PREGNANCY IN PATIENTS WITH A SYSTEMIC RV

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1 PREGNANCY IN PATIENTS WITH A SYSTEMIC RV Carole A. Warnes MD, FRCP Professor of Medicine, Mayo Clinic No disclosures 2013 MFMER slide-1

2 PREGNANCY : SYSTEMIC RV D - transposition after atrial switch procedure (Mustard / Senning) L- transposition (congenitally corrected TGA) Different hemodynamic situations Respond to stress differently 2013 MFMER slide-2

3 THE SYSTEMIC RV Contraction pattern of systemic RV Normal LV Systemic RV In contrast to LV, ventricular torsion virtually absent Systemic RV has predominant circumferential > longitudinal free wall shortening (like LV & opposite from normal RV ) Implies incipient myocardial dysfunction? Pettersen E, JACC MFMER slide-3

4 Stroke volume index (ml/m 2 ) Dobutamine Stress : CCTGA vs. Atrial Switch Rest Stress 60 stress rest rest stress * cctga + Atrial switch Fratz S: Circ, MFMER slide-4

5 DOBUTAMINE STRESS Atrial switch pts cannot those with CCTGA stroke volume vs. Atrial baffle constrains ventricular filling during stress : flow dominantly in early diastole baffles limit atrial contribution. Abnormal stroke volume responses during exercise & dobutamine stress not caused by contractile response but disruption of normal atrial function MFMER slide-5

6 d - TRANSPOSITION 2013 MFMER slide-6

7 d - TRANSPOSITION Mustard & Senning operations RV LV RV LV RV is the systemic ventricle How do they tolerate a pregnancy? Does pregnancy cause decline in hemodynamics? 2013 MFMER slide-7

8 PREGNANCY & d- TGA after atrial repair 68 pt, 139 pregnancies (103 completed) Mother Arrhythmias 22% Heart failure 15% NYHA Class 35% Persistent NHYA 8% Baby Fetal / neonatal mortality 10% Mean gest age 36 wk Prem delivery 35% SGA 25% Drenthen W, Eur Ht J, 05 Canobbio MN, AJC MFMER slide-8

9 So does pregnancy itself cause a deterioration in hemodynamics? 2013 MFMER slide-9

10 PATIENT JR Pre-pregnancy 32 wk pregnancy 2013 MFMER slide-10

11 PATIENT JR Before pregnancy 32 wk pregnancy 2013 MFMER slide-11

12 PATIENT JR Before pregnancy 32 wk pregnancy 2013 MFMER slide-12

13 SYSTEMIC RIGHT VENTRICLE Annular diln, altered ventricular geometry affects A-V valve coaptation 16 women Mustard op, 28 pgy. NYHA I = 21, NYHA II = 7 Mean F - Up 24 mos 6 women had FC during 7 pgy 2 needed CHF Rx during / soon after pgy 2 did not return to pre-pregnancy class Guedes A, JACC 2004

14 SYSTEMIC RIGHT VENTRICLE Mustard op : 16 women, 28 pregnancies RV dilatation (n=18) absent = 4 mild / mod = 12 severe = 2 Progressed in 5 (31%) : no recovery RV systolic dysfn (n=21) absent = 16 mild / mod = 4 severe = 1 Progressed in 4 (25%) : no recovery in 3 Guedes A, JACC 2004

15 SYSTEMIC RIGHT VENTRICLE Mustard op : 16 women, 28 pregnancies Tricuspid regurg ( n=20 ) absent = 8 mild = 9 moderate = 3 Progressed in 8 (50%) : no recovery in 3 Pre-pregnancy counseling should include possibility that pregnancy might cause decline in hemodynamics & impact long- term outcome Guedes A, JACC 2004

16 PREGNANCY : d-tga after atrial switch 60 pregnancies 34 women All FC I and II before 1 st pregnancy 44 successful 5 serious cardiac complications Acute ht failure 8 wk postpartum FC in 7 pregnancies (21% pt) No recovery in 5 systolic Ht failure fn. & cardiac in 4 arrest No recovery in 3 Cardiac arrest resuscitation SVT : Ht failure FC 18 mo later Premature birth 25% FC 9 mo later 11 miscarriages 5 abortions Outcomes unpredictable Life-threatening problems Occur even if FC I Trigas V, Circ J MFMER slide-16

17 d-transposition : atrial switch 10 women, 21 pregnancies : 14 live births 10 women serial echos dp/dt in 89% pregnancies : ( av = 1168 pre vs. 859 during ) RV fn postpartum BUT baseline function not regained Metz TD, Am J Obstet Gyn MFMER slide-17

18 d-transposition : atrial switch 10 women serial echos : degrees of TR Metz TD, Am J Obstet Gyn MFMER slide-18

19 SYSTEMIC RIGHT VENTRICLE : Atrial switch Does pregnancy impact long-term outcome? Pregnancy (n=19), 43 preg Post-op F - U 35.5 yr No pregnancy (n=15) Post-op F - U 33 yr Maternal comp within 12 mos Arrhythmias = 2 Heart failure = 3 Maternal death (SCD) = 2 1 at 27 wk 1 at 6 mo post partum Zentner D, Heart Lung & Circ MFMER slide-19

20 SYSTEMIC RIGHT VENTRICLE : Atrial switch Long-term outcomes Pregnancy (n=19), 43 preg Post-op F - U 35.5 yr Ht failure admission = 2 Ht failure Rx = 13 (68%) Ex duration (min) = 9 Pre-ex RVEF (n=9) = 46 Post-ex RVEF = 48 Deaths = 2 Zentner D, Heart Lung & Circ MFMER slide-20

21 SYSTEMIC RIGHT VENTRICLE : Atrial switch Long-term outcomes Pregnancy (n=19), 43 preg Post-op F - U 35.5 yr Ht failure admission = 2 Ht failure Rx = 13 (68%) No pregnancy (n=15) Post-op F - U 33 yr 0 3 (20%) (p < 0.01) Ex duration (min) = (p=ns) Pre-ex RVEF (n=9) = 46 Post-ex RVEF = 48 (n= 8) 51 (p=ns) Conclusion Apart Suggests from acute greater risks incidence during 56 gestation (p=ns) of there might be a permanent deterioration in women post in - RV pregnancy function Deaths clinical = 2 events (heart failure 1 (pneumonia) & SCD) Zentner D, Heart Lung & Circ MFMER slide-21

22 CONGENITALLY CORRECTED TGA RV is systemic ventricle Survival to adulthood Systemic AV valve regurg common Systemic ventricular failure NOT inevitable 2013 MFMER slide-22

23 PREGNANCY & CC TGA 22 women, 60 pregnancies 60 pregnancies (1 twin) 50 live births (83%) 11 spont unsuccessful 8 in 4 women no surgery 2 in 2 women with surgery Only 44 vaginal 1 CHF late pgy (EF 45%) 6 C section Systemic (88%) A-V valve (12%) regurg Valve replaced post partum Connolly H, JACC MFMER slide-23

24 PREGNANCY : SYSTEMIC RV Conclusions Rate of miscarriage, premature delivery & SGA offspring are higher in d-tga Maternal risks : arrhythmia, heart failure in d- TGA risks higher in those on cardiac meds pre-pregnancy but unpredictable Pregnancy outcomes appear better in those with CC - TGA if selected carefully 2013 MFMER slide-24

25 RECOMMENDATIONS : Systemic RV Pre- conceptual counselling should include possibility that pregnancy might cause a decline in hemodynamic status & impact long- term outcome Physicians should be alerted to the possibility of deterioration peri- and postpartum & should follow patients closely MFMER slide-25

Pregnancy outcomes in women after an arterial switch operation for transposition of the great arteries

Pregnancy outcomes in women after an arterial switch operation for transposition of the great arteries Department of Obstetrics and Gynecology 1) Department of Pediatrics 2) National Cerebral and Cardiovascular