Medical conditions pregnancy

Question 1

What is thrombotic thrombocytopenic purpura?

Answer 1

Condition that causes microvascular platelet aggregation. Systemic and extensive. Often involves CNS. (HUS less extensive consumption of platelets)

Question 2

When is TTP mostly seen and what symptoms does the patient have?

Answer 2

PP

Haemolytic anaemia
Thrombocytopenia
Fever
Neurological manifestations (headache, irritability, drowsiness, seizures, coma)
AKI

Question 3

How do you diagnose TTP?

Answer 3

Bilirubin and LDH raised
AKI
Thrombocytopenia

Coags normal

ADAMSTS-13 activity is <10% (inhibitor of vWF - cleaving protease - ADAMSTS-13) (Diffuse vascular endothelial insult, endoethelial cells secrete unsually large forms of vWF - these large multimers aggregate platelets)

Question 4

How do you diagnose aHUS?

Answer 4

In HUS, platelet aggregation is relatively less extensive, with predominantly renal involvement.
Diagnosis of exclusion, more likely if:
ADAMST-13 >10%
Serum Cr >177
LDH >1000
Hb <80
Feature persistent more than 72h PPw

Question 5

How should you manage TTS / HUS?

Answer 5

No effect on fetus

No evidence that delivery improves course of disease
Need to differentiate from HELLP
Aggressive treatment with FFP and plasmaphoresis can limit vascular injury and improve prognosis
Supportive therapy for AKI (sometimes dialysis)
Platelet transfusions contraindicated.

Question 6

What is the genetics of sickle cell anaemia?

Answer 6

Autosomal recessive - sickle gene affects the haemoglobin structure
HbSS = sickle cell anaemia
(Sickle cell trait asymptomatic apart from increased risk UTIs and microscopic haematuria)

Question 7

Clinical features of sickle cell disease

Answer 7

Anaemia - haemolytic
Painful vaso-occlusive crises
Infections (Loss of splenic function)
Acute chest syndrome - fever, tachypnoea, pleuritic chest pain, leukocytosis, anaemia, pumonary infiltrates)
Splenic sequestration - sickled red cells get stuck in spleen and damage it
Gallstones
Retinopathy
Leg ulcers
Aseptic necrosis of bone
AKI / kidney disease
Stroke
Pulm HTN

Question 8

What precipitates sickle crisis?

Answer 8

hypoxia
cold
acidosis
dehydration

Can cause
- Vaso-occlusive symptoms (tissue infarction, pain!)
- Splenic sequestration
- Aplastic (BM stops producing red cells - often ass with parvovirus)

Question 9

How do you diagnose sickle cell anaemia?

Answer 9

Electrophoresis

Question 10

What is the effect of sickle cells disease on pregnancy?

Answer 10

perinatal mortality 4-6 fold increase
Increased
- Miscarriage
- FGR (sickling infarcts, + anaemia)
- PTB
- APH and placental abruption
- Fetal distress
- CS
- Infection (UTIs, pneumonia, peurperal sepsis) (encapsulated organisms in hyposplenic individuals)
- Thromboembolic events

Question 11

What is the effect of pregnancy on sickle cell disease?

Answer 11

Complications more common
35% will have a crisis in pregnancy

Question 12

How should you manage sickle cell disease pre-pregnancy, antenatally, intrapartum, postpartum?

Answer 12

Pre pregnancy:
- Counsel re risks
- 5mg folic acid
- MDT: haematologists / obs
- Test partner - consider PGD if partner carrier
- ECHO to exclude pulm HTN
- Renal and LFTs annually
- Retinal screening
- Ferritin - to screen for iron overload - aggresive iron cehlation prior to concetion
- Alters meds - hydroxyurea is teratogenic - discontinue 3/12 prior to pregnancy. If become pregnant on it - structural abnormality scan but TOP not indicated as some cases no adverse effects.
- sometimes patients on ACEI for renal dysfunction - switch
- Only given iron if iron deficient
- Vaccinations: menigitis, pneumoniae, hemophilius influenzae

Pregnancy
- Penicillin prophylaxis throughout pregnancy - oral twice / day
Low dose aspirin
Hb and MSU every visit
Uterine artery dopplers, regular growth USS (2-4 weekly)
If hospital admission for LMWH
Manage crises aggressively: admission, treat pain (IV / SC morphine), adequate rehydration, early use of abx if infection suspected
Oxygen if sats <95% - pulse oximetry mandatory.
NSAIDs can be used between 12 and 28 weeks
(WCC raised in SCD so doesn’t necessarily indicate infection)
CXR: looking for acute chest syndrome - infiltrates, hypoxia - treat with blood transfusion or exchange transfuion, abx, resp support . LMWH until PE excluded.
Brain imaging if concerned re stroke
If acute anaemia - do reticulocyte count - if low could be parvovirus

intrapartum
- IOL 38-40/40 (no RCTs to support this)
- Avoid dehydration, hypoxia, sepsis, acidosis
- encourage epidural analgesia
NO is safe
CTG advisable
continuous sats monitoring - ABD and O2 replacement if <94%)

PP
- LMWH 7/7 for NVB, 6/52 CS
Contraception

Question 13

What will happen to a fetus that is alpha thalassaemia major?

Answer 13

Hydrops - no functional alpha genes so severely anaemic. Not compatible with life

Question 14

What is beta thalassaemia major?

Answer 14

Defective beta genes, inefficient carrying of oxygen, haemolysis because the alpha chains will hold onto the oxygen and this will cause damage to the red cells - haemolysis in BM or in spleen. These people only survive into 2nd or 3rd decade of life. Present wtih anaemia, hepatsplenomegaly, growth retardation, jaundice, haemochromotosis (arrhytmias, pericarditis, cirrhosis etc…). Initially HbF hangs around for first 6 months and then patients present.
Enlarged forehead and cheekbones because oof increased bone marrow production.
diagnose with electrophoresis

Question 15

Beta thalassaemia major
- Pre conception
- Antenatal
- Intrapartum
- PP

Answer 15

Most are subfertile. (can have damage to pituitary)

Periconceptual:
* Counsel risks - inheritance, worsening anaemia, cardiomyopathy, new development endocrinopathies secondary to no iron chelation in pregnancy (DM, hypothyroid), IUGR, PTB, CS for CPD
* Test partner, consider PIGD or gamete donation
* Aggressive Fe chelation, stop 3m prior to pregnancy
* Screen re Fe overload and end organ damage - TFT, echo, ECG, cardiac MRI, LFTs, liver USS, DEXA scan, fructossamine,
* RBC antibody (alloimmunity in 16.5%) and hepatitis screen
* High dose folate

Antenatal:
* MDT - high risk obs, MFM, haematology, anaesthetics, paediatrics, genetics
* High dose folate
* Fe only if low
* Aspirin if splenectomy or plt count >600, add LMWH if both (prothrombotic tendency due to abnormal red cell fragments)
* Fetal genotype if at risk - NIPT or CVS/amniocentesis
* Growth surveillance from 24/40
* Fructossamine (monthly) to assess for diabetes, or BSL monitoring - HbA1c less reliable if transfusion dependent
* Consider chelation after 20weeks - desferrioxamine
* Blood transfusions to aim >100g/L, 2-3 weekly Hb

Delivery:
* Aim VB
* CTG
* Cross match
* Active 3rd stage
* IV desferrioxamine (excess iron can cuase free radical damage and cardiac dysrhtymia in labour)

Postnatal:
* Breastfeeding support - OK to breastfeed on desferroxiamine)
* VTE prophylaxis (7/7 NVB, 6/52 CS)
* Contraception

Question 16

What are the causes of thrombocytopenia in pregnancy?

Answer 16

• Spurious result
• Gestational thrombocytopenia
• Immune thrombocytopenic purpura (ITP)
• HELLP
• DIC
• Sepsis
• Haemolytic uraemic syndrome (HUS) / thrombotic thrombytopenic purpura (TTP)
• HIV, drugs, infection
• SLE and APS
• Bone marrow suppression and folate deficiency

Question 17

Immune thrombocytopenia
- When to suspect
- Effect of pregnancy on ITP
- Effect of ITP on pregnancy

Answer 17

Diagnosis of exclusion, consider if diagnosed in early pregnancy (destruction by spleen after antibodies tag platelets)

Effect of pregnancy on ITP - doesn’t effect

Effect of ITP on pregnancy
- Capillary bleeding and purpura more common with counts <50, spontaneous mucous membrane bleeding <20
Antiplatelet IgG crosses placenta - causes fetal thrombocytopenia
0 - 1.5% risk fetal or neonatal ICH
- If a previous child is affected - high risk

Question 18

How should you manage ITP
- Maternal considerations
- Fetal considerations

Answer 18

Maternal considerations
- Exclude SLE and APLS
- Monthly plt count
- Treatment required in 1st and 2nd trimester if symptomatic with bleeding, count <20, interventional procedures required e.g. CVS
Aim counts >50 prior to delivery, >80 if want regional anaesthesia
Corticosteriods first line therapy 20-30mg prednisolone / day, then wean
IVIG in resistant cases - delays clearance of IgG coated platelets from maternal circulation (more rapid response but expensive) (lasts 2-3weeks)
Splenectomy in severe cases - if has had splenectomy - penicillin prophylaxis
If IVIG not succesful - methylprednisone, azathioprine, cyclosporin. plt transfusion last resort - increases Ab titres.

Fetal considerations
- Baby not at risk of bleeding before labour and delivery (Transfer increases towards end of pregnancy)
- ICH rates not reduced with CS
Cord platelet count immediately after delivery (nadir reached 2-5 days later though so observe over that time, IVIG if plt <20)
Avoid FSE, FBS, if plt count <80 in mother
avoid ventouse

Question 19

Causes of DIC

Answer 19

Haemorrhage e.g. abruption
PET / HELLP
AFE
Massive infection, particularly intrauterine infection
retention of dead fetus

Question 20

Pathogenesis of DIC

Answer 20

Endothelial injury
consumption of clotting factors and platelets
Fibrinolysis stimulated
Bleeding

Question 21

Diagnosis of DIC

Answer 21

Fibrinogen <2g/L
Thrombocytopenia
Prolonged clotting times - thrombin time, APTT, PT
Increased fibrinogen degradation products
Blood looks watery

Question 22

How should you manage DIC?

Answer 22

Treat underlying cause
Activate MTP
Coagulopathy treated with
- FFP (all coagulation factors)
Red cells
- Plt if plt <80 and ongoing bleeding
Cryoprecipitate (clotting factors)
Recombinant fibrinogen (if <1g/L and ongoing haemorrrhage)

Question 23

What is the inheritance of vWD?

Answer 23

Autosomal dominant

(vWF - adhesive protein, important role in plt function and stability of FVIII. Is required for binding of platelets to subendothelium after vessel injury)

Question 24

How is vWD diagnosed?

Answer 24

APTT prolonged
vWF reduced
VIII reduced

Question 25

vWD and pregnancy
- Effect of pregnancy on vWD
- Effect of vWD on pregnancy
- management

Answer 25

Effect of pregnancy on vWD
- pregnancy can normalise vWF and VIII levels, drop PP
Effect of vWD on pregnancy
- Early gestation vWF levels may not have increased enough - increased bleeding with ectopic, miscarriage, CVS
- No increased risk APH / miscarriage
- By 3rd trimester vWF increased enough that no need for alternate measures in labour
- active mx 3rd stage

Management
- Ascertain subtype of vWD pre-pregnancy and whether responds to DDAVP (type 1 responds to DDAVP - it stimulates release of vWF). Can give in pregnancy
- No aspirin or NSAIDs

Question 26

SLE (connective tissue disease - flares and remissions)

Periconceptual
Antenatal
Delivery
Postnatal

Answer 26

Periconceptual
* Enquire about systems affected: joint involvement (90% arthritis), skin (80%), raynauds, pleuritis, pericarditis, renal glomerulonpehritis, neurological (psychosis, seizures, chorea), haematoligcal (haemolytic anaemia, thrombocytopenia, lymphopenia, leukopenia)
* Counsel risks - higher if untreated or flare <6months or renal lupus. Miscarriage, early onset FGR, PTB, HTN, PET, VTE, Stillbirth, neonatal lupus.
* Review medications usually benefit > risk but NOT for methotrexate. Continue hydroxychloroquinine.
* Baseline ANA titre, c3/c4 (if low risk of flare), anti ds DNA (gives info about disease activity), CBC, Renal, LFTs, urine ACR (helps understand disease status) (may need serial measurements), anticardiolipin ab (risk of VTE)
* Anti Ro/La screen (30% have - if present 5% risk cutaneous lupus and 2% risk CHB)

Antenatal
* MDT - high risk obs, rheum, anaesthetics
* Aspirin (LMWH if APLs or prev VTE or lupus nephritis)
* Fetal heart auscultation if Anti Ro/La weekly from 16weeks
* FAS + uterine artery doppler
* Growth surveillance from 24weeks
* BP, PET surveillance
* Screen for flares (rising dsDNA titre indicates flare, also symptoms, or fall in complement levels - can help differentiate PET from lupus flare). Actively manage flares with corticosteroids. Azathioprine, NSAIDs and aspirin can also be used. Hydroxychloroquine should be continued
* Flares: UV light, viral infection

Delivery
* Aim VB
* IOL 38+
* CTG
* Active 3rd stage
* Hydrocortisone if high dose/long term steroids

Postnatal
* Neonatal review
* Breastfeeding support
* VTE prophylaxis
* Contraception

Question 27

How do you diagnose SLE?

Answer 27

No specific clinical or lab criteria
- normocytic normochormic anaemia, neutropenia, thrombocytopenia, raised ESR
Low complement
ANA - 96% positive
anti-dsDNA
antiSm
Glomerulonephritis occurs more commonly in women with antidsDNA and antiSm

Also may have anti - Ro and anti - La ab, or anti-cardiolipin ab

Question 28

SLE
- Effect of pregnancy on SLE
- Effect of SLE on pregnancy

Answer 28

Effect of pregnancy on SLE
- Flares more common 50%
- Flares more likely if disease active in last 6 months
- Can be difficult to diagnose
- lupus nephritis - if not well controlled, renal flare much more common
- Women should delay pregnancy until at least 6 months after a lupus nephritis flare

Effect of SLE on pregnancy
- Increased risk of miscarriage, fetal death, PET, PTB, FGR
This is related to anticardiolipin ab, or lupus anticoagulant, lupus nephritis or HTN and active disease at time of conception or first presentation of SLE in pregnancy
Renal lupus biggest risk factor for fetal loss, PET, FGR, PTB

Question 29

Anti Ro Ab (SSa+)
- What does it cause?

Answer 29

Neonatal lupus risk 5%
CHB 2%

Refer to MFM if has these Ab
FH auscultation from 16 weeks
Highest risk of CHB between 18 and 24/40
Hydrops and 15-20% mortality rate.
Once a fetal bradycardia is recognized, detailed scanning of the fetal heart, showing AV dissociation confirms CHB. This is permanent and can’t be reversed.
Salbutamol can be beneficial to the fetus if bradycardia is causing heart failure. If infants survive they will need pacemaker as the anitbodies cause permanent fibrosis.

Neonatal lupus: Erythematous skin lesions, will resolve within 4-6 months. Some hypopigmentation can last for up to 2 years.

Question 30

What is antiphospholipid syndrome?

Answer 30

Multisystem vasculopathy
- Increased risk VTE, miscarriage, thrombocytopenia

CLOT
- Coagulation defects
- Livedo reticularis
- Obstetric complications
- Thrombocytopenia

Other features
- Haemolytic anaemia
- Cerebral involvement: epilepsy, infarction, migraines
- Heart valve disease - mitral valve
- HTN
- Pulm HTN
- Leg ulcers

Question 31

How do you diagnose APLS?

Answer 31

Two or more positive readings for lupus anticoagulant and / or anticardiolipin and / or antibeta2glycoprotein1 at least 12/52 apart.
PLUS fulfill clinical criteria
- Thrombosis or pregnancy morbidity (3 consec miscarriages, 1 fetal death >10/40, ≥1 premature birth <34/40 due to PET or placental insufficiency

Question 32

Effect of pregnancy on APS and effect of APS on pregnancy

Answer 32

Effect of pregnancy on APS
- Thrombosis increased
- thrombocytopenia worsens

Effect of APS on pregnancy
- Miscsarriage
- IUGR (30%)
- Abruption
- SB
- PET
- PTB (30%)

Question 33

APLS

Periconceptual
Antenatal
Delivery
Postnatal

Answer 33

Periconceptual
* Screen for disease sequalae
* Counsel risks - miscarriage, early onset FGR, PTB, HTN, PET, abruption, VTE, stillbirth, neonatal lupus.
* Review medications
- May be on Warfarin - need to switch to aspirin and LMWH 5/52 prior to conception. If no hx thrombosis start on aspirin (CNP also says give LMWH if miscarriages with aspirin)
* Baseline CBC, Renal, LFTs, urine ACR
* Anti Ro/La screen (30% have - if present 5% risk cutaneous lupus and 2% risk CHB)

Antenatal
* MDT - high risk obs, haem, anaesthetics
* Aspirin + LMWH (prophylactic, intermediate if prev VTE (40 BD) and therapeutic if on treatment prior)
* Fetal heart auscultation if Anti Ro/La weekly from 16weeks
* FAS + uterine artery doppler
* Growth surveillance from 24weeks
* BP, PET surveillance
* Pscyhological support (especially if recurrent losses)
* Immunosupression - no good evidence.

Delivery
* Aim VB
* IOL 38+
* CTG
* Active 3rd stage

Postnatal
* Neonatal review
* Breastfeeding support
* VTE prophylaxis
* Contraception - avoid COCP
*Recommence Warfarin 5-7 days PP if was on, discontinue LMWH when INR >2

Question 34

What is scleroderma?

Answer 34

Non inflammatory disorder - widespread vasculpathy and fibrosis - autoimmune - obliteration of vessel lumens

Involves
- Skin (fixed mouth, facial expression)
- oesophagus
- lungs
- heart
- kidneys

Question 35

Scleroderma
- Effect of pregnancy on scleroderma
- Effect of scleroderma on pregnancy

Answer 35

Effect of pregnancy on scleroderma
- If renal invovlement risk of deterioration
- Raynauds improves
- Oesophagtitis worsens
- Those with interstital lung disease and pulm HTN - high risk PP deteroiration

Effect of scleroderma on pregnancy
- Success = 80%
- Increased risk PTB
- Increased risk PET / PGR / perinatal mortality
- IVL etc difficult
- GA difficult

Question 36

Scleroderma
- Pre pregnancy
- AN
- IP
- PP

Answer 36

Pre pregnancy
- Stablize disease
- Lung function tests and ECHO
- Multiple organ invovlement - advise against pregnancy

AN
- PPIs for oesphagitis
- MDT
- Regular BP
- Anesthetic review
- Steroids - avoid as can precipitate renal crisis

Question 37

What is Ehlers-Danlos syndrome

Answer 37

Group of disorders
Mostly autosomal dominantly inherited
Defects of collagen metabolism

Joint hypermobility, poor wound healing, easily bruised, rupture blood vessels / internal organs

Type 1: classic
Type III: commonest form - hypermobile - not assoicated with heart disease
Type IV: vascular - highest risk

Question 38

Risks of ehlers danlos syndrome in pregnancy
- Effect on EDS
- Effect on pregnancy

Answer 38

Pregnancy risks to EDS
Maternal mortality 25% in type IV - aortic rupture / visceral rupture. WHO GRADE 4 risk
increased joint and back pain

EDS risk to pregnancy
- Vascular EDS: uterine rupture, PTB, poor healing, severe tears, PPH
- Hypermobile EDS: PROM, preterm birth, precipitous labour, poor healing

Question 39

Management of pregnancy with EDS

Answer 39

Essential to know which type - refer to geneticist if don’t know which type
Terminate if eDS type IV- if dont terminate then offer ELCS at 34/40 (reduces risk of uterine and aortic rupture)
Increased resistance to local anaesthetics - refer to obs anaesthetics

Question 40

Rheumatoid arthritis

Periconceptual
Antenatal
Intrapartum
Postnatal

Risks, management

Answer 40

Periconceptual:
- Counsel risks: >50% improve in pregnancy, 90% flare PP. Atlanto-axial subluxation if GA (rare), PTB, IUGR, impacts of medications, GDM if on steroids, neonatal SLE if anti-Ro/La,
- Review meds: Azathioprine, hydroxycholorquinine, sulfasalazine okay. Biologics okay but avoid in late 3rd trimester (neonatal suppression effect). NSAIDs okay in 2nd trimester. Stop MTX and defer for 3months
- Anti Ro/La screen

Antenatal:
- MDT: High risk obs, rheumatology, obs physcians, anaesthetists
- Review joints
- BSL monitoring if on prednisone
- Monitor Hb and supplement

Intrapartum:
- Aim vaginal birth
- Stress dose monitoring if on prednisone
- Mindful of maternal positioning (hips)
- Alert anaesthetics (risk of axial/atlantosubluxation)

Postpartum:
- Re-establish medications
- Monitor for flare
- Breastfeeding support (meds dependent)
- VTE prophylaxis
- Contraception

Question 41

Which heart conditions are a contraindication to pregnancy? High mortality rate

Answer 41

Pulmonary hypertension
Eisenmengers syndrome
Peripartum cardiomyopathy (25-50% risk)
Previous MI (20-45%)
Class III - IV NYHA
Severe MS/ symptomatic AS
Severe aortic coarctation
Aorta >4.5cm in Marfans (>4cm HIGH risk also)
Vascular EDS

Question 42

Pulmonary hypertension
Pre-pregnancy
AN
IP
PP

Answer 42

Pre pregnancy
- Mortality rate 10-25%
-Actively advise against pregnancy
- TOP should be considered if get pregnant
(Even TOP ass with 7% risk mortality)

Issues
- Increasing R -> L shunt with those with Eisenmengers syndrome
- R heart failure
- Escalating pulmonary htn with crisis

Antenatal
- Continue therapies
- Phosphodiesterase inhibitors - sildenafil - promote blood vessel dilation
Stop endothelin receptor antagonists
Postanoid analogies - can continue
- Thormboprohylaxis not usually recommended
- Elective admission for bed rest, oxygen therapy, escalation of targeted therapies.
- Delivery: no evidence CS better
- Avoid hypovolaemia - maintain preload
- Avoid acidosis
- Avoid thromboembolism
- Avoid system vasodilation (caution with regional anaesthesia and syntocinon) DON’T give beta blocker - reduces afterload. can use nifedipine

Question 43

Marfans

Answer 43

Autosomal dominant
80% have cardiac involvement
- MV prolapse
- MV regurg
- Aortic root dilation
(>4cm very high risk, >4.5cm contraindication)
Risk aortic dissection and aortic rupture. (10% if >4cm)

Give beta blockers to reduce rate of aortic dilation
ECHO
ELCS if >4,5cm

Question 44

What do you do if mother or father has congenital heart disease

Answer 44

2-5% risk fetus having heart disease (higher if mother c.f father)
ASD 5-10%
AS 18-20%

Question 45

Mitral stenosis e.g. RHD

Answer 45

Death 10-20% if aortic root >4cm

Symptoms: asymptomatic, dyspnoiea, orthopnoea, PND, cough

Signs: mitral facies, cold peripheries, pulmonary oedema

Effects on rpegnancy
- Pulmonary oedema and deterioration, often precipitated y tachycardia (e.g. exercise, pain, anxiety, failure to adequately increase stroke volume)

Poor prognostic factors
- MV <1cm2
- Mod - severe symptoms pre pregnancy

Management
- Baseline ECG
- ECHO each trimester
- MDT care
- Optimise iron
- Aspirin and /or clexane
- Beta blockers
- Balloon valvotomy if non calcified valve
Surgery prior to pregnancy!!

IP
- Avoid fluid overload as well as severe maternal tachycardia
- Careful fluid balance
- Passive 2nd stage
- Early epidural
- NVB OK as long as can cardiac monitor
- IV access
- Continuous CTG
- Biggest risk to mother is second stage and immediately after delivery
- Consider frusemide if overload (e..g autotransfusion 3rd stage)
- Active mx 3rd stage but avoid syntometrine and carboprost
- Treat AF aggressively with digoxin and beta blockers
Avoid supine and lithotomy
Pulmonary oedema - treat with oxygen diamorphine and diuretics

PP
- Ongoing telemetry
- Strict fluid balance
- HDU cares
- VTE prophylaxis
- Advice for next pregnancy
- Consider bloods / BNP
- BF support
- Contraception

Question 46

NYHA

Answer 46

Class I: no breathlessness
Class II: Breathlessness on slight exertion
Class III: Breathlessness on mild exertion
Class IV: Breathlessness at rest

Question 47

Peripartum cardiomyopathy definition
risk factors
symptoms

Answer 47

HF development at end of pregnancy or in months following pregnancy, where no other cause is found

Risk factors
- Multiples
- HTN in pregnancy
- Muliparity
- AMA
- Afro-carribean

Symptoms
- Dyspnoea
- Reduced exercise tolerance
- Palpitation
- Pulmonary or peripheral oedema
- Symptoms related to peripheral cerebral emboli

Signs
- Tachycardia, tachypnoea
- Pulmonary oedema
- CHF
- Dysrhythmia
-

unknown aetiology ?autoimmune

Question 48

How do you diagnose peripartum cardiomyopathy?

Answer 48

BNP raised
ECHO: LVEF <45%, Global dilation with enlarged heart

Ddx
- PET
- Massive PP haemorrhage can cause transient decrease in LVEF

Question 49

How do you manage peripartum cardiomyopathy?

Answer 49

A. B, C
Elective delivery
Thromboprophylaxis
Diuretics
Vasodilators - hydralazine and / or nitrates
Cardioselective beta blockers: bisprolol, etc
digoxin if AF
inotropes if low EF and low BP
ACEI after delivery
Bromocriptine: can improve LVEF
ECMO, cardiac transplant severe cases

Prognosis
9% mortality
50% spont recovery
Depends on normalization of left ventricular size and function within 6 months after delivery
Risks if get pregnancy: arrhytmia, thromboembolism, MI, death

Counsel against future pregnancies if LV size and function doesn’t return to normal
- HIGH risk recurrence
- Worsening HF 50%
- 25% death

For those whose cardiomyopathy resolves, recurrence risk is unknown

Question 50

Causes of maternal collapse

Answer 50

Head: eclampsia, epilepsy, CVA, vasovagal
Heart: MI, arrhtymia, Peripartum cardiomyopathy, Congenital heart disease, dissection thoracic aorta
Hypoxia: asthma, PET, pulmonary oedema, anaphylaxis
Haemorrhage: Abruption, uterine atony, genital tract trauma, uterine rupture, uterine inversion, ruptured aneurysm
Whole body and hazards: hypoglycaemia, AFE, septic, trauma, complications anaesthesia, drug toxicity

Question 51

Cushings disease in pregnancy

Answer 51

Effect on pregnancy
- Increased miscarriage, IUFD, PTB, perinatal morbidity and mortality - partly explained by diabetes, hypertension
- Neonate at risk from adrenal insufficiency becasue of high maternal cortisol levels
- Severe PET
- Wound infection / poor healing
- women with treated Cushing’s do well in pregnancy

Mx
Surgery

Question 52

Hyperprolactinaemia

Question 53

Causes of seizures in pregnancy

Answer 53

Eclampsia
Cerebral vein thrombosis
PRES
TTP
Stroke
SAH
Drug and alcohol withdrawal
Hypoglycaemia
Hypocalcaemia
Hyponatraemia
Infections
Post dural puncture
Gestational epilepsy - seizures confined to pregnancy

(syncope / vasovagal)

Question 54

Epilepsy in pregnancy
- Risks

Answer 54

Effect of pregnancy on epilepsy
- Mostly no effect
- 2/3 won’t have seizure deterioration (particularly if no seizures in last year)
- Peripartum highest risk time
- Death (10 fold)

reasons for deterioration in seizure control - poor compliance, N+V = decreased drug levels, Increased volume of redistribution and increased drug clearance = decreased levels, lack of sleep, lack of absorption particularly in labour

Effect of epilepsy on pregnancy
- Long term cerebral damage not a feature as fetus resistant to short periods of hypoxia
- Small increase in micarriage, APH, PPH, hypertensive disorders, IOL, CS, FGR, PTB
- congenital abnormalities
- Genetics of epilepsy: 5% if either parent has epilepsy, 10% if one sibling, 20% if both parents

Question 55

Epilepsy
- Meds

Answer 55

Phenytoin, carbamazepine, sodium valproate, lamotrigine, toporimate, levetiracetam all cross placenta and are teratogenic

Lowest risk with carbamazepine, lamotrigine, levetiracetam

Major malformations
- NTDs
- Orofacial clefts
- Cardiac defects
- Urinary tract and skeletal malformations

Fetal valproate syndrome
- Abnormal facies

Riks highest with valproate

Risks increases with polypharmacy (10-15% with 2 drugs)

Should take 5mg FOLATE

Question 56

Epilepsy
- AN
- Delivery
- PN

Answer 56

5mg folic acid 12/40 prior to conception if on AEDs (continue throughout pregnancy)
If on carbamazepine, lamotrigine, levetiracetam continue on these if epilepsy well controlled
If on valproate ideally change, if unable wean to lowest dose possible as dose dependent effect (<600mg/day) and changes to a multiple times a day regimen to avoid peak concentrations
Advise relatives how to place woman in recovery position if has tonic clonic seziure
Bathe in shallow water
Prenatal screening for congenital abnormalities with NT scanning and fetal cardiology assessment
Often need to increase doses of AEDs - take baseline serum drug level to assess compliance and inform future changes in drug doses
If having frequent seizures may need to increase doses significantly, sometimes to doses above maximum for non pregnant women, especially lamotrigine.
If a woman is seizure free on an AED other than lamotrigine, no need to measure serial drug levels, or adjust dose unless she has a seizure

18 - 20/40 FAS assessing NTDs and cardiac anomalies

Monitor for anxiety and depression symptoms

Serial growth scans as increased risk SGA, particularly if taking AEDs. From 28/40

Vitamin K administered to mother to reduce risk of coagulopathy for enzyme inducing drugs - can give orally or IM

Intrapartum
- Epilepsy not an indication for IOL or CS
- Deliver in hospital
- 1-2% woman have a seizure in labour and 1-2% have postpartum seizure. Shoudn’t be left unattended in first 24 hours
- Uterine hypertonus can occur and cause fetal hypoxia
- Early epidural to address pain and lack of sleep
- continue regular AEDs in labour (consider IV if vomiting)
- Avoid dehydration
- Avoid pethidine / ketamine - lowers seizure threshold
- If seizure lasts more than 5 mins = status epilepticus. Left lateral tilt, maintain airway, oxygen, IV lorazepam (4mg) or PR diazepam (10-20mg)
- consider phenytoin if seizure still doesn’t terminate
- consider tocolytics if persistent uterine hypertonus

Postnatal management
- Day 3 highest risk
- Continue AEDs
- Neonates should definitely have vitamin K IM
- Neonate withdrawal symptoms: lethargy, difficulty in feeding, sedation, inconsolable crying
- BF encouraged (low secretion majority. Lamotrigine and phenobarbitone higher)
- don’t initiate lamotrigine postpartum as high transfer, but ok if on lamtorigine throughout pregnancy
- Slowly reduce AEDs over the first few weeks postnatally. Decrease lamotrigine faster
- Change nappies on floor
- contraception - avoid COC, POP, Jadelle in enzyme inducers - carbamazepine and phenytoin. Depo, mirena and copper ok
Lamotrigine - don’t use COC either

Question 57

Cerebral vein thrombosis
- What is it
- Symptoms
- DDx
- Management

Answer 57

Possible trauma to endothelial lining of cerebral sinuses and obstruction from clot. Oedema occurs and then venous haemorrhage can occur. Causes headache, seizures, impaired consciousness, signs of raised ICP, vomiting, photophobia , hemiparesis, fever, leukocytosis
DDx: eclampsia, SAH, reversible cerebral vasoconstriction syndrome and herpes encephalitis

Do CT venogram or MR venogram

Management: hydration and anticoagulation, thrombophilia screen

Question 58

Bells palsy

Answer 58

Unilateral LMN lesion of the facial nerve
Facial weakness, loss of power of frontalis muscle (can’t wrinkle forehead)
Ass pain around ear or loss of taste

Occurs secondary to latent herpes virus (HSV1 and herpes zoster)
- reactivated from cranial ganglia

Occ due to swelling in the region PP

dx: clinical

Management
- 95% improve spontaneously
- Short course of corticosteroids can be given
- Rule out Ramsay Hunt syndrome first - examine ear for vesicles

Question 59

Multiple sclerosis
- Definition
- Presentations

Answer 59

Chronic autoimmune demyelination of CNS (not PNS) - sensory or motor, relapsing, remitting course.
MRI brain - 2 separate lesions at one time

Genetic - ass with HLA system

Presentations
- Optic neuritis
- Neurogenic bladder dysfunction
- Impotence
- Sensory deficits
- UMN deficits - spasticity of legs
- Cerebellar involvement
- Vertigo

Question 60

Multiple sclerosis
- Effect of pregnancy on MS
- MS effect on pregnancy

Answer 60

Effect of pregnancy on MS
- Less likely to present or relapse in pregnancy, less disease activity
- Increased UTIs in those with neuropathic bladders
- Fatigue and balance can be an issue
- Rate of relapse increases in first 3/12 following pregnancy, then declines back to baseline at 10/12
- Long term outcomes not altered

MS doesn’t cause issues for pregnancy

Relapse can be treated with steroids
Biologics

Question 61

Cystic fibrosis - risks and contraindications to pregnancy

Answer 61

Dysfunction of all exocrine glands with abnormal mucus production

Malnutrition
Diabetes
Persistent and recurrent lung infections
Median age of death - 47

Effect of pregnancy on CF:
- Mortality increased (not significantly increased above age matched non pregnant woman)

Mortality increased in
- Moderate to severe lung disease - FEV1 <50-60%, no adverse effect of pregnancy on long term survivial.
- Maternal pulmonary hypertension
- Cyanosis
- Hypoxaemia

Pregnancy contraindicated if
- pulmonary hypertension
- Cor pulmonale (RHF secondary to pulmonary hypertension)
- FEV 1 <30-40% predicted
- Burkholderia cepacian recent infection as can cause rapid deterioration in lung function
- Cor pulmonale

Maternal morbidity
- Poor weight gain
- Deterioration in lung functino with worsening dyspnoea, exercise tolerance, oxygen saturations
- pulmonary infective exacerbations
- CCF

Effect of CF on pregnancy
- PTB 10-25%
- FGR - esp if chronic hypoxia and / or cyanosis and or FEV1 <50-60%

Question 62

Pre pregnancy counselling CF

Answer 62

Pregnancy contraindicated if
- pulmonary hypertension
- Cor pulmonale (RHF secondary to pulmonary hypertension)
- FEV 1 <30-40% predicted
- Burkholderia cepacian recent infection as can cause rapid deterioration in lung function
- Cor pulmonale

Safe if FEV1 >70-80%
Screen for diabetes
All offsprine will be carriers of CF so recommend partner carrier screening (1:25 risk of carrier in general population, there 2% risk of CF in baby if partner status unknown)

Question 63

CF
- AN
- IP
- PP

Answer 63

MDT care
Nutrition: 90% have pancreatic insufficiency and require enzyme supplements. Fat soluble vitamin supplements should be considered
Involve dietician

Pregnancy CI if
- FEV1 <40%
- Recent Burkholderia infection
- Pulm HTN

Pulmonary infections
- Chest physio
- Abx, avoid tetracyclines
- Aggressive treatment of infections - IV penicillins, aminoglycosides, cephalosporins if resistant pseudomonas
- Sometimes bronchodilators or steriods required
- Inhaled recombinant human DNAse - reduces viscosity in sputum, dornase alfa

Avoidance of prolonged hypoxia
- Consider admission for bed rest and oxygen therapy if persistent low oxygen sats
- Symptom deterioration may warrant delivery - most deliver vaginally at term
- Consider instrumental to shorten second stage - patients with CF are prone to pneumothoraces which prolonged pushing can cause

Regular growth assessment
- bed rest and oxygen supplementation may improve growth

BF
- Encourages
- Normal content to sodium and protein in breast milk

If has had lung transplant outcomes less favourable after delivery - rejection much more common. Very high risk of PTB

Question 64

Pulmonary hypertension
- Causes

• Risks
• Management in pregnancy

Answer 64

Mortality rate 10-25%
Pulm HTN can be idiopathic, drug related, CT disease such as scleroderma and SLE
Related to congenital heart disease such as ASD, VSD, Eisenmenger syndrome, L heart disease, lung disease such as CF, interstitial lung disease, hypoxia, sleep disordered breathing, chronic thromboembolic pulm HTN

Normally pulmonary vascular resistance will fall in pregnancy, with pulmonary hypertension this doesn’t occur so pulmonary blood flow cannot increase to match CO

Actively advise against pregnancy

If do get pregnant
- offer TOP
- TOP itself has a 7% risk of mortality
- Most women who die, do so soon after delivery
- Problems relate to right heart failure, increasing right ot left shunt in eisenmengers syndrome (cyanotic heart disease), escalating pulmonary hypertension with pulmonary hypertensive crises

Antenatal
- Targeted therapies
Phsophodiesterase inhibitors (sildenafil - promotes blood vessel dilation) safe in pregnancy
STOP endothelin receptor antagonists e.g. bosentan, ambrisentan
Safe to continue postanoid analogues e.g. iliprost, NO

Thromboprophylaxis not routinely recommended
Elective admission for bed rest and oxygen therapy
No evidence CS is better
Avoid hypovolaemia (monitor with ECHO)
Avoid acidosis
Avoid VTE
Avoid systemic vasodilation (careful with regional anaesthesia, syntocinon. Don’t given anything that decreases afterload (beta blockers). Can use nifedipine

women with congneital heart disease should be referred for fetal ECHO
- 2-5% risk of fetal cardiac disease, 5-10% if ASD, 20% if AS, Marfans and hypertrophic cardiomyopathy have AD inheritance

Question 65

Congenital aortic stenosis

Answer 65

Most is ass with bicuspid aortic value - risk of dilated ascending aorta

Significant obstruction aortic valve area <1cm2
Can get angina, hypertension, heart failure, sudden death
Inability to increase BP with exertion, impaired LV function or symptoms
Symptoms / HTN can be controlled with beta blockers
Can do balloon valvotomy if severe

Question 66

Hypertrophic cardiomyopathy
- Inheritance
- What it is
- Risks in pregnancy

Answer 66

AD
Abnormal thickeneing of myocardium
Sudden death if non sustained VT, failure of BP to increase during exercis
Well tolerated in pregnancy because of increase in LN size
Beta blockers can be used.

Question 67

Artificial heart valves in pregnancy

Answer 67

• Mechanical: high risk thrombosis
  Balance risks and benefits of warfarin vs LMWH (higher risk of clot with LMWH)
• Could use LMWH between 5 and 12 weeks and then switch back to warfarin
  Aim INR 2.5 - 3.5
  If using LMWH do SC dosing.
  If using warfain stop 10-14 days prior to delivery so effects in fetus wear off
  Abx prophylaxis not usually recommended
• Fetal warfarin syndrome

o 5-10% of those exposed at 6-9 weeks
o Nasal hypoplasia/absent nasal bridge
o Wide spaced eyes
o Microphthalmia
o Limb hypoplasia
o Stippled epiphysis
o Intellectual abnormality