Abstract
Cardiovascular disease is a leading cause of morbidity and mortality for pregnant patients. A significant portion of cardiac morbidity and mortality is preventable and related to poor or delayed recognition of clinical warning signs and oversights in management. The establishment of pregnancy heart teams facilitates multidisciplinary planning to improve management of people with cardiovascular disease. Core members of the pregnancy heart team include obstetricians, midwives, cardiologists, anesthesiologists, nurses, and patient care coordinators. Additional specialists and subspecialists are included on pregnancy heart teams based on individual patient needs. This review discusses the practical aspects of the pregnancy heart team’s approach to caring for pregnant people with cardiovascular disease.
1
Cardiovascular disease in pregnancy
Cardiovascular disease (CVD) is increasingly prevalent in pregnancy with data from the United Kingdom (UK), the United States (US), and Australia demonstrating that it is a leading cause of maternal morbidity and mortality [ ]. Racial disparities in pregnancy-related outcomes may also be exacerbated by CVD [ ]. Factors contributing to increased CVD among pregnant people include survival of patients with congenital heart disease to reproductive age, increasing prevalence of acquired CVD, and increasing coexistence of risk factors for CVD such as diabetes, obesity, and advanced maternal age [ ]. The complexity of the management of CVD in pregnancy has led to the emergence of cardio-obstetrics as a subspecialty and the establishment of pregnancy heart teams (PHTs).
The PHT is a multidisciplinary group that individualizes management plans for pregnant patients with CVD from preconception through pregnancy and postpartum. Multidisciplinary collaboration of the PHT is essential for safer management of pregnant patients with CVD. The purpose of this review is to discuss the different resources and personnel needed to create a PHT, and to discuss how the PHT approaches birth planning for pregnant people with CVD. Discrepancies in resources exist both between and within many countries and their health systems. The ability to adhere to recommendations may differ due to varying personnel and resource availability.
2
Resources
Comprehensive care for pregnant patients with CVD requires specific resources at the regional, hospital, and personnel level.
2.1
Regional resources
The California Maternal Quality Care Collaborative (CMQCC) is one of the best examples of how regional support can improve the care for pregnant patients with CVD [ ]. The CMQCC is a collective of state and federal government organizations, non-governmental organizations (NGOs), academic institutions, and insurance companies that was started in 2006 to address the increasing rate of maternal morbidity and mortality in California [ , ]. Since CVD is a leading cause of maternal mortality, the CMQCC developed infrastructure to carry out research and quality improvement initiatives and develop a toolkit detailing best practices for the management of CVD in pregnancy [ ]. Additionally, the CMQCC infrastructure provided education to improve provider recognition of symptoms of CVD in pregnancy. With the guidance of the CMQCC, maternal mortality in California decreased by 65% from 17/100,00 to 6/100,000 live births between 2006 and 2016 [ ]. While pregnancy-related mortality due to hypertensive disorders of pregnancy decreased in 2017–2019 in California, CVD remained the leading cause of pregnancy-related deaths [ ].
To replicate California’s focus on maternal morbidity and mortality, other states in the US have started perinatal quality collaboratives (PQCs) [ ]. In the US , funding for PQCs comes from federal and state governments, insurance companies, academic institutions, and NGOs. In other countries, the creation of and funding for similar programs would likely reflect the local healthcare system’s structure. The goal of regional support is to provide infrastructure to perform data collection, research, and quality improvement and improve maternal outcomes.
2.2
Hospital-level resources
Hospitals and medical centers where people with CVD give birth provide pivotal resources for safer care. Pregnant people with CVD may require diagnostic tests, monitoring, equipment, and specialized services such as echocardiography, cardiopulmonary bypass (CPB), and high acuity units (e.g. intensive care units, coronary care units, critical care units, or high dependency units). An essential step in caring for people with CVD is ensuring that the hospitals where patients deliver have the appropriate resources. Hospital classification systems such as the American College of Obstetrics and Gynecology’s (ACOG) Maternal Levels of Care or Australia’s Maternity and Neonatal Service Capability delineate what resources are available at specific hospitals by their designation [ , ]. Maternal hospital classification systems help providers determine whether they have adequate support at their hospital, or if a patient requires transfer to a higher level of care center [ ]. If not already present, governments and health systems should develop standardized hospital classification systems.
When patients do require transfer to a higher level of care it is important to have designated referral pathways to facilitate moving patients between hospitals, including in the emergency setting [ , ]. Medical transport, provider familiarity with regional hospitals, and transfer coordinators that facilitate transport logistics and between-hospital communication are important for transfer between facilities to occur with minimal barriers or delays.
2.3
Personnel: the pregnancy heart team
Managing pregnant patients with CVD requires a multidisciplinary team. The Cardiac Disease in Pregnancy (CARPREG) study followed pregnant patients with heart disease and demonstrated that “almost 50% of severe cardiac events were preventable, and most of these preventable events were due to provider management failures” [ ]. The PHT’s objective is to improve outcomes and decrease preventable cardiac morbidity and mortality by combining multidisciplinary expertise.
The PHT allows input from the different specialties involved in care of pregnant patients with CVD to provide comprehensive care for these complex patients. There are some specialties that are routinely represented whereas others may only be included for certain types of cardiac lesions. The core members of the PHT should include the pregnant person along with obstetric, midwifery, cardiology, anesthesiology, nursing, and patient care coordination representatives. Additional specialists such as cardiothoracic surgeons, hematologists, pulmonologists, intensivists, pharmacists, and others may be required on the PHT as needed ( Fig. 1 ).
2.3.1
Core members
2.3.1.1
Pregnant person
The pregnant person is the center and focus of the PHT. It is essential that the members of the PHT listen to patients. Decisions must consider the patient’s preferences and must be discussed with the patient using preferred language and appropriate level of medical literacy. Patients must be thoughtfully informed about their risks and the importance of reporting changes in their wellbeing or usual state of health.
2.3.1.2
Obstetricians
As physicians with longitudinal relationships with patients and expertise in the anatomy and physiology of pregnancy, obstetricians are well suited to coordinate care during pregnancy. Pregnancies in people with complex CVD with risks or involvement of the fetus and other comorbidities can benefit from consultations with subspecialists in maternal fetal medicine (MFM) and obstetric medicine.
2.3.1.3
Midwives
Midwives may collaborate with the high-risk providers and provide primary obstetric or labor care in consultation with other members of the pregnancy heart team.
2.3.1.4
Cardiologists
Cardiologists guide diagnosis and management of CVD. When possible, an obstetric cardiology subspecialist should be part of the PHT as they have experience in the nuanced management of CVD in pregnancy. Furthermore, management of patients with congenital CVD is improved with representation from congenital subspecialists since the longitudinal relationships that they have with their patients offer continuity of care. Other cardiology subspecialists such as electrophysiologists, interventional cardiologists, and heart failure specialists can be included on the PHT as needed.
2.3.1.5
Anesthesiologists
The PHT benefits from an anesthesiologist with expertise in obstetric anesthesiology. An additional (or dual-trained) anesthesiologist with cardiothoracic or intensive care anesthesiology training may be needed when specialized cardiac monitoring or interventions such as perioperative transesophageal echocardiography (TEE) or mechanical circulatory support ( MCS ) are required. When cardiothoracic or intensive care anesthesiologist are required, they can work as a team with the obstetric anesthesiologist to provide further expertise. For example, the obstetric anesthesiologist may optimize neuraxial techniques, maternal physiology, uterotonic selection, postpartum hemorrhage management, and obstetric crisis situations, while the cardiothoracic or intensive care anesthesiologist performs and interprets TEE along with refined vasoactive therapy regimens and cardiopulmonary support.
2.3.1.6
Nurses
Nurses are frequently patients’ primary point of contact during hospitalization. Labor nurses are of particular importance as they have the unique skills to care for pregnant patients during the peripartum and postpartum periods, and they are usually the first providers to detect changes in maternal or fetal status because of their longitudinal contact. Care for cardio-obstetric patients can also require additional specialized nursing including cardiac and intensive care nursing.
2.3.1.7
Patient care coordinators
Pregnant patients with complex cardiac disease may require substantial care coordination particularly when significant travel and/or financial resources are required for care delivery. Care coordinators that can help with the logistics of healthcare delivery are also valuable members of the PHT.
2.3.2
Individual specialists and subspecialists
2.3.2.1
Cardiothoracic surgeons
When patients with cardiac lesions such as severe valve disease may require surgical intervention, cardiac surgeons can help with the risk and benefit discussion, surgical planning, and postoperative management. Cardiothoracic surgeons should also be involved for logistical discussions when extracorporeal membrane oxygenation (ECMO) or other forms of MCS are under consideration.
2.3.2.2
Hematologists
Many people with CVD require antithrombotic medications (e.g. anticoagulation and antiplatelet medications). Hematologists’ expertise can help manage antithrombotic regimens and coordinate initiation and cessation of medications to facilitate labor, delivery, surgery, and neuraxial anesthesia. Choice and timing of antithrombotic regimens must consider thrombosis risk, teratogenicity risk, labor induction plan, and delivery timing.
2.3.2.3
Pulmonologists
Cardiac and pulmonary physiology are interdependent, and consequently lung pathology can affect cardiac pathology and vice versa. Having a pulmonologist on the PHT allows for optimization of a pregnant person’s pulmonary status if needed.
2.3.2.4
Intensivists
When patients with cardiac disease decompensate or have a high risk of decompensating, they may require intensive care. High acuity units provide increased monitoring and life sustaining measures such as mechanical ventilation, MCS, renal replacement therapy, and administration of vasoactive medications. It is valuable to have intensivist with expertise in obstetric critical care on the PHT because altered pregnancy anatomy and obstetric complications require specialized treatment and planning.
2.3.2.5
Neonatologists
Neonatologists may provide gestational-age expectations for the family if preterm birth is planned or anticipated or if the fetus has comorbidities.
2.3.2.6
Pharmacists
When the management of the cardio-obstetric patient requires complex pharmacological intervention, it is helpful to have the expertise of a pharmacist on the PHT. Pregnancy alters the pharmacokinetics and pharmacodynamics of many medications, and the effect and safety of various medications on the fetus and during breastfeeding must also be considered.
2.3.2.7
Additional specialists and subspecialists
Consultation with additional specialists and subspecialists including mental health providers and social workers may be required based on patients’ additional comorbidities and needs.
2.3.3
Team characteristics
Characteristics of an effective PHT include a safe and respectful environment and a culture of preparedness. Management of pregnant patients with CVD may not be straightforward, and there are limited data and research to guide decision making. Consequently, management decisions are often based on expert consensus. A safe and respectful environment facilitates members of PHT sharing expertise, feedback, and concerns. As a result, multidisciplinary collaboration is nurtured, and optimal management is produced.
Proper planning and effective communication are also essential components of a successful PHT. Management of complex patients requires that all members of the PHT receive and share information that can be nuanced and dynamic. Communication and planning by the PHT ensure that all resources are in place and contingency plans exist for anticipated emergency situations. It is recommended that PHTs participate in multidisciplinary simulation training to improve management of emergency situations. Simulation training can identify knowledge and skills gaps, and confirm that resources, workflows, and delegation of roles and tasks are seamlessly deployed in emergencies [ , ]. Furthermore, use of cognitive aids can verify that all appropriate steps are completed [ , ].
3
Pregnancy planning with the multidisciplinary pregnancy heart team
Ideally, the PHT follows pregnant patients from preconception through the postpartum period. The frequency with which the PHT meets and discusses each patient depends on the number of patients being followed, the severity of patients’ CVD, and the availability of the members of the PHT. Due to the evolving nature of pregnancy hemodynamics, it is helpful for a PHT to meet at least once per month so that new patients can be discussed and any changes to the management plans for existing patients can be communicated. PHTs can develop templates that can help streamline planning such as shown in Fig. 2 .
3.1
Preconception
Whether pregnancy is desired or not, preconception counseling should be prioritized for all patients with CVD that have reproductive capability [ ]. Preconception counseling relies on an established relationship with healthcare providers and consists of determining if or when the patient desires pregnancy, educating the patient on the implications of pregnancy in the context of their CVD, and discussing contraceptive options. If pregnancy is not desired or is contraindicated, it is essential to establish contraception. Choice of contraception can be tailored to optimize benefit while minimizing CV risk. For example, combined hormonal contraceptives may impart increased risk of thromboembolic complications and lipid abnormalities, while increased menstrual bleeding may occur in patients taking antithrombotic mediations with nonhormonal copper intrauterine devices or subdermal implants [ ].
Patients with CVD who desire pregnancy benefit from counseling to establish a plan. Preconception risk stratification is essential to determine likelihood of complications and need for resources. There are currently multiple risk stratification tools for cardiac disease in pregnancy including the modified World Health Organization (mWHO) Classification of CVD in Pregnancy, Cardiac Disease in Pregnancy Study (CARPREG II), and Zwangerschap bij Aangeboren HARtAfwijkingen (ZAHARA) tools [ ]. The mWHO classification is a validated, lesion-specific risk stratification tool that divides different cardiac lesions into five groups based on risk of cardiac events. The mWHO classification facilitates planning with its framework which can be used to determine resources required by subgroup ( Table 1 ) [ ]. The CARPREG II score predicts a patient’s individual risk of cardiac events based on risk factors such as use of anticoagulation, presence of a mechanical valve, or ventricular systolic dysfunction [ ]. The CARPREG II score incorporates features such as coronary artery disease which are not included in the mWHO classification in its risk stratification. When applicable, we suggest combining the mWHO and CARPREG II score so that risk stratification can incorporate both the individual’s specific lesion and risk factors.
| mWHO I | mWHO II | mWHO II-III | mWHO III | mWHO IV | |
|---|---|---|---|---|---|
| Diagnosis∗ | Small or mild
| Unoperated atrial or ventricular septal defect | Mild left ventricular impairment (EF >45%) | Moderate left ventricular impairment (EF 30–45%) | Pulmonary arterial hypertension |
| Successfully repaired simple lesions (atrial or ventricular septal defect, patent ductus arteriosus, anomalous pulmonary venous drainage) | Repaired Tetralogy of Fallot | Hypertrophic cardiomyopathy | Previous peripartum cardiomyopathy without any residual left ventricular impairment | Severe systemic ventricular dysfunction (ejection fraction <30%) | |
| Atrial or ventricular ectopic beats, isolated | Most arrhythmias (supraventricular arrhythmias) | Native or tissue valve disease not considered WHO I or IV (mild mitral stenosis, moderate aortic stenosis) | Mechanical valve | Aortic dilation >45 mm in Marfan syndrome or other HTAD, >50 mm in bicuspid aortic valve, Turner syndrome ASI >25 mm/m 2 , tetralogy of Fallot >50 mm | |
| Turner syndrome without aortic dilatation | Marfan syndrome or other HTAD without aortic dilatation | Systemic right ventricle with good or mildly decreased ventricular function | Vascular Ehlers–Danlos | ||
| Fontan circulation. If otherwise the patient is well and the cardiac condition uncomplicated | Severe re-coarctation of aorta | ||||
| Unrepaired cyanotic heart disease | Fontan with any complication | ||||
| Other complex heart disease | |||||
| Moderate mitral stenosis | |||||
| Severe asymptomatic aortic stenosis | |||||
| Moderate aortic dilatation (40–45 mm in Marfan syndrome or other HTAD; 45–50 mm in bicuspid aortic valve, Turner syndrome ASI 20–25 mm/m 2 , tetralogy of Fallot <50 mm) | |||||
| Ventricular tachycardia | |||||
| Risk | No increased risk of maternal mortality and no/mild increased risk in morbidity | Small increased risk of maternal mortality or moderate increase in morbidity | Intermediate increased risk of maternal mortality or moderate to severe increase in morbidity | Significantly increased risk of maternal mortality or severe morbidity | Extremely high risk of maternal mortality or severe morbidity |
| Maternal Cardiac Event Rate # | 2.5–5% | 5.7–10.5% | 10–19% | 19–27% | 40–100% |
| Pregnancy Heart Team Involvement @ | Referral to PHT as needed | Core PHT readily available | Core PHT physically present at all times. Additional members readily available at all times | Core PHT physically present and additional members readily available at all times | Core PHT physically present and additional members immediately or readily available as needed |
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