who, what, where management of pregnancy in women post ... may 091… · women post congenital...
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Who, what, where – Management of pregnancy in women post congenital cardiac surgery
- the cardiologist’s view
Dr. Aisling Carroll
Consultant Cardiologist
Adult Congenital Heart Disease
Clinical Lead ACHD & Obstetric Cardiology
Obstetric Anaesthetic Association
Scientific Meeting 2013, Bournemouth
No Disclosures
Overview
• Who?
• What?
• Where?
• How?
Leading Causes of Maternal Death
CMACE report 2011
Congenital Heart Disease and Maternal Death
2000-2002
• 1 Eisenmenger ASD (previously undiagnosed)
• 2 Repaired septal defects with progression of modest PHT
• 1 coarctation (previously undiagnosed)
• 1 Fontan
• 1 Pulmonary stenosis, Noonan
• 3 Valve/other
2006-2008
• 1 Thrombosed aortic valve
• 2 pulmonary hypertension
Scope of the Problem
• Congenital heart disease in 1% live births
• Survival to adulthood over 90%
• ACHD population increase ~1600pa, approx 159,000
• Many will develop ventricular dysfunction evolving into symptomatic heart failure
Verheugt et al, Int J Cardiol 08
Proportion of Patients in Contact with the
Healthcare System
Marelli et al, Circ 09
Median age at loss to follow-up from cardiology care: 15 yrs
Prevention of Maternal Mortality and Morbidity
• Accurate pre-conception counselling
• Many can have a successful pregnancy
• Unacceptable risk in others
• Risk assessment to make an informed decision
• Appropriate contraceptive advice
Approximate mortality risks in pregnancy
• Entirely healthy woman 1 in 20,000
• Population average 1 in 10,000
• Corrected Fallot 1 in 1,000
• Severe aortic stenosis 1 in 100
• Pulmonary hypertension 1 in 3
• Eisenmenger’s complex 1 in 2
Pre-pregnancy Counseling
• Risk assessment
• Cardiac investigations
• Full and frank discussion
• Appropriate contraception
• Medication adjustment
• When to contact Team if pregnant
Risk Assessment
• Define underlying cardiac lesion
• Maternal functional status
Distribution of Peak VO2 in Asymptomatic CHD Pts
– NYHA Class I
Risk Assessment
• Define underlying cardiac lesion
• Maternal functional status
• Need for further palliative or corrective surgery
• Additional associated risk factors
• Issues of maternal health and life expectancy
• Genetics
Predictors of Cardiac Events
1) Prior CHF, stroke or arrhythmia
2) Baseline NYHA class >II or cyanosis
3) Left heart obstruction
MVA <2cm2, AVA <1.5cm2
LVOT gradient >30mmHg by echo
4) ↓ systemic vent function (EF<40%)
Risk index predicts CV event rate
Siu et al: Circulation 2001
Actual vs Predicted Primary Cardiac Event Rates with Varying Numbers of Predictors
Siu, S. C. et al. Circulation 2001;104:515-521
23 yo
• VSD – repaired at 18months
Subsequent TR
Normal ventricular function
• Asymptomatic
AF in past – none for 2 years
• Considering pregnancy
What is the primary CV concern during pregnancy?
• 1) Heart failure
• 2) Arrhythmia
• 3) Paradoxical embolism
Predicted CV event rate 20%
due to arrhythmia history
What cardiac conditions are low risk?
Lesion Specific Maternal Risk
Mortality < 2%
Unoperated small septal
defects/PDA
Mild valvar stenosis
Most repaired septal
defects
Good coarctation repair
Most regurgitant lesions
Repaired Fallot
Mortality 2-10%
Expert advice & Mgt
Mechanical valve
Systemic R ventricle
Cyanotic, no PHT
Fontan circulation
Mortality 10-50%
Expert advice &
Mgt
Pulmonary
hypertension
↓ Ventricular
function
Aortic aneurysm
Severe L-sided
obstruction
Low risk Significant risk Contraindicated
24 yo PE teacher
Bicuspid Aortic Valve with Stenosis
Careful monitoring throughout pregnancy
C section in view of twins
Twins!
Marfan Syndrome
• Main maternal risk is Type A aortic dissection – 1%
• High risk:
• poor family history
• cardiac involvement
• aortic root > 4cm or rapidly expanding
• Appropriate counselling
• B blockers and elective section Elkayam et al. Ann Intern Med 1995;123:117-
22
Marfan SyndromeContraindications to pregnancy
Aorta >40mm
Decreased ventricular fn, EF<40%Class III-IV
Aortic Coarctation
MRA/CTA
• Delineate location, extent,
and severity of coarct
• Collaterals – flow direction
• Associated lesions
• Postop eval – aneurysm,
dissection, recoarctation
Compliments echo
Coarctation in Pregnancy
•50 pts had118 pregnancies resulting in 106 births
•11 miscarriages (9%), 4 premature (3%), 1 neonatal death
• CHD in 4 offspring
• CV complications infrequent
1 Turner pt dissection at 36 wk, 15 pt (30%) HTN pregnancy
• HTN related to coarctation
•11 (73%) had significant coarctation/recoarctation
Beauchesne, J Am Coll Cardiol 2001
Tetralogy of Fallot
• If radical repair……well tolerated
• If ventricular function good and no significant RVOTO
• Significant PR – may need bed rest and diuretics
33 yo, second pregnancy
Ebstein Anomaly
Free Tricuspid Regurgitation
Pregnancy in Ebstein
• 44 women - 111 pregnancies
• fetal loss (20%)
• premature birth (28%)
• LBW infants in cyanotic
• 6% CHD in offspring
• Pregnancy well tolerated
Connolly: JACC 1994
Where? How?
• Multidisciplinary involvement
• Regular review, low dose diuretic only
• Cardiac theatres
• Cardiac AND obstetric anaestheticteams
• Cardiac ITU for recovery
How?
“Over half of all maternal deaths could be eliminated by changes in patient,
provider and system factors”
Management Plan
Multidisciplinary Team
19 yo, 20/40, attends for fetal scan
Hypertrophic Cardiomyopathy
Hypertrophic Cardiomyopathy
• Most women tolerate pregnancy well
• Symptoms deteriorated < 10%
• Mortality increased but confined to patients at high risk (massive LVH, severe restrictive physiology)
• Risk of sudden death not increased by the pregnancy
• Careful fluid management in labour
Autore et al,JACC 2002 40:10:1864-69
Where?Congenital heart disease
Who?
Risk?
How?
What
conditions?
Outcomes?
Fontan Operation
Univentricular Heart
Double Inlet Left Ventricle
Tricupid Atresia
Other CHD with RV Hypo/Dysplasia
Concept:
Diverting all the blood from IVC, SVC, RA
into the Pul Artery bypassing the RV
Carroll A, Thorne S 2006
• Maternal risk:• If class I-II and good ventricular function not
excessively high
• Maintain filling pressures, avoid dehydration and vasodilation
• Fetal risk:• High risk fetal loss(30%)
• WarfarinLMWH
• Antiarrhythmics (arrhythmia risk vsteratogenicity)
Carroll et al, Medicine 06
Mustard/Senning – Systemic RV
Pulmonary venous baffle Systemic venous baffles
Congenitally Corrected Transposition of the Great Arteries
• Right ventricle supports systemic circulation
• Pregnancy well tolerated
• RV function may deteriorate
• Systemic AV valve regurgitation
• Complete heart block
Carroll et al, Medicine 06
Mechanical Valves
• Durable; need anticoagulation
• Fetal risk
• Warfarin
• Heparin
• Maternal Risk
• Valve thrombosis
• Maternal complications
Choice of Anticoagulation
• HEPARIN
• Safe for baby
• WARFARIN
• Safe for mother
Anticoagulation
• 1st trimester- controversial
• 2nd trimester- warfarin as per INR
• 3rd trimester-continue warfarin until 36/40
• Admit for heparin, plan delivery, stop peripartum and start 4-6 hours post delivery ( if no bleeding)
• C-section if labour while on warfarin (ICH)
Lesion Specific Maternal Risk
Mortality < 2%
Unoperated small septal
defects/PDA
Mild valvar stenosis
Most repaired septal
defects
Good coarctation repair
Most regurgitant lesions
Repaired Fallot
Mortality 2-10%
Expert advice & Mgt
Mechanical valve
Systemic R ventricle
Cyanotic, no PHT
Fontan circulation
Mortality 10-50%
Expert advice &
Mgt
Pulmonary
hypertension
↓ Ventricular
function
Aortic aneurysm
Severe L-sided
obstruction
Low risk Significant risk Contraindicated
Summary
• Who? Increasing population, all patients
should receive multi-disciplinary
counselling
• What? High or low risk, individualise
• Where? Estimate of maternal and foetal
risk
• How? Multidisciplinary approach - VITAL
Thank
you!
July 4th
November 7th
Next ACHD Study Days in Southampton