Euroecho2010 cyaotic-disease

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


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.


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%)


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


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


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


Classification



- TOFEb t i A l

pulm. Flow- TGA


P l At i




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


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



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


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


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


Classification



- TOFEb t i A l

pulm. Flow- TGA


P l At i




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)


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)


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


Indications for Surgical Interventions in TGA patients after Atrial Switch

ESC GUCH Guidelines2010


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


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


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



Classification



- TOFEb t i A l

pulm. Flow- TGA


P l At i




Single Ventricle Physiology andFontan Circulation

Biventricular repair can not be doneBiventricular repair can not be done



Tricuspid Atresia Single Ventricle




Indication for Intervention in Single Ventricles

ESC GUCHGuidelines2010


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


Glenn ShuntGlenn Shunt


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


Fontan Total Cavo-Pulmoner Anastomosis


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



Classification



- TOFEb t i A l

pulm. Flow- TGA


P l At i



Contributions Omaç Tüfekçioğlu M DContributions Omaç Tüfekçioğlu M.D.



Education

Euroecho2010 cyaotic-disease