Καρδιοπάθειες και κύηση. Υπάρχουν ενδείξεις διακοπής; Λαζαρίδου Φωτεινή, ΕΒ Καρδιολόγος Νοσοκομείο «Αγ Παύλος», Θεσσαλονίκη

Καρδιοπάθειες και κύηση. Υπάρχουν ενδείξεις διακοπής; Λαζαρίδου Φωτεινή, ΕΒ Καρδιολόγος Νοσοκομείο «Αγ Παύλος», Θεσσαλονίκη

~ 3% of women 18 44 years of age have cardiac disease ~ 1% of pregnancies are complicated by cardiac disease. Cardiac conditions account for 10 25% of maternal mortality in the USA In the past, rheumatic heart disease was the most common form of cardiac disease in pregnant women; it still predominates in developing countries and in immigrant populations in the USA. Advanced maternal age +underlying medical conditions (HT, DM, Chol) increase the incidence of acquired heart disease complicating pregnancy. Congenital heart disease in pregnancy is increasingly common as more affected women are surviving into reproductive age. US Department of Health and Human Services, Health Resources and Services Administration, Maternal and Child Health Bureau. Women s Health USA 2002. Rockville, MD: US Department of Health and Human Services; 2002. Burlew BS. Managing the pregnant patient with heart disease. Clin Cardiol 1990; 13:757-62. Koonin LM, Atrash HK, Lawson HW, Smith JC. Maternal mortality surveillance, United States, 1979-1986. MMWR Morb Mortal Wkly Rep 1991;40:1-13.

Maternal mortality rates from cardiac causes of death in the UK The progressive rise in cardiac deaths is more related to acquired disease, such as myocardial infarction and cardiomyopathy

The leading cardiac causes of maternal death in UK are now acquired disease, rising from 3.8 per million in 1990 to 20.8 per million in 2005. Myocardial infarction (MI), aortic dissection and cardiomyopathy are the main diseases. Rheumatic heart disease has re emerged as a cause of maternal health in immigrants Current Opinion in Obstetrics and Gynecology 2009, 21:508 513

Heart 2004;90:450 456

Hemodynamic alterations

Heart 2004;90:450 456

Predictors of cardiovascular complications during pregnancy in the CARPREG study The estimated risk of complications during pregnancy in patients with heart disease, but no risk factors, was 5%; with 1 risk factor, 27%; and with 2 risk factors, 75%.

Fetal complications/ prognosis Siu et al documented that the incidence of fetal complications (premature delivery and low birth weight) reached 20%, and severe complications (respiratory distress syndrome, intracranial bleeding and death) were confirmed in 5%. The factors that exacerbated fetal prognosis were NYHA class > II or cyanotic heart disease, anticoagulant therapy, smoking, multiple pregnancy and left ventricular obstruction In addition to the above factors, another study documented other risk factors; severe pulmonary regurgitation and right ventricular dysfunction Siu: Circulation 2001; 104: 515-21. Khairy: Circulation 2006; 113: 517-24.

Congenital heart lesions

Congenital heart disease is the most common congenital defect (approximately 1% of newborns are affected around the world). At least 85% of patients with congenital heart disease now survive to adulthood and half of them are women, most of reproductive age.

Non cyanotic heart disease: L R shunts Left to right shunts (ASD, VSD, PFO, and PDA) are sometimes diagnosed during pregnancy due to loud heart murmur from increased shunt volume caused by increased circulating blood volume. In pts with large shunt volume, decreases in peripheral vascular resistance balances out the increased blood volume. If no pulmonary hypertension then pregnancy, labor and delivery are completed without any complications. Risk of paradoxical embolism during labor and delivery VSD, 3 rd trimester VSD, 1 year after delivery In case of rapid bleeding, peripheral vasoconstriction may increase shunt blood flow, decrease cardiac output and cause congestive heart failure, shock and ventricular fibrillation. J Echocardiogr Vol.6, No.2, 2008 Cleveland clinic J of Medicine Vol 71, No 12, 2004

Aortic stenosis Most cases are congenital (bicuspid) or are associated with mitral stenosis AS is a risk factor for fetal complications. All pts with symptomatic AS should postpone pregnancy until after heart surgery. The risk is generally low if mean aortic gradient remains 50mmHg during pregnancy If pts remain severely symptomatic relieve aortic stenosis before delivery: Balloon aortic valvotomy in experienced centers Pregnant women with bicuspid AV associated with aortic dilatation are at risk for spontaneous aortic dissection, usually 3rd trimester, especially if there is an associated aortic coarctation For women planning pregnancy, prophylactic aortic root or ascending aorta surgery should be considered if the aortic diameter is >45mm, the rate of increase in diameter is 5 mm/year and/or there is worsening aortic regurgitation (AR).

AORTIC COARCTATION Maternal mortality in women with unrepaired aortic coarctation (CoA) has been estimated to be 3%. The main complications reported are associated with severe hypertension, including aortic dissection. MRI should be used before pregnancy, but can be safely performed during pregnancy if there is concern about recoarctation or aneurysm formation. Blood pressure should be controlled with b blockers. Changes in the aortic wall during pregnancy increase the risk inherent to CoA.

Pulmonary Stenosis (PS) Mild (<50mmHg), moderate (50 79mmHg) PS, or PS treated by valvuloplasty or surgery is well tolerated during pregnancy, with good maternal and fetal prognosis. However, in patients with severe PS, pregnancy can cause right sided heart failure or atrial arrhythmias.

Cyanotic heart disease without pulmonary hypertension During pregnancy, systemic vascular resistance decreases to elevate right to left shunt, thus exacerbating cyanosis. While the incidence of complications is high in pregnant women with cyanotic heart diseases, cyanosis is treatable and mortality risk is low. In patients without pulmonary hypertension, e.g., pulmonary atresia managed via Blalock Taussig shunt (BT), mortality during pregnancy is around 5%. Maternal risk for such patients depends largely on ventricular function (other risks hemohrrage, paradoxical embolism, heart failure, increasing cyanosis) Fetal prognosis is poor, and in severe cyanosis, fetal development is hindered (the rate of live births is 12% at 85% oxygen saturation). In one study of 96 pregnancies in 44 patients with cyanotic heart diseases, maternal cardiac complications were seen in 32%, and the rate of live births was 43%. Hemoglobin 16g/dL and oxygen saturation 85% were found to be prognosticators Circulation 1994;89:2673-2676

Cyanotic heart disease after surgical correction TOF In patients with good functional class (NYHA class I or II) and sinus rhythm, pregnancy and delivery are possible, but the incidence of miscarriage is high. The children of mothers with TOF have a 3% possibility of having some type of heart disease Repaired tetralogy of Fallot (TOF): In patients who underwent corrective surgery, pregnancy and delivery are possible. (delivery rate: 73%, cardiac events: 14%) [J Am Coll Cardiol 2004; 44: 174 80.]). Pregnancy risk factors included: residual VSD, moderate to severe pulmonary valve stenosis and insufficiency, aortic insufficiency, pulmonary hypertension, dilated aorta ( 40 mm), ventricular dysfunction, and past history of tachyarrhythmia.

Repaired cyanotic heart disease (TOF systemic RV) Pregnancy is well tolerated in women who have had radical repair of tetralogy of Fallot, as long as ventricular function is good and there is no significant right ventricular outflow tract obstruction (delivery rate: 73%, cardiac events: 14%) Pregnancy is likely to be well tolerated in asymptomatic patients with congenitally corrected transposition of the great arteries (ctga). However, systemic right ventricular function may deteriorate during pregnancy, presenting with heart failure With the exceptions of a narrowed pulmonary venous pathway or development of pulmonary vascular disease, pregnancy is usually well tolerated in NYHA class I II patients post Mustard. Post Fontan type operation: these patients are often advised against pregnancy. However, the maternal risk of pregnancy is not prohibitively high in NYHA class I II patients, provided ventricular function is good. Ventricular failure and arrhythmia are the main causes of maternal morbidity.

Marfan s syndrome During pregnancy, estrogen and other hormones make vessels more fragile, and as a result, in pregnant women with Marfan s syndrome, the risk for aortic dissection is higher than in non pregnant women. An aortic root diameter >40 mm in women with MFS identifies a high risk group, with risk of dissection during pregnancy ~ 10% (ESC2003). The 2010 ACC/AHA/American Association of Thoracic Surgeons (AATS) guidelines for the thoracic aorta note that it is reasonable to prophylactically replace the aortic root and ascending aorta if the aortic diameter exceeds 40 mm in women with MFS who are contemplating pregnancy. At <40 mm, normal delivery is possible. Treatment with beta blockers should be maintained during pregnancy J Echocardiogr Vol.6, No.2, 2008 Rev Esp Cardiol. 2006;59(9):971-84

Pulmonary hypertension Eisenmengers syndrome In patients with primary pulmonary hypertension, maternal mortality exceeds 50%, and pregnancy is contraindicated. In patients with Eisenmenger syndrome, maternal mortality ranges from 30 to 70%, and fetal mortality is as high as 50% Pulmonary hypertension is tolerated badly during pregnancy because of insufficient adaptation of the right heart to increases in cardiac output, in association with a poorly compliant pulmonary vasculature. The greatest risk occurs in the peripartum period and most deaths occur between 2 and 9 days postpartum Br J Obstet Gynaecol 1998; 105: 921-2. J Am Coll Cardiol 1999; 34: 1658. Br J Anaesth 2004; 93: 428 39

Rheumatic Heart Disease Aquired Heart Disease Cardiomyopathies

Valvuloplasty or surgery in all symptomatic patients should be considered before pregnancy Beta-blockers should be prescribed to all symptomatic woman or whose PAP> than 50 mm Hg Patients who are symptomatic despite medical treatment should be considered for balloon mitral valvuloplasty during pregnancy Rev Esp Cardiol. 2006;59(9):971-84

Algorithm for the management of mitral stenosis in the pregnant patient. MS, mitral stenosis; PBMV, percutaneous balloon mitral valvuloplasty.

ACC/ AHA 2006 guidelines for the management of patients with valvular heart disease: Circulation 2006; 114: e84-231.

Prosthetic heart valves Mechanical heart valves are associated with an increased incidence of thromboembolic events during pregnancy. The procoagulant state of pregnancy increases the risk of valve thrombus formation among women with mechanical valves. Approximately 30 to 35% of heterograft prostheses and 10 to 20% of homograft prostheses fail within 10 to 15 years of implantation

Anticoagulant regimen Choice of anticoagulant regimen is a balance between maternal and foetal risks. The incidence of valve thrombosis is lower with oral anticoagulants (warfarin) than with UFH or LMWH, but foetal risk is higher. Warfarin embryopathy occurs in 4 10% of cases and may be dose dependent, the risk being less at doses below 5mg daily Both UFH and LMWH are safer for the foetus as they do not cross the placenta

ESC 2007 and ACC/AHA 2006 guidelines recommend continuing oral anticoagulants until pregnancy is confirmed. Thereafter, the treatment options include (1) continuation of warfarin until week 36 [ (INR) 3.0], dose adjusted UFH [(APPT) twice control] or LMWH (anti Xa levels 0.7 1.2 U/ml 4 h after administration) until delivery, (2) dose adjusted LMWH/UFH 6 12 weeks and close to term with warfarin at other times and (3) dose adjusted LMWH throughout pregnancy. Low dose aspirin (75 to 162 mg/day) might be added to women deemed to be at high thromboembolic risk

Peripartum cardiomyopathy In peripartum cardiomyopathy, cardiac function recovers in about half of patients, but in this relatively rare disease (occurring in 1 in 3000 15 000 pregnancies), 20% of patients die or require a heart transplant. Maternal mortality is up to 20%. Adverse risk factors include age > 30 years, multiparity, twin pregnancy, and the 50% of women in whom left ventricular dilation and dysfunction persist. The risk for cardiac events during first or subsequent pregnancy is dependent on the severity of left ventricular dysfunction N Engl J Med 2001; 344: 1567-71.

Hypertrophic cardiomyopathy Among pregnant women with hypertrophic cardiomyopathy, maternal mortality is around 1%, and most patients can go through pregnancy. However, in about half of the patients who had symptoms before pregnancy, the NYHA functional class was exacerbated (NYHA class III/IV) during pregnancy. We often recognize elevated intraventricular pressure gradient and pulmonary hypertension in obstructive patients. Beta blockers should not be discontinued during pregnancy, avoiding severe vasodilatation, and vaginal childbirth should be indicated. J Am Coll Cardiol 2002; 40: 1864-9.

Dilated cardiomyopathy (DCM) In dilated cardiomyopathy, because prognosis is poor, especially for young patients, and most patients take medication such as an ACE inhibitor, very few patients become pregnant. Pregnancy is poorly tolerated in women with DCM. The risk of maternal death is approximately 7% if the patient is in NYHA functional class III or IV. Other adverse risks factors include EF < 20%, mitral regurgitation, right ventricular failure, atrial fibrillation, and systemic hypotension. Such patients should be counselled against pregnancy

Supraventricular arrhytmias The most common arrhythmia in women with structurally normal hearts is paroxysmal supraventricular tachycardia (PSVT), including AVNRT and AVRT. Management with vagal manouvres, adenosine, AV nodal blocking agents (ie,digoxin, beta blockers, or verapamil). If hemodynamic compromise is evident, DC cardioversion should be performed. AF and flutter during pregnancy are less common than PSVT and often occur in women with structural heart disease. In pregnant women with atrial fibrillation, a rhythm control strategy is preferred Pregnancy is a prothrombotic state, and thresholds for initiating anticoagulation are often lower than in the nonpregnant state. There is no standardized anticoagulation regime during pregnancy and the choice of anticoagulation should be tailored to the individual.

Ventricular arrhythmias Arrhythmias are the most common cardiac complication encountered during pregnancy in women with and without structural heart disease. Ventricular tachyarrhythmias are frequently associated with acquired or structural heart disease Monomorphic ventricular tachycardia without apparent structural heart disease is considered idiopathic. The most common type originates from the right ventricular outflow tract, and this form of ventricular tachycardia can often be successfully treated with beta blockers or verapamil. Women with the long QT syndrome are at risk for ventricular tachycardia, especially in the postpartum period. Ventricular tachyarrhythmias in the presence of structural heart disease are potentially lifethreatening and require immediate evaluation for hemodynamic instability to determine whether electrical cardioversion or defibrillation is indicated. Circulation. 2001;104(5):515.

Myocardial infarction Incidence of 6.2 in 100,000 pregnancies (data 2000 2002). mortality 37 50% Most cases occur in the third trimester and six week postpartum period. In a review of 103 cases from 1995 2005, coronary artery morphology was evaluated in 96 by arteriography or autopsy. coronary atherosclerosis with or without intracoronary thrombus (40 %), thrombus in a normal coronary artery (8 %), coronary artery dissection (27%),(near term or postpartum, affecting LAD, mortality 30 40%) spasm in (2%), emboli (2%), and normal coronary arteries (13%). J Am Coll Cardiol. 2008;52(3):171.

ACS management Low dose aspirin (75 to 162 mg/day), beta blockers, and nitrates Pregnancy is a relative contraindication to fibrinolytic therapy (postpartum bleeding). Heparin does not cross the placenta and thus does not affect the fetus directly. Bleeding complications (eg, threatened miscarriage) Angiotensin converting enzyme inhibitors, angiotensin II receptor blockers, and statins are contraindicated in pregnancy.

Infective Endocarditis Diagnosis and treatment are the same as outside pregnancy If gentamycin is used, drug levels should be measured (risk for foetal deafness) Decision for surgery should be made early as the foetal risk is determined on the maternal condition Antibiotic prophylaxis should be given in Prosthetic valves History of previous endocarditis

Cardiovascular drugs used during pregnancy

Title

Risk assessment before pregnancy Risk assessment can be aquired from Cardiovascular history and examination ECG Echo Arterial O2 saturation measurement Risk can be stratified according to Nature of lesion Maternal functional class or cyanosis Use of risk index (functional class, LV systolic dysfunction, left heart obstruction, cardiac event arrhythmia, stroke,pulmonary edema ) Maternal age Neonatal risk Risk for transmission (CHD) Anticipated maternal and paternal survival (CHD)