Pregnancy Management Guidelines in Women with Cardiac Diseases

1. Pregnancy Management Guidelines in Women with Cardiac Diseases BY Jameel Alata , MD Consultant pediatric cardiologist, KAAUH / KFSH&RC Jeddah, KSA. 32 ESC annual meeting in association with PACHDA Cairo, 22-25 FEB 2005

2. Introduction; • Pregnancy in most women with heart disease has a favourable maternal and fetal outcome. • With the exception of patients withEisenmenger syndrome, pulmonary vascular obstructive disease,and Marfan syndrome with aortopathy, maternal death during pregnancyin women with heart disease is rare. • However, pregnant womenwith heart disease do remain at risk for other complications includingheart failure, arrhythmia, and stroke.

3. Introduction; • Women with congenital heartdisease now comprise the majority of pregnant women with heartdisease seen at referral centres. • The next largest group includeswomen with rheumatic heart disease.

4. Introduction; • Approximately 10% of all maternal deaths in the United Statescan be attributed to cardiac disease. • In one study of 1,000pregnant women who had various types of cardiac disease andwere followed by the same health-care team over a 10-year period,more than 75% of the women had no complications during pregnancy. • The remaining 25%, the following complications wereseen most often:

5. Introduction; • Congestive heart failure, including pulmonary edema (12.3%) • Cardiac arrhythmias (6%) • Thromboembolism (1.9%) • Angina (1.4%) • Hypoxemia (0.7%) • Infective endocarditis(0.5%) • The overall maternal mortality rate in this group was 2.7%,and the stillbirth and spontaneous abortion rate was 7.7%.

6. Introduction; • Cardiac disease covers a wide range of conditions, includingcongenital heart disease, acquired disease such as rheumaticvalvular disease, and coronary disease. • It is estimated that1% to 3% of women either have cardiac disease entering pregnancyor are diagnosed with cardiac disease while they are pregnant. • The frequency of specific types depends on the patient populationand local conditions.

7. Introduction; • Advances in the diagnosis and treatmentof congenital heart disease have increased the survival rateof children affected with these disorders. • Pregnant women who have congenital heartdisease represent the largest number of patients seen at somereferral centers, comprising as many as 70% to 80% of all thecardiac patients. • It is estimated that 1in 10,000 pregnancies is associated with coronary heart disease,notably myocardial infarction.

8. Cardiovascular Physiology of Pregnancy; • Normal pregnancy is associated with of 30 to 50percent in blood volume and a corresponding increase in cardiacoutput. • These increases begin during the first trimester; thelevels peak by 20 to 24 weeks of pregnancy and then are eithersustained until term or decrease. • The heart rateincreases by 10 to 20 beats per minute, the stroke volume increases,and there is a substantial reduction in systemic vascular resistance,with decreases in blood pressure.

9. Cardiovascular Physiology of Pregnancy; • During labor, cardiac outputincreases; the blood pressure increases with uterine contractions. • Immediately after delivery, the cardiac filling pressure mayincrease dramatically due to the decompression of the vena cavaand the return of uterine blood into the systemic circulation. • The cardiovascular adaptations associated with pregnancy regressby approximately six weeks after delivery.

10. Cardiovascular Physiology of Pregnancy; • Functional Murmurs develop in nearly all women during pregnancy. • Echocardiography is warranted when diastolic murmurs, continuousmurmurs, or loud systolic murmurs (louder than grade 2 on the6-point scale) are detected or when murmurs are associated withsymptoms or an abnormal electrocardiogram

11. Cardiovascular Physiology of Pregnancy; • In normal pregnantwomen, serial echocardiography usually demonstrates minor increasesin the left and right ventricular diastolic dimensions, whichremain within the normal range. • Slight decrease in theleft ventricular end-systolic dimension and a minimal increasein the size of the left atrium is also noted. • The state of increased volumealso results in increased transvalvular flow velocities. • Minordegrees of atrioventricular valve regurgitation are normal.

12. Cardiovascular Physiology of Pregnancy; • During labour and delivery, pain and uterinecontractions result in additional increases in cardiac output ( 15% with each contraction )and blood pressure. • Immediately following delivery, relief ofcaval compression and autotransfusion from the emptied and contracteduterus produce a further increase in cardiac output ( upto 45 % ).

13. Other physiologic changes; • Hypercoaguble state. • Hypoalbumineamia. • Insulin resistant state. • Increased red c.ell mass • Increased ESR. • Increased renal blood flow ( 30 % ). • Increased hepatic clearance of medications.

14. Outcome of pregnancy with CHD

15. Outcome ; LT to RT shunts • In the absence of pulmonary hypertension, pregnancy, labour and delivery are well tolerated 2nd to attenuation of volume overload by peripheral vasodilation. • However arrhythmias, ventricular dysfunction, and progression of pulmonary hypertension may occur, especially when the shunt is large or when there is pre-existing elevation of pulmonary artery pressure. • In ASDs, paradoxical embolisation may be encountered if systemic vasodilatation and/or elevation of pulmonary resistance promote transient right to left shunting.

16. Outcome ;AS, COA & LVOTO • The absence of symptoms antepartum is not sufficient assurance that pregnancy will be well tolerated. • Pregnant women with severe aortic stenosis have limited ability to augment cardiac output, elevation of left ventricular systolic and filling pressures& non-compliant, hypertrophied ventricle is sensitive to falls in preload leads to: CHF , HYPOTENSION & or ISCHEMIA

17. Outcome ;AS, COA & LVOTO • In a compilation of many earlier small retrospective series, 65 patients were followed through 106 pregnancies with a maternal mortality of 11% and a perinatal mortality of 4%. • In 25 pregnancies managed recently, there was no maternal mortality but deterioration of maternal functional status occurred in 5 (20%). • In the absence of prosthetic dysfunction or residual aortic stenosis, patients with bioprosthetic aortic valves usually tolerate pregnancy well.

18. Outcome ;AS, COA & LVOTO • Ross procedure reported favourable maternal and fetal outcomes except in one woman who developed postpartum left ventricular dysfunction. • Pregnancy in a woman with a mechanical valve prosthesis carries increased risk of valve thrombosis as a result of the hypercoagulable state. • The magnitude of this increased risk (3-14%) is greater if subcutaneous unfractionated heparin rather than warfarin is used as the anticoagulant agent.

19. Outcome ; COA • Maternal mortality with uncorrected coarctation has been reported as 3% in an early series. • Aortopathy, or longstanding hypertension; aortic rupture accounted for eight of the 14 reported deaths and occurred in the third trimester as well as in the postpartum period.. • More recently, a preliminary report described encouraging maternal and fetal outcome in 87 pregnancies, with no maternal deaths and one early neonatal death.

20. Outcome ; COA • The management of hypertension in uncorrected coarctation is particularly problematic in pregnancy because satisfactory control of upper body hypertension may lead to excessive hypotension below the coarctation site, compromising the fetus. • Intrauterine growth restriction and premature labour and delivery are more common. Following coarctation repair, the risk of dissection and rupture is likely reduced but not eliminated.

21. Outcome ; Pulmonary stenosis • Mild pulmonic stenosis, or pulmonic stenosis that has been alleviated by valvuloplasty or surgery, is well tolerated during pregnancy and fetal outcome is favourable. • Severe pulmonic stenosis may be asymptomatic , but may precipitate right heart failure or atrial arrhythmias; such condition should be considered for correction before pregnancy. • Even during pregnancy, balloon valvuloplasty may be feasible .

22. Outcome; Cyanotic CHD • Uncorrected or palliated pregnant patients with cyanotic congenital heart disease such as tetralogy of Fallot, single ventricle,etc, the usual pregnancy associated fall in systemic vascularresistance and rise in cardiac output exacerbate right to leftshunting leading to increased maternal hypoxaemia and cyanosis. • Outcomes of 96 pregnancies in 44women with a variety of cyanotic congenital heart defects reporteda high rate of maternal cardiac events (32%, including one death).

23. Outcome; Cyanotic CHD • Prematurity (37%), and a low live birth rate (43%). • The lowestlive birth rate (12%) was observed in those mothers with an arterialoxygen saturation of  85%. • Pregnancy risk is low in women who havehad successful correction of tetralogy without residuals.

24. Outcome; Cyanotic CHD • For Atrial repair (Mustard orSenning procedure) 43 pregnancies in 31 women described in recent reports,showed one late maternal death. • There was a 14% incidenceof maternal heart failure, arrhythmias, or cardiac deterioration. • Few recipients of the current repair of choice for complete transposition thearterial switch procedure have yet reached reproductiveage.

25. Outcome; Cyanotic CHD • The Fontan operation eliminates cyanosis and volume overload . • A recent review of 33 pregnanciesin 21 women showed 15 (45%) term pregnancies with no maternal mortality althoughtwo women had cardiac complications . • The incidence of firsttrimester miscarriage was high (39%). • Since the 10 year survivalrate following the Fontan operation is only 60-80%, it is important to discuss prognosis during preconceptioncounselling.

26. Outcome; Marfan • Medial aortopathy resulting in dilatation, dissection,and valvar regurgitation risks are increased in pregnancy becauseof haemodynamic stress and perhaps hormonal effects. • Recently 45 pregnanciesin 21 patients reported no increase in obstetrical complicationsor significant change in aortic root size in the patients withnormal aortic roots.

27. Outcome; Marfan • The eight patients with adilated aortic root (> 40 mm) or prior aortic root surgery, threeof their nine pregnancies were complicated by either aortic dissection(two) or rapid aortic dilatation (one). • In contrast, women with little cardiovascular involvementand with normal aortic root diameter may tolerate pregnancy well. • Serial echocardiography should beused to identify progressive aortic root dilatation and prophylactic B-blockers should beadministered.

28. Outcome; L-TGA • Potential problems in pregnancy include dysfunction ofthe systemic Right ventricle and/or increased Systemic AtrioVentricularvalve regurgitation with heart failure, Atrial arrhythmias, andAV- block. • 41 patients,there were 105 pregnancies with 73% live births and no maternalmortality, although seven patients developed either heart failure,endocarditis, stroke, or myocardial infarction.

29. Outcome; Eisenmenger • A recent review of outcome of 125 pregnancies in patients with Eisenmenger syndrome, primary pulmonary hypertension, and secondarypulmonary hypertension showed; maternal mortality of 36%, 30%, and 56%,respectively. • The overall neonatal mortality was 13% • The preponderance of complicationsoccurs at term and during the first postpartum week.

30. Outcome; Eisenmeger • Preconceptioncounselling should stress the extreme pregnancy associated risks. • Termination of pregnancy should always be offered to such patients,as should sterilisation. • The vasodilation associated with pregnancywill increase the degree of right to left shunting in patientswith Eisenmenger syndrome, resulting in worsening of maternalcyanosis with poor fetal outcome

31. Outcome; RHC Disease • Mitral stenosis is the most common rheumatic valvar lesion encountered during pregnancy. • Patients withmild to moderate mitral stenosis, who are asymptomatic beforepregnancy, may develop atrial fibrillation and heart failure duringthe ante- and peripartum periods.

32. Outcome; RHC Disease • Earlier studies showed thatmortality rate increased with worsening antenatal maternal functionalclass. • A more recent study found no mortality but describedsubstantial morbidity from heart failure and arrhythmia.

33. Outcome; RHC Disease • Pregnant women whose dominant lesion is rheumatic aortic stenosis have a similar outcome to those with congenital aortic stenosis. • Severe aortic or mitral regurgitation is generally well toleratedduring pregnancy although deterioration in maternal functionalclass has beenobserved.

34. Outcome; Peripartum Cardiomyopathy • Unexplained left ventricularsystolic dysfunction, confirmed echocardiographically, presentingduring the last antepartum month or in the first five postpartummonths. • The relapserate during subsequent pregnancies is substantial in women withevidence of persisting cardiac enlargement or left ventriculardysfunction. • It remains unclear whether pregnancy is safe in thosewith recovery of systolic function.

35. Management • Risk stratification. • Counseling. • Antepartum management. • Multidisiplenary, high risk units. • Labour and delivery.

36. Risk stratification • The data requiredfor risk stratification can be acquired readily from a thoroughcardiovascular history and examination, 12 lead ECG, and transthoracicechocardiogram. • In patients with cyanosis, arterial oxygen saturationshould be assessed by percutaneous oximetry.

37. Risk stratification • Low risk • 1- Small left to right shunts.  • 2- Repaired lesions without residual cardiac dysfunction.  • 3- Isolated mitral valve prolapse without significant regurgitation.   • 4-Bicuspid aortic valve without stenosis.  • 5-Mild to moderate pulmonic stenosis. • 6-  Valvar regurgitation with normal ventricular systolic function.

38. Risk stratification • Intermediate risk • 1- Unrepaired or palliated cyanotic congenital heart disease  • 2- Large left to right shunt  • 3-Uncorrected coarctation of the aorta  • 4- Mitral or aortic stenosis  • 5- Mechanical prosthetic valves  • 6- Severe pulmonic stenosis • 7-  Moderate to severe systemic ventricular dysfunction  • 8- History of peripartum cardiomyopathy with no residual ventricular dysfunction

39. Risk stratification • High risk • 1- New York Heart Association (NYHA) class III or IV symptoms  • 2-Severe pulmonary hypertension  • 3- Marfan syndrome with aortic root or major valvar involvement  • 4-Severe aortic stenosis  • 5- History of peripartum cardiomyopathy with residual ventricular dysfunction

40. Counselling • In counselling, thefollowing six areas should be considered: • The underlying cardiaclesion, • Maternal functional status, • The possibility of furtherpalliative or corrective surgery, • Additional associated risk factors, • Maternal life expectancy , • Ability to care for a child, • The risk of congenital heart disease inoffspring.

41. Antepartum Management • Issues are: • Congestive heart failure, • Arrhythmias, • Thrombosis, • Emboli, and • Adverse effects ofAnticoagulants.

42. CHF • Activity limitation is helpful and in severely affected women with NYHA class IIIor IV symptoms, hospital admission by mid second trimester maybe advisable. • Pregnancy induced hypertension, hyperthyroidism,infection, and anaemia should be identified early and treatedvigorously.

43. CHF • For patients with important mitral stenosis, the useof  blockers or digoxin for control of heart rate should be considered. • Also offer empiric treatment with  blockers to patients withcoarctation and to Marfanpatients.

44. Arrhythmias • Arrhythmias in the form of premature atrial or ventricular beats are common in normal pregnancy. • Sustained tachyarrhythmias such as atrialflutter or atrial fibrillation should be treated promptly. • Electrical cardioversion is safe in pregnancy. • Digoxin and  blockers are antiarrhythmicdrugs of choice in view of their known safety profiles. Quinidine,adenosine, sotalol, and lidocaine are also "safe“. ( avoid teratogens and Amiodarone )

45. Anticoagulation • For pregnant women with mechnical valves mainly. • Warfarin more effective than Heparine , but embryopathic. • Should be stopped at least 2 wks before labour to avoid fetal brain bleeding.

46. Labour & delivery • Vaginal delivery is recommended with very few exceptions. • The only cardiac indications for caesarean section are aortic dissection,Marfan syndrome with dilated aortic root, and failure to switchfrom warfarin to heparin at least two weeks before labour. • Preterminduction is rarely indicated, but once fetal lung maturity isassured a planned induction and delivery in high risk situationswill ensure availability of appropriate staff and equipment.

47. Labour & Delivery • Invasive haemodynamic monitoringduring labour and delivery,is commonly utilised (intra-arterialmonitoring)with or without (concurrent pulmonary artery catheterisation). • Heparin anticoagulation is discontinued at least 12 hours before induction, or reversed with protamine if spontaneous labourdevelops, and can usually be resumed 6-12 hourspostpartum.

48. Labour & delivery • SBE prophylaxis: • Centreswith extensive experience in caring for pregnant women with heartdisease utilise endocarditis prophylaxis routinely, as an uncomplicateddelivery cannot always beanticipated. • Not AHA recommended if no infection site.

49. Labour & delivery • Epidural anaesthesia with adequate volume preloading is the technique of choice.( but can increase CHF & pulm oedema ) • Epidural fentanyl is particularly advantageousin cyanotic patients with shunt lesions as it does not lower peripheralvascular resistance.

50. Labour & delivery • Labour is conducted in the left lateral decubitus position. • Instrumentation to shorten 2nd stage is indicated. • Patients at intermediateor high risk may require monitoring for a minimum of 72 hourspostpartum. • Patients with Eisenmenger syndrome require longerclose postpartum observation, since mortality risk persists forup to sevendays.