Pregnant woman with congenital heart disease. A. Pijuan Domènech (U. de C. Congènites de l Adult) ACOR, Hospital Vall d Hebron Barcelona, Spain

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1 Pregnant woman with congenital heart disease A. Pijuan Domènech (U. de C. Congènites de l Adult) ACOR, Hospital Vall d Hebron Barcelona, Spain

2 Pregnant women with congenital heart disease Maternal mortality/10e6 Maternal mortality is increasing Around 70% of pregnancies with cardiac maternal disease Number of admissions in US in ACHD In 10 years twofold increase in pregnancy admissions in CHD patients Roos-Hessenlink et al;heart 2009:95:680-86; Opotowsky et al ;JACC 2009:54:460-7

3 Pregnant women with congenital heart disease Placental developement Low resistance Heart rate Sistolic volume Plasmatic volume IVC COMPRESSION CHAMBER AND AORTA DILATATION THROMBOSIS RISK X 6 DURING PREGNANCY Systemic vascular resistance Cardiac output Volume overload

4 Pregnant women with congenital heart disease pregnancy Arrythmia Heart Failure Mortality Risks for the mother delivery Risks for the fetus and neonate Transmission CHD Preterm SGA Mortality postpartum Long term implications

5 Pregnant women with congenital heart disease Aware risk Not aware High risk pregnancy Aw are risk Not avoid Avoid or delay Low risk pregnancy Not avoid Not aw are Avoid or delay Kovacs et al J Am Coll Cardiol 2008; 52: ACHD Guidelines J Am Coll Cardiol 2008

6 Can we predict the risk in CHD?Maternal risks From prospective studies... Risks for the mother FC>I o cyanosis LVOTO >30 mmhg peak Previous stroke, arrythmia o HF Systemic EF<40% Risks for the fetus From retrospective studies specific por CHD... Severe PR RV dysfunction Mechanical prosthesis SignificantL/RAVregurgitation Cardiac medication Complex CHD Khairy et.al.. Circulation 2006; 113: Drenthenet.al.. EHJ 2010; Siu S. et al. Circulation 2001;31: 104:

7 Risk factors for complications for the fetus Risks for the mother 13% CV complications FC>I o cyanosis LVOTO >30 LVOTO mmhg peak Previous stroke, arrythmia o HF Systemic EF<40% Risks for the fetus 20%Fetal complications Smoking Anticoagulation Twin pregnancy Age<18 or>35y Siu S. et al. Circulation 2001;31: 104:

8 Maternal complications in pregnant women with CHD Cardiac drugs only if necessary Pharmacokinetics Nitrates Hidralazine Furosemide ACEI ARBs Spironolactone 10% CV COMPLICATIONS 4,8% Heart failure 4,5% Arrythmia CV event N patients Cardiac complications Drenthen % Jastrow 317 7,4 Avila % Manso 83 10% Fesslova % Jastrow N V IJC 2010 Ford A, Obset Gynecol 2008;112:828 Rev Esp Cardiol 2008;61:(3) Fesslova V, IJC : Avila Clin Cardiol 2003; 26: Drenthen W.et.al. JACC 2007;49:

9 Obstetric care during pregnancy Risks for the mother Risks for the fetus High risk pregnancy Limited cardiac output IUGR 28 w-monthly SGA Preterm delivery 16% Offspring mortality 4% CBF Atosiban if PRM Atosiban Drenthen W.et.al. JACC 2007;49:

10 Risk of transmission of CHD Recurrence risk Global risk of transmission of 3,7% Especific CHD: AVSD 7-12% Left sided obstruction 5% Tetralogy of Fallot 5% TGA 0.5% NT thickness and normal cariotip 25% CHD Autosomal dominant Marfan syndrome 22q11 Delection Holt-Oram De novo mutations Risk transmission 50% Nucal translucency Fetal echo weeks Burn J Lancet 1998;351: Oyen N Circulation 2009; 120:

11 Gestational care in women with CHD Importance of first visit Number of visits Cardiac assessment arrythmia,sat, medications, Counselling prior to pregnancy Depends on residual lesions and cardiac status Always include 24 weeks Echo: increased velocity and gradients Weight control Identify pregnancy induced hypertension Anestesiology 32-34w Obstetrics ev 3-4 w until weeks de Once w 36-partum Heart Disease and Pregnancy P Steer, MA Gatzoulis,P Baker RCOG Press

12 Pregnant women with congenital heart disease Delivery Careful plan management Cardiology Obstetrics Anaesthesiology Minimize pain and anxiety Epidural Monitoring, fluid balance Left lateral position Spontaneous onset of labour Limit 2ond stage delivery Cardiac C- section: Heart failure Warfarine Aortic patients

13 Postpartum care of women with CHD Uterine contractions Autotransfusion Increase in CVP If high risk situation Cardiac enviroment CCU Balance fluid shift Pulmonary edema Postpartum haemorrhage Thrombosis risk LMH thromboprofilaxis Monitoring ECG,BP,CVP Steer P,Gatzoulis MA Pregnancy And Heart Disease Oxitocine perfusion

14 Long term implications of pregnancy in women with CHD Progression of the disease? Earlier cardiac intervention? Homograft deterioration? Guédès A JACC 2004 Uebing A IJC 2007

15 Pulmonary arterial Hypertension pregnancy Right to left shunt SVR Limited CO Heart failure, sudden death IUGR, prematurity POORLY TOLERATED delivery Cardiac C- section: Heart failure If possible vaginal epidural CVP, arterial line, O2 sat postpartum 80% mortality in Eisenmenger pospartum Thromboembolic events and haemorrage High level surveillance Cardiac enviroment Use of NO/Prostacicline/Sindenafil/Bosentan Bédard E et al ;EHJ 2009:30: Weiss BM et al ;JACC 1998;

16 Pregnant woman with CHD: Aortic patients Marfan syndrome pregnancy Risk of dissection 1% if Aortic root <40 mm DISSECTION type A PREGNANCY Marfan N pacients N=16 Mean Aortic root 4,8+/- 0,8 Cm Betablokers 10% if Aortic root >40 mm Echocardiogram assessment Bicuspid Aortic valve N=4 5,3+/-1,1 Cm BAV and Aortic dilatation No specific series for pregnancy >45 mm high risk?? Native severe Coarctation 100% Hypertension Risk of dissection If acute dissection Cardiac surgery Previous C-section if viability Risk fetal demise RISK OF DISSECTION Aortic coarctation repaired Always prior MRI irrespective of repair Hypertension and Preeclampsia 22% Stented coarctation: no series, 10 descriptions no complication delivery postpartum Cardiac C- section Arterial line if hypertension Anesthetic consultation Risk until 3 months Immer F,Ann Thorac Surg 2003; 76:309 Meijboom,Heart 2005; Vriend EHJ 2005;26: Beauchesne JL et al ;JACC 2001;38.172

17 Fontan patients and cyanotic congenital lesions without PAH Fontan syndrome Cyanotic congenital lesions without PAH SVR Thrombosis risk pregnancy SVR Right to left shunt Limited cardiac output Plasmatic volume Atrial arrythmia/hf Anticoagulation through pregnancy HIGH RISK PREGNANCY SVR Increasing cyanosis Heart failure Careful IUG monitoring LMH 3T Avoid volume loading AND volume depletion Avoid or SVR < 40 pregnancies reported No maternal mortality Spontaneus abortion 50% delivery postpartum Thromoboembolic events/lmh Venous lines with air filters If 02 Sat <85% high risk espontaneus abortion Walker F, Heart 2007 :93; 152 Drenthen, Heart 2006 : Cannobio M, JACC 1996 :28;763

18 Transposition of great arteries Systemic RV TGA and atrial switch pregnancy SVR Plasmatic volume Cardiac output? Congenitally corrected TGA Degree of systolic dysfunction Presence of TR Overall 20% morbidity Low mortality MODERATE RISK PREGNANCY Arrthymia De novo leaks RV dilatation and HF Pregnancy induced HT TGA and arterial switch 20 cases reported 10% complications but In high risk patients Connolly H et.al.. JACC 1999; 33: Cannobio MM et.al.. Am J Cardiol 2006; 98: postpartum Long term implications Drenthen W, EHJ 2005;26: Concerns regarding RV dilatation and deterioration after pregnancy Guedes A et.al.. JACC 2004 ; 44: Tobler D Am J Cardiol 2010;106:

19 Right sided stenotic and regurgitant lesions RVOT Obstruction No high risk No mortality Tetralogy of Fallot No mortality described 10% CV comp: LandRHF/Arrythmia Complications if BPS,reduced RVEF Severe PR SERIES IN TETRALOGY RV dysfunction Pedersen Meijer Greutman Khairy RVH, restrictive physiology? Should propose PVR before pregnancy? Controversy N pregnancies % Severe PR? 20%TAP 28% 40% 100% Cardiac complications 0% 12% 9,6% 27%HF 16%Arr Tricuspid regurgitation No specific series Ebstein anomaly Risk factors and cyanosis Meijer JM. et al Heart 2005;91: Pedersen 2008 Cardiol Young 2009; 18: Greutmann M EHJ 2010 ; 31: Khairy et.al.. Circulation 2006; 113:517-24

20 Pregnant women with congenital heart disease Left to right shunt pregnancy SVR Left to right shunt Plasmatic volume ASD Low risk of complications: 0.8% stroke Not closure if found during pregnancy... Preeclampsia/SGA/Fetal mortality VSD, PDA Low risk of complications Left sided regurgitant lesions LAVV regurgitation LOW RISK 17% worsening LAVV regurgitation 10% Arrythmias Repair performed: pregnancy 1year Yap S-C BJOG 2009:

21 Preconceptional counselling in women with CHD Prior to pregnancy Inform regarding risks for the mother Complications during pregnancy/postpartum Long term prognosis Transmission/Prematurity/SGA Inform risk for the fetus Age,drugs, smoking Minimize risk Ask actively pregnancy/contraception at the cardiac outpatient clinic Surgical approach/ Percutaneous approach/eps Avoid mechanical prosthesis Compare risk with general population/experience preconceptional clinic Explain measures for minimizing the complications: Care at tertiary center: Fetal echo/delivery/pospartum

22 Pregnant women with congenital heart disease THANK YOU!!!