Pregnancy Management Guidelines in Women with Cardiac Diseases

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Pregnancy Management Guidelines in Women with Cardiac Diseases BY BY Jameel Alata , MD Jameel Alata , MD Consultant pediatric Consultant pediatric cardiologist, cardiologist, KAAUH / KFSH&RC Jeddah, KAAUH / KFSH&RC Jeddah, KSA. KSA. 32 ESC annual meeting in association with PACHDA Cairo, 22-25 FEB 2005

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Pregnancy Management Guidelines in Women with Cardiac Diseases. BY Jameel Alata , MD Consultant pediatric cardiologist, KAAUH / KFSH&RC Jeddah, KSA. 32 ESC annual meeting in association with PACHDA Cairo, 22-25 FEB 2005. Introduction;. - PowerPoint PPT Presentation

Transcript of Pregnancy Management Guidelines in Women with Cardiac Diseases

Page 1: Pregnancy Management Guidelines in Women with Cardiac Diseases

Pregnancy Management Guidelines in Women with

Cardiac Diseases

BY BY

Jameel Alata , MDJameel Alata , MD

Consultant pediatric cardiologist,Consultant pediatric cardiologist,

KAAUH / KFSH&RC Jeddah, KSA.KAAUH / KFSH&RC Jeddah, KSA.32 ESC annual meeting in association with PACHDA

Cairo, 22-25 FEB 2005

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•Introduction;

Pregnancy in most women with heart disease has a Pregnancy in most women with heart disease has a favourable maternal and fetal outcome.favourable maternal and fetal outcome.

With the exception of patients withWith the exception of patients with Eisenmenger Eisenmenger syndrome, pulmonary vascular obstructive disease,syndrome, pulmonary vascular obstructive disease, and and Marfan syndrome with aortopathy, maternal death during Marfan syndrome with aortopathy, maternal death during pregnancypregnancy in women with heart disease is rare.in women with heart disease is rare.

However, pregnant womenHowever, pregnant women with heart disease do remain at with heart disease do remain at risk for other complications includingrisk for other complications including heart failure, heart failure, arrhythmia, and stroke. arrhythmia, and stroke.

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•Introduction;

Women with congenital heartWomen with congenital heart disease now disease now comprise the majority of pregnant women with comprise the majority of pregnant women with heartheart disease seen at referral centres. disease seen at referral centres.

The next largest group includesThe next largest group includes women with women with rheumatic heart disease.rheumatic heart disease.

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•Introduction;

Approximately 10% of all maternal deaths in the United Approximately 10% of all maternal deaths in the United StatesStates can be attributed to cardiac disease. can be attributed to cardiac disease.

In one study of 1,000In one study of 1,000 pregnant women who had various pregnant women who had various types of cardiac disease andtypes of cardiac disease and were followed by the same were followed by the same health-care team over a 10-year period,health-care team over a 10-year period, more than 75% more than 75% of the women had no complications during pregnancy.of the women had no complications during pregnancy.

The remaining 25%, the following complications wereThe remaining 25%, the following complications were

seen most often: seen most often:

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•Introduction; Congestive heart failure, including pulmonary edema (12.3%)Congestive heart failure, including pulmonary edema (12.3%)

Cardiac arrhythmias (6%)Cardiac arrhythmias (6%)

Thromboembolism (1.9%)Thromboembolism (1.9%)

Angina (1.4%)Angina (1.4%)

Hypoxemia (0.7%)Hypoxemia (0.7%)

Infective endocarditisInfective endocarditis (0.5%)(0.5%)

The overall maternal mortality rate in this group was 2.7%,The overall maternal mortality rate in this group was 2.7%, and the stillbirth and the stillbirth and spontaneous abortion rate was 7.7%.and spontaneous abortion rate was 7.7%.

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•Introduction;

Cardiac disease covers a wide range of conditions, Cardiac disease covers a wide range of conditions, includingincluding congenital heart disease, acquired congenital heart disease, acquired disease such as rheumaticdisease such as rheumatic valvular disease, and valvular disease, and coronary disease. coronary disease.

It is estimated thatIt is estimated that 1% to 3% of women either have 1% to 3% of women either have cardiac disease entering pregnancycardiac disease entering pregnancy or are or are diagnosed with cardiac disease while they are diagnosed with cardiac disease while they are pregnant.pregnant.

The frequency of specific types depends on the The frequency of specific types depends on the patient populationpatient population and local conditions. and local conditions.

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•Introduction;

Advances in the diagnosis and treatmentAdvances in the diagnosis and treatment of of congenital heart disease have increased the survival congenital heart disease have increased the survival raterate of children affected with these disorders. of children affected with these disorders.

Pregnant women who have congenital heartPregnant women who have congenital heart disease disease represent the largest number of patients seen at represent the largest number of patients seen at somesome referral centers, comprising as many as 70% referral centers, comprising as many as 70% to 80% of all theto 80% of all the cardiac patients.cardiac patients.

It is estimated that 1It is estimated that 1 in 10,000 pregnancies is in 10,000 pregnancies is associated with coronary heart disease,associated with coronary heart disease, notably notably myocardial infarction.myocardial infarction.

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•Cardiovascular Physiology of Pregnancy;

Normal pregnancy is associated with of 30 to 50Normal pregnancy is associated with of 30 to 50 percent in percent in blood volume and a corresponding increase in cardiacblood volume and a corresponding increase in cardiac

output.output.

These increases begin during the first trimester; theThese increases begin during the first trimester; the levels levels peak by 20 to 24 weeks of pregnancy and then are eitherpeak by 20 to 24 weeks of pregnancy and then are either

sustained until term or decrease. sustained until term or decrease.

The heart rateThe heart rate increases by 10 to 20 beats per minute, the increases by 10 to 20 beats per minute, the stroke volume increases,stroke volume increases, and there is a substantial reduction and there is a substantial reduction in systemic vascular resistance,in systemic vascular resistance, with decreases in blood with decreases in blood pressure. pressure.

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•Cardiovascular Physiology of Pregnancy;

During labor, cardiac outputDuring labor, cardiac output increases; the blood increases; the blood pressure increases with uterine contractions.pressure increases with uterine contractions.

Immediately after delivery, the cardiac filling pressure Immediately after delivery, the cardiac filling pressure maymay increase dramatically due to the decompression of increase dramatically due to the decompression of the vena cavathe vena cava and the return of uterine blood into the and the return of uterine blood into the systemic circulation.systemic circulation.

The cardiovascular adaptations associated with The cardiovascular adaptations associated with pregnancy regresspregnancy regress by approximately six weeks after by approximately six weeks after delivery.delivery.

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•Cardiovascular Physiology of Pregnancy;

Functional Murmurs develop in nearly all women Functional Murmurs develop in nearly all women during pregnancy.during pregnancy.

Echocardiography is warranted when diastolic Echocardiography is warranted when diastolic murmurs, continuousmurmurs, continuous murmurs, or loud systolic murmurs, or loud systolic murmurs (louder than grade 2 on themurmurs (louder than grade 2 on the 6-point scale) 6-point scale) are detected or when murmurs are associated withare detected or when murmurs are associated with

symptoms or an abnormal electrocardiogramsymptoms or an abnormal electrocardiogram

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•Cardiovascular Physiology of Pregnancy;

In normal pregnantIn normal pregnant women, serial echocardiography usually women, serial echocardiography usually demonstrates minor increasesdemonstrates minor increases in the left and right ventricular in the left and right ventricular diastolic dimensions, whichdiastolic dimensions, which remain within the normal range.remain within the normal range.

Slight decrease in theSlight decrease in the left ventricular end-systolic dimension and left ventricular end-systolic dimension and a minimal increasea minimal increase in the size of the left atrium is also noted.in the size of the left atrium is also noted.

The state of increased volumeThe state of increased volume also results in increased also results in increased transvalvular flow velocities.transvalvular flow velocities.

MinorMinor degrees of atrioventricular valve regurgitation are normal.degrees of atrioventricular valve regurgitation are normal.

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•Cardiovascular Physiology of Pregnancy;

During labour and delivery, pain and uterineDuring labour and delivery, pain and uterine

contractions result in additional increases in contractions result in additional increases in cardiac output ( 15% with each contraction )cardiac output ( 15% with each contraction ) and and blood pressure. blood pressure.

Immediately following delivery, relief ofImmediately following delivery, relief of caval caval compression and autotransfusion from the emptied compression and autotransfusion from the emptied and contractedand contracted uterus produce a further increase in uterus produce a further increase in cardiac output ( upto 45 % ).cardiac output ( upto 45 % ).

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•Other physiologic changes;

Hypercoaguble state.Hypercoaguble state. Hypoalbumineamia.Hypoalbumineamia. Insulin resistant state.Insulin resistant state. Increased red c.ell massIncreased red c.ell mass Increased ESR.Increased ESR. Increased renal blood flow ( 30 % ).Increased renal blood flow ( 30 % ). Increased hepatic clearance of medications.Increased hepatic clearance of medications.

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•Outcome of pregnancy with CHD

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•Outcome ; LT to RT shunts In the absence of pulmonary hypertension, pregnancy, In the absence of pulmonary hypertension, pregnancy,

labour and delivery are well tolerated 2labour and delivery are well tolerated 2ndnd to attenuation of to attenuation of volume overload by peripheral vasodilation.volume overload by peripheral vasodilation.

However arrhythmias, ventricular dysfunction, and However arrhythmias, ventricular dysfunction, and progression of pulmonary hypertension may occur, progression of pulmonary hypertension may occur, especially when the shunt is large or when there is pre-especially when the shunt is large or when there is pre-existing elevation of pulmonary artery pressure. existing elevation of pulmonary artery pressure.

In ASDs, paradoxical embolisation may be encountered if In ASDs, paradoxical embolisation may be encountered if systemic vasodilatation and/or elevation of pulmonary systemic vasodilatation and/or elevation of pulmonary resistance promote transient right to left shunting. resistance promote transient right to left shunting.

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•Outcome ;AS, COA & LVOTO

The absence of symptoms antepartum is not sufficient The absence of symptoms antepartum is not sufficient assurance that pregnancy will be well tolerated. assurance that pregnancy will be well tolerated.

Pregnant women with severe aortic stenosis havePregnant women with severe aortic stenosis have l limited imited ability to augment cardiac output, ability to augment cardiac output, eelevation of left levation of left ventricular systolic and filling pressures& ventricular systolic and filling pressures& nnon-compliant, on-compliant, hypertrophied ventricle is sensitive to falls in preload leads hypertrophied ventricle is sensitive to falls in preload leads to:to:

CHF , HYPOTENSION & or ISCHEMIA CHF , HYPOTENSION & or ISCHEMIA

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•Outcome ;AS, COA & LVOTO

In a compilation of many earlier small In a compilation of many earlier small retrospective series, 65 patients were followed retrospective series, 65 patients were followed through 106 pregnancies with a maternal mortality through 106 pregnancies with a maternal mortality of 11% and a perinatal mortality of 4%.of 11% and a perinatal mortality of 4%.

In 25 pregnancies managed recently, there was no In 25 pregnancies managed recently, there was no maternal mortality but deterioration of maternal maternal mortality but deterioration of maternal functional status occurred in 5 (20%).functional status occurred in 5 (20%).

In the absence of prosthetic dysfunction or In the absence of prosthetic dysfunction or residual aortic stenosis, patients with bioprosthetic residual aortic stenosis, patients with bioprosthetic aortic valves usually tolerate pregnancy well.aortic valves usually tolerate pregnancy well.

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•Outcome ;AS, COA & LVOTO

Ross procedure reported favourable maternal and fetal Ross procedure reported favourable maternal and fetal outcomes except in one woman who developed postpartum outcomes except in one woman who developed postpartum left ventricular dysfunction.left ventricular dysfunction.

Pregnancy in a woman with a mechanical valve prosthesis Pregnancy in a woman with a mechanical valve prosthesis carries increased risk of valve thrombosis as a result of the carries increased risk of valve thrombosis as a result of the hypercoagulable state. hypercoagulable state.

The magnitude of this increased risk (3-14%) is greater if The magnitude of this increased risk (3-14%) is greater if subcutaneous unfractionated heparin rather than warfarin subcutaneous unfractionated heparin rather than warfarin is used as the anticoagulant agent.is used as the anticoagulant agent.

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Outcome ; COA Maternal mortality with uncorrected coarctation has Maternal mortality with uncorrected coarctation has

been reported as 3% in an early series.been reported as 3% in an early series.

Aortopathy, or longstanding hypertension; aortic Aortopathy, or longstanding hypertension; aortic rupture accounted for eight of the 14 reported deaths rupture accounted for eight of the 14 reported deaths and occurred in the third trimester as well as in the and occurred in the third trimester as well as in the postpartum period..postpartum period..

More recently, a preliminary report described More recently, a preliminary report described encouraging maternal and fetal outcome in encouraging maternal and fetal outcome in 87 pregnancies, with no maternal deaths and one early 87 pregnancies, with no maternal deaths and one early neonatal death.neonatal death.

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•Outcome ; COA

The management of hypertension in uncorrected The management of hypertension in uncorrected coarctation is particularly problematic in coarctation is particularly problematic in pregnancy because satisfactory control of upper pregnancy because satisfactory control of upper body hypertension may lead to excessive body hypertension may lead to excessive hypotension below the coarctation site, hypotension below the coarctation site, compromising the fetus. compromising the fetus.

Intrauterine growth restriction and premature Intrauterine growth restriction and premature labour and delivery are more common. Following labour and delivery are more common. Following coarctation repair, the risk of dissection and coarctation repair, the risk of dissection and rupture is likely reduced but not eliminated. rupture is likely reduced but not eliminated.

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•Outcome ; Pulmonary stenosis

Mild pulmonic stenosis, or pulmonic stenosis that has been Mild pulmonic stenosis, or pulmonic stenosis that has been alleviated by valvuloplasty or surgery, is well tolerated alleviated by valvuloplasty or surgery, is well tolerated during pregnancy and fetal outcome is favourable. during pregnancy and fetal outcome is favourable.

Severe pulmonic stenosis may be asymptomatic , but may Severe pulmonic stenosis may be asymptomatic , but may precipitate right heart failure or atrial arrhythmias; such precipitate right heart failure or atrial arrhythmias; such condition should be considered for correction before condition should be considered for correction before pregnancy.pregnancy.

Even during pregnancy, balloon valvuloplasty may be Even during pregnancy, balloon valvuloplasty may be feasible .feasible .

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Outcome; Cyanotic CHD

Uncorrected or palliated pregnant patients with cyanotic Uncorrected or palliated pregnant patients with cyanotic congenital heart disease such as tetralogy of Fallot, single congenital heart disease such as tetralogy of Fallot, single ventricle,ventricle, etc, the usual pregnancy associated fall in etc, the usual pregnancy associated fall in systemic vascularsystemic vascular resistance and rise in cardiac output resistance and rise in cardiac output exacerbate right to leftexacerbate right to left shunting leading to increased shunting leading to increased maternal hypoxaemia and cyanosis.maternal hypoxaemia and cyanosis.

Outcomes of 96 pregnancies in 44Outcomes of 96 pregnancies in 44  women with a variety of women with a variety of cyanotic congenital heart defects reportedcyanotic congenital heart defects reported a high rate of a high rate of maternal cardiac events (32%, including one death).maternal cardiac events (32%, including one death).

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Outcome; Cyanotic CHD

Prematurity (37%), and a low live birth rate (43%).Prematurity (37%), and a low live birth rate (43%).

The lowestThe lowest live birth rate (12%) was observed in those live birth rate (12%) was observed in those mothers with an arterialmothers with an arterial oxygen saturation of  85%. oxygen saturation of  85%.

Pregnancy risk is low in women who havePregnancy risk is low in women who have had successful had successful correction of tetralogy without residuals.correction of tetralogy without residuals.

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Outcome; Cyanotic CHD

For For Atrial repair (Mustard orAtrial repair (Mustard or SenningSenning procedure) procedure) 43 pregnancies in 31 women described in recent 43 pregnancies in 31 women described in recent reports,reports, showed one late maternal death.showed one late maternal death.

There was a 14% incidenceThere was a 14% incidence of maternal heart of maternal heart failure, arrhythmias, or cardiac deterioration.failure, arrhythmias, or cardiac deterioration.

Few recipients of the current repair of choice for Few recipients of the current repair of choice for complete transposition thecomplete transposition the arterial switch arterial switch procedure have yet reached reproductiveprocedure have yet reached reproductive age. age.

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Outcome; Cyanotic CHD The Fontan operation eliminates cyanosis and volume The Fontan operation eliminates cyanosis and volume

overload .overload . A recent review of 33 pregnanciesA recent review of 33 pregnancies in 21 women showed in 21 women showed

15 (45%) term pregnancies with no maternal mortality 15 (45%) term pregnancies with no maternal mortality althoughalthough two women had cardiac complications .two women had cardiac complications .

The incidence of firstThe incidence of first trimester miscarriage was high trimester miscarriage was high (39%).(39%).

Since the 10 year survivalSince the 10 year survival rate following the Fontan rate following the Fontan operation is only 60-80%, it is importantoperation is only 60-80%, it is important to discuss to discuss prognosis during preconceptionprognosis during preconception counselling. counselling.

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Outcome; Marfan

Medial aortopathy resulting in dilatation, Medial aortopathy resulting in dilatation, dissection,dissection, and valvar regurgitation risks are and valvar regurgitation risks are increased in pregnancy becauseincreased in pregnancy because of haemodynamic of haemodynamic stress and perhaps hormonal effects. stress and perhaps hormonal effects.

Recently 45 pregnanciesRecently 45 pregnancies in 21 patients reported in 21 patients reported no increase in obstetrical complicationsno increase in obstetrical complications or or significant change in aortic root size in the patients significant change in aortic root size in the patients withwith normal aortic roots.normal aortic roots.

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Outcome; Marfan

The eight patients with aThe eight patients with a dilated aortic root dilated aortic root (> 40 mm) or prior aortic root surgery, three(> 40 mm) or prior aortic root surgery, three of their of their nine pregnancies were complicated by either aortic nine pregnancies were complicated by either aortic dissectiondissection (two) or rapid aortic dilatation (one).(two) or rapid aortic dilatation (one).

In contrast, women with little cardiovascular In contrast, women with little cardiovascular involvementinvolvement and with normal aortic root diameter and with normal aortic root diameter may tolerate pregnancy well.may tolerate pregnancy well.

Serial echocardiography should beSerial echocardiography should be used to identify used to identify progressive aortic root dilatation and prophylacticprogressive aortic root dilatation and prophylactic B-blockers should beB-blockers should be administered. administered.

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Outcome; L-TGA

Potential problems in pregnancy include Potential problems in pregnancy include dysfunction ofdysfunction of the systemic Right ventricle and/or the systemic Right ventricle and/or increased Systemic AtrioVentricularincreased Systemic AtrioVentricular valve valve regurgitation with heart failure, Atrial regurgitation with heart failure, Atrial arrhythmias, andarrhythmias, and AV- block. AV- block.

41 patients,41 patients, there were 105 pregnancies with 73% there were 105 pregnancies with 73% live births and no maternallive births and no maternal mortality, although mortality, although seven patients developed either heart failure,seven patients developed either heart failure,

endocarditis, stroke, or myocardial infarction.endocarditis, stroke, or myocardial infarction.

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Outcome; Eisenmenger

A recent review of outcome of 125 pregnancies in A recent review of outcome of 125 pregnancies in patients with Eisenmenger syndrome, primary patients with Eisenmenger syndrome, primary pulmonary hypertension, and secondarypulmonary hypertension, and secondary

pulmonary hypertension showed;pulmonary hypertension showed; maternal mortalitymaternal mortality of of 36%, 30%, and 56%,36%, 30%, and 56%,

respectively. respectively. The overall neonatal mortality was 13% The overall neonatal mortality was 13%

The preponderance of complicationsThe preponderance of complications occurs at occurs at term and during the first postpartum week.term and during the first postpartum week.

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Outcome; Eisenmeger

PreconceptionPreconception counselling should stress the counselling should stress the extreme pregnancy associated risks.extreme pregnancy associated risks.

Termination of pregnancy should always be Termination of pregnancy should always be offered to such patients,offered to such patients, as should sterilisation. as should sterilisation.

The vasodilation associated with pregnancyThe vasodilation associated with pregnancy will will increase the degree of right to left shunting in increase the degree of right to left shunting in patientspatients with Eisenmenger syndrome, resulting in with Eisenmenger syndrome, resulting in worsening of maternalworsening of maternal cyanosis with poor fetal cyanosis with poor fetal outcomeoutcome

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Outcome; RHC Disease

Mitral stenosis is the most common rheumatic Mitral stenosis is the most common rheumatic valvar lesion encountered during pregnancy. valvar lesion encountered during pregnancy.

Patients withPatients with mild to moderate mitral stenosis, mild to moderate mitral stenosis, who are asymptomatic beforewho are asymptomatic before pregnancy, may pregnancy, may develop atrial fibrillation and heart failure duringdevelop atrial fibrillation and heart failure during

the ante- and peripartum periods. the ante- and peripartum periods.

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Outcome; RHC Disease

Earlier studies showed thatEarlier studies showed that mortality rate mortality rate increased with worsening antenatal increased with worsening antenatal maternal functionalmaternal functional class.class.

A more recent study found no mortality but A more recent study found no mortality but describeddescribed substantial morbidity from heart substantial morbidity from heart failure and arrhythmia.failure and arrhythmia.

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Outcome; RHC Disease

Pregnant women whose dominant lesion is Pregnant women whose dominant lesion is rheumatic aortic stenosis have a similar outcome rheumatic aortic stenosis have a similar outcome to those with congenital aortic stenosis.to those with congenital aortic stenosis.

Severe aortic or mitral regurgitation is generally Severe aortic or mitral regurgitation is generally well toleratedwell tolerated during pregnancy although during pregnancy although deterioration in maternal functionaldeterioration in maternal functional class has beenclass has been

observed. observed.

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Outcome; Peripartum Cardiomyopathy

Unexplained left ventricularUnexplained left ventricular systolic dysfunction, systolic dysfunction, confirmed echocardiographically, presentingconfirmed echocardiographically, presenting during during the last antepartum month or in the first five the last antepartum month or in the first five postpartumpostpartum months. months.

The relapseThe relapse rate during subsequent pregnancies is rate during subsequent pregnancies is substantial in women withsubstantial in women with evidence of persisting evidence of persisting cardiac enlargement or left ventricularcardiac enlargement or left ventricular dysfunction. dysfunction.

It remains unclear whether pregnancy is safe in It remains unclear whether pregnancy is safe in thosethose with recovery of systolic function.with recovery of systolic function.

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Management

Risk stratification.Risk stratification. Counseling.Counseling. Antepartum management.Antepartum management. Multidisiplenary, high risk units.Multidisiplenary, high risk units. Labour and delivery.Labour and delivery.

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Risk stratification

The data requiredThe data required for risk stratification can for risk stratification can be acquired readily from a thoroughbe acquired readily from a thorough

cardiovascular history and examination, cardiovascular history and examination, 12 lead ECG, and transthoracic12 lead ECG, and transthoracic

echocardiogram.echocardiogram. In patients with cyanosis, arterial oxygen In patients with cyanosis, arterial oxygen

saturationsaturation should be assessed by should be assessed by percutaneous oximetry.percutaneous oximetry.

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•Risk stratification Low riskLow risk

1- Small left to right shunts. 1- Small left to right shunts. 

2- Repaired lesions without residual cardiac dysfunction. 2- Repaired lesions without residual cardiac dysfunction. 

3- Isolated mitral valve prolapse without significant regurgitation.   3- Isolated mitral valve prolapse without significant regurgitation.  

4-Bicuspid aortic valve without stenosis. 4-Bicuspid aortic valve without stenosis. 

5-Mild to moderate pulmonic stenosis.5-Mild to moderate pulmonic stenosis.

6-  Valvar regurgitation with normal ventricular systolic function.6-  Valvar regurgitation with normal ventricular systolic function.

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•Risk stratification Intermediate riskIntermediate risk

   1- Unrepaired or palliated cyanotic congenital heart disease 1- Unrepaired or palliated cyanotic congenital heart disease 

2- Large left to right shunt 2- Large left to right shunt 

3-Uncorrected coarctation of the aorta 3-Uncorrected coarctation of the aorta 

4- Mitral or aortic stenosis 4- Mitral or aortic stenosis 

5- Mechanical prosthetic valves 5- Mechanical prosthetic valves 

6- Severe pulmonic stenosis6- Severe pulmonic stenosis

7-  Moderate to severe systemic ventricular dysfunction 7-  Moderate to severe systemic ventricular dysfunction 

8- History of peripartum cardiomyopathy with no residual ventricular dysfunction8- History of peripartum cardiomyopathy with no residual ventricular dysfunction

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•Risk stratification High riskHigh risk

   1- New York Heart Association (NYHA) class III or IV symptoms  1- New York Heart Association (NYHA) class III or IV symptoms 

2-Severe pulmonary hypertension 2-Severe pulmonary hypertension 

3- Marfan syndrome with aortic root or major valvar involvement  3- Marfan syndrome with aortic root or major valvar involvement 

4-Severe aortic stenosis 4-Severe aortic stenosis 

5- History of peripartum cardiomyopathy with residual ventricular 5- History of peripartum cardiomyopathy with residual ventricular dysfunctiondysfunction

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Counselling

In counselling, theIn counselling, the following six areas should be following six areas should be considered: considered:

The underlying cardiacThe underlying cardiac lesion,lesion, Maternal functional status, Maternal functional status, The possibility of furtherThe possibility of further palliative or corrective palliative or corrective

surgery, surgery, Additional associated risk factors,Additional associated risk factors,

Maternal life expectancy ,Maternal life expectancy , Ability to care for a child,Ability to care for a child,

The risk of congenital heart disease inThe risk of congenital heart disease in offspring. offspring.

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Antepartum Management

Issues are:Issues are: Congestive heart failure, Congestive heart failure, Arrhythmias,Arrhythmias, Thrombosis,Thrombosis,

Emboli, andEmboli, and Adverse effects ofAdverse effects of Anticoagulants. Anticoagulants.

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CHF

Activity limitation is helpful and in severely Activity limitation is helpful and in severely affected women with NYHA class IIIaffected women with NYHA class III or IV or IV symptoms, hospital admission by mid second symptoms, hospital admission by mid second trimester maytrimester may be advisable. be advisable.

Pregnancy induced hypertension, Pregnancy induced hypertension, hyperthyroidism,hyperthyroidism, infection, and anaemia should be infection, and anaemia should be identified early and treatedidentified early and treated vigorously. vigorously.

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CHF

For patients with important mitral stenosis, For patients with important mitral stenosis, the usethe use of  blockers or digoxin for control of  blockers or digoxin for control of heart rate should be considered.of heart rate should be considered.

Also offer empiric treatment with Also offer empiric treatment with  blockers to patients with blockers to patients with coarctation and to coarctation and to MarfanMarfan patients. patients.

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Arrhythmias

Arrhythmias in the form of premature atrial or Arrhythmias in the form of premature atrial or ventricular beats are common in normal pregnancy. ventricular beats are common in normal pregnancy.

Sustained tachyarrhythmias such as atrialSustained tachyarrhythmias such as atrial flutter or atrial flutter or atrial fibrillation should be treated promptly. fibrillation should be treated promptly.

Electrical cardioversion is safe in pregnancy. Electrical cardioversion is safe in pregnancy.

Digoxin and  blockers are antiarrhythmicDigoxin and  blockers are antiarrhythmic drugs of drugs of choice in view of their known safety profiles. Quinidine,choice in view of their known safety profiles. Quinidine,

adenosine, sotalol, and lidocaine are also "safe“. ( avoid adenosine, sotalol, and lidocaine are also "safe“. ( avoid teratogens and Amiodarone )teratogens and Amiodarone )

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Anticoagulation

For pregnant women with mechnical valves For pregnant women with mechnical valves mainly.mainly.

Warfarin more effective than Heparine , but Warfarin more effective than Heparine , but embryopathic.embryopathic.

Should be stopped at least 2 wks before Should be stopped at least 2 wks before labour to avoid fetal brain bleeding.labour to avoid fetal brain bleeding.

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Labour & delivery

Vaginal delivery is recommended with very few Vaginal delivery is recommended with very few exceptions. exceptions.

The only cardiac indications for caesarean section The only cardiac indications for caesarean section are aortic dissection,are aortic dissection, Marfan syndrome with dilated Marfan syndrome with dilated aortic root, and failure to switchaortic root, and failure to switch from warfarin to from warfarin to heparin at least two weeks before labour. heparin at least two weeks before labour.

PretermPreterm induction is rarely indicated, but once fetal induction is rarely indicated, but once fetal lung maturity islung maturity is assured a planned induction and assured a planned induction and delivery in high risk situationsdelivery in high risk situations will ensure will ensure availability of appropriate staff and equipment.availability of appropriate staff and equipment.

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Labour & Delivery

Invasive haemodynamic monitoringInvasive haemodynamic monitoring during labour during labour and delivery,is commonly utilised (intra-arterialand delivery,is commonly utilised (intra-arterial

monitoring)with or without (concurrent monitoring)with or without (concurrent pulmonary artery catheterisation). pulmonary artery catheterisation).

Heparin anticoagulation is discontinued at least Heparin anticoagulation is discontinued at least 12 hours before induction, or reversed with 12 hours before induction, or reversed with protamine if spontaneous labourprotamine if spontaneous labour develops, and can develops, and can usually be resumed 6-12 hoursusually be resumed 6-12 hours postpartum. postpartum.

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Labour & delivery

SBE prophylaxis: SBE prophylaxis: CentresCentres with extensive experience in caring with extensive experience in caring

for pregnant women with heartfor pregnant women with heart disease disease utilise endocarditis prophylaxis routinely, as utilise endocarditis prophylaxis routinely, as an uncomplicatedan uncomplicated delivery cannot always bedelivery cannot always be

anticipated.anticipated. Not AHA recommended if no infection site. Not AHA recommended if no infection site.

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Labour & delivery

Epidural anaesthesia with adequate volume Epidural anaesthesia with adequate volume preloading is the technique of choice.( but preloading is the technique of choice.( but can increase CHF & pulm oedema ) can increase CHF & pulm oedema )

Epidural fentanyl is particularly Epidural fentanyl is particularly advantageousadvantageous in cyanotic patients with in cyanotic patients with shunt lesions as it does not lower peripheralshunt lesions as it does not lower peripheral

vascular resistance.vascular resistance.

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Labour & delivery

Labour is conducted in the left lateral decubitus Labour is conducted in the left lateral decubitus position.position.

Instrumentation to shorten 2Instrumentation to shorten 2ndnd stage is indicated. stage is indicated. Patients at intermediatePatients at intermediate or high risk may require or high risk may require

monitoring for a minimum of 72 hoursmonitoring for a minimum of 72 hours

postpartum. postpartum. Patients with Eisenmenger syndrome require Patients with Eisenmenger syndrome require

longerlonger close postpartum observation, since close postpartum observation, since mortality risk persists formortality risk persists for up to sevenup to seven days. days.

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Conclusion

Women who have survived congenital heart Women who have survived congenital heart disease into adulthood often have a strong desire disease into adulthood often have a strong desire to become pregnant. to become pregnant.

Optimum careOptimum care of these potentially complicated of these potentially complicated pregnancies can only be achievedpregnancies can only be achieved by a combined by a combined approach by cardiologists and obstetricians in approach by cardiologists and obstetricians in specialistspecialist centres with an understanding of the centres with an understanding of the obstetric and cardiac complicationsobstetric and cardiac complications that canthat can arise. arise.