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Multidisciplinary Management of Pregnancy in Complex Congenital Heart Disease: A Model for Coordination of Care Rachel C. Harris, MD,* Melissa H. Fries, MD,† Annelee Boyle, MD,† Hassan Adeniji-Adele, MD,‡ Zacharia Cherian, MD,§ Nancy Klein, BSN, RN,¶ and Anitha S. John, MD, PhD¶ *Harvard Medical School, Massachusetts General Hospital, Boston, Mass, USA; †Genetics and Fetal Medicine, Department of Obstetrics and Gynecology, ‡Obstetric Anesthesia, Department of Anesthesiology and §Department of Neonatology, Washington Hospital Center, Washington, DC, USA; ¶Division of Cardiology, George Washington University School of Medicine, Children’s National Medical Center, Washington, DC, USA ABSTRACT
With advancements in medical care, many women with complex congenital heart disease (CHD) are now living into adulthood and childbearing years. The strains of pregnancy and parturition can be dangerous in such patients, and careful interdisciplinary plans must be made to optimize maternal and fetal health through this process. Several large studies have been published regarding risk prediction and medical management of pregnancy in complex CHD, though few case studies detailing clinical care plans have been published. The objective of this report is to describe the process of developing a detailed pregnancy and delivery care plan for three women with complex CHD, including perspectives from the multidisciplinary specialists involved in the process. This article demonstrates that collaboration between specialists in the fields of cardiology, anesthesiology, high-risk obstetrics, maternal fetal medicine, and neonatology results in clinically successful individualized treatment plans for the management of pregnancy in complex CHD. Multidisciplinary collaboration is a crucial element in the management of pregnancy in complex CHD. We provide a template used in three cases which can serve as a model for the design of future care plans. Key Words. Complex Congenital Heart Disease; Pregnancy; Multidisciplinary; Delivery Plan
Introduction
Case 1
S
A 28-year-old gravida three para zero woman with mitral stenosis, subaortic stenosis, valvular aortic stenosis, and coarctation of the aorta (Shone’s syndrome) presented for management through her first pregnancy. She had undergone a total of 13 cardiac surgeries, including a lateral thoracotomy for coarctation repair and six midline sternotomies for valvular surgeries. She had a mechanical mitral valve placed at the age of 16 years and most recently had a subaortic resection and mechanical aortic valve placed at the age of 28 years. She also had a history of ventricular tachycardia. Her obstetrical history included a left oopherectomy secondary to left-sided ovarian torsion and two first trimester miscarriages. The patient had lifelong follow-up by a CHD specialist and received extensive prenatal counseling regarding the high risk of thrombosis associated with mechanical valves during pregnancy. However, the patient and her husband ultimately decided to proceed understanding these risks. Her
urvival for patients with congenital heart disease (CHD) has increased greatly, such that many women with CHD are living well into their childbearing years.1 Since the 1990s, the number of annual deliveries for women with CHD has dramatically risen by 34.9%.2 Unfortunately, this increase also brings a significantly higher risk of adverse maternal and fetal complications, over eight times that of their peers without CHD.2 As more women with complex CHD continue to undergo pregnancy and delivery, there is a growing need for further delineation of management strategies. Caring for these high-risk patients requires a multidisciplinary team approach. We present examples of a multidisciplinary team approach to three complex CHD patients, with attention to each case from the perspective of individual subspecialties. The following cases and sample care plans demonstrate a model that can be used for determining ideal care based on an individual woman’s cardiac risk.
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© 2014 Wiley Periodicals, Inc.
Management of Pregnancy in Congenital Heart Disease prepregnancy left ventricular ejection fraction (EF) was 55%, with normal mitral and aortic mechanical valve function. She was initiated on subcutaneous heparin injections (goal PTT 60–80) immediately after discovering she was pregnant. She had allergies to vitamin K and fresh frozen plasma. In addition, she was Rh negative, and had anti-Kell antibodies. Mexilitine was also stopped but furosemide and nadolol were continued due to a history of congestive heart failure (CHF) and ventricular arrhythmia. At 20 weeks estimated gestational age (EGA), a multidisciplinary team meeting was held. Over the course of her pregnancy, the patient developed worsening heart failure, and her anticoagulation also became more difficult to control. At 30 weeks gestation, she was admitted to the hospital for worsening heart failure (EF = 35–40%) and was diuresed with intravenous furosemide and received a course of betamethasone to promote fetal lung development. At 31 weeks EGA, the fetus was found to have intrauterine growth restriction, and the patient was taken for cesarean section using epidural anesthesia. She delivered a male infant weighing 1346 g with APGAR scores of 8 at 1 minute and 9 at 5 minutes. The infant remained in the neonatal intensive care unit for 6 weeks. Postpartum she suffered from CHF and was eventually discharged home after a 17-day hospitalization. At 6 weeks and 6 months postpartum, her left ventricular EF remained 45–50% and she was New York Heart Association (NYHA) Class I.
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25 mm Hg gradient across her pulmonary conduit. In addition, she was noted to have moderate pulmonary conduit insufficiency, neo-aortic root diameter of 37 mm, mild neo-aortic insufficiency, normal aortic valve gradient, and left ventricular diastolic dysfunction. She had not seen an adult congenital heart specialist since her cardiac catheterization. During the second trimester, she was noted to have progressive fatigue and edema, due to worsening conduit stenosis (peak gradient of 80 mm Hg) and diastolic dysfunction. At 26 weeks gestation, she began having nonsustained run of ventricular tachycardia (4–10 beats at 113 beats per minute). In addition, she also developed hypertension and was started on labetalol. At approximately 35 weeks EGA, an interdisciplinary team meeting was held, and a delivery care plan was formed. Three days following, the patient was admitted for increasing dyspnea, fatigue, and edema in addition to decreased amniotic fluid and poor fetal growth. She underwent an uncomplicated cesarean section delivery, using epidural anesthesia. Her infant weighed 2755 g with APGAR scores of 8 at 1 minute and 9 at 5 minutes. Postpartum, she was diuresed with i.v. furosemide for 5 days and was discharged with continued oral furosemide and labetalol. At 6 weeks postpartum, she was NYHA Class 1, and her pulmonary artery conduit peak gradient had decreased to 40 mm Hg and her other findings were stable.
Case 3 Case 2
A 28-year-old gravida three para one woman with a history of critical aortic coarctation and severe aortic stenosis with a bicuspid aortic valve presented during the second trimester of her third pregnancy. Prior procedures included a left subclavian flap repair of the aortic coarctation, multiple balloon aortic valvuloplasties, and a Ross procedure at 12 years of age. Four years prior, her first pregnancy was complicated by a 4th degree perineal tear, with significant blood loss requiring aggressive fluid resuscitation. Following delivery, the patient subsequently developed CHF and required diuretic therapy for 2 months postpregnancy. The patient’s second pregnancy resulted in a first trimester miscarriage. One year prior to her most recent pregnancy, she had a cardiac catheterization that revealed a
A 25-year-old gravida one para zero woman with a history of tetralogy of Fallot, pulmonary atresia, and right aortic arch was referred for management during her first pregnancy. She was status post–left modified Blalock–Taussig shunt and subsequently had a right ventricular pulmonary artery (RV-PA) conduit placement at 4 years of age, followed by conduit revision with a 22 mm aortic homograft and main pulmonary artery arterioplasty at 10 years of age. She presented to our clinic during her second trimester of pregnancy. Prior to conception, she was found to have proximal conduit obstruction and calcification. She underwent an attempt at transcatheter percutaneous pulmonary valve placement. Balloon dilation within the conduit resulted in right coronary artery compression and subsequent myocardial ischemia in addition to a small self-contained conduit tear. Congenit Heart Dis. 2014;9:E204–E211
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She was lost to follow-up after the attempt at the percutaneous pulmonary valve placement. At that time, an echo demonstrated elevated right ventricular pressures and an increasing gradient across the RV-PA conduit. By 30 weeks gestation, her echo showed worsening conduit obstruction and peak gradient of 80 mm. She also reported increased dyspnea on exertion and fatigue, and she was placed on modified bed rest restrictions. At this time, an interdisciplinary team meeting was held to discuss her management. Due to progressive symptoms and poor fetal growth, she underwent cesarean section delivery using epidural anesthesia at 36 weeks gestational age. The infant was a viable female, weight 2912 g, APGAR 8/9. She was managed with intravenous diuresis and discharged after 4 days with aspirin and lasix therapy. At 6 weeks postpartum, she continued to have fatigue with exertion and her peak RV-PA conduit gradient was 65 mm Hg. At 7 months postpartum, she underwent successful replacement of her RV-PA conduit with a 25 mm pulmonary homograft. Discussion
Pregnant women with CHD have a 10% risk of adverse maternal outcomes, and 4% risk of adverse neonatal outcomes.3 The maternal mortality rate of 0.15% for these women is significantly elevated compared with their peers without CHD.2 Additionally, women with complex CHD have over twice the risk of adverse outcomes compared with women with simple CHD.2 Multidisciplinary team management has been utilized successfully in other chronic conditions, such as adult CHF. A team approach that involved follow-up with collaborative team (nursing, cardiology, and primary care) has been shown to reduce mortality and CHF-related hospitalizations.4 In addition, adherence to medical therapy and quality of life were also improved in CHF patients who were part of a multidisciplinary protocol.5 The Table 1.
team structure involved collaboration between nursing and physicians to ensure adequate followup, coordination of visits, and a detailed medical algorhythm. Following these examples, we present three cases which illustrate the use of a multidisciplinary team (adult congenital cardiology, obstetrics, neonatology, anesthesia, and nursing) in managing high-risk patients with CHD through pregnancy. Following is an in-depth discussion of the components of the plan with each specialty’s concerns and input on management. Patient Selection for Multidisciplinary Management
There are several scoring systems available to risk stratify pregnant women with CHD. Risk factors identified by the CARdiac disease in PREGnancy (CARPREG) investigators and ZAHARA investigators are summarized in Table 1.6,7 Patients with one or more risk factors and those with moderate or severely complex CHD had a multidisciplinary team meeting to discuss management.8 Close contact was maintained between the high-risk obstetrical team and the adult congenital cardiology team for the duration of the pregnancy. Multidisciplinary meetings are usually held between 20 and 24 weeks, as the fetus approached viability and maternal complications during the pregnancy can be factored into the management plan. Discussions in cases 2 and 3 were delayed due to patient choice of management team and time of presentation, respectively. Patients were invited to the meeting to allow the opportunity to ask questions to the group. Mode of Delivery
Initial discussions began with time of delivery (EGA) and mode of delivery. Sample plans are included for both induced vaginal delivery (Table 2) and elective cesarean section (Table 3). Contingency plans were also discussed in case of fetal or maternal distress. Delivery between 37 and 40 weeks is usually planned, provided fetal and maternal stability. In
Risk Stratification Methods
Risk factors
CARPREG4*
ZAHARA5
Prior cardiac event or arrhythmia Baseline NYHA class >II or cyanosis Left heart obstruction Systemic ventricular ejection fraction 1 predictor have a risk of 67%. †In the setting of moderately complex congenital heart disease. AV, atrioventricular valve; CARPREG, CARdiac disease in PREGnancy; CHD, congenital heart disease; NYHA, New York Heart Association.
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Management of Pregnancy in Congenital Heart Disease Table 2.
Template for Pregnancy Care Plans, Induced Vaginal Delivery Description
Sample Plan
Scheduled Vaginal Delivery Induction Hospital Admission Determine timing and workup at admission
Hemodynamic Monitoring Cardiology
Anesthesia
Determine type of cardiac monitoring, location of monitoring, type of staffing. Cardiology Consult; Anticipated medical management during and post delivery Type of pain control and any special considerations
1. 2. 3. 1. 2. 1. 2. 3. 1. In 2. 3.
Rate of fluid administration based on cardiac lesion (ie, IVF at 100 cc/hr, IV TKVO, or no restriction) Recommendations for endocarditis prophylaxis.
4. 1. 2. 1.
Second Stage of Labor
Specify agents to be used for induction and location of delivery Specify if assisted or nonassisted.
2. 1. 2. 1.
Post-partum
Location of recovery for baby and mother.
Fluid Management Endocarditis Prophylaxis
Induction
Spontaneous Labor Prior to Induction General management Same as above Hemodynamic Monitoring Same as above Arrest of Labor; Failure to Progress Attempt to further induce Additional agents to use Cesarean Section Location of surgery and type of support
1. 2. 2.
Admit EGA 39 weeks Type and cross Hold heparin anticoagulation, PT/PTT. CVP and arterial line placement Return to L&D for monitoring by CCU nursing. ACHD consult on admit Lasix IV x 1 post delivery Antiarrhythmic medications to have on hold Epidural placement case of heparin use: If PTT elevated, Fentanyl PCA until PTT normal, then epidural. If PTT normal, will place epidural for pain control; slow epidural dosing with no boluses. Protamine available if indicated. IVF at 100 cc/hr Foley placement, ins & outs monitoring. At second stage of labor: Ampicillin 2 g IV, and Gentamicin 1.5 mg/kg IV. Six hours later: additional Ampicillin 1 gm. Oxytocin, AROM when indicated. Delivery in L&D OR. Assisted with vacuum or forceps to minimize maternal stress Administer RhoGAM. Baby to newborn nursery. Transfer to CCU for further monitoring for 24 hours for cardiac care and diuresis.
Same as above Anesthesia will place CVP and arterial line; CCU nurse for monitoring Utilize uterotonic agents: pitocin, cytotec 1. Main OR or Cardiac OR/cardiac anesthesia depending on maternal stability 2. NICU support
Fetal Distress Cesarean Section
Location and support needed
L&D OR, with NICU support
Maternal Distress Cesarean Section
Location of surgery and type of support
Main or Cardiac OR depending on maternal state; NICU and cardiac anesthesia support
EGA, estimated gestational age; PT/PTT, prothrombin time/partial thromboplastin time; CVP, central venous pressure; L&D, labor and delivery; CCU, cardiac care unit; ACHD, adult congenital heart disease; IVF, intravenous fluids; IV, intravenous; TKVO, to keep vein open; AROM, artificial rupture of membranes; OR, operating room; NICU, neonatal intensive care unit.
most cases a vaginal delivery is preferred as it has been associated with smaller shifts in blood volume, fewer clotting or bleeding complications, and lower risk of infection.9 In some cases, vaginalassisted delivery methods, including vacuum devices and forceps, are recommended to decrease maternal strain.10,11 Certain positioning such as left lateral decubitus can minimize fluctuations in cardiac output by eliminating uterine pressure upon the inferior vena cava (IVC).10,12 Due to the increased risks of fluid shifts and blood loss, cesarean delivery is usually recommended to be reserved for cases of maternal hemodynamic deterioration, fetal distress, obstet-
rical indications, severe obstructive lesions, severe pulmonary hypertension, or an unstable aorta (≥40 mm in Marfan syndrome).9–11 Elective cesarean section may also be utilized for patients on warfarin anticoagulation for artificial heart valves to minimize the time off of anticoagulation. In case 1, the patient was at higher risk for bleeding due to prior abdominal surgery and allergies to reversal agents; therefore, the decision was made to attempt an induced vaginal delivery initially. Due to fetal compromise and maternal condition, as also occurred in case 3, delivery was via elective cesarean instead. For case 2, cesarean section was Congenit Heart Dis. 2014;9:E204–E211
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Harris et al. Pregnancy Care Plans, Scheduled Cesarean Section Description
Sample Plan
Scheduled Cesarean Section Hospital Admission Determine timing and workup at admission Hemodynamic Monitoring Cardiology
Determine type of cardiac monitoring, location of monitoring, type of staffing. Cardiology Consult; Anticipated medical management during and post delivery
Anesthesia Fluid Management
Type of pain control and any special considerations Rate of fluid administration based on cardiac lesion (ie, IVF at 100 cc/hr, IV TKVO, or no restriction)
Endocarditis Prophylaxis Post-partum
Recommendations for endocarditis prophylaxis. Location of recovery for baby and mother.
1. Admit evening before EGA 37 weeks, type & screen, make NPO at midnight. 1. Arterial line placement prior to Cesarean Section 1. ACHD Consult on admit 2. Recovery with telemetry monitoring on Cardiac floor post delivery 1. Epidural anesthesia for cesarean section. 1. IVF at 100 cc/hr of section, Foley placement, hourly ins & outs monitoring. Blood products on call. 1. No prophylaxis needed 1. Transfer mother to cardiac floor for further monitoring/diuresis for 24 hours. 2. Baby to visit mother on cardiac unit
Spontaneous Labor Prior to Scheduled Cesarean section General management Same as above Hemodynamic Monitoring Same as above
Same as above Same as above
Fetal Distress Cesarean Section
Location of surgery and support needed
L&D OR, with cardiac anesthesia support
Maternal Distress Cesarean Section
Location of surgery and support needed
Cardiac or Main OR; NICU, Cardiology, and Cardiac Anesthesia support
EGA, estimated gestational age; NPO, nothing by mouth; ACHD, adult congenital heart disease; IVF, intravenous fluids; IV, intravenous; TKVO, to keep vein open; L&D, labor and delivery; OR, operating room; NICU, neonatal intensive care unit.
initially scheduled due to the severity of the patient’s prior perineal tear. Cardiac Management
Women with CHD of moderate or greater complexity should ideally be followed at a congenital heart center. Optimally, counseling regarding risks of pregnancy and potential risks to the fetus should be discussed prior to undertaking a pregnancy. Careful review of maternal medications should be performed, with discontinuation and substitution of medications not approved for use in pregnancy. In case 1, the patient had been on mexilitine for a remote history of ventricular tachycardia. This was discontinued, but after weighing the risks and benefits, the nadolol was continued. Offspring of women with CHD are at risk for their own CHD, with an average rate of 3–5%.13,14 Certain anomalies and some associated genetic syndromes such as 22q11.2 deletion syndrome carry a much higher risk of recurrence.15 Therefore, genetic counseling should be offered to these patients prior to the pregnancy so that individual risk can be determined. Fetal echocardiography at 18–22 weeks is recommended to allow for identification of and treatment planning for any structural abnormalities.13 Congenit Heart Dis. 2014;9:E204–E211
During labor and delivery, augmented cardiac contractility and heart rate can increase cardiac output and myocardial oxygen demand. When combined with acutely increasing pulmonary artery pressures due to acidosis and hypercarbia, these hemodynamic factors may result in heart failure if not recognized.9 Given these factors, fluid administration was discussed as part of the care plan and recommendations were individualized for each patient. The type of cardiac monitoring during labor and delivery depends on the underlying cardiac disease and maternal condition. For case 1, a central venous pressure line and an arterial line were placed due to the patient’s CHF, ventricular arrhythmias, and concern for worsening during the labor and delivery process. Most patients do not require aggressive monitoring, but if there are concerns of rapidly worsening maternal status due to poor cardiac function, arrhythmias, pulmonary hypertension, or severe obstruction, more intensive monitoring should be considered. Following delivery, cardiac output increases again from the release of pressure upon IVC and autotransfusion from the contracted uterus of up to half a liter.10,12,16 Because of the extensive fluid shifts postpartum, patients may need to be observed in a cardiac unit after delivery depending
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Management of Pregnancy in Congenital Heart Disease on their heart disease.12,16 Recommendations for the above cases were for telemetry monitoring following delivery. All presented cases spent 1–4 days on a cardiac unit for heart failure management prior to being transferred to the postpartum unit. All patients required immediate intravenous diuretic therapy after delivery. Case 1 had the added complication of two mechanical valve prostheses. Maintaining adequate anticoagulation presents a challenge during pregnancy due to increased prothrombotic and decreased fibrinolytic activity.17 The maternal mortality rate due to prosthetic valve thrombosis during pregnancy is approximately 1–4%, and careful coordination between teams is necessary to manage anticoagulation during the labor and delivery process.12 During pregnancy and periods of CHF, anticoagulation levels can be quite variable and need to be closely monitored. Recommendations for starting and stopping anticoagulation were determined at the time of the multidisciplinary meeting and written into the care plan.
Endocarditis prophylaxis at the onset of labor is recommended for women with evidence of infection or in whom a complicated delivery course cannot be excluded, as well as women with mechanical heart valves or conduits.10,18,19 In case 1, the patient had mechanical heart valves, and in cases 2 and 3, the patients had valved conduits.
pulmonary vascular resistance. Additionally, there can be a large increase in pulmonary arterial pressure during laryngoscopy and intubation, further reasons to avoid general anesthesia.9 Extensive monitoring by the anesthesia team is another crucial component in the care of the pregnant woman with CHD. Some recommended forms of monitoring for women with complex CHD include: arterial O2 saturation, cardiac rhythm, blood pressure, central venous, and arterial pressure.9,20,21 Recommendations for the type of monitoring were made by both cardiology and anesthesia at the time of the meeting as discussed previously. In cases where maternal decompensation is a concern, cesarean section is preferably performed in the main operating room, where the expertise of a cardiac anesthesia team is readily available. Many maternal morbidities occur peripartum, due to the large hemodynamic fluctuations during delivery, and close monitoring should be continued. Anticoagulation with heparin may be safely continued throughout pregnancy up until 12 hours before induction to allow partial reversal for epidural placement and delivery. If the patient presents with bleeding while on heparin, reversal with protamine sulfate is recommended.22 This easy reversal with protamine is part of the reason heparin was chosen for case 1, as the patient would not be able to be quickly reversed if she were on warfarin due to her allergy to both vitamin K and plasma blood products.
Anesthesia and Cardiac Monitoring during Delivery
Fetal and Neonatal Considerations
Choice, route, and timing of anesthesia during delivery for women with CHD are important considerations for the anesthesia team. Due to release of catecholamines, pain and uterine contractions lead to tachycardia, hypertension, and overall increased cardiac output.10,20 Because this extra stress may result in arrhythmias, ischemia, or heart failure, care must be taken to avoid this through the use of effective and early anesthesia.16,20 Because it carries less cardiovascular risks, neuraxial anesthesia (epidural) is the primary anesthesia of choice for both cesarean sections and vaginal deliveries.20 The epidural is slowly titrated to maintain systemic vascular resistance (SVR) or avoid a sudden drop in SVR. Especially for women with pulmonary artery hypertension, general anesthesia during pregnancy increases their risk of death fourfold due to a depression in cardiac contractility by the volatile agents and increases in
Pregnant women with CHD have approximately a 12–16% risk of premature birth.3,7 Similarly, women with complex CHD have higher rates of small for gestational age children.3,7 The CARPREG study identified maternal NYHA class, cyanosis, left heart obstructive lesions, smoking, multiple gestations, and anticoagulation as risk factors for neonatal complications.6,7 Further study showed that maternal risk factors for neonatal complications included: at birth cyanotic disease, the use of cardiac medications, and mechanical valve prosthesis.7,13 Through the pregnancy, the fetus must be monitored for intrauterine growth retardation, as chronic maternal hypoxemia and uteroplacental insufficiency may lead to fetal compromise.3,13,14,16 During delivery, both the mother and the fetus should be monitored. Signs of fetal compromise such as a decrease in the heart rate variability,
Endocarditis Prophylaxis
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Nursing
Nursing staff from all of the above disciplines also attended these multidisciplinary meetings and were critical in instituting the recommended care plans. When necessary, cardiac nurses monitor the patient while on the obstetrical service prior to delivery. As all of these reported cases needed at least 24-hour recovery on a cardiac unit, obstetrical nurses provided additional visits and expertise to the cardiac nursing staff. Nurses from the neonatology division facilitated visits to the mother’s bedside in the cardiac care unit so that adequate bonding time between mother and baby could be established. Nursing staff from the adult congenital program distributed the care plan to all on-call doctors and services involved. Additionally, they communicated with the patients on a regular basis, especially as the due date approached. Discussions included follow-up of visits, lab testing and results, medication changes as ordered by attending, and assessing the patient’s status. Nurses also communicated with the specialists involved and provided updates and reports as needed. Finally, in the first 2 months following discharge, nursing staff continued to correspond with the patient regularly to assess her condition and help coordinate appointments.
General Considerations and Conclusion
Obstetric management of the patient with a congenital heart anomaly requires early onset of care and regular follow-up. Ideally, all women would establish care with an adult congenital heart specialist and obstetrician prior to conception. Preconception and genetic counseling is useful to discuss risks of pregnancy to the mother and the fetus. The initial consultation should include a detailed history and physical exam, and review of cardiac reports and medications. All patients require a fetal echocardiogram. In high-risk cases, patients should deliver at institutions with a labor and delivery unit, neonatal intensive care unit, and a cardiac intensive care unit with specialists in CHD. Risks for subsequent pregnancies should Congenit Heart Dis. 2014;9:E204–E211
Harris et al. be discussed with the patient and contraception recommendations should be offered prior to discharge. Women with CHD represent a high-risk population of patients to care for during pregnancy. Input from multiple subspecialties is needed for individualized coordination of care to ensure optimal care for both mother and infant. Author Contributions Rachel C. Harris: Concept/design, data collection, data analysis/interpretation, drafting article, critical revision of article. Melissa H. Fries/Nancy Klein/Hassan Adeniji-Adele/ Zacharia Cherian/Annelee Boyle: Critical revision of article, approval of article. Anitha S. John: Concept/Design, data collection, data analysis/interpretation, drafting article, critical revision of article, approval of article.
Corresponding Author: Anitha S. John, MD, PhD, Division of Pediatric Cardiology, Children’s National Medical Center, 111 Michigan Avenue, NW, Washington, DC 20010, USA. Tel: 202-476-3543; Fax: 202-4765700; E-mail: [email protected] Conflict of interest: None, self-funded. No relationships with industry. Disclosures of grants or other funding: None, self-funded. Accepted in final form: December 9, 2013.
References
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Multidisciplinary Management of Pregnancy in Complex Congenital Heart Disease: A Model for Coordination of Care Rachel C. Harris, MD,* Melissa H. Fries, MD,† Annelee Boyle, MD,† Hassan Adeniji-Adele, MD,‡ Zacharia Cherian, MD,§ Nancy Klein, BSN, RN,¶ and Anitha S. John, MD, PhD¶ *Harvard Medical School, Massachusetts General Hospital, Boston, Mass, USA; †Genetics and Fetal Medicine, Department of Obstetrics and Gynecology, ‡Obstetric Anesthesia, Department of Anesthesiology and §Department of Neonatology, Washington Hospital Center, Washington, DC, USA; ¶Division of Cardiology, George Washington University School of Medicine, Children’s National Medical Center, Washington, DC, USA ABSTRACT
With advancements in medical care, many women with complex congenital heart disease (CHD) are now living into adulthood and childbearing years. The strains of pregnancy and parturition can be dangerous in such patients, and careful interdisciplinary plans must be made to optimize maternal and fetal health through this process. Several large studies have been published regarding risk prediction and medical management of pregnancy in complex CHD, though few case studies detailing clinical care plans have been published. The objective of this report is to describe the process of developing a detailed pregnancy and delivery care plan for three women with complex CHD, including perspectives from the multidisciplinary specialists involved in the process. This article demonstrates that collaboration between specialists in the fields of cardiology, anesthesiology, high-risk obstetrics, maternal fetal medicine, and neonatology results in clinically successful individualized treatment plans for the management of pregnancy in complex CHD. Multidisciplinary collaboration is a crucial element in the management of pregnancy in complex CHD. We provide a template used in three cases which can serve as a model for the design of future care plans. Key Words. Complex Congenital Heart Disease; Pregnancy; Multidisciplinary; Delivery Plan
Introduction
Case 1
S
A 28-year-old gravida three para zero woman with mitral stenosis, subaortic stenosis, valvular aortic stenosis, and coarctation of the aorta (Shone’s syndrome) presented for management through her first pregnancy. She had undergone a total of 13 cardiac surgeries, including a lateral thoracotomy for coarctation repair and six midline sternotomies for valvular surgeries. She had a mechanical mitral valve placed at the age of 16 years and most recently had a subaortic resection and mechanical aortic valve placed at the age of 28 years. She also had a history of ventricular tachycardia. Her obstetrical history included a left oopherectomy secondary to left-sided ovarian torsion and two first trimester miscarriages. The patient had lifelong follow-up by a CHD specialist and received extensive prenatal counseling regarding the high risk of thrombosis associated with mechanical valves during pregnancy. However, the patient and her husband ultimately decided to proceed understanding these risks. Her
urvival for patients with congenital heart disease (CHD) has increased greatly, such that many women with CHD are living well into their childbearing years.1 Since the 1990s, the number of annual deliveries for women with CHD has dramatically risen by 34.9%.2 Unfortunately, this increase also brings a significantly higher risk of adverse maternal and fetal complications, over eight times that of their peers without CHD.2 As more women with complex CHD continue to undergo pregnancy and delivery, there is a growing need for further delineation of management strategies. Caring for these high-risk patients requires a multidisciplinary team approach. We present examples of a multidisciplinary team approach to three complex CHD patients, with attention to each case from the perspective of individual subspecialties. The following cases and sample care plans demonstrate a model that can be used for determining ideal care based on an individual woman’s cardiac risk.
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Management of Pregnancy in Congenital Heart Disease prepregnancy left ventricular ejection fraction (EF) was 55%, with normal mitral and aortic mechanical valve function. She was initiated on subcutaneous heparin injections (goal PTT 60–80) immediately after discovering she was pregnant. She had allergies to vitamin K and fresh frozen plasma. In addition, she was Rh negative, and had anti-Kell antibodies. Mexilitine was also stopped but furosemide and nadolol were continued due to a history of congestive heart failure (CHF) and ventricular arrhythmia. At 20 weeks estimated gestational age (EGA), a multidisciplinary team meeting was held. Over the course of her pregnancy, the patient developed worsening heart failure, and her anticoagulation also became more difficult to control. At 30 weeks gestation, she was admitted to the hospital for worsening heart failure (EF = 35–40%) and was diuresed with intravenous furosemide and received a course of betamethasone to promote fetal lung development. At 31 weeks EGA, the fetus was found to have intrauterine growth restriction, and the patient was taken for cesarean section using epidural anesthesia. She delivered a male infant weighing 1346 g with APGAR scores of 8 at 1 minute and 9 at 5 minutes. The infant remained in the neonatal intensive care unit for 6 weeks. Postpartum she suffered from CHF and was eventually discharged home after a 17-day hospitalization. At 6 weeks and 6 months postpartum, her left ventricular EF remained 45–50% and she was New York Heart Association (NYHA) Class I.
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25 mm Hg gradient across her pulmonary conduit. In addition, she was noted to have moderate pulmonary conduit insufficiency, neo-aortic root diameter of 37 mm, mild neo-aortic insufficiency, normal aortic valve gradient, and left ventricular diastolic dysfunction. She had not seen an adult congenital heart specialist since her cardiac catheterization. During the second trimester, she was noted to have progressive fatigue and edema, due to worsening conduit stenosis (peak gradient of 80 mm Hg) and diastolic dysfunction. At 26 weeks gestation, she began having nonsustained run of ventricular tachycardia (4–10 beats at 113 beats per minute). In addition, she also developed hypertension and was started on labetalol. At approximately 35 weeks EGA, an interdisciplinary team meeting was held, and a delivery care plan was formed. Three days following, the patient was admitted for increasing dyspnea, fatigue, and edema in addition to decreased amniotic fluid and poor fetal growth. She underwent an uncomplicated cesarean section delivery, using epidural anesthesia. Her infant weighed 2755 g with APGAR scores of 8 at 1 minute and 9 at 5 minutes. Postpartum, she was diuresed with i.v. furosemide for 5 days and was discharged with continued oral furosemide and labetalol. At 6 weeks postpartum, she was NYHA Class 1, and her pulmonary artery conduit peak gradient had decreased to 40 mm Hg and her other findings were stable.
Case 3 Case 2
A 28-year-old gravida three para one woman with a history of critical aortic coarctation and severe aortic stenosis with a bicuspid aortic valve presented during the second trimester of her third pregnancy. Prior procedures included a left subclavian flap repair of the aortic coarctation, multiple balloon aortic valvuloplasties, and a Ross procedure at 12 years of age. Four years prior, her first pregnancy was complicated by a 4th degree perineal tear, with significant blood loss requiring aggressive fluid resuscitation. Following delivery, the patient subsequently developed CHF and required diuretic therapy for 2 months postpregnancy. The patient’s second pregnancy resulted in a first trimester miscarriage. One year prior to her most recent pregnancy, she had a cardiac catheterization that revealed a
A 25-year-old gravida one para zero woman with a history of tetralogy of Fallot, pulmonary atresia, and right aortic arch was referred for management during her first pregnancy. She was status post–left modified Blalock–Taussig shunt and subsequently had a right ventricular pulmonary artery (RV-PA) conduit placement at 4 years of age, followed by conduit revision with a 22 mm aortic homograft and main pulmonary artery arterioplasty at 10 years of age. She presented to our clinic during her second trimester of pregnancy. Prior to conception, she was found to have proximal conduit obstruction and calcification. She underwent an attempt at transcatheter percutaneous pulmonary valve placement. Balloon dilation within the conduit resulted in right coronary artery compression and subsequent myocardial ischemia in addition to a small self-contained conduit tear. Congenit Heart Dis. 2014;9:E204–E211
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She was lost to follow-up after the attempt at the percutaneous pulmonary valve placement. At that time, an echo demonstrated elevated right ventricular pressures and an increasing gradient across the RV-PA conduit. By 30 weeks gestation, her echo showed worsening conduit obstruction and peak gradient of 80 mm. She also reported increased dyspnea on exertion and fatigue, and she was placed on modified bed rest restrictions. At this time, an interdisciplinary team meeting was held to discuss her management. Due to progressive symptoms and poor fetal growth, she underwent cesarean section delivery using epidural anesthesia at 36 weeks gestational age. The infant was a viable female, weight 2912 g, APGAR 8/9. She was managed with intravenous diuresis and discharged after 4 days with aspirin and lasix therapy. At 6 weeks postpartum, she continued to have fatigue with exertion and her peak RV-PA conduit gradient was 65 mm Hg. At 7 months postpartum, she underwent successful replacement of her RV-PA conduit with a 25 mm pulmonary homograft. Discussion
Pregnant women with CHD have a 10% risk of adverse maternal outcomes, and 4% risk of adverse neonatal outcomes.3 The maternal mortality rate of 0.15% for these women is significantly elevated compared with their peers without CHD.2 Additionally, women with complex CHD have over twice the risk of adverse outcomes compared with women with simple CHD.2 Multidisciplinary team management has been utilized successfully in other chronic conditions, such as adult CHF. A team approach that involved follow-up with collaborative team (nursing, cardiology, and primary care) has been shown to reduce mortality and CHF-related hospitalizations.4 In addition, adherence to medical therapy and quality of life were also improved in CHF patients who were part of a multidisciplinary protocol.5 The Table 1.
team structure involved collaboration between nursing and physicians to ensure adequate followup, coordination of visits, and a detailed medical algorhythm. Following these examples, we present three cases which illustrate the use of a multidisciplinary team (adult congenital cardiology, obstetrics, neonatology, anesthesia, and nursing) in managing high-risk patients with CHD through pregnancy. Following is an in-depth discussion of the components of the plan with each specialty’s concerns and input on management. Patient Selection for Multidisciplinary Management
There are several scoring systems available to risk stratify pregnant women with CHD. Risk factors identified by the CARdiac disease in PREGnancy (CARPREG) investigators and ZAHARA investigators are summarized in Table 1.6,7 Patients with one or more risk factors and those with moderate or severely complex CHD had a multidisciplinary team meeting to discuss management.8 Close contact was maintained between the high-risk obstetrical team and the adult congenital cardiology team for the duration of the pregnancy. Multidisciplinary meetings are usually held between 20 and 24 weeks, as the fetus approached viability and maternal complications during the pregnancy can be factored into the management plan. Discussions in cases 2 and 3 were delayed due to patient choice of management team and time of presentation, respectively. Patients were invited to the meeting to allow the opportunity to ask questions to the group. Mode of Delivery
Initial discussions began with time of delivery (EGA) and mode of delivery. Sample plans are included for both induced vaginal delivery (Table 2) and elective cesarean section (Table 3). Contingency plans were also discussed in case of fetal or maternal distress. Delivery between 37 and 40 weeks is usually planned, provided fetal and maternal stability. In
Risk Stratification Methods
Risk factors
CARPREG4*
ZAHARA5
Prior cardiac event or arrhythmia Baseline NYHA class >II or cyanosis Left heart obstruction Systemic ventricular ejection fraction 1 predictor have a risk of 67%. †In the setting of moderately complex congenital heart disease. AV, atrioventricular valve; CARPREG, CARdiac disease in PREGnancy; CHD, congenital heart disease; NYHA, New York Heart Association.
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Management of Pregnancy in Congenital Heart Disease Table 2.
Template for Pregnancy Care Plans, Induced Vaginal Delivery Description
Sample Plan
Scheduled Vaginal Delivery Induction Hospital Admission Determine timing and workup at admission
Hemodynamic Monitoring Cardiology
Anesthesia
Determine type of cardiac monitoring, location of monitoring, type of staffing. Cardiology Consult; Anticipated medical management during and post delivery Type of pain control and any special considerations
1. 2. 3. 1. 2. 1. 2. 3. 1. In 2. 3.
Rate of fluid administration based on cardiac lesion (ie, IVF at 100 cc/hr, IV TKVO, or no restriction) Recommendations for endocarditis prophylaxis.
4. 1. 2. 1.
Second Stage of Labor
Specify agents to be used for induction and location of delivery Specify if assisted or nonassisted.
2. 1. 2. 1.
Post-partum
Location of recovery for baby and mother.
Fluid Management Endocarditis Prophylaxis
Induction
Spontaneous Labor Prior to Induction General management Same as above Hemodynamic Monitoring Same as above Arrest of Labor; Failure to Progress Attempt to further induce Additional agents to use Cesarean Section Location of surgery and type of support
1. 2. 2.
Admit EGA 39 weeks Type and cross Hold heparin anticoagulation, PT/PTT. CVP and arterial line placement Return to L&D for monitoring by CCU nursing. ACHD consult on admit Lasix IV x 1 post delivery Antiarrhythmic medications to have on hold Epidural placement case of heparin use: If PTT elevated, Fentanyl PCA until PTT normal, then epidural. If PTT normal, will place epidural for pain control; slow epidural dosing with no boluses. Protamine available if indicated. IVF at 100 cc/hr Foley placement, ins & outs monitoring. At second stage of labor: Ampicillin 2 g IV, and Gentamicin 1.5 mg/kg IV. Six hours later: additional Ampicillin 1 gm. Oxytocin, AROM when indicated. Delivery in L&D OR. Assisted with vacuum or forceps to minimize maternal stress Administer RhoGAM. Baby to newborn nursery. Transfer to CCU for further monitoring for 24 hours for cardiac care and diuresis.
Same as above Anesthesia will place CVP and arterial line; CCU nurse for monitoring Utilize uterotonic agents: pitocin, cytotec 1. Main OR or Cardiac OR/cardiac anesthesia depending on maternal stability 2. NICU support
Fetal Distress Cesarean Section
Location and support needed
L&D OR, with NICU support
Maternal Distress Cesarean Section
Location of surgery and type of support
Main or Cardiac OR depending on maternal state; NICU and cardiac anesthesia support
EGA, estimated gestational age; PT/PTT, prothrombin time/partial thromboplastin time; CVP, central venous pressure; L&D, labor and delivery; CCU, cardiac care unit; ACHD, adult congenital heart disease; IVF, intravenous fluids; IV, intravenous; TKVO, to keep vein open; AROM, artificial rupture of membranes; OR, operating room; NICU, neonatal intensive care unit.
most cases a vaginal delivery is preferred as it has been associated with smaller shifts in blood volume, fewer clotting or bleeding complications, and lower risk of infection.9 In some cases, vaginalassisted delivery methods, including vacuum devices and forceps, are recommended to decrease maternal strain.10,11 Certain positioning such as left lateral decubitus can minimize fluctuations in cardiac output by eliminating uterine pressure upon the inferior vena cava (IVC).10,12 Due to the increased risks of fluid shifts and blood loss, cesarean delivery is usually recommended to be reserved for cases of maternal hemodynamic deterioration, fetal distress, obstet-
rical indications, severe obstructive lesions, severe pulmonary hypertension, or an unstable aorta (≥40 mm in Marfan syndrome).9–11 Elective cesarean section may also be utilized for patients on warfarin anticoagulation for artificial heart valves to minimize the time off of anticoagulation. In case 1, the patient was at higher risk for bleeding due to prior abdominal surgery and allergies to reversal agents; therefore, the decision was made to attempt an induced vaginal delivery initially. Due to fetal compromise and maternal condition, as also occurred in case 3, delivery was via elective cesarean instead. For case 2, cesarean section was Congenit Heart Dis. 2014;9:E204–E211
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Harris et al. Pregnancy Care Plans, Scheduled Cesarean Section Description
Sample Plan
Scheduled Cesarean Section Hospital Admission Determine timing and workup at admission Hemodynamic Monitoring Cardiology
Determine type of cardiac monitoring, location of monitoring, type of staffing. Cardiology Consult; Anticipated medical management during and post delivery
Anesthesia Fluid Management
Type of pain control and any special considerations Rate of fluid administration based on cardiac lesion (ie, IVF at 100 cc/hr, IV TKVO, or no restriction)
Endocarditis Prophylaxis Post-partum
Recommendations for endocarditis prophylaxis. Location of recovery for baby and mother.
1. Admit evening before EGA 37 weeks, type & screen, make NPO at midnight. 1. Arterial line placement prior to Cesarean Section 1. ACHD Consult on admit 2. Recovery with telemetry monitoring on Cardiac floor post delivery 1. Epidural anesthesia for cesarean section. 1. IVF at 100 cc/hr of section, Foley placement, hourly ins & outs monitoring. Blood products on call. 1. No prophylaxis needed 1. Transfer mother to cardiac floor for further monitoring/diuresis for 24 hours. 2. Baby to visit mother on cardiac unit
Spontaneous Labor Prior to Scheduled Cesarean section General management Same as above Hemodynamic Monitoring Same as above
Same as above Same as above
Fetal Distress Cesarean Section
Location of surgery and support needed
L&D OR, with cardiac anesthesia support
Maternal Distress Cesarean Section
Location of surgery and support needed
Cardiac or Main OR; NICU, Cardiology, and Cardiac Anesthesia support
EGA, estimated gestational age; NPO, nothing by mouth; ACHD, adult congenital heart disease; IVF, intravenous fluids; IV, intravenous; TKVO, to keep vein open; L&D, labor and delivery; OR, operating room; NICU, neonatal intensive care unit.
initially scheduled due to the severity of the patient’s prior perineal tear. Cardiac Management
Women with CHD of moderate or greater complexity should ideally be followed at a congenital heart center. Optimally, counseling regarding risks of pregnancy and potential risks to the fetus should be discussed prior to undertaking a pregnancy. Careful review of maternal medications should be performed, with discontinuation and substitution of medications not approved for use in pregnancy. In case 1, the patient had been on mexilitine for a remote history of ventricular tachycardia. This was discontinued, but after weighing the risks and benefits, the nadolol was continued. Offspring of women with CHD are at risk for their own CHD, with an average rate of 3–5%.13,14 Certain anomalies and some associated genetic syndromes such as 22q11.2 deletion syndrome carry a much higher risk of recurrence.15 Therefore, genetic counseling should be offered to these patients prior to the pregnancy so that individual risk can be determined. Fetal echocardiography at 18–22 weeks is recommended to allow for identification of and treatment planning for any structural abnormalities.13 Congenit Heart Dis. 2014;9:E204–E211
During labor and delivery, augmented cardiac contractility and heart rate can increase cardiac output and myocardial oxygen demand. When combined with acutely increasing pulmonary artery pressures due to acidosis and hypercarbia, these hemodynamic factors may result in heart failure if not recognized.9 Given these factors, fluid administration was discussed as part of the care plan and recommendations were individualized for each patient. The type of cardiac monitoring during labor and delivery depends on the underlying cardiac disease and maternal condition. For case 1, a central venous pressure line and an arterial line were placed due to the patient’s CHF, ventricular arrhythmias, and concern for worsening during the labor and delivery process. Most patients do not require aggressive monitoring, but if there are concerns of rapidly worsening maternal status due to poor cardiac function, arrhythmias, pulmonary hypertension, or severe obstruction, more intensive monitoring should be considered. Following delivery, cardiac output increases again from the release of pressure upon IVC and autotransfusion from the contracted uterus of up to half a liter.10,12,16 Because of the extensive fluid shifts postpartum, patients may need to be observed in a cardiac unit after delivery depending
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Management of Pregnancy in Congenital Heart Disease on their heart disease.12,16 Recommendations for the above cases were for telemetry monitoring following delivery. All presented cases spent 1–4 days on a cardiac unit for heart failure management prior to being transferred to the postpartum unit. All patients required immediate intravenous diuretic therapy after delivery. Case 1 had the added complication of two mechanical valve prostheses. Maintaining adequate anticoagulation presents a challenge during pregnancy due to increased prothrombotic and decreased fibrinolytic activity.17 The maternal mortality rate due to prosthetic valve thrombosis during pregnancy is approximately 1–4%, and careful coordination between teams is necessary to manage anticoagulation during the labor and delivery process.12 During pregnancy and periods of CHF, anticoagulation levels can be quite variable and need to be closely monitored. Recommendations for starting and stopping anticoagulation were determined at the time of the multidisciplinary meeting and written into the care plan.
Endocarditis prophylaxis at the onset of labor is recommended for women with evidence of infection or in whom a complicated delivery course cannot be excluded, as well as women with mechanical heart valves or conduits.10,18,19 In case 1, the patient had mechanical heart valves, and in cases 2 and 3, the patients had valved conduits.
pulmonary vascular resistance. Additionally, there can be a large increase in pulmonary arterial pressure during laryngoscopy and intubation, further reasons to avoid general anesthesia.9 Extensive monitoring by the anesthesia team is another crucial component in the care of the pregnant woman with CHD. Some recommended forms of monitoring for women with complex CHD include: arterial O2 saturation, cardiac rhythm, blood pressure, central venous, and arterial pressure.9,20,21 Recommendations for the type of monitoring were made by both cardiology and anesthesia at the time of the meeting as discussed previously. In cases where maternal decompensation is a concern, cesarean section is preferably performed in the main operating room, where the expertise of a cardiac anesthesia team is readily available. Many maternal morbidities occur peripartum, due to the large hemodynamic fluctuations during delivery, and close monitoring should be continued. Anticoagulation with heparin may be safely continued throughout pregnancy up until 12 hours before induction to allow partial reversal for epidural placement and delivery. If the patient presents with bleeding while on heparin, reversal with protamine sulfate is recommended.22 This easy reversal with protamine is part of the reason heparin was chosen for case 1, as the patient would not be able to be quickly reversed if she were on warfarin due to her allergy to both vitamin K and plasma blood products.
Anesthesia and Cardiac Monitoring during Delivery
Fetal and Neonatal Considerations
Choice, route, and timing of anesthesia during delivery for women with CHD are important considerations for the anesthesia team. Due to release of catecholamines, pain and uterine contractions lead to tachycardia, hypertension, and overall increased cardiac output.10,20 Because this extra stress may result in arrhythmias, ischemia, or heart failure, care must be taken to avoid this through the use of effective and early anesthesia.16,20 Because it carries less cardiovascular risks, neuraxial anesthesia (epidural) is the primary anesthesia of choice for both cesarean sections and vaginal deliveries.20 The epidural is slowly titrated to maintain systemic vascular resistance (SVR) or avoid a sudden drop in SVR. Especially for women with pulmonary artery hypertension, general anesthesia during pregnancy increases their risk of death fourfold due to a depression in cardiac contractility by the volatile agents and increases in
Pregnant women with CHD have approximately a 12–16% risk of premature birth.3,7 Similarly, women with complex CHD have higher rates of small for gestational age children.3,7 The CARPREG study identified maternal NYHA class, cyanosis, left heart obstructive lesions, smoking, multiple gestations, and anticoagulation as risk factors for neonatal complications.6,7 Further study showed that maternal risk factors for neonatal complications included: at birth cyanotic disease, the use of cardiac medications, and mechanical valve prosthesis.7,13 Through the pregnancy, the fetus must be monitored for intrauterine growth retardation, as chronic maternal hypoxemia and uteroplacental insufficiency may lead to fetal compromise.3,13,14,16 During delivery, both the mother and the fetus should be monitored. Signs of fetal compromise such as a decrease in the heart rate variability,
Endocarditis Prophylaxis
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Nursing
Nursing staff from all of the above disciplines also attended these multidisciplinary meetings and were critical in instituting the recommended care plans. When necessary, cardiac nurses monitor the patient while on the obstetrical service prior to delivery. As all of these reported cases needed at least 24-hour recovery on a cardiac unit, obstetrical nurses provided additional visits and expertise to the cardiac nursing staff. Nurses from the neonatology division facilitated visits to the mother’s bedside in the cardiac care unit so that adequate bonding time between mother and baby could be established. Nursing staff from the adult congenital program distributed the care plan to all on-call doctors and services involved. Additionally, they communicated with the patients on a regular basis, especially as the due date approached. Discussions included follow-up of visits, lab testing and results, medication changes as ordered by attending, and assessing the patient’s status. Nurses also communicated with the specialists involved and provided updates and reports as needed. Finally, in the first 2 months following discharge, nursing staff continued to correspond with the patient regularly to assess her condition and help coordinate appointments.
General Considerations and Conclusion
Obstetric management of the patient with a congenital heart anomaly requires early onset of care and regular follow-up. Ideally, all women would establish care with an adult congenital heart specialist and obstetrician prior to conception. Preconception and genetic counseling is useful to discuss risks of pregnancy to the mother and the fetus. The initial consultation should include a detailed history and physical exam, and review of cardiac reports and medications. All patients require a fetal echocardiogram. In high-risk cases, patients should deliver at institutions with a labor and delivery unit, neonatal intensive care unit, and a cardiac intensive care unit with specialists in CHD. Risks for subsequent pregnancies should Congenit Heart Dis. 2014;9:E204–E211
Harris et al. be discussed with the patient and contraception recommendations should be offered prior to discharge. Women with CHD represent a high-risk population of patients to care for during pregnancy. Input from multiple subspecialties is needed for individualized coordination of care to ensure optimal care for both mother and infant. Author Contributions Rachel C. Harris: Concept/design, data collection, data analysis/interpretation, drafting article, critical revision of article. Melissa H. Fries/Nancy Klein/Hassan Adeniji-Adele/ Zacharia Cherian/Annelee Boyle: Critical revision of article, approval of article. Anitha S. John: Concept/Design, data collection, data analysis/interpretation, drafting article, critical revision of article, approval of article.
Corresponding Author: Anitha S. John, MD, PhD, Division of Pediatric Cardiology, Children’s National Medical Center, 111 Michigan Avenue, NW, Washington, DC 20010, USA. Tel: 202-476-3543; Fax: 202-4765700; E-mail: [email protected] Conflict of interest: None, self-funded. No relationships with industry. Disclosures of grants or other funding: None, self-funded. Accepted in final form: December 9, 2013.
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