Pregnancy and congenital heart disease (1)

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Pregnancy in congenital heart disease:

Pregnancy in congenital heart disease Moderator-: Dr. V. Hariharan Dr.Mithilesh kumar DNB Trainee Indraprastha Apollo hospital 28-08-12



Introduction :

Introduction Approximately 2% of pregnancies involve maternal cardiovascular disease With the declining incidence of rheumatic heart disease in Western countries, most maternal cardiac disease is now congenital in origin. Incidence of congenital heart disease in india-3.9/1000 live births (MAMC 1993)

Introduction :

Introduction Some patients will present for the first time in pregnancy with symptoms and learn that they have congenital heart disease. Patients even with repaired defects may encounter cardiac problems during pregnancy, the most common being heart failure and arrhythmias. All patients, with or without cardiac repair, should have a detailed evaluation and appropriate counseling before pregnancy is considered.


HEMODYNAMIC CHANGES IN PREGNANCY Changes are profound and begin early in the first trimester. The plasma volume begins to increase in the sixth week of pregnancy and by the second trimester approaches 50% above baseline. The plasma volume then tends to plateau until delivery. This increased plasma volume is followed by a slightly lesser rise in red cell mass , which results in the relative anemia of pregnancy. The heart rate begins to increase to about 20% above baseline to facilitate the increase in cardiac output.




HEMODYNAMIC CHANGES IN PREGNANCY Uterine blood flow increases with placental growth, and there is a fall in peripheral resistance . This decreased peripheral resistance may result in a slight fall in blood pressure, The venous pressure in the lower extremities rises, which is why approximately 80% of healthy pregnant women develop pedal edema. “The adaptive changes of a normal pregnancy result in an increase in cardiac output, which begins in the first trimester and by the end of the second trimester approaches 30% to 50% above baseline .”


HEMODYNAMIC CHANGES IN PREGNANCY Labour , particularly the second stage, is associated with a further increase in cardiac output as pain increases heart rate via the sympathetic response and stroke volume is augmented by autotransfusion during contractions. In the absence of haemorrhage there is also significant autotransfusion after delivery. Thus the later stages of labour are a period of high risk for pulmonary oedema

Structural changes to the heart and great vessels:

Structural changes to the heart and great vessels Echo diameters and orifice areas of all four valves increase, causing a higher incidence of valvular regurgitation Changes in the extracellular matrix of the aortic media increase compliance but also, in combination with the increased cardiac output, the risk of dissection. 80% of healthy pregnant women have a third heart sound and >90% a soft ejection systolic murmur at the left sternal edge.



High-Risk Pregnancies:

High-Risk Pregnancies Pulmonary hypertension Dilated cardiomyopathy (EF <40%) Symptomatic obstructive lesions:- Aortic stenosis Mitral stenosis Pulmonary stenosis Coarctation of the aorta Marfan syndrome with aortic root >40 mm Cyanotic lesions Mechanical prosthetic valves

High-Risk Pregnancies:

High-Risk Pregnancies In some situations, the maternal risk of pregnancy is very high, and the patient should be counseled to avoid pregnancy and sometimes even to consider termination of pregnancy if it occurs . Pulmonary HTN higher than 60% to 70% of the systemic pressure are likely to be associated with maternal compromise; in these circumstances, pregnancy is best avoided. Women who have a LVEF <40% from any cause are not likely to withstand the volume load that pregnancy imposes and should be advised not to become pregnant.

High-Risk Pregnancies:

High-Risk Pregnancies Because pregnancy is associated with a decrease in peripheral resistance, symptomatic patients with significant stenotic cardiac lesions are likely to deteriorate during a pregnancy. Patients with a dilated aortic root >40 mm are vulnerable to progressive aortic dilation, dissection, and rupture during pregnancy eg:- Marfan syndrome. This occurs due to increased stroke volume and gestational hormonal changes causing histologic abnormality in the aortic media. Estrogen inhibits collagen and elastin synthesis in the aorta, and progesterone has been shown to accelerate the deposition of noncollagenous proteins in the aortas of rats.

Atrial Septal Defect:

Atrial Septal Defect Secundum ASD is one of the most common congenital heart defects. Patients with even a large secundum ASD tolerate pregnancy without complication unless there is coexistent pulmonary hypertension or atrial fibrillation. Give meticulous attention to maternal leg veins, particularly during peridelivery, because deep venous thrombosis could precipitate a paradoxical embolus and stroke. Elective closure of an atrial septal defect by device or operative repair is preferable before pregnancy is contemplated.

Ventricular Septal Defect:

Ventricular Septal Defect Patients with small defects usually tolerate pregnancy without difficulty. In the setting of a large ventricular septal defect and pulmonary hypertension, patients should be counseled not to proceed with a pregnancy

Patent Ductus Arteriosus:

Patent Ductus Arteriosus Small ducts with normal or near-normal pressures usually cause no risk during pregnancy. large shunt- volume load of pregnancy might precipitate left ventricular failure. With pulmonary hypertension:- avoid pregnancy.

Congenital Aortic Stenosis:

Congenital Aortic Stenosis Usually occurs secondary to a bicuspid aortic valve. Detailed 2D-echo assessment of the valve function should be performed before pregnancy is contemplated. associated aortic dilation or ascending aortic aneurysm may be present. Pregnancy is contraindicated if the aortic dimension is > 4.5 cm.

congenital Aortic Stenosis:

congenital Aortic Stenosis Mild aortic stenosis – pregnancy tolerated Moderate stenosis - sometimes well tolerated, In the absence of symptoms & with a normal exercise test without ST-T wave changes severe aortic stenosis - valve area <1 cm 2 or a mean gradient >50 mm Hg should be counseled not to have a pregnancy

Coarctation of the Aorta:

Coarctation of the Aorta A significant coarctation causes diminished flow to both the uterus and fetus, result in small-for-dates babies or even fetal loss. Because of the associated aortopathy, the entire aorta is vulnerable to dilation, aneurysm, and dissection. Blood pressure should be controlled to below 130/80 with B -blockers as the first line agent vaginal delivery with assisted second stage to avoid aortic wall stress is the method of choice Angioplasty and stenting of coarctation during pregnancy and the puerperium is not recommended because of the increased predisposition to dissection during this period Most women, however, will have a successful pregnancy with proper care.

Pulmonary Stenosis:

Pulmonary Stenosis Pulmonary stenosis is usually well tolerated during pregnancy, particularly if the right ventricular pressure is less than 70% of systemic pressure and sinus rhythm is maintained. If necessary, balloon pulmonary valvuloplasty can be performed, with shielding of the fetus from radiation.

Aortopulmonary Window:

Aortopulmonary Window Aortopulmonary windows which were successfully treated in childhood without the development of other symptoms pose no significant problems for pregnancy. However, pregnancy is dangerous for patients with aortopulmonary windows, with significant risks for both mothers and their unborn children

Cyanotic Heart Disease:

Cyanotic Heart Disease Cyanosis poses risks for both mother and fetus. The decrease in peripheral resistance that accompanies pregnancy augments the right-to-left shunt and may exaggerate the maternal cyanosis. Because of the erythrocytosis that accompanies cyanosis and the propensity to thrombosis, women who develop venous thrombosis are at risk of paradoxical embolus and stroke. Maternal hypoxia imposes a pronounced handicap to fetal growth and survival

Cyanotic Heart Disease:

Cyanotic Heart Disease Presbitero and colleagues have evaluated 44 women with 96 pregnancies (excluding patients with Eisenmenger syndrome) and confirmed earlier study findings that the degree of maternal cyanosis has a profound impact on fetal outcome.

Fetal Outcome in Cyanotic Congenital Heart Disease and Its Relationship with Maternal Cyanosis:

Fetal Outcome in Cyanotic Congenital Heart Disease and Its Relationship with Maternal Cyanosis NO. OF PREGNANCIES NO. OF LIVE BIRTHS PERCENTAGE BORN ALIVE Hemoglobin, g/dL* ≤ 16 28 20 71 17-19 40 18 45 ≥ 20 26 2 8 Arterial oxygen saturation (%)† ≤ 85 17 2 12 85-89 22 10 45 ≥ 90 13 12 92 Presbitero P, Somerville J, Stone S, et al: Pregnancy in cyanotic congenital heart disease. Outcome of mother and fetus. Circulation 89:2673, 1994

Ebstein Anomaly:

Ebstein Anomaly In the absence of maternal cyanosis, right-sided heart failure, or arrhythmias, pregnancy is usually well tolerated Cesarean section may be selected as the safest form of delivery.

Eisenmenger syndrome:

Eisenmenger syndrome

Eisenmenger syndrome:

Eisenmenger syndrome If the patient chooses to proceed with pregnancy against medical advice, careful monitoring by health professionals is essential. Minimal activity and bed rest are usually recommended, and treatment with anticoagulants and oxygen therapy may be prescribed. Cesarean section may be selected as the safest form of delivery. Reduced activity and careful monitoring for a couple of weeks after giving birth are necessary to guard against sudden death.

Tetralogy of Fallot:

Tetralogy of Fallot women with a good surgical repair, good exercise capacity, and minimal residua, pregnancy may be well tolerated An occasional patient not had prior surgery or palliated with a surgically created shunt (e.g., Blalock-Taussig), pregnancy may pose a risk, depending on the degree of cyanosis. 2D-echo:- look for residual pulmonary regurgitation and associated RV dysfunction and tricuspid regurgitation. The volume load of pregnancy may not be well tolerated in these circumstances offer pre-pregnancy counseling to look for 22q11 deletion. If there is no parental chromosomal abnormality and no family history of other congenital cardiac disease, the risk of the fetus having a congenital cardiac anomaly is approximately 5% to 6%.

Transposition of the Great Arteries (D-Transposition):

Transposition of the Great Arteries (D-Transposition ) Patients who have received the arterial switch (Jatene) repair for TGA-D,, are not expected to have significant cardiac problems during pregnancy All patients repaired with Mustard or Senning atrial baffle procedure are at risk of developing right ventricular and tricuspid valve dysfunction as well as arrhythmias, such as atrial flutter. These symptoms will significantly increase the risks of pregnancy. Therefore, these patients require careful evaluation before conception and regular monitoring if they become pregnant. However, there is an increased risk of prematurity or low birth weight Dysfunction of the systemic ventricle may be a contraindication to pregnancy.

Coarctation of aorta:

Coarctation of aorta assess coarctation repair site to exclude residual or recurrent coarctation or aneurysm formation ( repaired with a patch are more susceptible) assess the entire aorta to rule out dilation or aneurysm formation. Outcome - favorable. For patients with mild dilation of the aorta, vaginal delivery with a short second stage is reasonable, if evidence of aortic instability, a cesarean section is preferable.

Univentricular Heart and Fontan Operations:

Univentricular Heart and Fontan Operations These women have an increased risk of atrial arrhythmias, which may cause profound hemodynamic deterioration. vulnerable to thrombosis in the Fontan circuit because of the sluggish flow and prothrombotic state of pregnancy. Function of the single ventricle may deteriorate because of the volume load of pregnancy the risk of miscarriage also appears to be increased ( 30% rate of first trimester fetal loss)

Univentricular Heart and Fontan Operations:

Univentricular Heart and Fontan Operations Maternal outcome again depends on functional capacity and ventricular function, which is more likely to be adequate if the single ventricle is morphologically left. If these are satisfactory and the woman accepts the 30% rate of first trimester fetal loss, twice that of the general population, then there is no reason to advise against pregnancy successful pregnancies under careful medical supervision have been reported for patients with various forms of single ventricle, both with and without the Fontan.

Pulmonary Hypertension:

Pulmonary Hypertension carries a high mortality when it is associated with pregnancy The most common cause in childbearing years is secondary to a shunt in the setting of congenital heart disease (e.g., VSD, PDA OR ASD). When the pulmonary hypertension >60% of systemic levels, pregnancy is more likely to be associated with complications. In the setting of severe pulmonary vascular disease (Eisenmenger syndrome) maternal mortality may approach 50%. The volume load of pregnancy may compromise the poorly functioning right ventricle and precipitate heart failure. The fall in peripheral resistance augments right-to-left shunting and may precipitate more cyanosis

Pulmonary Hypertension:

Pulmonary Hypertension The time around labor and delivery is particularly dangerous, and the highest incidence of maternal death is during parturition and the puerperium. There may be an abrupt decrease in afterload as the baby is delivered, and hypovolemia from blood loss can cause hypoxia, syncope, and sudden death. Vagal responses to pain may also be life-threatening. Death may also occur from pulmonary embolism or in situ pulmonary infarction

Pulmonary Hypertension:

Pulmonary Hypertension Women who elect to continue should be admitted for bed rest from the third trimester and monitored for at least two weeks ante partum, as this is a period of continued risk of sudden death. Cesarean section delivery is probably preferable with cardiac anesthesia. No studies have suggested a more favorable outcome with the use of pulmonary vasodilators, although case reports have suggested a more successful maternal outcome. Nitric oxide can be administered and successful pregnancy has also been reported with intravenous epoprostenol. Sildenafil has also been used, but with all these agents, maternal death may still occur days or weeks after delivery

Management During Pregnancy:

Management During Pregnancy MEDICAL THERAPY Healthy patient little or no specific treatment require careful monitoring. NYHA Class I or II need to limit strenuous exercise adequate rest supplementation of iron and vitamins to minimize the anemia of pregnancy low-salt diet if there is concern about ventricular dysfunction regular cardiac and obstetric evaluations NYHA Class III or IV hospital admission for bed rest close monitoring require early delivery if there is maternal hemodynamic compromise.

Surgical management During Pregnancy:

Surgical management During Pregnancy Cardiac surgery is seldom necessary during pregnancy and should be avoided whenever possible. There is a higher risk of fetal malformation and loss if cardiopulmonary bypass is performed in the first trimester; if it is performed in the last trimester, there is a higher likelihood of precipitating premature labor. The “optimal time” appears to be between 20 and 28 weeks of gestation. Obstetric monitoring of the fetus during the procedure is recommended so that fetal bradycardia may be dealt with promptly and uterine contractions may be controlled. Despite these interventions, the current risk of fetal loss is still at least 10% and is probably higher when the cardiac surgery is emergent.

Management of Arrhythmias:

Management of Arrhythmias supraventricular and ventricular ectopic beats require no therapy The most common arrhythmia is atrial reentry tachycardia. Atrial fibrillation is usually an indication that there is underlying structural heart disease. Treatment is same as for nonpregnant women with added concern about the effects of medications on the fetus Use lowest dose of medication Electrical cardioversion - If the arrhythmia is unresponsive to medical therapy Ventricular tachycardia – rare, electrical cardioversion should be performed if there is hemodynamic compromise

ACC/AHA Recommendations for Treatment Strategies for Supraventricular Tachycardias During Pregnancy:

ACC/AHA Recommendations for Treatment Strategies for Supraventricular Tachycardias During Pregnancy INDICATION CLASS I (INDICATED ) CLASS IIa (STRONG SUPPORTIVE EVIDENCE ) CLASS IIb (WEAK SUPPORTIVE EVIDENCE ) CLASS III (NOT INDICATED ) Acute conversion of PSVT Vagal maneuver Adenosine Direct-current cardioversion Metoprolol,* propranolol * Verapamil Prophylactic therapy Digoxin Metoprolol* Propranolol* Sotalol,* flecainide Quinidine, propafenone , verapamil Procainamide Catheter ablation Atenolol Amiodarone

Labor and Delivery:

Labor and Delivery The hemodynamic changes during labor and delivery are abrupt. With each uterine contraction, up to 500 mL of blood is released into the circulation, prompting a rapid increase in cardiac output and blood pressure. During a normal vaginal delivery, approximately 400 mL of blood is lost. In contrast, with a cesarean section, about 800 mL of blood is often lost and may pose a more significant hemodynamic burden to the parturient . After delivery of the baby, there is an abrupt increase in venous return, in part because of autotransfusion from the uterus but also because the baby no longer compresses the inferior vena cava. In addition, there continues to be autotransfusion of blood in the 24 to 72 hours after delivery, and this is when pulmonary edema may occur.

Labor and Delivery:

Labor and Delivery For most patients with cardiac disease, a vaginal delivery is feasible and preferable Delivery can be accomplished in the left lateral position so that the fetus does not compress the inferior vena cava, thereby maintaining venous return. The second stage should be assisted, if necessary (e.g., forceps or vacuum extraction), to avoid a long labor. Blood and volume loss should be replaced promptly. For those patients with tenuous hemodynamics, Swan-Ganz catheterization before active labor facilitates optimization of the hemodynamics and should be continued for at least 24 hours after delivery, when pulmonary edema commonly occurs.

Cardiac indications for caesarean section:

Cardiac indications for caesarean section immature fetal liver does not metabolize warfarin as rapidly as the mother's liver. After discontinuation of warfarin, reversal of anticoagulation occurs more rapidly in the mother, whereas reversal of anticoagulation in the fetus may take up to 1 week because of the immature fetal liver. Vaginal delivery when the fetus is anticoagulated is contraindicated because of the risk of fetal hemorrhage


Contraception For women with congenital heart disease, appropriate contraceptive advice should be given before they become sexually active. Because pregnancy may pose a high risk of morbidity and even mortality. Patients need to be given detailed advice about various contraceptive methods and their effectiveness,and each patient should understand the relative risks and benefits of each modality

Barrier Contraception:

Barrier Contraception safe for all cardiac patients added benefit of protection against sexually transmitted diseases failure rate of approximately 15 pregnancies/100 woman-years

Hormonal methods:

Hormonal methods The oestrogen component of the combined oral contraceptive (COCP) confers an increased risk of thrombosis. It is therefore contraindicated in women with high thrombotic :- -pulmonary hypertension, -the Fontan circulation, - dilated cardiac chambers with the risk of atrial fibrillation - in cyanosed patients in whom paradoxical embolism may occur

Hormonal methods:

Hormonal methods The standard progesterone-only ‘‘mini’’ pill is safe, but is less reliable than the COCP Recommended progesterone- - daily oral desogestrel - three monthly depot medroxyprogesterone, - and the subcutaneous implant Implanon

Implanon :

Implanon long acting reversible hormonal contraceptive subdermal implant contains 68 milligrams of etonogestrel (a progestin)which is released over a three failure rate- 0.0449%, year period Implanon is the method of choice for complex congenital heart disease

Intrauterine devices :

Intrauterine devices not contraindicated insertion can be associated with bacteraemia and a vasovagal response. Antibiotic prophylaxis protects against endocarditis Vasovagal can be life threatening in Fontan circulation or Eisenmenger syndrome. Thus, IUCD is best avoided.


Sterilisation Sterilisation by tubal ligation may be appropriate for women in whom pregnancy would be high risk. may be performed laparoscopically or through a laparotomy There may be risk of cardiac instability, paradoxical embolism, cardiac anesthesia may be preferable Sterilisation of the male partner is notgenerally advised if he has a much longer potential lifespan than his partner and may therefore wish to father children in a subsequent relationship. More recently, tubal sterilization has been accomplished with the use of an intrafallopian plug, which is inserted endoscopically( Mayo Clin Proc 2008; 83:431 )


Contraception-summary COCP contraindicated if tendency to thrombosis or cyanosed daily oral desogestrel and Implanon safe for all and highly effective IUCD needs antibiotic prophylaxis for insertion and beware of vasovagal. Best avoided.

Recurrence risk if one sibling has cardiovascular anomaly:

Recurrence risk if one sibling has cardiovascular anomaly anomaly Suggested risk % VSD 3.0 ASD 3.0 PDA 2.5 TOF 2.5 PULM. STENOSIS 2.0 CoA 2.0 AS 2.0 TGA 1.5 AV CANAL DEFECT 2.0 T.ATRESIA 1.0 EBSTEIN ANOMALY 1.0 HLHS 2.0 PULM. ATRESIA 1.0 PERSISTENT TRUNCUS ARTERIOSUS 1.0


AFFECTED OFFSPRING GIVEN ONE PARENT WITH A CHD Defect Mother affected % Father affected % AS 13.0-18.0 3.0 ASD 4.0-4.5 1.5 AV CANAL DEFECT 14.0 1.0 CoA 4.0 2.0 PDA 3-5-4.0 2.5 PULM. STENOSIS 4.0-6.5 2.0 TOF 6.0-10.0 1.5 VSD 6.0 2.0


conclusion Advances in paediatric cardiology and cardiac surgery are resulting in a growing population of young women with congenital heart disease well enough to contemplate pregnancy. Although certain conditions are still associated with a high maternal mortality. Appropriate pre- pregnancy assessment and risk stratification should be made. A careful discussion of the maternal and fetal risks should be made at the time of pre-pregnancy counseling, and if the mother is going to pursue a pregnancy, a strategy should be outlined regarding the frequency of follow-up by the cardiologist, and a plan should be put in place for labor and successful delivery


problems Few evidence-based guidelines are available most published data involve isolated case reports or small cohort studies. Few physicians have expertise or training to manage such patients What is the ideal management strategy ???



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