congenital heart diseases ii

Congenital Heart DiseasesCongenital Heart DiseasesIIII

Samer Abbas, MDSamer Abbas, MD

Cardiology UIC/ChristCardiology UIC/Christ

02/27/200802/27/2008

ClassificationClassification

Cyanosis with low Cyanosis with low pulm flow:pulm flow:

- TOF- TOF

- Ebstien Anamoly- Ebstien Anamoly

- Hypoplastic of RV- Hypoplastic of RV

- Tricuspid atresia- Tricuspid atresia

- Pulm atresia- Pulm atresia

Cyanosis with high Cyanosis with high pulm flow:pulm flow:- TGATGA- Double outlet V.Double outlet V.- Double inlet VDouble inlet V- TAPVD.TAPVD.

Eisenmenger SyndromeEisenmenger SyndromeIn 1897 Victor Eisenmenger published In 1897 Victor Eisenmenger published a paper entitled “Congenital Defects of a paper entitled “Congenital Defects of the Ventricular Septum.”the Ventricular Septum.”

In 1958, Paul Wood summarized In 1958, Paul Wood summarized Eisenmenger’s accounts:Eisenmenger’s accounts:““The patient was a powerfully built man of 32 The patient was a powerfully built man of 32 who gave a history of cyanosis and moderate who gave a history of cyanosis and moderate breathlessness since infancy. He managed breathlessness since infancy. He managed well until January of 1894 when dyspnea well until January of 1894 when dyspnea increased and edema set in. Seven months increased and edema set in. Seven months later he was admitted to the hospital in a state later he was admitted to the hospital in a state of heart failure……He improved with rest and of heart failure……He improved with rest and digitalis, but collapsed and died suddenly on digitalis, but collapsed and died suddenly on November 13 following a large hemoptysis”November 13 following a large hemoptysis”

Eisenmenger SyndromeEisenmenger Syndrome

Large Large left to rightleft to right shunt:shunt:– severe pulmonary severe pulmonary

vascular disease leading vascular disease leading to shunt reversalto shunt reversal

Initial reversibleInitial reversible changes:changes:– Medial hypertrophy of the Medial hypertrophy of the

pulmonary vasculaturepulmonary vasculature– Intimal proliferation Intimal proliferation

Eisenmenger SyndromeEisenmenger SyndromeProgressive irreversible Progressive irreversible changes:changes:– plexiform lesionsplexiform lesions– necrotizing arteritisnecrotizing arteritis

As the increased PVR As the increased PVR approaches or exceeds the SVR approaches or exceeds the SVR the shunt is reversed.the shunt is reversed.

As R to L shunting develops As R to L shunting develops cyanosis appears.cyanosis appears.

Most patients will develop Most patients will develop exertional dyspnea and impaired exertional dyspnea and impaired exercise tolerance.exercise tolerance.

Eisenmenger SyndromeEisenmenger SyndromePalpitations occur in >50% of patients Palpitations occur in >50% of patients – A. fib/flutter in 40% A. fib/flutter in 40% – VT in 10%)VT in 10%)

Hemoptysis in ~20%Hemoptysis in ~20%PE, angina, syncope, endocarditis ~10%PE, angina, syncope, endocarditis ~10%Signs of PHTNSigns of PHTN– RV heave, palpable PRV heave, palpable P22, and right sided S, and right sided S44

Pulmonary ejection click and a soft scratchy Pulmonary ejection click and a soft scratchy SEM SEM – dd/t dilated pulmonary trunk/t dilated pulmonary trunk

High pitched decrescendo diastolic murmur High pitched decrescendo diastolic murmur (Graham-Steele) audible in most patients (Graham-Steele) audible in most patients Usually no peripheral edema until R HF ensuesUsually no peripheral edema until R HF ensues


ECG shows RVH, RAE and RADECG shows RVH, RAE and RADAtrial arrhythmias may be presentAtrial arrhythmias may be present


CXR reveals prominent central pulmonary arteries and CXR reveals prominent central pulmonary arteries and decreased vascular markings (pruning) of the peripheral vesselsdecreased vascular markings (pruning) of the peripheral vessels

Eisenmenger SyndromeEisenmenger SyndromeLarge variation in life expectancy in adults with Large variation in life expectancy in adults with Eisenmenger syndromeEisenmenger syndromeRate of survival among patients with Eisenmenger Rate of survival among patients with Eisenmenger syndrome is:syndrome is:– 80% at 10 years80% at 10 years– 77% at 15 years77% at 15 years– 42% at 25 years42% at 25 years

Recent study of 109 adults revealed following as Recent study of 109 adults revealed following as independent predictors of mortality:independent predictors of mortality:– Age at presentationAge at presentation– Supraventricular arrhythmiasSupraventricular arrhythmias– Poor NYHA functional class (III or IV)Poor NYHA functional class (III or IV)

Eisenmenger SyndromeEisenmenger Syndrome Pregnancy is discouraged due to high maternal (50%) and Pregnancy is discouraged due to high maternal (50%) and fetal (60%) mortality.fetal (60%) mortality.CVA may occur secondary to paradoxical emboliCVA may occur secondary to paradoxical emboliCerebral abscessesCerebral abscessesAvoid intravascular volume depletion, heavy exertion, high Avoid intravascular volume depletion, heavy exertion, high altitudes, and use of vasodilatorsaltitudes, and use of vasodilatorsIV epoprostenol may be beneficial in decreasing PVRIV epoprostenol may be beneficial in decreasing PVRPhlebotomy with isovolumic replacement is recommended Phlebotomy with isovolumic replacement is recommended for patients with moderate to severe symptoms of for patients with moderate to severe symptoms of hyperviscosity and an elevated hematocrit >65%hyperviscosity and an elevated hematocrit >65%Prevention of iron deficiency is important Prevention of iron deficiency is important Supplemental oxygen reduces episodes of dyspnea Supplemental oxygen reduces episodes of dyspnea Lung transplantation (with repair of cardiac defect) or Lung transplantation (with repair of cardiac defect) or heart/lung transplantation is an optionheart/lung transplantation is an option

Tetralogy of FallotTetralogy of Fallot

Etienne-Louis Arthur Etienne-Louis Arthur Fallot made the first Fallot made the first published bedside published bedside diagnosis that was diagnosis that was proven at post-proven at post-mortem in 1888 and mortem in 1888 and called the condition called the condition of ‘of ‘maladie bleuemaladie bleue’.’.

EpidemiologyEpidemiologyPrevalence varies from 0.26-0.48/1000 live births.Prevalence varies from 0.26-0.48/1000 live births.

Exact incidence of TOF with PS versus pulmonary Exact incidence of TOF with PS versus pulmonary atresia or absent pulmonary valve is not known as most atresia or absent pulmonary valve is not known as most studies lump them together.studies lump them together.

Most recent study is the BWIS (Baltimore –Washington Most recent study is the BWIS (Baltimore –Washington Infant Study)Infant Study)

--Incidence 0.33/1000 live birthsIncidence 0.33/1000 live births

-Fifth most common defect overall (6.8% of all CHD)-Fifth most common defect overall (6.8% of all CHD)

-Most common cyanotic CHD-Most common cyanotic CHD

-TOF with PS 0.26/1000 live births (5.4% of all CHD)-TOF with PS 0.26/1000 live births (5.4% of all CHD)

-Suggestion of male predominance ( p=NS)-Suggestion of male predominance ( p=NS)

GeneticsGenetics

Syndromes associated with TOF:Syndromes associated with TOF:– DiGeorge’s DiGeorge’s – VelocardiofacialVelocardiofacial– Alagille’sAlagille’s– Trisomy 21/18/13Trisomy 21/18/13– Cat EyeCat Eye– San Luis ValleySan Luis Valley

Mutations of Nkx 2.5 may be associated with Mutations of Nkx 2.5 may be associated with TOF.TOF.

MorphologyMorphology

Ventricular Septal Defect– 80% of cases fibrous

continuation between the mitral, tricuspid, and aortic valves

– 20% cases there is a muscular rim around the defect.

– Rarely subarterial defect due to hypoplasia of the outlet septum.

– The pulmonary valve annulus tends to be hypoplastic or atretic.


Overriding AortaOverriding Aorta– Degree of override can Degree of override can

vary from an exclusive vary from an exclusive connection of the right connection of the right ventricle to an ventricle to an exclusive connection exclusive connection to the left ventricle.to the left ventricle.

Pulmonary Stenosis:Pulmonary Stenosis:– InfundibularInfundibular– valvularvalvular


Concentric right ventricular hypertrophy: – Secondary to RV

outflow obstruction

Associated LesionsAssociated LesionsPulmonary valvar stenosisPulmonary valvar stenosis

Pulmonary atresiaPulmonary atresia

Absence of pulmonary valve leafletsAbsence of pulmonary valve leaflets– pulmonary insufficiency in utero and marked dilatation of the pulmonary insufficiency in utero and marked dilatation of the

pulmonary trunk.pulmonary trunk.

Right aortic archRight aortic arch

Patent foramen ovalePatent foramen ovale

Atrial Septal Defect ( Pentalogy of Fallot)Atrial Septal Defect ( Pentalogy of Fallot)

Anomalous origin of the LAD from the RCA.Anomalous origin of the LAD from the RCA.

On ExamOn Exam

Cyanosis of varying degreesCyanosis of varying degrees

ClubbingClubbing

Accentuated RV impulseAccentuated RV impulse

Second heart sound usually single and loud. If mild PS Second heart sound usually single and loud. If mild PS with normal valve apparatus S2 normally split.with normal valve apparatus S2 normally split.

Pulse pressure may be wide in the presence of Pulse pressure may be wide in the presence of aortopulmonary collaterals or PDA.aortopulmonary collaterals or PDA.

On ExamOn ExamSystolic Murmur:Systolic Murmur: – Upper left sternal border, crescendo-decrescendo or Upper left sternal border, crescendo-decrescendo or

plateauplateau– Intensity inversely proportional to severity of the right Intensity inversely proportional to severity of the right

to left shuntto left shunt– In PS with intact septum murmur directly proportional In PS with intact septum murmur directly proportional

to the severity of stenosisto the severity of stenosis– Murmur decreases in intensity during hypercyanotic Murmur decreases in intensity during hypercyanotic

spellsspells

Diastolic Murmur:Diastolic Murmur:– If AI concomitantly heard in TOF with APV syndromeIf AI concomitantly heard in TOF with APV syndrome

Tetralogy of FallotTetralogy of Fallot

CatheterizationCatheterization

Surgical repairSurgical repair

Closure of VSDClosure of VSD

Relieving the RVOTORelieving the RVOTO– Pulmonary valvotomyPulmonary valvotomy– Resection of infundibular muscleResection of infundibular muscle– RVOT or subannular patchRVOT or subannular patch– Transannular patch Transannular patch

only when PV annulus is restrictiveonly when PV annulus is restrictive– Pulmonary valve implantation Pulmonary valve implantation

usually in adolescents and adultsusually in adolescents and adults– Extracardiac conduit between the RV and PAExtracardiac conduit between the RV and PA

Angioplasty or patch augmentation of the central pulmonary Angioplasty or patch augmentation of the central pulmonary arteriesarteries

– Closure of ASD or PFOClosure of ASD or PFO

Complications After RepairComplications After Repair

Mechanical problemsMechanical problems

Significant Pulmonary Significant Pulmonary regurgitationregurgitation

RV DilatationRV Dilatation

Restrictive RVRestrictive RV

Residual RVOTOResidual RVOTO

Aneurysmal dilatation Aneurysmal dilatation of the RVOT Residual of the RVOT Residual VSDVSD

AR with or without AR with or without root dilatationroot dilatation

LV dysfunctionLV dysfunction

Infective endocarditisInfective endocarditis

Complications after repairComplications after repair

Electrical ProblemsHeart block: RBBB, Bifascicular.Complete heart block is rare.

Supraventricular arrythmia (A.flutter/A.fib)

Relatively common. A.flutter-SCD,1:1.

NSVT: Significance uncertain, ? SCD

VT

SCD

Risks for SCD/VTRisks for SCD/VT

Older age at repair

Male gender

Worse heart failure NYHA Class

Chronic RV volume overload

Presence of PR

QRS duration >180 msec

Transient complete heart block lasting more than 3 days after repair.

Management of the complicationsManagement of the complicationsSignificant PR and RV dilatationSignificant PR and RV dilatation – Pulmonic valve replacement with homograft or Pulmonic valve replacement with homograft or

porcine bioprosthesisporcine bioprosthesis– Timing of surgery is debated. Selection of Timing of surgery is debated. Selection of

asymptomatic patient ?criteria.asymptomatic patient ?criteria.– Might TV annuloplasty if concomitant moderate to Might TV annuloplasty if concomitant moderate to

severe TR.severe TR.

Restrictive RVRestrictive RV– Diuretics in the post-operative period.Diuretics in the post-operative period.

Residual RVOTOResidual RVOTO– Indicated if RVSP>2/3 systemic pressure.Indicated if RVSP>2/3 systemic pressure.– Surgery or balloon angioplasty with stenting.Surgery or balloon angioplasty with stenting.

Management of complicationsManagement of complications

Aneurysmal dilatation of the RVOTAneurysmal dilatation of the RVOT

Residual VSD:Residual VSD:– Surgical correction if Qp/Qs>2:1 or 1.5-2:1 with LV Surgical correction if Qp/Qs>2:1 or 1.5-2:1 with LV

dilatation or dysfunctionor H/O paradoxical embolidilatation or dysfunctionor H/O paradoxical emboli

Dilatation of aortic root:Dilatation of aortic root:– Aortic root replacement when ascending aorta>55 Aortic root replacement when ascending aorta>55

mm and/or in the presence severe AI.mm and/or in the presence severe AI.

Management of the ComplicationsManagement of the Complications

Heart block:Heart block:– Patients with bifascicular block with prolonged PR Patients with bifascicular block with prolonged PR

(>0.20 msec) should have holter or EPS. If true (>0.20 msec) should have holter or EPS. If true trifascicular block is documented then pacemaker.trifascicular block is documented then pacemaker.

SVTSVT– Correction of target residual hemodynamic lesions.Correction of target residual hemodynamic lesions.– RF ablation for a.flutter or reentrant tachycardia either RF ablation for a.flutter or reentrant tachycardia either

percutaneously or during surgery.percutaneously or during surgery.– Biatrial maze for a.fib.Biatrial maze for a.fib.– Anti-arrhythmic meds /antitachycardia pacemakers Anti-arrhythmic meds /antitachycardia pacemakers

can be used as adjunctivetools.can be used as adjunctivetools.

Management of ComplicationsManagement of ComplicationsVTVT– Correction of the hemodynamic lesionsCorrection of the hemodynamic lesions– RF ablation of VT focusRF ablation of VT focus– Role of AICD (?)Role of AICD (?)– Antiarrhythmic meds for symptomatic patients.Antiarrhythmic meds for symptomatic patients.

SCDSCD– Resuscitated SCD-AICDResuscitated SCD-AICD– Role of prophylactic AICD (?)Role of prophylactic AICD (?)

Fontan procedure-requiring patientFontan procedure-requiring patient

Tricuspid atresiaTricuspid atresia

Hypoplastic left ventricleHypoplastic left ventricle

Double inlet ventricleDouble inlet ventricle

IsomerismIsomerism

Tricuspid atresiaTricuspid atresia

Tricuspid AtresiaTricuspid Atresia

On EchoOn Echo

Hypoplastic Left HeartHypoplastic Left Heart

Double Inlet VentricleDouble Inlet Ventricle

Fontan procedureFontan procedure

10 year survival is 60-70%10 year survival is 60-70%

Poor prognosisPoor prognosis– 5 year survival 46-59%5 year survival 46-59%

Fontan PatientFontan PatientLong term complications includeLong term complications include– Atrial flutter or fibrillationAtrial flutter or fibrillation

Incidence increases with timeIncidence increases with time

– Right atrial thrombus formationRight atrial thrombus formation

– Obstruction of the Fontan circuitObstruction of the Fontan circuitShould be r/o in all pts presenting with atrial arrhythmiasShould be r/o in all pts presenting with atrial arrhythmias

– Ventricular dysfunctionVentricular dysfunction

– Protein losing enteropathyProtein losing enteropathyEtiology unclear: pleural effusions, ascites, edema and chronic Etiology unclear: pleural effusions, ascites, edema and chronic diarrhea. Low S.Albumin, high diarrhea. Low S.Albumin, high αα-1 antitrypsin stool clearance.-1 antitrypsin stool clearance.

Management of ComplicationManagement of Complication

In patients with atrial fibrillation and flutterIn patients with atrial fibrillation and flutter

– Prompt TEE followed by cardioversionPrompt TEE followed by cardioversion

– Maintenance of sinus with drugs ( Amiodarone) <50% Maintenance of sinus with drugs ( Amiodarone) <50% success rate.success rate.

– Transcatheter atrial ablation <50% success rateTranscatheter atrial ablation <50% success rate

– Recalcitrant cases surgical revision with Recalcitrant cases surgical revision with antiarrhythmic surgeryantiarrhythmic surgery

Management of ComplicationsManagement of Complications

In patients with established thrombusIn patients with established thrombus– Thrombolytic therapyThrombolytic therapy

– Surgical thrombectomySurgical thrombectomy

– Conversion of Fontan circuitConversion of Fontan circuit

– Long term anticoagulationLong term anticoagulation

– Many centers prophylactically anticoagulate all Many centers prophylactically anticoagulate all patients with Fontan.patients with Fontan.

Management of complicationsManagement of complications

In patients with Fontan obstructionIn patients with Fontan obstruction– Surgical revisionSurgical revision– Balloon angioplastyBalloon angioplasty

Protein losing enteropathyProtein losing enteropathy– Creation of a fenestration in the atrial septumCreation of a fenestration in the atrial septum– Heparin SQHeparin SQ– IV Octreotide IV Octreotide – PrednisonePrednisone

In ECHOIn ECHO

The presence or absence of right atrial stasis, The presence or absence of right atrial stasis, thrombus, patency of a fenestration, and Fontan thrombus, patency of a fenestration, and Fontan circuit obstruction should be sought. circuit obstruction should be sought.

Superior and inferior vena cavae biphasic and Superior and inferior vena cavae biphasic and pulmonary artery triphasic flow patterns suggest pulmonary artery triphasic flow patterns suggest unobstructed flow in the Fontan circuit.unobstructed flow in the Fontan circuit.

Mean gradient between the Fontan circuit and Mean gradient between the Fontan circuit and the pulmonary artery of the pulmonary artery of 2 mm Hg2 mm Hg or more may or more may represent significant obstruction. represent significant obstruction.

Conduit or Lateral Tunnel FontanConduit or Lateral Tunnel Fontan

Fenestration FontanFenestration Fontan

TGATGA

D loopD loop

L loopL loop

RV follow the LoopRV follow the Loop– D loop------RV on the rightD loop------RV on the right– L loop------RV on the leftL loop------RV on the left

L TGAL TGA

Congenitally Congenitally corrected TGA.corrected TGA.

Ventricular inversionVentricular inversion

D TGAD TGAIncidence 20-30/1000,000 live births.Incidence 20-30/1000,000 live births.

Without treatment 30% die within 1st week, 50% within Without treatment 30% die within 1st week, 50% within 1st month, 70% in 6 months and 90% in first year.1st month, 70% in 6 months and 90% in first year.

With current medical and surgical interventions 90% With current medical and surgical interventions 90% early and midterm survival.early and midterm survival.

In complete Transposition the connection between atria In complete Transposition the connection between atria and ventricles are normal.and ventricles are normal.

The connections between the ventricles and the great The connections between the ventricles and the great arteries are discordant.arteries are discordant.

D TGAD TGA

SurgerySurgery

Most commonly done procedures were Most commonly done procedures were atrial atrial switchswitch operations: operations:

– Blood redirected at atrial level with a baffle made of Blood redirected at atrial level with a baffle made of dacron or pericardium dacron or pericardium (Mustard)(Mustard)

– With atrial flaps With atrial flaps (Senning)(Senning) to achieve physiological to achieve physiological correctioncorrection

SurgerySurgery

Current practice is theCurrent practice is the arterial switcharterial switch operation operation developed in 1980’s.developed in 1980’s.

Blood is redirected at the level of the great Blood is redirected at the level of the great artery artery – the morphological left ventricle becomes the subaortic the morphological left ventricle becomes the subaortic

ventricle ventricle – the morphological right ventricle becomes the the morphological right ventricle becomes the

subpulmonic ventricle.subpulmonic ventricle.

Atrial Switch Surgery (Mustard/Senning Atrial Switch Surgery (Mustard/Senning Procedure)Procedure)

Mustard ProcedureMustard Procedure

Modified Arterial Switch OperationModified Arterial Switch Operation

Consequences of MustardConsequences of Mustard

Most patients reaching adulthood have NYHA I/II Most patients reaching adulthood have NYHA I/II symptoms over the next 25 years.symptoms over the next 25 years.

50% develop moderate systolic dysfunction of the RV 50% develop moderate systolic dysfunction of the RV but only few present with CHF.but only few present with CHF.

1/3rd have severe systemic TR.1/3rd have severe systemic TR.

Atrial flutter arises in 20% by age 20.Atrial flutter arises in 20% by age 20.

50% patients have sinus node dysfunction by age 20.50% patients have sinus node dysfunction by age 20.

Baffle leak or obstruction can also occur.Baffle leak or obstruction can also occur.

Baffle LeakBaffle Leak

Consequences of Arterial SwitchConsequences of Arterial Switch

Data about long term consequences still Data about long term consequences still unavailable.unavailable.

Concerns include:Concerns include:– supra neopulmonary artery stenosissupra neopulmonary artery stenosis– ostial coronary artery disease ostial coronary artery disease – progressive neoaortic valve regurgitationprogressive neoaortic valve regurgitation

TreatmentTreatment

Use of ACE inhibitors suggests a benefit in non-Use of ACE inhibitors suggests a benefit in non-randomized studies.randomized studies.– randomized study using randomized study using Ramipril Ramipril is underwayis underway..

Failed Mustard or Senning might be a reason for heart Failed Mustard or Senning might be a reason for heart transplantation.transplantation.

Alternatively, atrial baffle can be converted to an arterial Alternatively, atrial baffle can be converted to an arterial switch after retraining the LV with a pulmonary band.switch after retraining the LV with a pulmonary band.– No data available for outcomesNo data available for outcomes

TV replacement is not recommended since it worsens RV TV replacement is not recommended since it worsens RV function.function.

Ebstein’s anomalyEbstein’s anomalyMay be associated with:

•PFO•ASD

•Most commonly secundum rarely primum

•VSD•with or without pulmonary atresia

•Pulmonary outflow obstruction•PDA•Coarctation of the aorta•LV cardiomyopathy

Ebstein’s AnomalyEbstein’s AnomalyPrevalencePrevalence 1:50,000 to 100,000. Occurs with higher 1:50,000 to 100,000. Occurs with higher

frequency in infants of mothers who took Lithium in frequency in infants of mothers who took Lithium in early pregnancy.early pregnancy.

Clinical FeaturesDepends on the severity of the pathology and the magnitude of right to left intraatrial shunting.

In milder forms of the condition incidental murmur and arrhythmia may be the only features.

In severe forms of the disease cyanosis is seen and depends on the extent of right to left shunting.

Increased incidence of WPW syndrome.

EchoEchoIncrease in RV volumeIncrease in RV volume

Paradoxical septal motionParadoxical septal motion

Increase in TV excursionIncrease in TV excursion

Delay in TV closure Delay in TV closure compared to MV closurecompared to MV closure

Decreased TV EF slopeDecreased TV EF slope

Abnormal anterior Abnormal anterior position of the TV in position of the TV in diastolediastole

Prognosis and ManagementPrognosis and Management

Survival at 1 year 67% and 10 year 59 %.Survival at 1 year 67% and 10 year 59 %.

Survival after Tricuspid bioprosthesis: 10 yr 93% Survival after Tricuspid bioprosthesis: 10 yr 93% and 15 yr 81%. 94% in NYHA I/II.and 15 yr 81%. 94% in NYHA I/II.

Management consists of surgical repair of the Management consists of surgical repair of the TV or replacement in older individuals.TV or replacement in older individuals.

PregnancyPregnancy

BP decrease early in pregnancy ~10 mmHg, BP decrease early in pregnancy ~10 mmHg, usually secondary to decreased SVRusually secondary to decreased SVR

This increases flow across right to left shunts.This increases flow across right to left shunts.

30-50% increase in intravascular volume. Poorly 30-50% increase in intravascular volume. Poorly tolerated by women with valvular lesions and tolerated by women with valvular lesions and LV/RV dysfunction.LV/RV dysfunction.

Marked fluctuations occur in CO during normal Marked fluctuations occur in CO during normal labor and delivery.labor and delivery.

PregnancyPregnancy

Major maternal cardiac risk factorsMajor maternal cardiac risk factorsPulmonary HTNPulmonary HTN -Maternal mortality 30-50% with Eisenmenger syndrome.-Maternal mortality 30-50% with Eisenmenger syndrome.

Maternal CyanosisMaternal Cyanosis-Cyanotic worse then acyanotic. -Cyanotic worse then acyanotic. -Erythrocytosis worsens outcome. -Erythrocytosis worsens outcome. -Goal Hct<65-Goal Hct<65

Poor maternal functional classPoor maternal functional class -Difficult to assess in cyanotic CHD.-Difficult to assess in cyanotic CHD.

H/O ArrhythmiaH/O Arrhythmia-Antiarrhythmic drugs can be used with accepted risk for debilitating -Antiarrhythmic drugs can be used with accepted risk for debilitating symptomssymptoms

AnticoagulationAnticoagulation

Specific PatientsSpecific PatientsTOF TOF – Uncorrected TOF associated with worsening cyanosis. Uncorrected TOF associated with worsening cyanosis.

Susceptible to Infective endocarditis. Susceptible to Infective endocarditis. – Corrected TOF usually uncomplicated pregnancyCorrected TOF usually uncomplicated pregnancy

Ebstein’s AnomalyEbstein’s Anomaly-Worsening RV function in pregnancy-Worsening RV function in pregnancy-Atrial tachyarrhythmias/ WPW-Atrial tachyarrhythmias/ WPW-Cyanosis might be manifest for the first time-Cyanosis might be manifest for the first time-Increased risk of paradoxical embolization and hypoxemia-Increased risk of paradoxical embolization and hypoxemia-Acyanotic women with Ebstein’s tolerate pregnancy well if -Acyanotic women with Ebstein’s tolerate pregnancy well if sinus rhythm is maintained.sinus rhythm is maintained.-Repaired Ebstein’s significantly reduces risks-Repaired Ebstein’s significantly reduces risks

Specific patientsSpecific patients

Congenitally corrected TGA (L-TGA)Congenitally corrected TGA (L-TGA)-Increased risk of ventricular volume overload-Increased risk of ventricular volume overload-Increased risk of heart block-Increased risk of heart block

Complete TGAComplete TGA-Atrial baffle- Risks are related to functional status of the -Atrial baffle- Risks are related to functional status of the RV, PAP and rhythm disturbances.RV, PAP and rhythm disturbances.

-Not enough experience with arterial switch.-Not enough experience with arterial switch.

FontanFontan-Usually leads to uncomplicated pregnancy.-Usually leads to uncomplicated pregnancy.-Risk of thrombus formation.-Risk of thrombus formation.

Labor and deliveryLabor and deliveryWomen with unoperated or operated CHD who are functionally Women with unoperated or operated CHD who are functionally normal can be allowed to progress to spontaneous labornormal can be allowed to progress to spontaneous labor

If functional adequacy of heart and lungs in doubt: If functional adequacy of heart and lungs in doubt: -Controlled induction-Controlled induction-Oxytocin low dose for induction.-Oxytocin low dose for induction.-Avoid maternal pushing-Avoid maternal pushing-Assisted delivery with low forceps or vacuum extraction.-Assisted delivery with low forceps or vacuum extraction.

AnesthesiaAnesthesia-IM/IV narcotics safe.-IM/IV narcotics safe.-Epidural safe to use :caution systemic venous pooling and -Epidural safe to use :caution systemic venous pooling and increased thromboembolism risk.increased thromboembolism risk.

Labor and deliveryLabor and delivery

No indication for routine Swan GanzNo indication for routine Swan Ganz

Routine endocarditis prophylaxisRoutine endocarditis prophylaxis

Cesarean delivery reserved for obstetric indicationsCesarean delivery reserved for obstetric indications-A/W twice the amount of blood loss-A/W twice the amount of blood loss-Increased risks of wound and uterine infection-Increased risks of wound and uterine infection-Increased incidence of post-op thrombophlebitis.-Increased incidence of post-op thrombophlebitis.

BreastfeedingBreastfeeding-Affects cardiac reserve and risks mastitis and -Affects cardiac reserve and risks mastitis and bacteremia.bacteremia.

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congenital heart diseases ii

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