Enfermedad Cardiovascular Materna
Enfermedad Cardiovascular Materna
Enfermedad Cardiovascular Materna
13-11-e651
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Article
maternal-fetal medicine
Educational Gaps
Fetuses and newborns of mothers with cardiovascular disease face a range of risks related to the type of maternal disease, the drugs used to manage it, and the inheritability of the disease.
Author Disclosure Drs Hill and Pacheco have disclosed no nancial relationships relevant to this article. This commentary does contain a discussion of an unapproved/ investigative use of a commercial product/ device.
Abstract
Although cardiac lesions in pregnancy are often well tolerated, those that are not warrant a cardiac evaluation as well as careful management in the antepartum and peripartum periods. Congenital heart disease cases comprise the majority of pregnant cardiac patients, and the lesions can require careful monitoring. Women who have valvular disease often tolerate pregnancy well, but there are specic lesions that require close monitoring throughout the pregnancy. Finally, peripartum cardiomyopathy, although rare, is of great concern and must be managed quickly and appropriately. We will review concerns and treatment for pregnant women who have congenital heart disease, valvular lesions, and peripartum cardiomyopathy, as well as provide guidance for drug therapy in the pregnant patient who has cardiac disease.
Objectives
1. Identify common congenital heart lesions seen in the pregnant patient and understand basic management for each lesion. 2. Understand medical management for valvular cardiac disease in pregnancy. 3. Classify heart lesions according to patient symptoms. 4. List medications that should not be used for cardiac patients in pregnancy.
Introduction
Cardiac disease in the pregnant patient is encountered more and more often because of women surviving prior cardiac surgeries as well as the advances in assisted reproductive medicine. In fact, more than one half of cardiac patients managed obstetrically have congenital heart disease (CHD). Currently, the incidence of cardiac disease in pregnancy is w4% but notably the morbidity can be much higher (34%). The leading causes for maternal mortality consist of cardiomyopathy, pulmonary hypertension, aortic dissection, and myocardial infarction. Many physiologic changes occur in the pregnant patient (Table 1). Although total body volume increases, red blood cell volume increases at a lower rate, causing a dilutional aneAbbreviations mia. This anemia allows the pregnant woman to have less AS: aortic stenosis viscous blood to better perfuse her placenta to nurture ASD: atrial septal defect the fetus. The heart compensates for the overall increased CHD: congenital heart disease volume during pregnancy with decreased blood pressure NYHA: New York Heart Association (nadir at the late second to early third trimester) and dePDA: patent ductus arteriosus creased systemic vascular resistance (SVR). Moreover, PVR: pulmonary vascular resistance the cardiac output is greatly increased, starting as early as SVR: systemic vascular resistance 8 weeks gestation. UFH: unfractionated heparin Patients who have cardiac disease in pregnancy should VSD: ventricular septal defect be classied according to any limitations in their physical
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Table 1.
CHD lesions but should be strongly considered for patients who have Eisenmenger syndrome because they have a higher rate of thromboembolism. Each lesion is discussed briey in the following text.
activity as well as symptoms they experience at rest or with physical activity. The basic criteria for the New York Heart Association (NYHA) classication system can be found in Fig 1. This classication system may be used in preconceptional counseling for patients who have known cardiac disease. We will address patients who have cardiac disease encountered in the obstetric world, including those who have CHD, valvular disease, and cardiomyopathy, as well as discuss medications used in pregnancy.
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Table 2.
Cardiac Lesion Patent ductus arteriosus Atrial septal defect Ventricular septal defect Coarctation Tetralogy of Fallotc Eisenmenger syndromec Marfan syndrome
CCBcalcium channel blocker; HRheart rate; PVRpulmonary vascular resistance. a Can lead to increase in left-to-right shunt. b If pulmonary hypertension is present, avoid increase in PVR (ie, hypoxemia, nitrous oxide, hypercarbia, vasoconstrictors) because it can worsen a right-to-left shunt. c No epidural; may consider with Tetraology of Fallot if no intracardiac shunt is present.
Tetralogy of Fallot
The tetrad of VSD, overriding aorta, right ventricular hypertrophy, and pulmonary stenosis often has been corrected surgically by the time a patient reaches childbearing age and thus is well tolerated in pregnancy. These women should be monitored for pulmonary regurgitation because it places them at the greatest risk for complications such as arrhythmia and heart failure. Echocardiograms should be performed on these women to assess for any remaining cardiac shunts that could place them at increased risk for pulmonary hypertension.
treatment for this diagnosis. SVR decreases in pregnancy. If a woman experiences hypertension, she will have an increase in SVR, which could then lead to a worsening of a left-to-right shunt. Equally, if the pulmonary vascular resistance (PVR) falls, a left-to-right shunt can worsen. Therefore, increases in SVR and decreases in PVR should be avoided in patients who have a PDA, ASD, or VSD. When a patient has already experienced reversal of a shunt, and thus has a right-to-left shunt with pulmonary hypertension, it is important to avoid increases in PVR (metabolic acidosis, hypoxia, or lung hyperination), which could worsen a right-to-left shunt.
Eisenmenger Syndrome
This syndrome occurs when a left-to-right intracardiac shunt (PDA, ASD, or VSD) shifts to a right-to-left shunt. This happens when the pulmonary vasculature system is overloaded, resulting in pulmonary hypertension, which provides enough pressure to reverse the shunt. In severe cases, a heartlung transplant may be the ultimate
Marfan Syndrome
Marfan syndrome is an autosomal dominant condition that will be accompanied by cardiac complications 80% of the time. The main concern is a dilation of the aortic root and therefore risk of dissection or rupture. It is recommended that women undergo repair before
Table 3.
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pregnancy if the dilation of the aorta is more than 4.5 cm. Risk of rupture when the aorta is not dilated is extremely low (<1%). In labor, women should be encouraged to minimize the use of the Valsalva maneuver. Women should be counseled strongly regarding the high risk of having a neonate who has Marfan syndrome (Table 3).
Ebstein Anomaly
Ebstein anomaly is rarely seen in the pregnant population, and reported cases resulted in no maternal complications and neonatal complications of prematurity and congenital car- Figure 2. Maternal mortality associated with cardiac lesions in pregnancy. AS[aortic diac abnormalities. This anomaly is stenosis; ASD[atrial septal defect; PDA[patent ductus arteriosus; MI[myocardial dened as apical displacement of infarction; MS[mitral stenosis; NYHA[New York Heart Association; VSD[ventricular the tricuspid septal leaet and is al- septal defect. ways accompanied by tricuspid regurgitation, which can lead to right atrial dilation. One counseled on the risk of recurrence of CHD in the fetus half of these patients have a co-existing ASD or PDA. depending on the type of maternal CHD (Table 3). Maternal mortality risks should also be reviewed and can vary widely, from less than 1% with ASD and VSD up to 25% Transposition of the Great Vessels to 50% in patients who have pulmonary hypertension Patients who have complete transposition of the great ves(Fig 2). sels who reach childbearing age have undergone corrective procedures, most commonly an atrial switch (Mustard procedure). These women tolerate pregnancy well but can eventually experience irreversible right ventricular dysfunction. Finally, when counseling a mother who has CHD, she should be aware that she has approximately a 5% chance of having a child who has CHD. Also, the mother should be
Table 4.
ARaortic regurgitation; ASaortic stenosis; EFejection fraction; LVleft ventricular; MRmitral regurgitation; MSmitral stenosis; NYHANew York Heart Association.
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a valvular lesion in pregnancy should be counseled regarding their risks during the pregnancy. After identifying the lesion and the NYHA classication, one can further classify the patient into low or high maternal and/or fetal risks (Table 4). NYHA I patients fall into the low-risk category. Conversely, patients who fall into NYHA III or IV carry higher risks during pregnancy. Patients who have tricuspid lesions usually have an isolated lesion, which is well tolerated during pregnancy. Regurgitant lesions in the mitral and aortic valves, mitral valve prolapse, and pulmonary stenosis also cause minimal concern in the pregnant patient. Moreover, these lesions rarely require treatment throughout pregnancy or during labor. Stenotic lesions of the mitral and aortic valve are most concerning in pregnancy and will be addressed specically in this section. General management criteria for patients who have valvular disease in pregnancy can be found in Table 5.
Mitral Stenosis
Mitral stenosis is the most common valvular lesion found in pregnancy. The stenotic mitral valve causes decreased left ventricle lling and can ultimately lead to pulmonary hypertension and right ventricle failure. Individuals who have mitral stenosis warrant an echocardiogram and an electrocardiogram. Patients must be carefully monitored for pulmonary edema and can experience atrial brillation and arrhythmias. Anticoagulation should be considered, especially if the patient experiences atrial brillation. Medications that cause tachycardia (eg, terbutaline) should be avoided, and an assisted second stage of labor to decrease use of Valsalva maneuvers should be strongly considered.
and is decreased to one fourth the normal 3 to 4 cm2 in diameter, then the disease is considered severe, and surgical repair is recommended. The cardiac output for these patients is xed, due to the stenotic aortic valve, making proper perfusion of the mother, as well as the fetus, difcult. For this reason, patients who have severe AS (aortic valve <1 cm2) should limit their physical activity. Patients should have an electrocardiogram to look for arrhythmias and an echocardiogram to evaluate the aortic valve and ejection fraction. It is imperative to maintain an appropriate preload as these patients will not be able to increase cardiac output, which is needed to maintain perfusion during the physiologic stresses of pregnancy. For the same reasons, hypotension and bradycardia should be avoided. Use of a narcotic epidural is possible, but great care must be taken to avoid hypotension with placement of any epidural catheter. Patient symptoms should be monitored often to precisely decide on NYHA classication of the pregnant patient who has AS, and placental perfusion will need to be evaluated throughout the pregnancy. Counseling patients who have a valvular lesion varies depending on the type of lesion and degree of effect. Low-risk lesions such as issues with the tricuspid valve, pulmonary stenosis, mitral valve prolapse, and regurgitant lesions of the mitral or aortic valve are well tolerated in pregnancy and often do not require further evaluation. Stenotic lesions of the mitral or aortic valve can cause complications and must be more carefully managed during pregnancy. Combining maternal NYHA classication as well as maternal history with the valvular heart lesion (Table 4) can help in counseling the pregnant patient regarding maternal and fetal risks.
Aortic Stenosis
Pregnant patients who have aortic stenosis (AS) generally tolerate pregnancy well if their disease is mild (valvular area >1.5 cm2). If the valve becomes more stenotic
Peripartum Cardiomyopathy
Diagnosis of peripartum cardiomyopathy classically is the development of cardiac failure (ejection fraction <45%)
Table 5.
Lesion PS MR AR MS ASa
ARaortic regurgitation; ASaortic stenosis; HRheart rate; MRmitral regurgitation; MSmitral stenosis; SVRsystemic vascular resistance; PSpulmonary stenosis; PVRperipheral vascular resistance. a No epidural.
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from the last month of pregnancy up to 5 months postpartum with no identiable cause for cardiac failure. This condition can lead to maternal mortality, and maternal death is most common in the immediate postpartum period. Management details regarding the patient who has peripartum cardiomyopathy can be found in Fig 3. Anticoagulant therapy is not prophylactically warranted unless the patient has a known cardiac thrombus or coronary artery disease.
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embryopathy, is estimated by Hirsch and Fuster to be 4% to 10%. Moreover, use of warfarin during embryogenesis in the rst trimester is related to higher miscarriage rates. During the latter part of pregnancy, use of warfarin can have a deleterious effect on the fetus, sometimes resulting in intracranial hemorrhage. It is also important to note the long half-life of warfarin, especially when compared with the other anticoagulant choices (Table 6). In a study by Chan et al, warfarin was found to be more effective than UFH in preventing venous thromboembolism in patients who have older-generation mechanical heart valves. With the risks of warfarin, and the availability of other anticoagulant agents that are highly effective in preventing venous thromboembolism, warfarin is not routinely used in the pregnant patient.
parentheses. The classes are: (A), human studies show no risk to fetus; (B), animal studies show risk or no risk to fetus, but human studies show no risk to fetus; (C), animal studies have shown adverse risk to fetus, but human studies are lacking; (D), human studies show evidence of fetal risk. Class C and D may have medical benets for the mother that could outweigh risk to fetus. IUGR[intrauterine growth retardation.
Warfarin, however, crosses the placenta and is usually not used in pregnancy. Use in the rst trimester warrants concern for fetal malformations such as nasal and limb hypoplasia, optic atrophy, and impaired mental status. The risk of this syndrome, referred to as warfarin
Table 6.
Anticoagulation Medications
Anticoagulant Unfractionated heparin Low-molecular-weight heparin Warfarin Half-Life 6090 min 4.5 h (single dose) 7 h (multiple doses) 1.52.5 d
Pregnant patients who have cardiac lesions do not require antibiotic prophylaxis. Currently, the American College of Obstetricians and Gynecologists, as well as the American Heart Association, recommend systemic bacterial endocarditis prevention with a vaginal or cesarean delivery only if infection is present in specic cardiac lesions (Table 7). The antibiotics recommended are as follows: ampicillin, cefazolin, ceftriaxone, clindamycin, or amoxicillin. Because cefazolin, ceftriaxone, and clindamycin do not cover enterococcus, vancomycin should be added to those regimens. Antibiotics should be administered 30 to 60 minutes before delivery.
Conclusions
Cardiac lesions in pregnancy are often well tolerated, but those that are not warrant a cardiac evaluation as well as careful management in the antepartum and peripartum periods. Many patients, specically those who have peripartum cardiomyopathy, are at a higher risk of death in
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Cardiac Lesions Requiring Systemic Bacterial Endocarditis Prophylaxis When Infection Is Present
Table 7.
Prosthetic cardiac valve or prosthetic material for valve repair History of infective endocarditis Unrepaired cyanotic CHD (palliative shunts, conduits) Completely repaired CHD with prosthetic materials if done <6 mo ago Repaired CHD with residual defects
CHDcongenital heart disease.
the immediate postpartum period. One cannot forget that the postpartum period is an important time to watch the cardiac patient closely because third-spacing and increased cardiac output can put added stress on an already inadequately functioning heart. It is important to properly identify the exact cardiac lesions a pregnant woman presents with and closely monitor her symptoms (with assistance of NYHA classication) so that she can be properly counseled, managed, and treated throughout her pregnancy.
Suggested Reading
American College of Obstetricians and Gynecologists. Practice bulletin no. 120: use of prophylactic antibiotics in labor and delivery. Obstet Gynecol. 2011;117:14721483
American College of Cardiology/American Heart Association Task Force on Practice Guidelines; Society of Cardiovascular Anesthesiologists; Society for Cardiovascular Angiography and Interventions; Society of Thoracic Surgeons; Bonow RO, Carabello BA, Kanu C, et al. ACC/AHA 2006 guidelines for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (writing committee to revise the 1998 Guidelines for the Management of Patients With Valvular Heart Disease): developed in collaboration with the Society of Cardiovascular Anesthesiologists: endorsed by the Society for Cardiovascular Angiography and Interventions and the Society of Thoracic Surgeons. Circulation. 2006;114(5):e84e231 Bonow RO, Carabello BA, Chatterjee K, et al. 2008 Focused update incorporated into the ACC/AHA 2006 Guidelines for the Management of Patients With Valvular Heart Disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to revise the 1998 guidelines for the management of patients with valvular heart disease) endorsed by the Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. J Am Coll Cardiol. 2008;52(13):e1e142 Chan WS, Anand S, Ginsberg JS. Anticoagulation of pregnant women with mechanical heart valves: a systematic review of the literature. Arch Intern Med. 2000;160(2):191196 Chang J, Elam-Evans LD, Berg CJ, et al. Pregnancy-related mortality surveillanceUnited States, 1991-1999. MMWR Surveill Summ. 2003;52(2):18 Donnelly JE, Brown JM, Radford DJ. Pregnancy outcome and Ebsteins anomaly. Br Heart J. 1991;66(5):368371 Francois K. Postpartum hemorrhage. In: Foley MR, Strong TH, Garite TJ, eds. Obstetric Intensive Care Manual. 3rd ed. New York, NY: The McGraw-Hill Companies Inc; 2010: 2737 Hirsch J, Fuster V. Guide to anticoagulant therapy. Part 2: oral anticoagulants. American Heart Association. Circulation. 1994; 89(3):14691488 Lupton M, Oteng-Ntim E, Ayida G, Steer PJ. Cardiac disease in pregnancy. Curr Opin Obstet Gynecol. 2002;14(2):137143 Martin SR, Hill AJ, Foley MR. Cardiac disease in pregnancy. In: Queenan JT, Spong CY, Lockwood CJ. eds. Queenans Management of High-Risk Pregnancy. 6th ed. Oxford: WileyBlackwell; 2012:260291 Simpson LL. Maternal cardiac disease: update for the clinician. Obstet Gynecol. 2012;119(2 pt 1):345359 Vandvik PO, Lincoff AM, Gore JM, et al; American College of Chest Physicians. Primary and secondary prevention of cardiovascular disease: antithrombotic therapy and prevention of thrombosis, 9th ed: American College of Chest Physicians Evidence-Based Clinical Practice Guidelines [published correction appears in Chest. 2012;141(4):1129]. Chest. 2012;141 (suppl 2):e637Se668S
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5. In terms of cardiac medications used in pregnancy, which of the following is correct? A. Adenosine should not be administered to a pregnant patient who is experiencing supraventricular tachycardia. B. Angiotensin converting enzyme inhibitors and angiotensin receptor blockers should be avoided in the rst trimester because they can lead to congenital malformations and in the later trimesters the fetus can experience impaired renal function that can result in oligohydramnios. C. Antibiotic prophylaxis should not be used in pregnant women with cardiac lesions even if they have infective endocarditis. D. Unfractionated heparin and low molecular weight heparin are safe for the fetus because they readily cross the placenta. E. Warfarin is a safe drug to use for anticoagulation during all stages of pregnancy.
Cardiovascular Disease in Pregnancy Alexandria J. Hill and Luis D. Pacheco Neoreviews 2012;13;e651 DOI: 10.1542/neo.13-11-e651
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