Rheumatic Heart Disease in Pregnancy PMMRC June 2015 Dr Claire McLintock Faith Mahony

Aim Improve the safety and quality of maternity care in Australia and New Zealand Background Uncommon conditions are difficult to study little research available Clinical practice not based on robust evidence base

AMOSS Conditions Completed Ongoing Peripartum hysterectomy Eclampsia Antenatal pulmonary embolism Placenta accreta/increta/percreta Gestational breast cancer Rheumatic heart disease Ongoing Blood transfusion >5 units in <4 h Amniotic fluid embolism

Rheumatic Fever Let’s concentrate on the who -the majority of patients with MS have developed it as a result of RF - an autoimmune disease caused by antibodies to an untreated streptococcal sore throat. I

Rheumatic Fever Incidence Aboriginal & Torres Strait Islanders 150-320 375 65 Pacific Island 96 Maori 65 per 100 000 population aged 10-15y European <1

North-South Gradient 2006-2010 RF admissions age 0-24 y (per 100 000) 0-1 2-5 6-15 16-25 >25 New Zealand Child and Youth Epidemiology Service www.otago.ac.nz/nzcyes

RF Admissions by Age NZ 2006-2010 NZCYES 2012 www.otago.ac.nz/nzcyes

RF Admissions by Ethnicity 0-24 Year Olds, NZ 2006-2010 NZCYES 2012 www.otago.ac.nz/nzcyes

Duration of Secondary Prophylaxis Benzyl penicillin im injection every 28 days RHD category Recommendation None or Mild Minimum 10 yrs Mod Until age 30 yrs then reassess Severe Until age 40 yrs (reassess age 30 yrs)

heart rate cardiac output plasma volume In pregnancy there are increases in plasma volume, heart rate and cardiac output. The increase in HR means that there is less time for diastolic filling of the ventricle, which in women with mitral stenosis prevents an increase in cardiac output and with the increase in plasma volume combine to increase the gradient across the valve, cause more pulmonary hypertension and thereby worsening of clinical symptoms. Women who were previously asymptomatic can develop symptoms and this seems to occur at the time of the second increase in heart rate around week 20-24. The inability to increase cardiac output with MS leads to symptoms of fatigue, shortness of breath, orthopnoea and PND.

Research questions Which women are affected? How severe is the RHD? previous RHD surgery, valvotomy What are the outcomes for mother & baby? Cardiac decompensation Intensification of therapy Surgical intervention Adverse infant outcomes

Data Collection Prospective identification of pregnant women with RHD Oct 1st 2012 -Dec 31st 2014 Sources PMMRC Local Coordinators Cardiologists, MFM, Midwives, Obstetric Specialists DHB decision Data collection and data entry NZ RHD coordinator Demographics Cardiac status Medical complication Pregnancy outcomes

Recruitment Pregnancies N=186* Fulfill AMOSS criteria n=146 History RF only or trivial RHD n=34 No echo results n=5 Lost to follow-up n=1 *14 women 2 pregnancies, 2 women 3 pregnancies, 1 twin pregnancy

63% of cases in Auckland Region Location Oct 12- Dec 14 Northland 5% Waitemata 4% Auckland 27% Manukau 32% Tauranga 1% Lakes 3% Waikato 7% Gisborne 5% New Plymouth 1% Hawkes Bay 5% Wanganui 1% Hutt 1% Wellington 5% 63% of cases in Auckland Region Christchurch 3% 69% Upper North Island

Ethnicity SE Asian 3 NZ European 4

Age Range 15-43 y Median 27 y

Age of last episode RF (or 1st episode) 43% initial RF or recurrence ≥18 years

Severity of RHD Valve Lesion

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Maternal Outcome Maternal outcome N (%) Maternal death Surgical intervention in pregnancy 6 (3.4%) Initiation of cardiac medications in pregnancy 36 (25%) 1st diagnosis of RHD during index pregnancy or postpartum* 16 (11%) antenatal, n=12 postpartum, n=4 *cardiac decompensation postpartum in 4 women with previously unrecognised RHD

Outcomes in Pregnancy in Women with No Previous Cardiac Surgery RHD disease severity Number of Pregnancies Clinical decompensation in pregnancy or postpartum (n, %) Description of RHD valve lesion in women with decompensation Mild 43 Moderate 41 6 (14.6%) Moderate AR x2 Moderate MR Moderate MS+AR Moderate AR+MR Moderate MS+MR Severe 38 21 (55.3%) Severe MR (n=13) Severe AR (n=1) Severe MS (n=6) Severe MS+MR (n=1) AR – aortic regurgitation MR – mitral regurgitation MS – mitral stenosis All 7 women with severe MS decompensated. 5 required surgery during pregnancy or postpartum

Outcomes in women with severe RHD Valve lesion Pregnancies (N) Complications N, % Description Severe MR 26 13 (50%) Diuretics, b-blockers Severe MS - isolated 6 6 (100%) Balloon mitral valvotomy (n=3) Valve replacement surgery (n=3) Severe MS + severe MR 1 1 (100%) Severe AR + trivial or mild MR 3 Severe AR + moderate MR AR – aortic regurgitation MR – mitral regurgitation MS – mitral stenosis

Outcomes in Pregnancy in Women with Previous Cardiac Surgery 24 women previous cardiac surgery RHD status at start of index pregnancy Mild (n=15) cardiac meds in pregnancy (n=2, 13.3%) Moderate (n=6) cardiac meds in pregnancy (n=3, 50%) Severe (n=3) cardiac meds in pregnancy (n=1, 33%)

Infant Outcomes Outcome N (%) Mode of birth Miscarriage or termination 14 (9.6) Stillbirth 2 (1.5) Preterm birth 15 (11.4) Birthweight (mean, range) 3295 g (410-5300) Mode of birth Spontaneous vaginal birth, unassisted 88 (60.3) Assisted birth (forceps, ventouse) 12 (9.1) Induction of labour 42 (32.8) Caesarean section 31 (23.5)

Risk stratification and optimal care for women with RHD in pregnancy Education about prevalence of RHD in NZ Improve health literacy to reduce incidence RF