selective complex medical disorders in pregnancy

1. GUIDE- DR.R.PATEL(MD)
RMO –DR PARTHA
Complex medical
disorders in pregnancy

2. DISCUSSION OF KEY CAUSES OF
MATERNAL MORTALITY DUE TO
PREEXISTING AND PREGNANCY
SPECIFIC MEDICAL DISEASES.
INTRODUCTION

3. MATERNAL DEATH
• A FIFTH OF WORLDWIDE MATERNAL DEATH OCCUR IN
INDIA.
• INDIAN MMR REDUCED FROM 212/10000 LIVE BIRTHS
IN 2007 TO 178 (COMPAREDTO 10/10000 IN UK) IN
2012.
• THE FIFTH UN MDG OF MMR 109/10000 NOT
REACHED.
• DATA COLLECTED BY RGI SHOW THAT THE MAJORITY
OF MATERNAL DEATHS IN INDIA ARE DUETO
“DIRECT”(OR OBSTETRIC) CAUSES PRIMARILY
HAEMORRHAGE, HYPERTENSION AAND SEPSIS WITH
16% CLASSIFIED AS “INDIRECT” DUETO MEDICAL
DISEASESTHAT ARE PREEXISTING OR OCCURING IN
PREGNANCY.

4. USING THE WHO NEAR-MISS TOOL
OVER A 10 YEAR PERIOD, A
SINGLE CENTRE IN SOUTH INDIA
SHOWED EVEN HIGHER VALUE OF
48.1% OF MATERNAL DEATHS AS
DUE TO “INDIRECT” CAUSES

5. INDIRECT CAUSES OF MATERNAL DEATH SOUTH INDIA
INDIRECTCAUSES OF
MATERNAL DEATH
n
% of all maternal
deaths
Maternal infectious and
parasitic diseases(mainly
pulm.TB,scrub typhus
and malaria
35 16.5
cardiovascular 28 13.2
Hepatic disorders 24 11.3
hematological 6 2.8
neurological 4 1.9
respiratory 3 1.4
renal 2 0.9
Total 102 48.11
Additional other maternal
diseases classifiable
elsewhere
67 31.6

6. DISORDERS AFFECTING
PREGNANCY……………
 CARDIAC DISEASES (VALVULAR HEART DISEASES,
PREGNANCY WITH PROSTHETIC HEART VALVES,
ANTICOAGULATION IN PREGNANCY, PERIPARTUM
CARDIOMYOPATHY..)
 HEPATIC DISEASES (HEPATITIS E VIRUS
INFECTION, HELLP SYNDROME, ACUTE FATTY LIVER OF
PREGNANCY, OBSTETRIC CHOLESTASIS..)
 PRESCRIBING ( DRUG SAFETY)

7. CARDIAC DISEASE IN
PREGNANCY

8. CARDIOVASCULAR
DISORDERS
about 1% of pregnancies
complicated by heart
diseases
leading cause of maternal mortality
Mortality rate 50% in case pulmonary
hypertension

9. Physiologic adaptation to pregnancy
 Increase blood volume on 40 -50 %
 Increase cardiac output 30-50%
 Decreased systemic vascular resistance
 The heart elevated upward and rotated forward to the
left
 Pulse increase about 10-15 beat/min after 14-20 weeks,
palpitation
 Disturbed rhythm: arrhythmia, premature atrial
contractions, premature ventricalar systole
 Increase clot factors (VII,VIII, IX, X, fibrinogen)
 Cardiac output changes during labor and birth
 Intravascular volume changes just after childbirth
Cardiac hypertrophy

10. Physiologic adaptation to pregnancy
 If cardiac changes are not well tolerated
cardiac failure can develop during pregnancy,
labour, postpartum
 If myocardial disease develops, valvular
disease exists or congenital heart defect is
present, cardial decompensation is
anticipated

11. Percent change in heart rate, stroke volume,
and cardiac output measured in the lateral
position throughout pregnancy compared with
pregnancy values

12. Hemodynamic changes post partum
Blood shifting “auto-transfusion”
(from the contracting uterus to the
systemic circulation)
Increase in effective blood volume
Substantial increase in LV filling pressure, SV and CO
Clinical deterioration
Blood loss
during delivery-
• HR and CO return to pre-labor values within 1 hour. MAP and SV within 24 hours.
• Hemodynamic adaptation persists post partum and return to pre-pregnancy values
within 12-24 weeks after delivery.
Increase in venous return
(relief of caval compression)

13. History
Exercise capacity
Current or past evidence of HF
Associated arrhythmias
Physical exam
Cardiac Hemodynamics
Severity of heart disease, PA pressures
Echo, MRI.
Exercise testing
Useful if the history is inadequate to allow assessment of functional capacity
During pregnancy
Evaluate once each trimester and whenever there is change in symptoms
Multidisciplinary approach, Fetal Echo
Beforeconception
During Labor & Delivery
Multidisciplinary approach (Obstetrician, Cardiologist, Anesthesiologist)
Tailor management to specific needs

14. CARDIAC DISEASE
1. Rheumatic 80- 90% of Heart Disease in
pregnancy
2. Congenital  10-35% of HD in pregnancy

15. High-risk pregnancy
 Pulmonary HTN and Eisenmenger’s syndrome.
 Symptomatic obstructive cardiac lesions:
■ MS,AS, PS, uncorrected coarctation of the aorta.
 Marfan’s Syndrome with dilated aortic root(>45
mm)
 Systemic ventricular dysfunction with
* NYHA class iii-iv
* LVEF < 30 %
 Patients with prosthetic valves.
 Significant uncorrected Cyanotic Heart Disease.
 Aortic dilatation more than 50 mm in aortic
disease associated with bicuspid aortic valve.

16. Contraindications to Pregnancy
Lesion Maternal
death rate
(%)
• Severe Pulmonary Hypertension 50
• Severe obstructive lesions:
AS,PS, HOCM, Coarctation.
17
• Systemic Ventricular Dysfunction,
NYHA class III or IV
7

17. Maternal cardiac disease risk
group
 Group I (mortality rate 1%)
■ Corrected tetralogy Fallot
■ Pulmonic/tricuspid disease
■ Mitral stenosis
■ Patern ductus arteriosus
■ Ventricular septal defect
■ Atrial septal defect
 Group II (mortality rate 5-15%)
■ Mitral stenosis with atrial fibrillation
■ Uncorrected tetralogy Fallot
■ Aortic coarctation (uncomplicated)
■ Marfan syndrome with normal aorta
 Group III (mortality rate 20-50%)
■ Aortic coarctation (complicated)
■ Myocardial infarction
■ Marfan syndrome with aortic involvement
■ Pulmonary hypertension

18. The indications forTermination of pregnancy:
Because of high maternal risks, MTP is indicated in:
1.Eisenmenger’s syndrome.
2.Marfan’s syndrome with aortic involvement
3.Pulmonary hypertension.
4.Coarctation of aorta with valvular involvement.
• Termination should be done before 12 weeks of
pregnancy.

19. Mitral Stenosis
 The pressure gradient across the narrow valve
increases secondary to the increased heart rate and
blood volume
 Left atrial pressure increases, back pressure into the
lungs causes breathlessness, swelling in the legs
and may lead to atrial arrhythmias.
 Stretching of the atrium can also occur causing
palpitations and arrhythmia.

20. MITRAL STENOSIS
 90%
 During pregnancy  CO increase obstruction
worsens
 Asymptomatic pt.  symptomatic
 Symptoms of cardiac decompensations or
pulmonary edema appear as pregnancy progresses
in Pt. with severe Mitral stenosis(valve diameter
less than 1 cm2)  Atrial fibrillation exacerbate
the change so it would be recommended to
start pregnant women with moderate or severe
MS on beta blockers in the 1st trimester aiming to
keep the maternal heart rate at 60-70 beats/min.

21. Mitral Stenosis
 Vaginal delivery can be permitted in most
patients with judicious use of fluids and
avoidance of supine and lithotomy positions.
 Hemodynamic monitoring is recommended
(Swan) and should be continued several hours
following delivery

22. Treatment in presence of
decompensation
 Furosemide –to treat pulmonary edema
 Beta blockers—to control heart rate
 Digoxin—who cannot tolerate beta blockers, in AF.
 Prophylactic LMWH—enlarged left atrium, to prevent
atrial thrombus
 ***
 Surgical treatment– in persistent decompensation or
further deterioration. percutaneous balloon mitral
valvoplasty if mitral valve is pliable without significant
regurgitation.MVR in severely stenotic and calcified
valve.

23. Aortic Stenosis
 AS lead to obstruction to
left ventricular ejection
 Mild AS is usually tolerated
 Moderate to severe AS is
likely to be associated with
symptomatic deterioration
during pregnancy
 Women with valve area
<1.0 should consider valve
replacement prior to
pregnancy

24. Aortic Stenosis
 Symptoms often develop in the 2nd and 3rd trimester
■ Exertional dyspnea
■ Chest pain
■ Syncope
 Fetal effects included
■ Intrauterine growth retardation
■ Premature delivery
■ Reduced birth weight
■ Increase in cardiac defects

25. Marfan Syndrome
 Autosomal dominant genetic disorder
characterized
■ weakness of the connective tissue,
■ resulting in joint deformities,
■ ocular lens dislocation,
■ weakness of aortic wall and root
 Mitral valve prolapse (90%)
 Aortic insufficiency (25%) risk of
aortic dissection and rupturing
 Pregnancy in patients with Marfan poses 2
problems
■ Cardiovascular complications of the mother
■ Risk of having a child who inherits Marfan’s
syndrome
 Cardiovascular problems
■ Dilation of the ascending aorta, may lead to
development of aortic regurgitation and
heart failure
■ Proximal and distal dissections of the aorta
with possible involvement of the coronaries

26. Marfan’s Syndrome
Management
■Vigorous physical activity should be avoided
■Beta blockers (reduces the rate of aortic dilation)
■If substantial dilation/dissection should occur,
depending on the stage of pregnancy
 therapeutic abortion,
 early delivery or
 surgical intervention should be considered

27. Eisenmenger Syndrome
 Right-to-left or bidirectional shunting at
atrial or ventricular level and combined with
elevated pulmonary vascular resistance
 High risk of maternal (30-50%) and fetal
(50%) morbidity and mortality
 Pregnancy is contraindicated (contraception
or termination of pregnancy)
 Death usually (75%) occurs between the first
few days and weeks after delivery, but the
cause is unclear

28. Eisenmenger Syndrome
 Patients should be monitored closely for any signs of
deterioration
 Early elective hospitalization is recommended
 Activity is strictly limited
 Hemodynamic monitoring is required
 Anticoagulant???
 Prophylaxis of hypovolemia
 Oxygen
 Epidural analgesia

29. PROSTHETIC VALVES AND
PREGNANCY
Anticoagulation

30. Valve replacement

31. Mechanical Valves and
Anticoagulation during
Pregnancy
 Heparin may not prevent valve thrombosis:
?how much ?route.
 Adequate anticoagulation difficult.
 Heparin can produce osteoporosis.
 Warfarin can cause embryopathy.
 Baby ASA safe + probably beneficial.
1-4% mortality in pregnant women with
mechanical valve prosthesis,Whatever the
anticoagulation regimen.
No Ideal Solution

32. ANTI-Xa LEVELS for
maintenance of effective
anticoagulation with LMWH
INDICATION FORTHERAPEUTIC
LMWH
PEAK anti-Xa LEVEL (4 HRS POST
DOSE ) (RECOMMENDED(IU/ML)
VENOUSTHROMBOEMBOLISM 0.6-0.9
AORTIC METALVALVE 0.8-1.0
MITRAL METALVALVE 1.0-1.2

33. Warfarin vs. Heparin
Warfarin
 Crosses the placenta.
 ↑early abortion, prematurity,
and embryopathy when used in
1st trimester (6th–12th weeks).
 CNS & Eye abnormalities (2nd &
3rd trimester).
 Bleeding in the fetus (especially
at delivery)
■ Should be stopped before
delivery.
Heparin
 Does not cross the placenta
 No teratogenicity
 No fetal bleeding
 Twice daily SC injection
 Risk of osteoporosis
■ <2% symptomatic fractures.
■ but 30% decrease in bone density.
 Risk for thrombocytopenia
 ↑↑ Risk of thrombosis
“warfarin embryopathy”: Nasal hypoplasia, Bone epiphysis, optic atrophy,
blindness, seizures.
Overall risk around 5%. Decreases with the use of UFH in the first 3 months

35. Low-dose ASA
 The additional use of low-dose aspirin should
be considered, particularly in
 Women with high-risk valves.
 Patients with cyanosis.
 Patients with intra-cardiac shunts.
 Women with previousTIAs and/or strokes.
 And women with atrial fibrillation.

36. LMWH
 Do not cross the placenta.
 Do not require frequent PTT monitoring
 and have a longer half-life than UFH.

37. Peripartum Cardiomyopathy
 A form of dilated CMP with LV systolic dysfunction that results in
the signs and symptoms of heart failure
 Criteria
■ Development in last month of pregnancy or the first 5 months
after delivery
■ Absence of heart disease prior to last month of pregnancy
■ Absence of identifiable cause of heart failure
■ LV systolic dysfunction
 Etiology is unknown
 Theories
■ Genetic predisposition
■ Autoimmunity
■ Viral infection

38. Peripartum Cardiomyopathy
 Associated risk factors:
■ Age - over 35
■ twin pregnancy
■ gestational hypertension
■ Multiparity
■ African-american race
■ use of tocolytic therapy
 Mortality rate 25-50%

39. Peripartum Cardiomyopathy
 Clinical findings
■ Left ventricular failure
 Dyspnea
 Fatigue
 Edema
 Enlarged heart
 Tachycardia
 arrhythmias
Management
■Acute heart failure treatment with O2, ,diuretics, digoxin and
vasodilators (hydralazine is safe)
■Because of the increased incidence of thromboembolic
events, anticoagulation therapy is recommended

40. Pregnancy result in case of
Cardiovascular Disorders
 miscarriages
 Preterm labor and birth
 IUGR
 Congenital heart lesions (4-16%)
 Maternal mortality

41. HEPATIC DISEAES
 HELLP SYNDROME
 ACUTE FATTY LIVER OF PREGNANCY
 OBSTETRIC CHOLESTASIS
 HEPATITIS E VIRUS INFECTION

42. HELLP SYNDROME
HEMOLYSIS
ELEVATED LIVER ENZYMES( TRANSAMINASES
ARE MILDLY RAISED,AST>70 U/L)
LOW PLATELETS (<100*10^9/L)
HELLP syndrome
thought to be a severe form of pre-eclamptic
liver dysfunction but it can occur in
normotensive patients as well.

43. Majority of patients with HELLP syndrome present
in 3rd trimester, but up to 1/4th of these patients can
present only in the immediate post-partum period.
Antenatal pre-eclampsia is known to occur in most
of these patients with postpartum presentation.
Absence of hemolysis; i.e. ELLP syndrome
and partial HELLP syndrome are other diagnoses
that can be entertained.
Partial HELLP syndrome
elevated liver enzymes (serum aspartate
aminotransferase > 40 U/L),
low platelets (<150*109/L)
with or without evidence of hemolysis.

44. Severe thrombocytopenia
portends poor prognosis and urgent need for
management.
Abnormally elevated prothrombin time
Signifies an underlying DIC
Haemolytic uremic syndrome and thrombotic
thrombocytopenic purpura
albeit uncommon in pregnancy, are other mimics
of HELLP syndrome.

45. Prognosis
Maternal mortality
0 to 15%.
Maternal morbidity
acute renal failure,
hepatic infarct,
hepatic hematoma,
hepatic rupture,
disseminated intravascular coagulation,
post-partum hemorrhage,
Pulmonary edema and rarely liver cell failure.
The mortality in patients with any of these severe
complications is higher.
There are usually no long term maternal
complications.

46. Management
1. close monitoring
2. optimisation of blood pressure
3. seizure prophylaxis with MgSO4.
4. Delivery
 only definitive management.
The decision to deliver is to be balanced with
fetal maturity
urgent delivery
near term (>34 weeks)
fetal distress
Systemic complications as DIC
renal failure etc.

47. 5. delivery be delayed with careful monitoring.
Only in a small subset with mild/asymptomatic
HELLP syndrome in early pregnancy
6. Steroids
should be considered only for fetal lung maturity.
7. The route of delivery
usually by Caesarean section
but is decided by the Obstetricians on a case-to-
case basis.

48. Acute fatty liver of
pregnancy

49. Rare
1 in 7000 to 1 in 20,000 pregnancies
Potentially lethal
for both the mother and fetus, especially if
diagnosis is delayed.
Commoner in:
primigravidae (although this predilection is not
as marked as in pre-eclampsia).
An association with
male fetuses (ratio 3:1)
multiple pregnancy (20% of cases).
Maternal mortality:
10% to 20%
Perinatal mortality:
20% to 30%

50. Diagnosis
DD:
HELLP syndrome
Profound hypoglycaemia (70%)
Marked hyperuricaemia (which is out of
proportion to the other features of
preeclampsia (90%)
Coagulopathy (90%) in the absence of
thrombocytopenia.

51. ABOUBAKRELNASHAR

52. Management
Expeditious delivery:
improved prognosis for mother and baby.
Multidisciplinary team in an intensive care setting.
Coagulopathy and hypoglycaemia
should be treated aggressively before delivery.
Large amounts of 50% glucose may be needed
to correct the hypoglycaemia
fresh frozen plasma and albumin should be
given as necessary.

53. The best markers of severity:
– Prothrombin time
– Glucose
– Acidosis and raised lactate
– Encephalopathy.

54. Plasmapheresis
has been used in some cases.
N-acetylcysteine (NAC)
an antioxidant and glutathione precursor,
promotes selective inactivation of free radicals
logical tt in hepatic failure
 Multiple system failure:
ventilation and dialysis.

56. Clinical features
1. Pruritus:
Severe
Limbs& trunk, particularly the palms &soles
2nd 3rdhalh of pregnancy (usually during the T) .
2. Insomnia and
common.
3. ±Excoriations
malaise:
No rash.
4. LFT:
abnormal.
5. ± dark urine, anorexia
6. Malabsorption of fat
steatorrhoea.

57. Diagnosis
exclusion. 3 steps:By
1.
2.
a.
History of pruritus without rash
Abnormal liver function tests
Transaminases:
Moderate (<3fold) elevation
(ALT is the most sensitive)
b. Alkaline phosphatase:
Raised beyond normal pregnancy values
c. Gamma-glutamyl transpeptidase (ƔGT):
Raised in 20%
d. Bilirubin:
Mild elevation: less common
e. Serum total bile acid: Increased
Primary bile acids (cholic acid& chenodeoxycholic
may increase 10 to100-fold
acid):

58. 3. Exclusion of other causes of
liver function.
a. Liver ultrasound:
Gallstones without evidence of
obstruction does not exclude a
itching& abnormal
extra-hepatic
diagnosis of OC
Gallstones are common in women with OC
b. Viral serology:
for hepatitisA, B, C & E, EBV, CMV
c. Liver autoantibodies:
for pre-existing liver disease:
anti-smooth muscle antibody: chronic
hepatitis
active
anti-mitochondrial antibodies: primary
biliary
cirrhosis.

59. Factors affecting:
1. Maternal symptoms or transaminase levels:
relation.
2. Serum maternal bile acids:
<40 micromol/I: No increase in fetal risk Risk
of fetal complications (PTL, asphyxia,
meconium) increased by 1-2% per additional
micromol/l of serum bile acids.
No

60. Management
1. Counselling
:Risks to the fetus
Close surveillance.
2. Liver function tests:
Including prothrombin
3. Fetal well-being
Monitored at frequent
CTG
US:
Fetal growth
Liquor
volume
time& bile acids: weekly.
intervals.
umbilical artery Doppler blood-flow

61. 4. Delivery
at 37-38 w, or when fetal lung maturity is evident
{decrease perinatal mortality}.
Close monitoring during induction&
risk of fetal distress}
labour {high
The neonate should receive i.m. vit K

62. DRUGS
 TOPICAL EMOLLIENTS like CALAMINE
 VIT K(10 mg ORALLY/DAY)
 ANTI HISTAMINES
 URSO DEOXYCHOLICACID(UDCA)
 RIFAMPICIN

63. HEPATITIS
E
INFECTION

64. HEPATITIS E INFECTION CAN BE
6
4
UNEVENTFUL
• In general, hepatitis E is a self-limiting viral infection
followed by recovery. Prolonged viraemia or faecal
shedding are unusual and chronic infection does not
occur.
• Occasionally, a fulminant form of hepatitis develops, with
overall patient population mortality rates ranging between
0.5% - 4.0%. Fulminant hepatitis occurs more
frequently in pregnancy and regularly induces a
mortality rate of 20% among pregnant women in
the 3rd trimester.

65. Hepatitis E -
Clinical Features
 Incubation period:
 Case-fatality rate:
 Illness severity: increases with age
 Chronic sequelae: none identified
Average 40 days
Range 15-60 days
Overall, 1%-3%
Pregnant women,
15%-25%

66. • Among pregnant women, the case fatality rate is
20%, and this rate increases during the second and
third trimesters. Reported causes of death include
encephalopathy and disseminated intravascular
coagulation. The rate of fulminant hepatic failure in
infected pregnant women is high.
HIGHER FATALITIES IN
PREGNANT WOMEN

67. • Liver transplant recipients may be at a greater risk for
HEV infection, which can lead to chronic hepatitis.
However, if the patient has antibodies against HEV,
the risk of reactivation is extremely low
LIVER TRANSPLANT PATIENTS AT
RISK
HEPATITISCAN GO INTO CHRONIC PHASE IN
IMMUNOSUPPRESSED.

68. THANKS