN -TEACHING COURSE 2010
Indication for Intervention in Single Ventricles
ESC GUCHGuidelines2010
EUROECHO CONGRESS - COPENHAGEN -TEACHING COURSE 2010
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
EUROECHO CONGRESS - COPENHAGEN -TEACHING COURSE 2010
Glenn ShuntGlenn Shunt
EUROECHO CONGRESS - COPENHAGEN -TEACHING COURSE 2010
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
EUROECHO CONGRESS - COPENHAGEN -TEACHING COURSE 2010
Fontan Total Cavo-Pulmoner Anastomosis
EUROECHO CONGRESS - COPENHAGEN -TEACHING COURSE 2010
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
EUROECHO CONGRESS - COPENHAGEN -TEACHING COURSE 2010
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
EUROECHO CONGRESS - COPENHAGEN -TEACHING COURSE 2010