RHD & Pregnancy – Script SLIDE 1 Welcome to RHD Australia’s Health Provider Education series. Educational resources for clinicians by clinicians. RHD Australia is an initiative of Baker IDI, Menzies School of Health Research and James Cook University and is supported by the Commonwealth Department of Health and Ageing This module will investigate how pregnancy impacts on women with rheumatic heart disease. SLIDE 2 This presentation was developed with the assistance of Jaye Martin, and Barry Walters. Jaye is a consultant physician based in Broome, Western Australia, and provides specialist services across the region, travelling by 4WD and light aircraft. Her particular interests are chronic disease management, viral hepatitis, HIV, rheumatic heart disease and diabetes. Barry is Clinical Associate Professor of Obstetric Medicine at the University of Western Australia in Perth, and Adjunct Professor of Medicine at the University of Notre Dame Australia. He is the current President of the Society of Obstetric Medicine of Australia and New Zealand. SLIDE 3 The learning objectives for this presentation are firstly to understand how rheumatic heart disease can impact on the health of pregnant women. Secondly, you will gain an appreciation of the significance of valve disease in pregnancy, especially mitral stenosis and aortic stenosis. Thirdly, to be able to identify which pregnant women will be most at risk of complications from their rheumatic heart disease, and additionally to know which medications are safe, and which to avoid. You will gain an understanding of anticoagulation issues associated with pregnancy, and finally you will have learnt to appreciate the overriding importance of pre conception counselling in rheumatic heart disease SLIDE 4 The take home messages for this module are that the normal changes associated with pregnancy can have an adverse effect on women with rheumatic heart disease, that pre conception counselling is essential for all women with rheumatic heart disease, and that pregnancy poses a significant threat to women with rheumatic heart disease. Anticoagulation is always difficult to manage throughout pregnancy, and it can be hazardous for both baby and mother. Early referral to a specialist obstetrician familiar with rheumatic heart disease is advisable, as the clinical management of pregnancy associated with rheumatic heart disease is difficult. Lastly, contraception and the future management of their rheumatic heart disease should be discussed with women following delivery and before discharge from hospital. SLIDE 5 There will be a significant number of abbreviations used during this presentation, so let’s take a look at them before proceeding further. AF refers to atrial fibrillation – This is an abnormal and irregular heart rhythm that can occur in people with rheumatic heart disease especially when it affects the mitral valve AR is used for aortic regurgitation, this is a leaking aortic valve that links the left ventricle with the aorta. ARF refers to acute rheumatic fever – This condition can cause heart inflammation and lead to RHD AS is used for aortic stenosis, a sticking aortic valve that makes it difficult for blood to cross it. CO is used for cardiac output and this is the volume of blood pumped by the heart in one minute. Echo is an abbreviation for echocardiogram or heart ultrasound H2 refers to the second heart sound heard when auscultating or listening to the heart. This sound is associated with the closing of the aortic and pulmonary valves. H3 refers to the third heart sound – this can be a normal finding in young people and pregnant women, but is usually associated with heart problems in older people. It is an extra heart sound that occurs at the beginning of diastole, when the heart is relaxing and filling with blood, after the second heart sound or H2. It either occurs as a result of rapid filling of the ventricle (and this is why it can be normal in young or pregnant people) or because the ventricle is abnormal, as can occur in heart failure or following a heart attack. LA refers to the left atrium – one of the four chambers of the heart that is linked to the left ventricle via the mitral valve LV is used for left ventricle – the major pumping chamber of the heart that pushes blood across the aortic valve into the aorta and then the rest of the body LMWH is the abbreviation for low molecular weight heparin – the lighter component of heparin that is often used because dosing and administration is easier MS refers to mitral stenosis, the sticking of the mitral valve so that it does not let blood pass easily across it. MR is used for mitral regurgitation, a leaking mitral valve that lets blood leak back into the left atrium from the left ventricle MV is used for mitral valve, a valve commonly damaged by rheumatic heart disease MVA is the abbreviation for mitral valve area, which is a measure of how wide the mitral valve can open, and this measurement is used to assess severity of MS. NYHA is the abbreviation for the New York Heart Association – the organisation that developed a measure of shortness of breath in heart disease PASP is for pulmonary artery systolic pressure – this is also sometimes called RVSP or right ventricular systolic pressure and is the measure of pulmonary pressure and gauges pulmonary hypertension. PBMV is the abbreviation for percutaneous balloon mitral valvotomy – a relatively non-invasive technique for dealing with mitral stenosis PND is used for paroxysmal nocturnal dysponea – episodes of shortness of breath at night often associated with heart failure or mitral valve disease PR is used for pulmonary regurgitation – a leaking pulmonary valve that links the right ventricle and the pulmonary artery RHD refers to rheumatic heart disease – the development of permanent heart valve damage following repeated episodes of ARF. TR is used for tricuspid regurgitation – a leaking tricuspid valve that lets blood from the right ventricle leak back into the right atrium. SV is used for stroke volume – the volume of blood the heart pumps every beat. Stroke volume multiplied by heart rate equals CO or cardiac output SVR is used for systemic vascular resistance – the resistance the heart must work against based on all the blood vessels in the body. SLIDE 6 For those who would like more information regarding the management of acute rheumatic fever and rheumatic heart disease, this can be found in the Australian Guidelines for the Prevention, Diagnosis and Management of ARF and RHD. These guidelines have been substantially updated and revised in 2012, are available at the RHD Australia website. SLIDE 7 There is also a quick reference guide that provides an excellent summary of these guidelines. SLIDE 8 The Guidelines also provide a synopsis of the key points for the management of pregnancy for women with rheumatic heart disease. SLIDE 9 More information regarding a broad range of aspects of the prevention, diagnosis and management of acute rheumatic fever and rheumatic heart disease can be found at the RHD Australia website in the Health Provider Education modules. These will be regularly updated and expanded. SLIDE 10 The normal haemodynamics of pregnancy In order to understand the impact of pregnancy upon women with rheumatic valvular heart disease, it is first necessary to understand the normal haemodynamic changes that occur during pregnancy. These include a 50% increase in both blood volume and cardiac output at the same time as a reduction in systemic vascular resistance or SVR. Blood pressure tends to fall in the first and second trimesters of pregnancy. It is also important to remember that following birth there is an increase in the circulating blood volume and venous blood returning to the heart, as blood from the contracting uterus is returned to the circulation. SLIDE 11 This slide demonstrates graphically the effects of various stages of gestation upon heart rate, stroke volume and the product of these, the cardiac output. You can see that these all gradually increase throughout pregnancy, peaking late in the third trimester. There is also a rapid fall in heart rate, stroke volume and consequently of course, cardiac output following delivery. SLIDE 12 It’s also important to note that plasma volume increases by about 50% throughout pregnancy, and this is what is responsible for the so-called physiological anaemia of pregnancy, where the same number of red blood cells and amount of haemoglobin is diluted by a larger volume of plasma. There can also be a real reduction in total amount of haemoglobin associated with falling iron stores. You’ll see from this graph that iron supplementation reduces the fall in haematocrit somewhat, but does not completely compensate for this effect. SLIDE 13 The normal ECG in pregnancy. There are also ECG changes that can occur during pregnancy, and again many of these can be normal. These changes include a sinus tachycardia, mild S-T segment depression, either left or right axis deviation, and also non-specific T-wave changes.. Both atrial and ventricular ectopics can increase and these can also be a normal manifestation of pregnancy. SLIDE 14 Clinical findings in pregnancy. There are also a number of findings on examination of the cardiovascular system that would not be considered normal in a non-pregnant woman, but again, can be normal during pregnancy. These are listed on this slide and include increased splitting of H2 or the second heart sound, a third heart sound and a mid-systolic murmur. Less commonly a continuous venous hum, known as a mammary souffle ( pronounced souf), can also be heard on auscultation of the precordium or front of the chest. Pre-existing stenotic murmurs, that is, mitral and aortic stenosis become louder, due to the increased cardiac output, but regurgitant murmurs conversely may be quieter due to decreased systemic vascular resistance and greater forward and less abnormal backward or regurgitant flow. SLIDE 15 There are also some echocardiographic changes that occur during pregnancy that again can be normal. These include mild ventricular enlargement, mild tricuspid and pulmonary regurgitation, and mild mitral and aortic regurgitation. SLIDE 16 Cardiac risk in pregnancy. It is important to understand how the presence of pre-existing heart valve disease can increase the risk of adverse events during pregnancy. The most concerning valve lesion is mitral stenosis. This slide graphically illustrates this and shows a patient with mitral stenosis. You’ll note the mitral valve mean gradient increases during pregnancy compared to the post-partum state as does the pulmonary pressure demonstrated by the right ventricle systolic pressure or RVSP. You will see from the bar chart on the right side of this slide that the risk of adverse events during pregnancy, both maternal and foetal, is proportional to the severity of mitral valve disease. Of particular note, there is almost an 80% risk of an adverse event occurring if mitral stenosis is severe pre-pregnancy. SLIDE 17 Valvular disease in pregnancy. The principles of management of women with valvular heart disease during pregnancy can be divided into four categories. These are: 1. The vital role of pre-pregnancy planning and counselling, 2. Stratifying a woman’s risk of having an adverse event during pregnancy as a consequence of her heart disease 3. Defining optimal antenatal management and where this care is best provided. Contingencies for complications need to be included at this stage. Finally, 4. Where and how the delivery should occur in order to ensure a good outcome both for mother and child. SLIDE 18 Pre-pregnancy planning and counselling. Ideally, women with RHD should be assessed and counselled prior to becoming pregnant. This of course implies that all pregnancies, including in women with RHD, are planned, which of course is not always the case. It is therefore important to consider this in all women of child-bearing age with known RHD, whether they are sexually active or not and to discuss whether contraception is required. Whilst the choice of contraception should be discussed with woman and individualised to their preferences, long acting devices such as Implanon or Depo-Provera are preferred in this setting because of their low failure rate. If significant valvular heart disease is present, it is best to discuss this with the woman prior to conception. The implication that severe valvular disease might lower her chances of a successful pregnancy will need to be talked over. It is important to reinforce that most women with RHD can have children; with the main issue being whether the valve disease is best addressed prior to conception. Possible procedures for advanced disease may include percutaneous balloon mitral valvuloplasty, valve repair or valve replacement. If there is a possibility that any of these procedures may be necessary, then referral to a cardiologist prior to conception is advised. Pre-conception counselling is also an opportunity to review how well secondary prophylaxis with benzathine penicillin has been delivered. It is also an opportunity to monitor progression and severity of valvular heart disease with echocardiography, and to ensure specialist reviews are up to date. SLIDE 19 Risk stratification. Assessing the significance and severity valvular heart disease requires a number of assessments. The first is history. Symptoms including poor exercise tolerance and shortness of breath on exertion, at night (called paroxysmal nocturnal dyspnoea), or when lying down (called orthopnoea), can all indicate moderate to severe valve disease. The New York Heart Association has a functional classification that can be used to grade the severity of heart disease based upon how short of breath people become on exertion. This ranges from one, where there are no symptoms, through to 4 where there are symptoms at rest. Sometimes getting an accurate assessment of shortness of breath can be difficult. Going for a brisk walk with the patient, doing a formal assessment of exercise tolerance with a six minute walking test, or asking family or local health care staff can therefore be helpful. Echocardiography iscrucial . In particular, information is sought about severity of the mitral and aortic valve disease, the size and function of the left ventricle and the pulmonary artery pressure. A poorly functioning left ventricle and an elevated pulmonary pressure is always concerning. Finally if the woman has been pregnant previously, then her past obstetric history is important. An adverse event in a previous pregnancy is highly predictive of adverse events in future pregnancies. This is particularly the case if there has been no treatment to address the valve disease in the interim. Whilst an earlier uncomplicated pregnancy might be reassuring it should be kept in mind that valve disease can progresses. An earlier uncomplicated pregnancy should not necessarily reassure you that the current pregnancy will be similarly uncomplicated. SLIDE 20 The N.Y.H.A. classification. This slide illustrates in more detail, the New York Heart Association classification of heart disease based on symptom severity. Grade 1 disease describes heart disease with no significant symptoms, grading through mild symptoms for Grade 2, to significant symptoms limiting normal activity for Grade 3, and onto severe symptoms comprising significant breathlessness at rest, with the patient often being bedbound. Grade 4 symptoms indicate severe valvular heart disease requiring intervention. SLIDE 21 Maternal outcome and valve disease. This slide stratifies the risk of adverse maternal outcomes according to the New York Heart Association classification Grades 1 to 4. It shows that there is a tendency for any valvular heart disease to worsen during pregnancy, with women moving from stage 1 and 2 N.Y.H.A. symptoms through to Grade 3 or even Grade 4. Whilst this may occur with aortic and pulmonary valve disease, it is far more prevalent in mitral valve disease. SLIDE 22 So when should you worry. The main factors that should ring alarm bells when assessing a woman with rheumatic valvular heart disease who is either pregnant, or wishes to become pregnant are listed here. Factors that predict increased risk during pregnancy include decreased left ventricular systolic function on echo and significant aortic and/or mitral stenosis particularly when associated with moderate or severe pulmonary hypertension. Symptomatic heart disease before pregnancy, or heart failure either before or during pregnancy, are also poor prognostic indicators. Risk is also significantly increased if the patient already has a mechanical valve in situ, and atrial fibrillation is present. Both these conditions require anticoagulation which in itself adds additional risk to pregnancy and delivery. SLIDE 23 Maternal and foetal outcomes in patients with RHD. This slide depicts the increased risk to both maternal and foetal outcomes during pregnancy in women with RHD compared to matched control women without RHD. There is a 38% increased risk of heart failure developing during pregnancy, and a 35% risk of maternal hospitalisation during pregnancy, versus 2% in matched controls for women without RHD. Similarly, there is a fourfold increase in the risk of pre-term delivery in women with RHD and one in five babies will have intrauterine growth retardation in this group. It is important to note that these unfavourable outcomes mostly apply to women with moderate or severe mitral and/or aortic stenosis. SLIDE 24 The importance of RHD in obstetric care, particularly for Aboriginal and Torres Strait Islander women living in northern and Central Australia, is demonstrated here. The Indigenous populations of northern and Central Australia, those from Far North Queensland, the Northern Territory and the Kimberley region of Western Australia, have the highest prevalence of rheumatic heart disease in Australia. In the Kimberley approximately one in fifty Aboriginal Australians has RHD, with more women than men being affected. This bar chart shows that the majority of Kimberley women with rheumatic heart disease are under the age of 40, and therefore, of child-bearing age. SLIDE 25 We will now look at 3 case studies to illustrate some of the points mentioned. The first case is that of a 21yr old from Beagle Bay community. Beagle Bay is a small Aboriginal community two hours’ drive north of the main Kimberley town of Broome in northern Western Australia. This lady had known RHD and a pregnancy 12 months ago. She developed pulmonary oedema during her first labour and now presented requesting advice for another pregnancy. She fortunately realised that pregnancy required careful planning, but at the same time, was anxious to become pregnant and was refusing contraception. She frequently did not attend her planned medical appointments, and her secondary prophylaxis with benzathine penicillin had been spasmodic. So what issues should we consider discussing with this lady, and how should we approach management at this time? SLIDE 26 Clinical examination may include the following. A history should be taken regarding smoking and alcohol and a plan put in place to address these if needed. It’s important to reinforce that there is no safe level of drinking or smoking in pregnancy and that their effect can be particularly damaging before a woman even knows she is pregnant. She may need to lose or gain some weight. Poor maternal nutrition and being underweight or overweight can be important contributors to intrauterine growth retardation. Dental review is important at this time and frequently overlooked. A dental review may reduce the risk of infective endocarditis in the setting of RHD. If no recent ECG and echocardiogram, these will also be required, as well as a close review of her medication. She may be on any or several of the medications listed on this slide, and consideration needs to be given to whether these medications can or should be continued during pregnancy, whether they should be replaced with an alternative, and whether the medications should be discontinued prior to conception. SLIDE 27 The ECG of this case study confirmed that she was in sinus rhythm, and the echocardiogram showed mixed mitral valve disease with severe mitral regurgitation, and moderate mitral stenosis with a mitral valve area of 1.8cm2. She also had pulmonary hypertension with a pulmonary artery systolic pressure of 50 mm of mercury and the left atrial diameter was increased at 53mm. SLIDE 28 Assessing the risk and benefit of many drugs used in managing heart disease in the setting of pregnancy is difficult. Whilst definite advice is often lacking non-selective beta blockers, frusemide, digoxin, vasodilators such as hydralazine, nifedipine, verapamil and nitrates are all considered to be safe in pregnancy. Heparin is also safe, and warfarin after the first trimester may also be considered reasonable to continue. The issue of warfarin, particularly in the first trimester, will be discussed in more detail later. However, ACE inhibitors and the related angiotensin receptor blockers are absolutely contraindicated in pregnancy, as are other agents such as lipid lowering statin drugs. SLIDE 29 We will now move onto a second case study, that of a 23yr old woman from a community called Balgo in the remote East Kimberley. Balgo is located on the northern edge of the Tanami and Great Sandy Deserts, about 3½hrs drive south of the nearest town, Halls Creek. Halls Creek has a population of about 2,000 people and a small community hospital with no resident specialist staff. Balgo is 1,000km by road or about 3hrs by light aircraft from the major centre of Broome. This particular lady was physically active, with no exertional symptoms suggestive of heart disease, although she did have a history of significant alcohol use. At the age of 14 she was said to have had mild mitral stenosis noted on an echocardiogram. She presented now pregnant at 20 weeks gestation for her first ante natal visit. On examination there was a pansystolic murmur and possibly also a mid-diastolic murmur and a loud pulmonary component of the second heart sound. SLIDE 30 Her echocardiogram showed that she had mitral stenosis. SLIDE 31 So what are the main issues of concern in this particular patient? Is the mitral stenosis important and what is her prognosis? How should the mitral stenosis be managed during pregnancy, during labour, delivery and in the post-partum period? SLIDE 32 In order to understand the management of mitral stenosis in pregnancy, it is first important to understand why mitral stenosis is concerning in pregnancy. It is important because the pressure gradient across the mitral valve increases during pregnancy. In turn this worsens the functional severity of the mitral stenosis, because of the normal increase in heart rate and blood volume associated with pregnancy. This leads to an increase in left atrial pressure with associated shortness of breath, and an increased risk of pulmonary oedema, atrial fibrillation and other arrhythmias, as well as pulmonary hypertension. SLIDE 33 So how can mitral stenosis be managed during pregnancy? During pregnancy, mild to moderate mitral stenosis is usually managed medically. However if there is moderate to severe mitral stenosis with a mitral valve area less than 1.5cm2, consideration should be given to percutaneous balloon mitral valvuloplasty. This is particularly important to consider in symptomatic women and in those who have an echocardiogram which demonstrates a raised pulmonary artery systolic pressure above 50 mm of mercury. SLIDE 34 Medical management of mitral stenosis during pregnancy includes the use of beta blockers or digoxin for rate control of atrial fibrillation. DC cardioversion can be performed during pregnancy and should be considered if atrial fibrillation is inadequately controlled on medications, though often its benefit will only be temporary. Betablockers may also be useful in sinus rhythm if there is an associated tachycardia and symptoms. In this case slowing the rate can allow greater time for left ventricular filling across the stenosed mitral valve. It is important to avoid anaemia, so timely intervention with iron and folic acid supplements is essential. Ante natal care can be performed in the usual way in the community, but consideration should be given to transfer and admission in the third trimester to a larger centre with more experience in the management of valvular heart disease during delivery and better facilities. If atrial fibrillation is present then anticoagulation is required using low molecular weight heparin. Low molecular weight heparin should also be administered if the echocardiogram shows a dilated left atrium, left atrial thrombus,and if there is a history of previous thrombosis or embolic stroke. Monitoring of the efficacy of low molecular weight heparin can be difficult in pregnancy where normal weight based dosing may not be adequate. Advice should be sought from specialists regarding the need for regular factor Xa monitoring to direct low molecular weight heparin dosing. SLIDE 35 More severe degrees of mitral stenosis may require management with bed rest and diuretics for left ventricular failure. While beta blockers have the benefit of preventing tachyarrhythmias by slowing the heart rate and optimising left ventricular filling, procedural intervention is usually required for symptomatic disease. SLIDE 36 Getting back to our patient from Balgo, what was the outcome with her pregnancy? She could not be found for follow up after her echocardiogram. The assistance of the local clinic staff, Halls Creek Hospital, the community midwife and the local police were required to track her down. Fortunately she was found, and because she had severe mitral stenosis, she agreed to be transferred to Perth where she underwent a percutaneous balloon mitral valvuloplasty. Her baby was delivered by a normal vaginal delivery, and there were no adverse maternal or foetal outcomes. However she was pregnant again when next seen for review by the visiting specialist in Balgo a few months later. SLIDE 37 We will now discuss the management of aortic stenosis in pregnancy. Aortic stenosis related to RHD is far less common than mitral stenosis. Mild to moderate aortic stenosis is usually well tolerated in pregnancy. However percutaneous trans-luminal aortic valvuloplasty should be considered if symptoms are severe. Aortic stenosis should be managed medically, in a similar way to the medical management of mitral stenosis, using diuretics if heart failure develops, and drugs such as beta blockers or digoxin for rate control of atrial fibrillation if needed. It is important to note that cardiac surgery, that is, valve replacement, should be avoided if possible during pregnancy, because there is a high risk of foetal loss. This applies equally to both the aortic and mitral valve. SLIDE 38 We will now look at our third case study, that of a 25yr old woman in her first pregnancy with no known previous cardiac history. She lives in the community of Looma, which again is a small Aboriginal community about 100km from the nearest significant regional town of Derby in the Kimberley region of Western Australia. Derby has a population of about 4,000 people with a base hospital and resident medical officers, but no resident specialists. This lady had undergone an uneventful pregnancy, except for developing anaemia, with her haemoglobin falling to 80g/litre. She presented to the hospital in Derby during labour. SLIDE 39 The labour was prolonged, and required a forceps delivery. During the delivery she sustained a second degree tear, and had a significant loss of blood approximating about 5 litres. She was given IV crystalloids and packed red cells as volume resuscitation and responded well. In the immediate post-partum period, she developed a fever and tachycardia, and was noted to have a very poor urine output. She was assessed and thought to be dehydrated, and therefore was given an intravenous fluid challenge. SLIDE 40 Following the intravenous fluid she developed significant shortness of breath, and her oxygen saturations dropped to 90-92%. She was noted on examination to be in pulmonary oedema, and also had sacral oedema, a raised JVP, and a tachycardia of 104 beats per minute. A pan-systolic murmur consistent with mitral regurgitation was noted. SLIDE 41 This slide shows her chest x-ray, which confirms the presence of pulmonary oedema. It also suggests that she has a dilated left atrium. SLIDE 42 She had an urgent echocardiogram, which showed moderate aortic regurgitation with moderate to severe mitral regurgitation However there were no significant signs of pulmonary hypertension, with normal right heart pressures and only mild tricuspid regurgitation. SLIDE 43 Mitral and aortic regurgitation. Mitral regurgitation is the most common valvular lesion in RHD. It is usually well tolerated during pregnancy unless there is a sudden deterioration as may occur with the rupture of a chordae tendon. Pulmonary oedema is treated in the usual way with diuretics. Whilst vasodilators are rarely required they may be needed for control of systemic hypertension. It should be remembered that ACE inhibitors and angiotensin receptor blockers are usually contraindicated in pregnancy. SLIDE 44 The normal haemodynamic changes associated with the post-partum period are outlined here. There is an increase in systemic venous return with relief of compression of the inferior vena cava after delivery, combined with a phenomenon known as autotransfusion. This refers to the contracting uterus returning blood to the systemic circulation with an associated increase in circulating blood volume. While this effect is potentially counteracted by blood loss during delivery, this is often less than the increase associated with autotransfusion. The end result is a substantial increase in ventricular filling pressures, cardiac output and total peripheral resistance. The outcome of this can be pulmonary oedema, particularly if other factors come into play. It is important to note that this haemodynamic adaptation can take 6-12 weeks post-partum to return to pre-pregnancy values, so this increased risk of pulmonary oedema can persist for some weeks following delivery. SLIDE 45 The tendency to pulmonary oedema may be exacerbated if the woman also has anaemia, infection or tachycardia, or suffered from pre-eclampsia. This bar chart shows that the plasma oncotic pressure in pregnancy is reduced compared to the non-pregnant state, particularly if pre-eclampsia has occurred. Thus for the same increase in left atrial and left ventricular filling pressure the risk of pulmonary oedema will be higher as fluid is more likely to leak from the pulmonary vessels into the lung. SLIDE 46 This chart illustrates graphically the relative importance of contributing factors that predispose women to pulmonary oedema in the post-partum period. You will see that one of the most important factors is iatrogenic or health provider induced fluid overload. This is almost as important as the contribution of pre-existing cardiac disease, and more important than preeclampsia, which in itself is also significant contributor. Interestingly, the use of tocolytics is relevant in about 25% of women who develop post-partum pulmonary oedema contributing the same percentage as patients with pre-existing cardiac disease. SLIDE 47 This slide illustrates the strain that is put on a woman’s heart during labour. You will see that during a uterine contraction the cardiac output increases markedly, but falls back to baseline 24hrs after delivery. SLIDE 48 It is also important to note that the cardiac output is dependent upon the woman’s position during labour. Particularly in women with known valvular heart disease, it is recommended they be positioned as shown in the diagram in the bottom right hand corner of the slide, that is, lying not absolutely supine, but rolled slightly to the right, which can be facilitated by placing a wedge under the back on the left hand side. SLIDE 49 This slide lists the risk factors associated with heart failure during pregnancy. The left hand column lists maternal factors such as age, obesity, presence of other heart disease or hypertension, presence of other chronic diseases such as diabetes and being an Aboriginal or Torres Strait Islander woman. Use of amphetamines and cocaine are also risk factors. The right hand column lists pregnancy associated factors, including severe pre-eclampsia, sepsis, anaemia, use of tocolytics or steroids, low albumin, twin pregnancies and the use of crystalloid intravenous fluids. SLIDE 50 It is important to note that women with mitral stenosis tolerate tachycardia poorly during delivery, whereas women with aortic stenosis tolerate hypovolemia poorly. It is very important that women with high risk mitral and/or aortic stenosis undertake delivery in a planned fashion, in a centre with experience in managing such deliveries. It cannot be overemphasised how important it is to consider early transfer for valvuloplasty or high risk cardiac surgery if there is concern that this may be needed. SLIDE 51 This slide lists the important predictors of an adverse pregnancy outcome in women with heart disease. The most important factors are any previous cardiac event or arrhythmia, New York Heart Association functional class greater than 2, or the presence of cyanosis, a presence of left heart obstruction, defined as a mitral valve area less than 2cm2 and/or an aortic valve area of less than 1.5cm2, and finally, the presence of systemic ventricular dysfunction with a left ventricular injection fraction less than 40%. SLIDE 52 This bar chart shows the risk of a cardiac event according to the presence of these 4 mentioned risk factors. If none of these risk factors are present, then only a very small percentage of pregnancies will be affected by a significant cardiac event, whereas if more than one of these risk factors are present, then 60% or more pregnancies can be expected to be complicated by a cardiac event. SLIDE 53 Therefore, early referral to a regional centre for ongoing care should be considered if the woman is thought to be of intermediate or high cardiac risk, defined as a risk score of one or more of these factors present, or with risk factors specific to the particular valve lesion they possess. Conversely, delivery in a community hospital can occur if there is a low cardiac risk, that is, a risk score of zero with no lesion-specific risk factors. SLIDE 54 In general terms, vaginal delivery is encouraged and should be the aim in all women except those with severe mitral stenosis and severe pulmonary hypertension. Usually these conditions will require an elective caesarean section in a tertiary hospital. Cardiac monitoring can be non-invasive in the presence of mild and moderate valvular heart disease. Antibiotic prophylaxis is not typically required, but should be given if there is prolonged labour and/or ruptured membranes. Principles of management include aiming for as short a second stage of labour as possible, and managing labour with a multi-disciplinary team of experts. There should however be a low threshold for obstetric intervention, and close post-partum monitoring is absolutely essential because of the increased risk of pulmonary oedema. SLIDE 55 As a general principle cardiac valve surgery should be avoided during pregnancy. This is because cardio pulmonary bypass in pregnancy is associated with an up to 5% risk of maternal mortality, and a very high foetal mortality rate of 16-33% depending upon the case series examined. SLIDE 56 Possible procedures for the management of valvular disease in pregnancy include valvuloplasty which should be done by a percutaneous approach wherever possible. Other options include valve repair and valve replacement only if this is not possible, but these later two options involve surgery with its attendant risk to mother and baby. SLIDE 57 This survival curve demonstrates that the long term outcome of percutaneous balloon mitral valvotomy undertaken in pregnancy is very similar in women who were pregnant at the time of the procedure compared to women who were not pregnant when the procedure was performed. SLIDE 58 So we will now move onto valve replacement with a mechanical or bioprosthetic valve. One of the most important considerations after the decision has been made that a valve needs to be replaced is to consider the type of valve, a bio prosthetic valve or a mechanical heart valve. The advantage of a bioprosthetic valve is that anticoagulation is not required; however, these valves will need to be replaced earlier than a mechanical valve. It is important to remember that most women undergoing these procedures will survive, and will need to have a repeat valve replacement at some stage in their life if a bioprosthetic valve is used. Whilst mechanical valves may last longer they are associated with a higher thromboembolic risk and anticoagulation will be required. The safe management of anticoagulation in remote communities is always difficult, and requires substantial support from primary health services. Further information regarding the management of anticoagulation in RHD can be found elsewhere in this education series. There is also a higher risk of infective endocarditis with mechanical valves and this should be consider particularly if there is a history of infective endocarditis. SLIDE 59 The use of anticoagulation in pregnancy is a difficult and challenging. When anticoagulation is required, early discussion with a specialist physician, haematologist or obstetrician is essential. The two agents currently used for anticoagulation in pregnancy are heparin, either low molecular weight or unfractionated, and warfarin. The risk of thromboembolism if anti-coagulation is inadequate needs to be balanced against the risk of bleeding associated with over anti-coagulation, and the possible risk of teratogenicity from the use of warfarin in pregnancy. SLIDE 60 Ideally, interventions for advanced rheumatic heart disease that do not require anticoagulation, such as valvuloplasty, valve repairs or bioprosthetic valves, are preferable in women who plan to become pregnant. However you will no doubt encounter women with mechanical heart valves who require anticoagulation who either are pregnant or wish to become pregnant. It is therefore necessary to weight up the risks and benefits of using heparin or warfarin, or a combination of both throughout the pregnancy. While warfarin is associated with foetal damage especially if given in the first trimester, recent evidence indicates this may be avoided if the warfarin dose can be kept to 5mg or less during pregnancy. However, there is still a risk of intra cranial haemorrhage to the foetus if warfarin used. The maternal risks of anticoagulation have already been highlighted, that is, bleeding in the case of over anticoagulation versus thromboembolism, particularly valve thrombosis and stroke if anticoagulation is inadequate. Essentially there are three options for anticoagulation in pregnancy, as detailed on the next slide. SLIDE 61 The first is to continue warfarin at a dose of 5mg or less daily throughout pregnancy, changing to intravenous heparin or low molecular weight heparin at 36-37 weeks, until elective delivery. This option is the best option for the heart valve, providing anticoagulation is adequate on this low dose of Warfarin, but of course is not such a good option for the baby. The second option therefore is to plan pregnancy, and use low molecular weight Heparin during the first trimester, changing to Warfarin at 13 weeks, and continuing Warfarin until Week 36. Low molecular weight heparin should then be introduced from Week 36 onwards, until labour and factor Xa levels used to monitor adequacy of anticoagulation with the low molecular weight heparin. The third option is to use low molecular weight heparin throughout pregnancy, again with factor Xa monitoring. This third option would seem to be the safest for the baby, but there is doubt that it is a good an option for the mother in terms of adequate anticoagulation, and at the present time there is no data to confirm or refute this suggestion. Low molecular weight heparin should be stopped for delivery, and for 24hrs post-partum, then re-introduced whilst the mother is commencing warfarin. Low molecular weight heparin can then be discontinued after five days and when INR levels are adequate on warfarin therapy. SLIDE 62 So, in drawing to a close, let’s revisit our take home message from the beginning of the presentation. Normal changes associated with pregnancy can have an adverse impact on women with rheumatic heart disease, pre conception counselling is vital for all women with rheumatic heart disease, pregnancy poses a significant threat to women with severe rheumatic heart disease, management of anticoagulation in pregnancy is difficult and risky, management of pregnancy in women with rheumatic heart disease can be difficult and requires early referral to specialists, and finally, the issue of contraception should be discussed prior to hospital discharge postpartum. SLIDE 63 More information regarding a broad range of aspects of the prevention, diagnosis and management of acute rheumatic fever and rheumatic heart disease can be found at the RHD Australia website in the Health Provider Education modules. These will be regularly updated and expanded. SLIDE 64 You can also register at the Health Provider Education website for additional resources. To download this and other PowerPoint presentations for your own use in your local practice, and additional assessment items for training providers, and if you would like to be notified about new modules and updates, please ‘Like’ us on Facebook at the provided address below. SLIDE 65 And finally, for those of you who would like to test your knowledge regarding the information presented in this module please go to the brief self-assessment quiz at the link provided on this website.