• 1.
  

  a) T b) T c) T d) F e) F

Bosentan induces the cytochrome P450 enzymes CYP2C9 and CYP3A4, so women with pulmonary arterial hypertension receiving bosentan need an increased dose of desogestrel. They should also be advised to use a supplementary form of contraception, such as condoms, as the risks associated with contraceptive failure in women with pulmonary hypertension are extremely serious. Combined hormonal contraceptives should be avoided in women with a history of thromboembolic disease, regardless of their cardiac history; however, levonorgestrel-containing contraceptives may carry a slightly lower risk of thromboembolic disease compared with other combined hormonal contraceptives. There is no contraindication for progestogen-only contraception in women taking long-term anticoagulation therapy for metallic prosthetic cardiac valves. After unprotected sexual intercourse, a copper intrauterine device can be placed up to 120 h after intercourse to be effective for emergency contraception. The Nexplanon ^® contraceptive implant is subdermal and only needs to be replaced every 3 years.

• 2.
  

  a) T b) F c) T d) F e) T

Biosynthetic grafted-tissue heart valves (either from pigs or humans) do not need anticoagulation, but the bioprosthesis deterioration accelerates during pregnancy. Women with metal prosthetic heart valves must continue full anticoagulation throughout pregnancy as the risk of valve thrombosis is extremely high. A peak anti-Xa level measured 4 h after administering low molecular weight heparin should be between 0.8 and 1.2 IU/ml.

Statins are contraindicated in pregnancy. Given the scarcity of available data, it is generally advisable to avoid use of statins in women who are planning pregnancy in order to reduce the risks of teratogenicity as much as possible. Warfarin is associated with a risk of embryopathy between 6 and 12 weeks of gestation. The teratogenic risk of chondrodysplasia punctata, nasal hypoplasia, growth restriction, short proximal limbs and other abnormalities is about 5%. The risk of embryopathy with warfarin is low in patients taking warfarin doses of 5 mg/day or less, so continuation of oral anticoagulants may be considered during the first trimester if the warfarin dose required for therapeutic anticoagulation is less than 5 mg per day after discussing the risks and benefits with the patient.

• 3.
  

  a) T b) F c) T d) F e) F

In women of child-bearing age, the main cause of aortic stenosis is congenital bicuspid aortic valves, and women can be asymptomatic, even with severe aortic stenosis. Clopidogrel may be used in pregnancy but it must be stopped before delivery, as there is an increased risk of bleeding. Several studies have attributed epidural haematoma to clopidogrel, and regional anesthesia is generally contraindicated in women taking this medication. The risk is increased in older multigravid women, smokers, and obese women, as well as in women with diabetes, hypertension hypercholesterolaemia, or a family history of coronary artery disease. The most common cardiac abnormality in women with Marfan syndrome is mitral valve prolapse and regurgitation. Adenosine is the preferred drug of choice to treat supraventricular tachycardias in pregnancy; however, either propranolol or verapamil can be used for the acute termination of supraventricular tachycardias, or for those who do not respond to vagal manoeuvres. For prevention of further tachycardias, beta-blockers or verapamil may be used. Propafenone and amiodarone should be avoided.

• 4.
  

  a) F b) F c) F d) T e) T

The numbers of women becoming pregnant with both congenital and acquired heart disease are rising. Eisenmenger syndrome has a higher risk of perinatal mortality than transposition of the great arteries. Women with an atrial switch – Mustard or Senning procedure are at risk of this cardiac complication. A failure to raise heart rate appropriately during cardio-pulmonary exercise testing is indeed associated with adverse cardiac events during pregnancy and severe pulmonary regurgitation is also a predictor of poor outcome in women with Tetralogy of Fallot.

• 5.
  

  a) T b) F c) T d) F e) F

Prior cardiac events (e.g. heart failure, transient ischemic attack, or stroke before pregnancy) or arrhythmias do form part of the scoring system as do women with cyanotic heart disease or NYHA class II disease. All the parameters for left outflow obstruction listed are indeed included. 50% is normal; ≤40% is the correct number. A total score of 1 indicates a 75% chance of a serious cardiac event in pregnancy.

• 6.
  

  a) T b) T c) T d) T e) T

All of the conditions are at increased risk of aortic dissection in pregnancy.

• 7.
  

  a) F b) F c) F d) T e) T

The chance of a mother with congenital heart disease, having a fetus with congenital heart disease is about 5%. The chance of a mother with Marfan syndrome having a child with Marfan syndrome is one in two as it is autosomal dominant. Recurrence of Turners is sporadic and therefore there is no increase in risk. Both d and e are correct.

• 8.
  

  a) F b) T c) T d) F e) F

Although a lack of symptoms is reassuring, it does not exclude the presence of other important risk factors. Indeed, symptoms in congenital heart disease relate very poorly to formal effort capacity or underlying disease severity. People with Tetralogy of Fallot may have Di George syndrome, which, if present, has a 50% recurrence risk (inherited in a dominant manner). A cardiac magnetic resonance scan will be needed to document the function of the pulmonary valve and the size and function of the right ventricle.

Pulmonary hypertension is rare in Tetralogy unless there are aorto-pulmonary collaterals or a previous Waterston shunt. There is no specific gestation that is optimal for delivery. A spontaneous onset of labour is preferable in most circumstances.

• 9.
  

  a) F b) F c) F d) F e) F

Although the cardiac condition is important, delivery is usually determined by obstetric indications. Caesarean section is not inherently safer than vaginal delivery. Each delivery plan must be tailored to the individual set of circumstances of the patient. A slow incremental regional anaesthetic is frequently used in women with congenital heart disease, and is recommended in many as the optimal form of pain relief. Only a minority of women will require invasive monitoring. Babies of women at a lower risk can safely be delivered in their local unit with an established delivery care plan in place.

• 10.
  

  a) F b) T c) T d) F e) T

Women with an unoperated VSD usually have near normal haemodynamics with a good cardiac output and no increased risk of growth restriction. Beta-blockers are frequently associated with intrauterine growth restriction and third-trimester growth scans should be carried out. The Fontan circulation is often associated with limited cardiac output and reduced oxygen saturations. These women usually have a normal haemodynamic response to pregnancy. The reason for the association of severe pulmonary regurgitation is unclear but probably relates to reduced cardiac output and right ventricular impairment.

• 11.
  

  a) T b) F c) T d) T e) T

As Hart et al. show, aortic compliance increases. This is thought to be subsequent to the hormonal changes that influence the connective tissue. Selected individuals are suggested for follow up in a specialist centre, particularly those at high risk of dissection (e.g. a family history for dissection or sudden death, women with Ehlers–Danlos syndrome or Loeys–Dietz syndrome, and indeed all women with aortic disease); these women are all at risk of developing complications. Manalo–Estrella and Barker revealed the histo-pathological changes to the medial layer changes during pregnancy. Hypertrophy and hyperplasia of smooth muscle cells and loss of structure of the elastic fibres are both seen, and this might contribute to the vulnerability of the aortic wall.

• 12.
  

  a) F b) T c) T d) T e) F

This recommendation (40 mm) was used in past decades because of the advice of Pyeritz. Recently, the guidelines state that pregnancy should be discouraged if the aortic diameter is greater than 45 mm. If the aortic diameter is between 40 and 45 mm, several other risk factors need to be considered (i.e. prior dissection and family history). It seems that the changes of aortic diameter are partly irreversible if present according to the largest and most recent study by Donnelly et al. Evidence is contradictory, however, with other studies reporting no relevant changes specifically caused by pregnancy. In cases of a type A dissection during pregnancy, management does not differ from the general guidelines on aortic dissection. If the fetus is considered viable however, one might first carry out a Caesarean section followed by aortic repair, but only if the mother is relatively stable and Caesarean section can be carried out urgently in the cardiac theatre. Caesarean section is only carried out if the aortic diameter exceeds 45 mm, and might be considered (depending on further risks) if it is larger than 40 mm. Women with an aortic diameter smaller than 40 mm may have a vaginal delivery.

• 13.
  

  a) F b) T c) T d) T e) T

Dissection occurs more often in the third trimester and postpartum, however, few studies have reported on the timing of dissection. Bicuspid aortic valve is present in about 30% of women with Turner syndrome. It is suggested that this is indeed related to a higher risk of dissection in this population. Coarctation is seen in about 10–12% of women with Turner syndrome and in the small numbers of women with Turner syndrome actually having a dissection. It has been suggested that extra caution should be taken with pregnancy. An elongated aortic arch is seen in about 47% of women and is found to be associated with a higher risk of dissection.

• 14.
  

  a) T b) F c) T d) F e) T

This is correct according to current European guidelines. In addition, a class IIb indication exists for women with an aortic diameter between 40 and 45 mm. Epidural puncture is impeded owing to a potential dural ectasia, but this can be diagnosed before pregnancy or delivery with magnetic resonance imaging. Moreover, a puncture can be carried out with ultrasound. To minimise haemodynamic changes, it is preferable to give epidural anaesthesia and a shortened second stage, as there is still a concern in women with an elevated risk of aortic complications. Nitroprusside has adverse effects on the fetus. Instead the treatment of choice is hydralazine, followed by a beta blocker.

• 15.
  

  a) F b) F c) F d) F e) T

Successful regional anaesthesia has been described in all of the first three conditions. In women with Fontan circulation, regional anaesthesia is the technique of choice. In these women, venous return to the lungs is essentially passive, and anything that raises intrathoracic pressure, such as positive pressure ventilation, should be avoided. Although many prefer general anaesthesia for women with pulmonary hypertension, carefully performed regional anaesthesia has been successful. Traditionally, aortic stenosis was considered a contraindication to regional anaesthesia, particularly single shot spinal techniques. This was due to the relatively ‘fixed’ cardiac output and inability to compensate for a large reduction in systemic vascular resistance (SVR). Carefully titrated epidural anaesthesia, however, is generally considered safe. Women with decompensated heart failure may have epidural anaesthesia if vaginal delivery is planned. Symptoms will worsen significantly, however, if they have to lie flat for Caesarean section. Full anti-coagulation is one of the absolute contraindications to regional anaesthesia owing to the risk of epidural haematoma.

• 16.
  

  a) T b) F c) F d) T e) F

The physiological effects of oxytocin are well documented. It causes a marked reduction in SVR of up to 50%, with a compensatory rise in heart rate and cardiac output. Although common practice in the UK, a bolus dose of oxytocin should be avoided in cardiac parturients if possible. If bleeding is particularly problematic, a small bolus dose carefully titrated to effect can be used in conjunction with a phenylephrine infusion. Ephedrine is a vasopressor with both beta and alpha agonist activity. It may, therefore, worsen a tachycardia caused by oxytocin. Phenylephrine is the vasopressor of choice to counteract the unwanted effects of oxytocin. Studies have shown that a bolus dose of oxytocin can cause ST changes on electrocardiography. These changes are more pronounced when larger doses are used. Ergometrine is an alpha-agonist that can cause coronary vasospasm and pulmonary artery vasoconstriction. It should, therefore, be avoided in women with pulmonary hypertension or ischaemic heart disease.

• 17.
  

  a) F b) T c) F d) T e) F

Most guidelines recommend that women with a previous venous thromboembolism (VTE) that was associated with the COC pill should take antenatal and postpartum thrombo-prophylaxis. For women taking a prophylactic dose of LMWH, epidural, spinal analgesia or anaesthesia is considered safe as long as there is a gap of at least 12 h between the last dose of LMWH. This should be discussed with women in the lead up to delivery. An option for women who wish to ensure that they can have an epidural is to switch to prophylactic dose unfractionated heparin, which has a shorter half life, and the anticoagulant effect can be determined by measurement of the activated partial thromboplastin time. Women with a previous VTE associated with a temporary risk factor that is no longer present have a very low risk of recurrence in pregnancy, 0% in 3 published studies. The recurrence in women with previous idiopathic or hormonally-related event (pregnancy or COC) ranges from 2–10%. In women considered to be at increased risk of VTE, where thromboprophylaxis is recommended, should start it as soon as pregnancy is recognised given the timing of presentation of VTE throughout pregnancy. Both LMWH and warfarin are safe for breast feeding.

• 18.
  

  a) F b) F c) T d) F e) F

There seems to be a dose relationship with the risk of warfarin fetopathy (i.e. central nervous system defects, late fetal loss and stillbirth), which is likely to reflect the fact that warfarin and vitamin K antagonists directly cross the placenta. The fetus is vitamin K naïve, with low levels of coagulation factors, so the dose of warfarin that would cause a therapeutic level of anticoagulation in the mother will produce major over-anticoagulation in the fetus. Although warfarin is effective at preventing thromboembolic complications, these have been described in 1 out of 25 pregnancies. Non-compliance with treatment is also a major issue, as women are concerned with the adverse effect of this drug on their baby and choose not to take it. Warfarin embryopathy occurs after exposure during 6–9 weeks gestation, and is not described in women who stop the drug before 6 weeks gestation. Women who take warfarin can safely conceive on warfarin but must be advised to seek medical review as soon as they have missed a period or have regular pregnancy tests to ensure that options for ongoing anticoagulant can be discussed and a plan developed. As long as anticoagulation is stopped in preparation for delivery, the mode of delivery can be determined by obstetric indications. Delivery must be planned with either an induction of labour or elective Caesarean section. Women taking therapeutic doses of LMWH do have the option of regional analgesia or anaesthesia, but there must be a 24-h gap between the last dose of LMWH and placement of the neuraxial catheter.

• 19.
  

  a) F b) T c) T d) F e) T

Lateral tilt has been shown to increase maternal blood pressure and cardiac output, in addition to fetal oxygenation, non-stress test, and fetal heart rate. At tilt angles of over 30°, the unconscious patient will slide or roll off the incline plane. At 15°, 30°, and 45° of tilt aorto-caval compression can still be found. One study found that the maximal cardiac index was achieved only with the full left lateral position, a position that is incompatible with resuscitation. The maximum resuscitative force that can be applied during chest compression in the tilted woman has been shown to decline as the degree of tilt increases. Left lateral tilt has never been shown to cause fetal trauma. Efforts at maternal lateral tilt may result in delays, interruptions and reduced force of chest compressions and, therefore, are not ideal in the cardiac arrest situation.

• 20.
  

  a) T b) T c) T d) F e) F

Appropriately performed left uterine displacement will relieve aorto-caval compression, whilst allowing for ideal chest compressions. Lateral uterine displacement has been shown to reduce hypotension related to aorto-caval compression. The maximum resuscitative force that can be applied during chest compression in the tilted woman has been shown to improve as the degree of lateral tilt decreases towards the supine. Fetal heart rates are not monitored during active resuscitation. It is important to maintain the direction of displacement in a lateral, not a downward, direction to relieve aorto-caval compression.

• 21.
  

  a) T b) F c) F d) F e) T

The first responder will activate the maternal cardiac arrest team, document the time of onset of the maternal arrest, and start chest compressions. The second responder should provide aorto-caval decompression, ideally with manual left uterine displacement. The adult resuscitation team will manage the usual measures for adult resuscitation. Modifications to adult resuscitation in the pregnant woman include placing the hands slightly higher on the sternum for chest compression. The responders should not delay defibrillation or administration of the usual resuscitation drugs. Magnesium has been reported to cause cardiac arrest in pregnancy. Therefore, for women receiving magnesium, the magnesium should be immediately stopped and empiric treatment should be given, in the form of calcium. As part of the 2010 American Heart Association guidelines, the first algorithm for the management of cardiac arrest in pregnancy was published.

• 22.
  

  a) F b) T c) T d) F e) F

If non-invasive methods of relieving aorto-caval compression prove inadequate, peri-mortem Caesarean section can be life saving for both mother and baby, and should be considered as one of the important treatment options for the management of cardiac arrest in pregnancy.

Peri-mortem Caesarean section should be considered when spontaneous circulation does not return after 4 mins of resuscitation to avoid hypoxic brain injury. Peri-mortem Caesarean section should be carried out at the site of the maternal cardiac arrest; the woman should not be transported to an operating room, as this causes delays to peri-mortem Caesarean section and results in decreased cardiopulmonary resuscitation quality. Many case reports have shown sudden and dramatic return of spontaneous circulation in women only after peri-mortem Caesarean section has been carried out. A sudden improvement in haemodynamics after peri-mortem Caesarean section has been described in many case reports. One study found that implementation of a training course on managing maternal peri-mortem Caesarean section emergencies resulted in both an increased awareness of the potential maternal benefit of peri-mortem Caesarean section and also found an increase in the number of peri-mortem Caesarean sections carried out.

• 23.
  

  a) F b) F c) T d) F e) F

In Eisenmenger syndrome, intra-cardiac right- to-left shunting will increase because systemic vascular resistance falls whereas pulmonary vascular resistance remains elevated. Women with Eisenmenger syndrome often have thrombocytopaenia and thrombopathy; therefore, bleeding risk is often elevated, and especially the risk of hemoptysis is high. Right-to-left-shunting increases and oxygen saturation will therefore decrease. In many women with pulmonary hypertension, the pulmonary pressures will increase during pregnancy, as they have no capability for pulmonary vasodilatation to accommodate for the increased blood volume and cardiac output. Local thrombosis may contribute to increase of pulmonary artery pressures also in group 1.

• 24.
  

  a) F b) F c) F d) T e) F

Pulmonary hypertension is not a predictor of mortality but of heart failure as described in the systematic review published here. In our systematic review, and in others, pulmonary hypertension associated with congenital heart disease has a higher mortality than idiopathic pulmonary hypertension. Although mortality seems lower with milder hypertension, this is not significant in any study. d is indeed also true as described in the chapter. There are no predictors in the two mentioned reviews.

• 25.
  

  a) F b) F c) F d) F e) T

Endothelin receptor antagonists and not tadalafil are fetotoxic. Calcium channel blockers are contra-indicated in Eisenmenger syndrome: they can give vasodilation with increased right-to-left-shunting and have no proven efficacy in this group. They are indicated in other patients with group 1 pulmonary arterial hypertension when they are responders to a vasodilatation test. Advanced pulmonary hypertension therapies are only proven beneficial in women with group 1 pulmonary hypertension. Answer d is false because individuals with Eisenmenger also have a high bleeding risk and anticoagulants should only be given on an individual basis. Answer e is true and this was a result from the systematic review published in this chapter.

• 26.
  

  a) T b) F c) F d) F e) F

Endothelin receptor blockers indeed reduce the efficacy of oral contraceptives. There is yet no proof that the advanced therapies are the determinant of an improved prognosis. Better management of the pregnancies, including early planned delivery, may also contribute. Termination may also have a high risk of haemodynamic deterioration and should be carried out in an expert centre. Caesarean section is not contraindicated but is associated with haemodynamic fluctuations that are often more pronounced than in vaginal delivery; therefore, vaginal delivery is also an option. Additionally general anaesthesia has been associated with higher mortality risk. Indeed there is no significant health risk for the woman with condom use but the efficacy of these contraceptives is suboptimal and they are therefore not advised in women with a high mortality risk.

• 27.
  

  a) T b) T c) T d) T e) F

Women with severe mitral stenosis tolerate the haemodynamic changes of pregnancy poorly and an intervention such as valvuloplasty or valve replacement should be carried out before a pregnancy to decrease the risk of subsequent cardiac events. In pregnancy, both the heart rate and blood volume increase. The increased heart rate limits the diastolic ventricular filling time. These factors result in increased left atrial pressures which can precipitate atrial arrhythmias, pulmonary oedema, or both. Medical treatment for pregnant women with severe mitral stenosis should be aimed at slowing the heart rate and thereby lengthening the diastolic filling period. In women who remain symptomatic despite medical treatment, percutaneous balloon valvuloplasty should be considered if the valve is suitable for the procedure. Anticoagulation with heparin or warfarin is required in women with mitral stenosis who develop atrial fibrillation because of the increased risk of left atrial thrombus.

• 28.
  

  a) T b) F c) T d) F e) T

The hypercoagulable state of pregnancy is associated with increased risk of thromboembolic events and, therefore, therapeutic anticoagulation is essential for all women with mechanical valves. The guidelines suggest that various anticoagulant regimens are possible, and include recommendations for the use of warfarin, unfractionated heparin or low molecular weight heparin in pregnant women with mechanical valves. In pregnant women with mechanical valves using low molecular weight heparin, dosing should be based on maternal weight and peak anti-Xa levels. Regular assessment of peak anti-Xa level is recommended to ensure that the low molecular weight heparin dosing is adequate. Maternal INR measurements offer no guide to fetal INR effects. If warfarin has not been transitioned to heparin at least 2 weeks before labour begins, a Caesarean delivery is indicated because of the potential risk of intracranial haemorrhage in the anticoagulated fetus.

• 29.
  

  a) T b) F c) T d) F e) F

Physiological adaptation to pregnancy causes a greater stroke volume and increased cardiac output. Sinus tachycardia facilitates this, and volume increases in the left ventricle, as well as slight cardiac rotation due to an increasingly gravid abdomen, will cause a small shift in axis towards the left. Right bundle branch block may be a non-pathological finding if identified pre-pregnancy but should not develop during pregnancy. Often if developing during pregnancy it may be due to right heart strain and in the setting of acute chest pain should prompt consideration of pulmonary embolism. ST segment depression is an indicator of myocardial ischaemia. Left bundle branch block is a pathological finding and if associated with pain may be due to acute (anterior) STEMI, but may also be seen in cardiomyopathies – such as peri-partum cardiomyopathy.

• 30.
  

  a) T b) F c) F d) F e) T

Previously venous thromboembolic events – namely pulmonary embolism – were the biggest cause of maternal death in the UK. However, the most recent enquiry into maternal causes of death (CMACE) revealed ischaemic heart disease had taken over as the biggest cause of mortality. This is in part due to the aggressive risk assessment and prevention strategy of VTE but also due to the increasing incidence of IHD during pregnancy. Atherosclerotic coronary disease is consistently identified as the biggest cause of these ACS episodes during pregnancy and hence traditional atherosclerotic risk factors, such as smoking and diabetes, are just as important during pregnancy as outside. Studies have identified that pre-eclampsia can cause troponin elevations – but not normally into the cut off for a diagnosis of ACS. Coronary dissection has been identified as a common cause of ACS during pregnancy though remains a rare cause outside of pregnancy.