Maternal Medicine Flashcards
Respiratory Indications for ECMO
ARDS
Pneumonia (infectious/aspiration/interstitial)
Severe asthma
Thoracic trauma causing lung contusion
Alveolar or pulmonary haemorrhage
Severe inhalation injury
Air leak syndromes (bronchopleural fistula)
Acute airway obstruction
Lung transplant
Cardiac indications for ECMO
Acute MI
Myocarditis
Cardiac arrest
Post heart transplant
Post cardiotomy
Hypothermia with cardiac instability
Postpartum acute cardiomyopathy
Drug intoxication
Septic cardiomyopathy
Arrhythmic storm
Interventional cardiac procedures
When to consider ECMO
When estimated mortality is in excess of 50-80% despite optimal medical management
Complications of ECMO
Intracranial haemorrhage
DIC
HIT
Bleeding
Hypertrophic cardiomyopathy incidence
0.1-0.5% of women
Symptoms/signs of hypertrophic cardiomyopathy
Syncope
Chest pain
Ejection systolic murmur
Pan-systolic murmur
Heart failure
Arrhythmias
Treatment of hypertrophic cardiomyopathy in pregnancy
B-blockers
Incidence of sarcoidosis in pregnancy
1 in 2000
Sarcoidosis site involvement
Lungs (1st)
Skin (commonest extra-pulmonary)
Heart (50%)
Brain
Eyes
Joints
Liver (20% hepatomegaly)
Pulmonary sarcoidosis presents as
Interstitial lung disease with fibrosis 20%
Pulmonary hypertension
Skin manifestations of sarcoidosis
Granuloma (lupus pernio)
Erythema nodosum
Erythema multiforme
Nummular eczema
Rate of myocardial infiltration with sarcoidosis
2-7%
Rate of CNS involvement with sarcoidosis and commonest site
5-13%
Cranial nerves
Pathogenesis of sarcoidosis
Th1 cell mediated
TNF-a
Interferon-y
Influx of CD14 macrophages
Investigations for sarcoidosis
CXR/CT
broncheolar lavage
Transbronchial biopsy
Cardiac MRI
ECG/echo
MRI head
EMG for myopathy
When is pregnancy is contraindicated in insterstitial lung disease?
When FVC<1-1.5L
Course of sarcoidosis in pregnancy
Majority have no change or improvement
Some worsen
Postnatal flare
Effects of sarcoidosis on pregnancy
PET
VTE
FGR
C/S
PPH
Baseline tests for sarcoidosis
FBC
U+E
LFT
Calcium
ACE
ECG
Echo if cardiac sarcoidosis is known
24hr tape if palpitations
Mycophenelate mofetil risks in pregnancy
Microtia (no ear cartilage)
Facial clefts
Micrognathia
Miscarriage
FGR
Washout period for mycophenelate
6 weeks
Washout period for MTXA
4 weeks
Give high dose folic acid
Leflunomide washout period
11 days with cholestyramine
When to discontinue adalimumab
28 weeks
When to discontinue infliximab
20 weeks
AN management of sarcoidosis
Aspirin
VTE
Growth scans
Enhanced BP monitoring
Investigate tachycardia and palpitations
Haemophilia A
Factor VIII deficiency
Haemophilia B
Factor IX deficiency
Impact of pregnancy on factor VIII
Increases x 3
Impact of pregnancy on factor IX
No effect
Normal range for factor VIII and IX outside of pregnancy
0.5-2.0 iu/ml
There is no family history in _____ of haemophilia patients
50%
Obligate haemophilia carrier status
Affected father
OR
Affected son and relative in maternal line
Risk of haemophilia in a male baby after a spontaneous affected sibling
45%
Classification of haemophilia A
Severe - <0.01 iu/ml
Moderate - 0.01 - 0.05 iu/ml
Mild - 0.06 - 0.4 iu/ml
Risk of resistance to clotting factor treatment
40%
Prenatal diagnosis of severe haemophilia
PGD
cell free fetal DNA for fetal sex
CVS for male fetus 11-14 weeks
Amniocentesis 3rd trimester to inform delivery options
Risk of intracranial and extra cranial haemorrhage with haemophilia
RR 56% for ICH
RR 92% for ECH
Antepartum management of haemophilia
No ECV in severe haemophilia
TXA
Levels 0.5 iu/ml to cover surgical procedures and miscarriage
DDAVP with 1L fluid restriction in 24 hours to increase factor VIII
Recombinant factor VIII if DDAVP ineffective
Recombinant factor IX if <0.5 iu/ml (measure before and after, then 4-6h after rx)
Mode and timing of delivery with haemophilia
C/S for affected male babies/status unknown/severe haemophilia
No ventouse/midcavity NBFD
No FSE in severe or moderate haemophilia
Intracranial haemorrhage risk with ELLSCS vs forceps in haemophilia
OR 0.69 vs 2.8
(4x less)
Optimal level of factor VIII after DDAVP treatment
1.0 iu/ml
When to give DDAVP and TXA intrapartum for haemophilia
As close to delivery as possible
Postpartum management of haemophilia
Maintain factor VIII/IX above 0.5 for 3 days after SVD, 5 days after operative delivery
TXA until lochia is minimal
No VTE if level <0.6
Investigation of the neonate in haemophilia carrier mothers
Cord blood testing in males
Re-test 3-6 months
Follow up with haematology
Cranial USS screening prior to discharge if moderate/severe haemophilia
MRI head if symptoms/signs of ICH
Treatment in neonate with haemophilia
Short term prophylaxis if moderate/severe or preterm or trauma at delivery
Types of vWD
Type 1 - partial quantitative
Type 2 - qualitative
Type 3 - severe quantitative
Commonest bleeding disorder
vWD
Level of care for vWD according to type
Normal unit with haem input for mild/moderate type 1
Specialist input for type 2/3 or severe type 1
Target level for factor VIII and vWF:RCo
0.5 to cover for spontaneous miscarriage and surgical procedures
Prevalence of VWD
1 in 1000
Mode of inheritance for vWD
Type 1 - variable penetrance with multiple mutations
Type 2B - dominant
Type 2N - recessive
Type 3 - recessive
Prevalence of type 3 vWD
1 in 1 million
Higher in consanguineous communities
Antenatal Treatment of vWD
DDAVP
Restrict fluid 1 litre in 24 hours
Contraindication to DDAVP
PET
Arterial disease
Uncontrolled hypertension
Side effects of DDAVP in VWD
Type 2B can develop thrombocytopenia
Intrapartum management of type 2/3 vWD
Avoid FBS, FSE, ECV, ventouse and mid cavity forceps
Postpartum care in vWD
TXA for 14 days
Keep factor VIII above 0.5 for 3 days after SVD or 5 days after operative delivery
Neonatal management with VWD
Oral vit K
Cord blood testing in type 2/3
Retest at 3-6 months for type 2/3
Cranial US +/- MRI head for type 2/3
Inform parent of signs for ICH
Dose of DDAVP
0.3 micrograms/kg of pregnancy body weight
Treatment for acute haemorrhage in vWD
DDAVP
Viral inactivated concentrate with VWF and factor VIII (hep A and parvo resistant to inactivation)
Factor XI deficiency inheritance
Autosomal dominant
Autosomal recessive
Factor XI prevalence
1 in 1 million general population
8% Ashkenazi Jews heterozygous
0.2 - 0.5% Ashkenazi Jews homozygous
Factor XI maintenance levels
0.5 iu/ml
Effects of pregnancy on Factor XI levels
No effect
Antepartum monitoring of factor XI
Booking
3rd tri
Prior to invasive procedures
Bleeding risk with factor XI deficiency
Type O
High risk phenotype
Testing in neonate for factor XI deficiency
No testing unless mum is homozygous or compound heterozygosity
Management of factor XI deficiency
Exclude compounding vWF/thrombocytopenia
Can give TXA OR factor XI concentrate (both together increase risk of VTE) or FFP
What are the rare bleeding disorders?
Deficiencies of fibrinogen
Factor II, V, VII, X and XIII
Combined factor V and VIII deficiencies
Congenital deficiency of vit K dependent factors
Maintenance level for the rare bleeding disorders
Aim >0.2 iu/ml
Management of rare bleeding disorders
TXA 15-20 mg/kg or 1g QDS for minor bleeds
+/- factor replacement
Factor II deficiency is AKA
Prothrombin deficiency
Management of prothrombin (factor II) deficiency
If bleeding/labour or for c/s and <0.2/ml give prothrombin complex concentrate 10-20 iu/kg at 48 hour intervals
Maintain level >0.2 for 3 days minimum
If already receiving prophylactic prothrombin complex then continue through pregnancy
Management of factor V deficiency
If activity <0.2/bleeding/delivery then give 15-25ml/kg FFP
Further FFP at 10ml/kg 12 hourly to maintain activity 0.2-0.4 for 3 days
Consider platelets for severe bleeding or c/s
Factor VII deficiency management
Recombinant factor VIIa 15-30 micrograms/kg every 4-6 hours to maintain level >0.2
3-5 days after c/s
Only in response to severe bleeding for other women
Severe factor X deficiency management
Antenatal prophylaxis 2-3x/week for recurrent bleeding or adverse pregnancy outcome with prothrombin complex concentrate
Give 20-40iu/kg prothrombin complex concentrate for bleeding or c/s to maintain activity >0.4
10-20 iu/kg daily for 3 days maintenance
Severe factor XIII deficiency management
Factor XIII plasma concentrate prophylaxis every 14-21 days
Maintain activity >0.2
Consider additional 10-40 iu/kg in established labour/ prior to c/s
Factor V and factor VIII deficiency management
Consider FFP 15-25 ml/kg in established labour or before c/s. Maintain level 0.2-0.4 iu/ml
Consider factor VIII if less than 0.5 iu/kg in third trimester
Fibrinogen disorders and their inheritance
Afibrogenaemia - recessive (bleeding)
Hypfribrinogenaemia with or without qualitative defects (dysfribrinogenaemia) - dominant (variable bleeding/thrombosis risk)
Pregnancy outcomes with fibrinogen disorders
APH
PPH
VTE
pregnancy loss
ICH
Umbilical bleeding
Management of fibrinogen deficiency
Maintain levels 0.5-1 g/litre antepartum and 3 days post partum
TXA for minor bleeding
Fibrinogen concentrate 50-100mg/kg through pregnancy 2x/week - consider LMWH if low risk for bleeding
What is Bernard Soulier syndrome? (BSS)
Deficiency of membrane GP Ib-IX-V complex
Causes abnormal adhesion of platelets
Inheritance of BSS
Autosomal recessive