2. Pregnancy in women with heart disease fetus point Dr. M.Moshfeghi OBS&GYN fellowship of perinatology Shariati.Hospital,TUMS

3. Improved techniques of surgical repair have resulted in women with congenital heart disease surviving to bear children

4. ■ Preconceptional Counseling Women with severe heart disease will benefit from counseling before undertaking pregnancy,

5. significant worsening of NYHA class in 4.4 percent of pregnancies in which the baseline class was I or II.

6. The risk to the woman should be spelled out as clearly as possible.

7. A woman may require a prosthetic valve in the future. should be advised to have her family before valve replacement with its associated anticoagulant risk

8. One of the most important questions is whether the mother’s heart disease is hereditary, ?

9. Congenital Heart Disease in Offspring inherited as polygenic Environmental factors living at higher altitudes (> 3600 meters) and was presumably due to lower oxygen concentrations

10. Recurrence in the fetus IS 2.7percent – with concordance (recurrence of the same parental or sibling defect) in approximately one-third

11. The risk of recurrence was greater if the mother rather than the father had congenital heart disease (5.7 versus 2.2 percent)

12. cardiomyopathies, especially hypertrophic inherited in a mendelian manner. FAMILIAL dilated cardiomyopathy has also been described. Approximately 20% of idiopathic cardiomyopathy is inherited. strong familial tendency in, such as PDA and atrial septal defect (ASD)

13. the risks to her fetus of drugs, other therapies, and diagnostic tests ? Echocardiography poses no threat to the fetus, but radiation incurred with radionuclide angiography, cardiac catheterization with contrast angiography, or computed tomography pose a potential hazard to the fetus.

14. CARDIOVASCULAR DRUGS Some of the drugs commonly used in the management of cardiovascular disease have potentially harmful effects on the developing embryo and fetus

15. Warfarin embryopathy, consisting of nasal hypoplasia, optic atrophy, digital abnormalities, and mental impairment, occurs in a minority of cases. A risk of 4% to 10%. embryopathy is less likely if the warfarin dosage is 5 mg/day or less

16. β-Adrenergic blocking agents, for hypertension and tachyarrhythmia, associated with neonatal respiratory depression, bradycardia, and hypoglycemia when administered late in pregnancy

17. The thiazide harmful effects on the fetus in the third trimester impair normal expansion of plasma volume. Rarely, severe neonatal electrolyte imbalance, jaundice, thrombocytopenia, liver damage, and even death have been reported.

18. angiotensin-converting enzyme (ACE) inhibitors profound and deleterious effect on fetal renal Function oligohydramnios, neonatal renal failure. absolutely contraindicated during pregnancy

19. Hydralazine, vasodilator and oral antihypertensive agent, can be substituted for ACE inhibitors during pregnancy.

20. Amlodipine is another alternative to ACE inhibitors, and it can be used with hydralazine if needed

21. Digoxin© Maternal and fetal arrhythmias, heart failure No evidence for unfavorable side effects on the fetus

22. PERIPARTUM MANAGEMENT CONSIDERATIONS In some, pregnancy termination may be advisable

23. MAJOR MATERNAL&FETAL CARDIAC RISKS — Pulmonary hypertension (pulmonary vascular disease) Maternal cyanosis Poor maternal functional class History of arrhythmia Maternal anticoagulants

24. Pulmonary hypertension Eisenmenger syndrome, Preterm delivery and fetal growth retardation at least 50 percent, 15 to 25 percent of pregnancies progressing to term

25. Cyanosis — The arterial oxygen saturation before pregnancy important predictors of poor fetal and maternal outcomes The likelihood of a live birth was much lower in women with a resting arterial oxygen saturation below 85 percent

26. Oxygen administration — The value of antepartum oxygen in cyanotic women is unproven. There is little convincing evidence that oxygen benefits the mother, and there is no evidence that a favorable effect is exerted on a growth- retarded fetus,

27. Pregnancy is contraindicated in women with Eisenmenger syndrome because of high maternal mortality risk, fetal risks, risk of thromboembolism

28. CARPREG risk index Four predictors of cardiac events were identified: (NYHA class III or IV) or cyanosis Previous cardiac events (eg, heart failure, transient ischemic attack, stroke) or arrhythmia Left heart obstruction (mitral valve 30 mmHg) Left ventricular systolic dysfunction (EF<40 %)

29. CARDIAC SURGERY DURING PREGNANCY — perform reparative cardiac surgery before conception., eliminates the fetal risk

30. Cardiac surgery during pregnancy The maternal risks are about the same as those in nonpregnant women but cardiopulmonary bypass during pregnancy incurs risk for the fetus

31. Fetal risks nonpulsatile blood flow reduced uteroplacental flow during cardiopulmonary bypass, deep hypothermia preterm labor and delivery If cardiopulmonary bypass is needed, high blood flow (2.5 L/min per m2) and mean arterial blood pressure greater than 70

32. SO If a woman presents early in pregnancy with one of the high risk valve lesions, we recommend termination of pregnancy followed by reparative surgery before another attempt at pregnancy.

33. If the mother declines termination, we manage the patient medically and operate only for refractory NYHA class III or IV symptoms It is preferable to delay surgery, if possible, until the fetus is viable; a cesarean delivery can then be performed as part of a combined procedure

34. During surgery, foam wedges should be placed The fetal heart rate should be monitored continuously. Fetal bradycardia often responds to an increase in pump flow rate. In viable fetuses, prolonged bradycardia (less than 80 beats per minute) that is unresponsive to increased pump flow rates is an indication for cesarean delivery if the fetus is at a viable gestational age

35. Cesarean delivery has been successfully performed while the mother was on cardiopulmonary bypass the catastrophe of sudden maternal death, immediate postmortem caesarean delivery is an important option. an infant survival of approximately 15 percent, cesarean delivery should be performed within four minutes of maternal cardiac arrest The resulting decompression of the gravid uterus aids maternal resuscitation

36. Fetal heart rate monitoring — FHR should be documented pre- and post- operatively at all gestational ages. Maternal hemodynamic stability does not guarantee that placental perfusion and fetal oxygenation are optimal. Intraoperatively, fetal heart rate monitoring can be a more sensitive assessment of maternal cardiorespiratory status than maternal vital signs.

37. We suggest continuously monitoring all viable fetuses (greater than 23 to 24 weeks of gestation) throughout surgery, if technically possible.. For abdominal operations, some centers use transvaginal ultrasound to monitor fetal heart rate.

38. reduced variability with induction of general anesthesia,. Baseline FHR may also decrease, but within the normal range. Vasoactive and chronotropic-modifying agents cross the placenta and can produce changes in the fetal heart rate If bradycardia, tachycardia, or repetitive decelerations occur, the anesthesiologist should optimize uteroplacental oxygen delivery and blood flow by ensuring there is no aortocaval compression;

39. FETAL RISK — functional class of the mother, maternal cyanosis maternal medications ……………expose the fetus to IUGR.

40. Overall fetal outcomes : miscarriage in 15 percent Fetal mortality was 1.7 percent perinatal mortality was 2.3 percent (compared to an expectation of less than 0.5 percent in the general population),

41. adverse neonatal outcomes in 28 percent of pregnancies preterm (21 percent), SGA (8 percent), IUFD(3 percent), IVH and neonatal death (1.4 percent each). An adverse neonatal outcome was independently predicted by a basal left ventricular outflow gradient >30 mmHg

42. Maternal cyanosis IUGR 37% prematurity fetal wastage a live birth 43% The mean birth weight of full term 2575 g compared to a normal term 3500 g.

43. . We suggest that antepartum fetal monitoring begin at 28 weeks of gestation ultrasound examinations at 28 and 34 weeks of gestation to screen for fetal growth retardation, with additional assessment if growth delay is diagnosed

44. Cyanotic congenital heart disease A successful pregnancy was unlikely if hemoglobin ≥ 20 g/dL (live birth rate of 8 percent) maternal arterial oxygen saturation was ≤ 85% (live birth rate of 12 percent).

45. Complete heart block congenital or acquired. The congenital form called the neonatal lupus syndrome trans-placental passage of maternal anti-Ro/SSA and anti-La/SSB antibodies.

46. Congenital third degree (complete) atrioventricular block present with fetal bradycardia between 18 and 28 weeks of gestation (95 percent) are due to neonatal lupus,

47. PREGNANCY — Approximately 50 percent of patients with congenital complete heart block are female survival into childbearing age is anticipated most of these patients will have indications for pacemaker insertion

48. syncopal episodes occasionally first occur during gestation and the heart and circulation may not respond adequately to the acute circulatory demands of labor and delivery

49. Management of congenital CHB in utero and in the perinatal period steroid therapy if associated with anti-Ro/SSA and anti-La/SSB antibodies, and isoproterenol and/or pacemaker insertion immediately after birth.isoproterenol

50. warfarin potentially teratogenic. Embryopathy sixth to ninth weeks of toxicity after this period is still possible

51. Fetal hemorrhage, increases the risk of hemorrhagic fetal death during vaginal delivery. To minimize this risk, warfarin should be discontinued after 34 to 36 weeks of gestation and/or cesarean delivery should be considered

52. , infusion of fresh frozen plasma into the mother does not reliably reverse fetal anticoagulation. A cesarean delivery may prevent hemorrhagic fetal death, and fresh frozen plasma should be administered to the neonate

53. For women who are taking long-term vitamin K antagonists and are attempting to become pregnant, the 2012 ACCP Guidelines have made a weak suggestion in favor of performing frequent pregnancy tests and substituting treatment with LMW heparin as soon as pregnancy is achieved, rather than switching to a LMW heparin preparation while attempting pregnancyheparin

54. We believe that this is a reasonable option for a woman who meets all of the following criteria: She has regular monthly menstrual cycles. She agrees to have a blood pregnancy test within the first seven days of the missed first day of expected menses. She can be switched to a LMW heparin preparation promptly if the pregnancy test is positive, and will have a second blood pregnancy test if the first test is negative and menses have not begun within 10 days of the missed first day of expected menses.heparin She understands the baseline risk of birth defects (3 percent) in the population and the further increased risk and types of embryopathy if she continues to take her long-term vitamin K antagonist during or after the sixth week of pregnancy (ie, ≥14 days after the missed first day of expected menses).

55. Antepartum management Serial ultrasound fetal growth, fetal behavior (FPP), amniotic fluid, blood flow. The frequency is based upon the severity of findings and whether the examinations are being done to monitor fetal well-being (one to seven times per week) or fetal growth (every two to four weeks).

56. Doppler velocimetry of the umbilical artery is recommended for monitoring pregnancies in which growth restriction is diagnosed

57. Timing of delivery Remote from term, normal umbilical artery flow by Doppler velocimetry is reassuring We would immediately deliver any pregnancy ≥32 weeks with reversed flow, under 34 weeks with absent flow

58. Delivery of the late preterm (34 to 36 6/7ths weeks) or early term (37 0/7ths to 37 6/7ths weeks) IUGR fetus is recommended if there are additional risk factors for adverse outcome, such as maternal medical/obstetrical disorders, arrest of growth over a three- to four-week interval, and/or absence or reversal of Doppler flow in the umbilical artery

59. For pregnancies with mild growth restriction at 34 to 37 weeks, normal umbilical artery Doppler flow, and no additional maternal/fetal risk factors, delivery can be delayed until 38 to 39 6/7ths weeks, when pulmonary maturity is likely Delivery at 37 to 38 weeks is reasonable if umbilical artery flow is decreased.

60. INTRAPARTUM MANAGEMENT — perform continuous intrapartum fetal monitoring to detect nonreassuring fetal heart rate patterns. Umbilical cord blood analysis should be considered as a component of establishing baseline neonatal status.

61. INDICATIONS FOR FETAL ECHOCARDIOGRAPHY Familial risk factors Maternal risk factors Fetal risk factors

62. Familial risk factors First or second degree relatives with congenital heart disease (eg, the fetus’ siblings, parents, and grandparents) Syndromes including congenital heart disease (eg, Noonan, tuberous sclerosis, Holt-Oram, velocardiofacial [DiGeorge] syndrome)

63. Maternal risk factors Maternal congenital heart disease Cardiac teratogen exposure (eg, lithium, folate antagonists, organic solvents thalidomide, anticonvulsants, isotretinoin, paroxetine, warfarin )lithium thalidomide isotretinoin paroxetine warfarin Maternal medical illness (eg, diabetes, phenylketonuria, anti Ro/SSA or anti La/SSB antibodies) Exposure to prostaglandin synthetase inhibitors (can cause premature closure of the ductus arteriosus in the third trimester) Rubella infection in the first trimester In vitro fertilization

64. Fetal risk factors Suspected cardiac anomaly during basic sonogram Extracardiac anomaly Aneuploidy Nonimmune hydrops Arrhythmia Abnormal fetal situs Increased nuchal translucency at 11 to 14 weeks of gestation Chromosomal abnormality Monochorionic twins, with or without twin-twin transfusion syndrome

65. Fetal karyotype, with screening for deletion in 22q11.2 when conotruncal anomalies are present

66. Simpson (2012) recommends cesarean delivery for women with the following: (1) dilated aortic root >4 cm or aortic aneurysm (2) acute severe congestive heart failure; (3) recent myocardial infarction; (4) severe symptomatic aortic stenosis; (5) warfarin administration within 2 weeks of delivery; (6) need for emergency valve replacement immediately after delivery

70. Indications for Fetal Echocardiography Fetal Arrhythmias Extracardiac anomalies Hydrops Hydramnios Growth restriction Chromosomal defects Increased nuchal translucency thickness at 11 + 0 - 13 + 6 weeks' gestation Trucuspid regurgitation at 11 + 0 - 13 + 6 weeks' gestation Retrograde flow in the ductus venosus at 11 + 0 - 13 + 6 weeks' gestation Abnormal heart at 18-20 weeks routine scan (usually abnormal four-chamber view)