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