Heart Disease in Pregnancy - RCP London

Heart Disease in Pregnancy

Dr Laurence O’Toole MD FRCP

Consultant Cardiologist

Sheffield Teaching Hospitals NHS FT

Royal College of Physicians Regional Update Tankersley Manor

22nd May 2017

Cardiac Sepsis

Neurological Other indirect causes

Thromboembolism Psychiatric

Amniotic fluid embolism Haemorrhage

Early pregnancy deaths Indirect malignancy

Anaesthesia Pre-eclampsia

0 1 2 Ratio per 100, 000 maternities

Cardiovascular disease is the commonest cause of maternal death in UK & Ireland

Pregnancy makes significant demands on the cardiovascular system

• Reduced systemic vascular resistance – 30% reduced at 8/40, nadir at 24/40

• Rise in cardiac output – 40% for a singleton, 50-60% for a twin pregnancy

• Increased heart rate – 10 to 20 bpm in early 3rd trimester

• Increased maternal blood volume – 40% for singleton pregnancy and 66% for a twin pregnancy

– plasma volume increases 50% & red cell mass by 30%

– blood volume peaks at 32/40

• Reduced colloid oncotic pressure – 15% fall in blood albumin level at 24 weeks

• IVC obstructive pressure from the gravid uterus – 8% of women hypotensive when supine

• Arterial tree remodelling

• Hypercoagulable state – Reduced tPA, Protein C & S production

– Increased TPA inhibitor, factors V,VII, VIII, IX, X, XII and vWF

Weeks

Labour and delivery make very significant demands on the cardiovascular system

• Labour increases CO by a further 10-30% in 1st stage and 50% in 2nd stage – 500ml auto transfusion which each uterine contraction

– Sympathetic stimulation from pain & anxiety

• Post-delivery there is a marked increase in CO (80% in first hour)

– Uterine involution

– Relief of aorto-caval compression

• It takes 6 months for restoration of normal physiology

Timing of maternal deaths due to cardiac causes (n=153: 2009-2014)

% of all deaths

Who can’t tolerate a pregnancy?

Fixed Cardiac Output

Susceptible Vasculature

Who can’t tolerate a pregnancy?


Severe systemic ventricular dysfunction (LVEF <30%)

Pulmonary arterial hypertension

Obstructive symptomatic left heart lesion (e.g. AS or mitral stenosis)


Marfan ascending aorta over 45mm diameter

Bicuspid aortic valve over 50mm diameter

Native severe aortic coarctation

WHO Class IV risk

Who probably can’t tolerate a pregnancy?


Systemic right ventricle

Fontan circulation

Cyanotic heart disease

Other complex congenital heart disease

LVEF 30-40%

Asymptomatic severe aortic stenosis


Aortic dilatation: 40–45 mm in Marfan syndrome

45–50 mm in BAV aortopathy

Mechanical valve

WHO Class III risk

Which groups of woman are at risk?

Complex Adult Congenital Heart Disease

Prosthetic Mechanical Heart Valves

Personal or family history of cardiomyopathy or aortopathy

Survivors of childhood cancers (the ‘late effects’ group)

Immigrants from areas with high prevalence of rheumatic fever

IVF pregnancies – Health Tourists

Past cardiac ischaemic events or multiple risk factors for such

General principles of management of pregnancy in women with heart disease: (1)

• Ideally all women with congenital or acquired heart disease should receive multi-disciplinary preconception counselling before pregnancy

• Guidance must be given early.

“A teenager with a late repair of a VSD and persisting pulmonary hypertension died three days after delivery….despite full ITU support. It is unclear if she received pregnancy or contraceptive counseling from paediatric cardiology services. She was referred to the adult congenital heart disease clinic when she found herself pregnant and elected to continue the pregnancy. With increasing symptoms, she was delivered at 34/40.”


• Early review and risk assessment by a MDT (obstetricians, anaesthetists & cardiologists) required.

• Optimise mother’s CV status during pregnancy

• Monitor for deterioration

• Address foetal issues

• Develop a clear, well documented and widely distributed plan for labour and the puerperium


• Mode of delivery? – ‘Spontaneous vaginal delivery with low-dose regional anaesthesia and

careful fluid management preferred in most cases’

– But the C/section rate in women with significant heart disease is high

• Induction may be appropriate • (eg optimisation of timing of delivery in relation to anticoagulation or

availability of specific medical staff or for deteriorating maternal function

• Effective pain relief important – Reduces rise in CO from pain and anxiety

– Low-dose regional anaesthesia

• Limit maternal effort – The managed second stage • Assisted delivery (Ventouse/Forceps)


• Place of delivery – ?is a cardiac theatre appropriate

• Post labour HDU/ICU/CCU • usually 24 to 48 hours

• Senior post-partum obstetric and cardiology review important

• Early cardiology follow up

• Early discussion about risks of future pregnancy & robust contraception

The majority of pregnant women who die of a cardiovascular disease have not been identified as being ‘at risk’.

Causes of Maternal Cardiovascular Death MBRRACE 2009-2014

0 5 10 15 20 25 30 35

SADS/MNH

Cornary Artery Disease

Cardiomyopathy

Aortic Dissection

Valvular Heart Disease

Pulmonary Hypertension

Essential HT

%age of all CV maternal deaths

153 maternal deaths from CV disease

Sudden Cardiac Death with Morphologically Normal Heart

Diagnosis of exclusion (includes a negative drug screen)

Mortality rate in pregnancy is probably the same as the non-pregnant rate

81% arrested out of hospital and few had prodromal symptoms

A young Caucasian woman had a normal delivery of her third baby. Two weeks postpartum she collapsed at home but despite exemplary resuscitation could not be revived. Her autopsy was ‘negative’; her BMI was normal, and her heart was normal weight. Review by a cardiac pathologist confirmed the likelihood that she died from SADS/ MNH since all other possible causes had been excluded.

Sudden Cardiac Death with Morphologically Normal Heart

Screening of proband and first degree relatives will identify a genotype for an ion channelopathy in about 50% of families

The Ion channelopathies

Long QT syndromes

Brugada syndrome

Catecholaminergic Polymorphic VT

Progressive Cardiac Conduction Defect

Idiopathic VF

Wolfe-Parkinson-White (with AF)

MBRRACE review screening for inherited cardiac conditions largely not done and only 2 of 53 identified with a disorder

Aortic Dissection

Risk increased 25 times in late pregnancy

Usually aortic root dilatation leading to tamponade

40% of the mothers who died presented with premonitory chest pain that went unrecognised

A woman who had delivered a few days earlier presented by ambulance in the middle of the night to the Emergency Department with severe sudden onset chest and neck pain. Her pregnancy had been complicated by disabling symphysis pubis dysfunction. On arrival at A&E she was anxious and described severe pain starting in the neck and radiating in waves to the chest and back. She was clear that the pain was different to the ‘all over pain’ of her symphysis pubis dysfunction. She was seen by junior doctors who considered pulmonary embolism or anxiety attack as possible diagnoses. They decided on the latter and sent her home without investigation or senior review. She died suddenly at home a few days later. Post mortem confirmed dissection of the entire aorta with a bicuspid aortic valve.

Aortic Dissection

Predisposing conditions

Marfan syndrome

Bicuspid aortic valve with aortopathy

Familial thoracic aortic syndrome

Vascular Ehlers-Danlos syndrome

Loeys-Dietyz syndrome

Coarctation of the aorta esp. complex repairs

Aortitis

In MBRRACE, in 15 of 21 maternal deaths no cause established.

CARDIAC ISCHAEMIA

34 deaths

Half atherosclerotic:

10 acute MI, 6 sudden deaths with CAD+

Almost all of the atherosclerotic deaths were in women with risk factors

Mean age 34, BMI 27, half smokers

One third coronary artery dissection

Mean age 34, half smokers, BMI 24

One-sixth other causes – arteritis, Takotsubo CM, myocardial bridging

CARDIOMYOPATHY

MBRRACE data

27 deaths from cardiomyopathy

Most frequent mode of death was out of hospital arrest after delivery

9 considered peri-partum cardiomyopathy

‘Consider cardiomyopathy in the ∆∆ dyspnoea in pregnancy’

Orthopnoea and PND are ‘not symptoms of pregnancy’

‘Don’t withold investigations and treatment on the grounds of pregnancy and breast-feeding’

CARDIOMYOPATHY

38 years old nurse at 33/40 6 weeks of dyspnoea BP 96/40 mmHg; p 120 bpm; 3rd HS with soft systolic murmur Sinus tachy on ECG P oedema on CXR Increasing diuretic requirements over next 48 hours Day 4: Urgent elective LCSC after placement of IABP Hypotensive++ over next two weeks Discharged home at 3 weeks

CARDIOMYOPATHY

Shown with permission

During her pregnancy a woman presented on five occasions within two weeks to different hospitals and her GP complaining of cough, dyspnoea and orthopnoea. She was noted to be tachycardic. She was prescribed multiple courses of antibiotics to which she failed to respond but no further investigations were done. Eventually a diagnosis of peripartum cardiomyopathy was made and she was delivered by Caesarean section. Despite insertion of an intra-aortic balloon pump, Extracorporeal Membrane Oxygenation (ECMO) and attempted Left Ventricular Assist Device (LVAD) insertion, she died shortly postpartum.

CARDIOMYOPATHY

CARDIOMYOPATHY 29 years old primip 4 weeks post partum 3 presentations with dyspnoea 2 courses of antibiotics Echo eventually Moved to CCU given diuretics, ACEi and … bisoprolol 2.5 mg stat Hypotension+ Inotropic support induced VT Moved to NGH Survived with support One year on.. Current VO2 max 20.3 LV no improvement

PERIPARTUM CARDIOMYOPATHY

Idiopathic Cardiomyopathy in the last trimester to 6/12 postpartum

1 in 3000 pregnancies

More often in..

African ancestry

Age >30 years

Multiparity

Preg induced hypertension

Usually partial to complete recovery of LV function in 2 to 6 months

Subsequent Pregnancies after Peripartum Cardiomyopathy

J Am Coll Cardiol 2014;64:1629–36

VALVULAR HEART DISEASE

Regurgitant lesions not causing symptoms before pregnancy are generally well tolerated

Symptomatic or severe obstructive lesions are not well tolerated.

Mechanical heart valve prostheses represent very high risk pregnancies

Problems with Warfarin and Pregnancy

1) Warfarin Embryopathy Incidence 5-10% of pregnancies Possibly dose related [??<5 mg warfarin daily safer] 2) Foetal loss (Chan et al 2000) 29% on warfarin 16% on LMWH in weeks 6 to 12/40 3) Warfarin to term Prematurity 45% Low birth weight 50% ICH 3%

VALVULAR HEART DISEASE

• Surveyed the 210 Consultant-led obstetric units in the UK

• Poor maternal outcomes

• Death / ICU admission / Valve thrombosis

• Valve dysfunction resulting in HF or death

• Bleeding requiring blood transfusion or return to theatre

• Poor foetal outcomes

• Miscarriage and stillbirth / Neonatal death

• Foetal abnormality / Apgar score <7 at 5 minutes / Neonatal unit

58 UK maternities with a mechanical prosthetic valve in situ Anticoagulation regime LMWH 71% First trimester LMWH then Warfarin 16% Warfarin throughout 12% 42% presented after 7 weeks gestation 19% not referred to a tertiary care unit 45% Vaginal delivery and 53% Caesarian section

Vause et al BrJOG Dec 2016

58 UK maternities with a mechanical prosthetic valve in situ 5 deaths (9%) 2 valve thrombosis 2 immediately post partum 1 embolic stroke Serious maternal morbidity 41% Mitral position 57% Aortic position 39% Dual valves (n=4) 75% Caesarian section 40% Vaginal delivery 33% Poor foetal outcomes in 43% 1 foetal warfarin embryopathy

Vause et al BrJOG Dec 2016

58 UK maternities with a mechanical prosthetic valve in situ Only 16 of 58 28% women had a good maternal and foetal outcome By anticoagulation regime… Good maternal and foetal outcome LMWH throughout 8 of 41 (20%) First tri LMWH then warfarin 5 of 9 (56%) Studies with warfarin anticoagulation principally Maternal death Bleed Thrombosis HF ROPAC 1.4% 23% 6.1% 7.5% Lawley CM et al. 1.8% 11% 14%

Reason for Referral to JOCC 1 Jan 2012 – 22 May 2015 348 patients seen in 618 clinic visits.

0 10 20 30 40 50 60 70 80

arrhythmia (including 4 long QTc, 3 congenital HB, 2 ablations, 4 accessory pathways)

Asystole at GA induction

Cardiomyopathy - (including two resolved, 5 pregnancy associated, 2 CTX associated)

Chest pain

Complex medical history (including 6 ACS, 1 OOHCA, 6 Marfan's/EDS, 1 Turner's, 1…

Corrected congenital lesion (including 3 Tetrallogy of Fallot)

Family history (2 cardiac, 7 medical)

Fatigue/dizziness

Known valvular lesion

Ventricular systolic dysfunction/hypertrophy

Murmur (2 were childhood only)

Unclear indication

Palpitations - (including 4 pts with previous ablation/significant family history

Other (1 panic attack, 1 past recreational drug use)

Pre-eclampsia

SOB

syncope (including 9 POTS)

Valvular replacement (6 tissue, one metalic)

ASD/VSD (restrictive/repaired/Spontaneously closed)

Attendances Jan 2012 to May 2015


CONCLUSION

Women with heart disease need expert multidisciplinary assessment at the onset of reproductive capacity or at diagnosis of CV disease and before each pregnancy

Pregnancy represents a significant cardiovascular stress for women with significant heart disease

Specialist multidisciplinary assessment in pregnancy is best practice


CONCLUSION

Women with heart disease need expert multidisciplinary assessment at the onset of reproductive capacity or at diagnosis and before pregnancy

Pregnancy represents a significant cardiovascular stress

Specialist multidisciplinary assessment in pregnancy is best practice

• Early recognition

• Prompt multidisciplinary risk assessment

• Optimisation

• Monitoring

• Delivery planning

• Surveillance and optimisation post delivery

General principles of management of pregnancy in women with heart disease

Heart Disease in Pregnancy - RCP London

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