The high-risk birthing population includes the pregnant cardiac patient at risk for maternal mortality because of altered cardiac anatomy and physiology or concurrent cardiovascular comorbidities. Intensive medical management and surveillance, coordination of multidisciplinary care, and referral to tertiary or quaternary levels of care can create health system challenges in caring for the pregnant cardiac patient.1 At Einstein/Montefiore, we established our maternal-fetal medicine (MFM) Cardiology Joint Program in February, 2015, in response to the devastating statistics reported in January, 2015, Pregnancy associated deaths in the United States on the rise, majority due to cardiovascular disease in pregnancy.2 We acknowledged that there was a need for our rising pregnant patients with known heart disease and/or suspected cardiac pathology. The aim was to establish a multidisciplinary program to optimize the care of high-risk pregnant patients with known or suspected cardiac disease because there is a real potential for communication gaps when patients are seen separately in contrast with parallel visits by different specialists.
We were successful with co-departmental support from both Obstetrics and Gynecology and Women’s Health and Cardiology. We structured a multidisciplinary office in the cardiology outpatient patient care rooms with obstetric examination facilities, same day maternal electrocardiogram, echocardiogram and other vascular testing. Through dialog in the same room with Maternal Fetal Medicine and Cardiology specialists, management in pregnancy and peripartum were fully discussed. Obstetric Anesthesia consultations are performed in the same establishment along with laboratory studies. Referrals of select patients with known or suspected cardiac disease who are pregnant, planning a pregnancy or who had a complicated postpartum course attended our outpatient setting for consultation. Referring providers included Ob/Gyn, Cardiology and Primary Care providers. As a multidisciplinary team, the highest risk patients were identified. Our program is unique because 2 subspecialists interview and examine the patient together simultaneously. We then advance our understanding of the pregnant cardiac patient, appreciate each other’s concerns and provide a platform for student, resident and fellow education and role modeling.
The inpatient arm of our program consists of monthly meetings in our cardiac intensive care unit conference room. Attendants include nursing from labor and delivery and cardiac intensive care unit, Obstetric Anesthesia, Maternal Fetal Medicine, Cardiology, Neonatology (Fig. 1). We utilize a checklist (Fig. 2) to discuss our highest risk patients to delineate delivery plans both intrapartum and postpartum. We further assess and stratify their morbidity and mortality risk during delivery as low, moderate or high based on their disease and comorbidities. We review common medications administered on the labor floor and think through how the patient’s cardiac pathology will respond. We review their cardiac imaging to have a full understanding of the patient’s disease and think through how we will administer regional anesthesia and furthermore, how to monitor in the immediate postpartum period. This model aligns with the European Society of Cardiology latest recommendations of 2018.3
Notably, the majority of our high-risk patients are undiagnosed before pregnancy, and therefore, there is an urgent need for a timely multidisciplinary delivery plan (Fig. 3). The MFM Cardiology visit for some was the first foray into cardiology after a hiatus or ever, representing a pivotal opportunity to engage and retain women who need lifelong cardiac care. A summary of our new consults and their type of cardiac disease over the first 5 years is summarized in Table 1.
TABLE 1 -
The Maternal-Fetal Medicine Cardiology Joint Program: 2015-2019
||Frequency 2015 (N=61) [n (%)]
||Frequency 2016 (N=75) [n (%)]
||Frequency 2017 (N=101) [n (%)]
||Frequency 2018 (N=110) [n (%)]
||Frequency 2019 (N=147) [n (%)]
||Syncope, palpitations, murmur, shortness of breath
||TOF, Marfan, VSD, Ebstein, subaortic, AV canal defect, uncorrected PDA
||Prior peripartum cardiomyopathy, new diagnosis, heart transplant
||Brugada, WPW, prolonged QT, heart block, SVT
||Rheumatic, septic infection
||CHTN, preeclampsia, PP eclampsia, prior PE, carotid disease, MI, pulmonary HTN, SLE
Albert Einstein College of Medicine, Bronx, NY.
ACHD indicates adult congenital heart disease; AV, atrioventricular; CHTN, chronic hypertension; HTN, hypertension; MI, myocardial infarction; PDA, patent ductus arteriosus; PE, pulmonary embolism; PP, postpartum; SLE, systemic lupus erythematosus; SVT, supraventricular tachycardia; TOF, tetralogy of fallot; VSD, ventricular septal defect; WPW, wolff parkinson white syndrome.
Here is a report on our 5-year experience and 5 key points we have found essential for care of the pregnant cardiac patient. Of note, chapters to follow will detail some of these key points in further depth.
Cardiac Risk Assessment for Maternal Mortality Should Begin Preconception, Particularly for Adult Congenital Heart Disease Patients
There is great opportunity for cardiologists to refer all women of childbearing age with high-risk cardiac disease to their Ob-Gyn colleagues for preconception counseling, including the implementation of a contraceptive plan. Cardiologists should be aware that their female patients have potential for a high-risk pregnancy and anticipate that their patient may need guidance regarding family planning. Cardiologists must be aware of physiological changes in pregnancy and the highest risk lesions; namely, Marfan syndrome and other connective tissue disorders, that is, Loews Dietz, with aortopathy and other high-risk aortopathies, severe pulmonary hypertension [World Health Organization (WHO) group I], mechanical valves, peripartum cardiomyopathy (PPCM) with residual left ventricular dysfunction and acute coronary syndromes during pregnancy.4 There are at least 4 predictive models, CARPREG I, CARPREG II, ZAHARA, and WHO which are used to score mortality risk and can be employed by the cardiologist to quantify patients’ risk. We recommend using these risk estimates for guidance when appropriate, but ultimately risk assessment is based on multidisciplinary clinical judgment, as some women with understudied phenotypes might not be captured by a 1-time score. Similarly, previously healthy women may present with new onset PPCM after delivery, disease not predicted by the available scoring systems.
Cardiologists may be counseling high-risk women that they should avoid pregnancy, particularly on certain medications (statins, angiotensin converting enzyme inhibitors, warfarin) but implementing a contraception plan is likely beyond the scope of their usual practice. This is where comanagement can be impactful. Family planning services should be accessible to high-risk women as they will likely use >1 contraceptive method over the course of their reproductive life and require serial evaluation. There may be misconceptions that physicians and patients have about contraceptive choices for high-risk women and the Ob-Gyn community can help dispel myths leading to underutilization. The female cardiac patient has many contraceptive options including oral contraceptives (combined or progesterone only), long acting reversible contraceptives, emergency contraception and permanent sterilization. WHO and the Centers for Disease Control (CDC) publish a document that evaluates the safety for all contraceptive options per each chronic condition, including cardiac disease.5 The Medical Eligibility Criteria is numerated 1 to 4, 1 corresponding to no restriction and 4 an unacceptable health risk. The female cardiac patient scores 1 or 2 for all contraceptive choices (Fig. 4). Cardiologists may be unaware of this scoring tool.
We recommend all cardiac patients of reproductive age be referred to family planning before, during and/or after pregnancy for detailed options counseling which is educative and supportive of women’s family planning. This kind of approach might inspire a high-risk woman to contemplate pregnancy at a safer time when postoperative for a corrective cardiac procedure or surgery, for example. In addition, women may be interested in reproductive technologies such as surrogacy or preimplantation genetic diagnosis with subsequent embryo selection to avoid inheritance of congenital disease. Engaging subspecialists with family planning or reproductive endocrinology expertise early in the contemplative phase may alert the high-risk woman to alternatives that she might not have considered. Indeed, an encouraging area of growth in our program has been in the number of preconception consults for women diagnosed with high risk cardiac disease, growing from 4.8% in the first year 10% in the fifth year, but clearly there is room for earlier referral as approximately one third of our consultations are for pregnant women with congenital heart disease. This group in particular should be routinely engaged in preconception counseling, an opportunity for providers caring for these patients through their transition from pediatric to adult cardiology care. In addition, as MFM subspecialists encounter potentially high-risk patients, they should consider referring to their cardiology colleagues for evaluation and management. Over time, these dialogs facilitate mutual understanding and promote cross-collaboration for advancement of patient care and research.
High-risk Cardiac Disease in Patients Contemplating Pregnancy or Who Are Currently Pregnant Should Have Expedited, Streamlined Evaluation by Cardiology and MFM Specialists
We strongly recommend that tertiary facilities with resources to care for the high-risk pregnancy develop multidisciplinary teams consisting of at least a MFM subspecialist and a cardiologist, working in partnership to evaluate and mitigate the patient’s risk of mortality and morbidity in pregnancy. At our institution, we employ a Heart Team approach to expedite care for the patient through a joint office visit with both the MFM and cardiologist present and echocardiography available. The Heart Team has usually been reserved for the evaluation of high risk patients by both cardiology and cardiothoracic surgery, often at the same visit, an approach supported by both American College of Cardiology/American Heart Association and European Society of Cardiology guidelines4 for surgical pregnant patients, in which the team consists of “cardiology, MFM/obstetrics, anesthesiologists, cardiothoracic surgery, and other subspecialists, as needed.” We apply the Heart Team approach for even our nonsurgical high-risk pregnant women.
Obstetricians Should Be Astute to Abnormal Physical Examination, Laboratory, or Imaging Findings That Might Point to High-risk Cardiac Disease in Their Patients
Normal cardiovascular changes in pregnancy include an increased cardiac output, expanded blood volume and reduced systemic vascular resistance that commences in the second trimester and peaks in the third.6 These changes contribute to the optimal development of the fetus and protect the mother from delivery risks. The consequences of these significant changes are physical examination findings, laboratory changes, and image findings that often mimic cardiac pathology. It is common to hear a systolic ejection murmur or exaggerated splitting in a pregnant patient. However, we encourage obstetricians to listen to their patients’ heart every trimester and refer to cardiology for further evaluation of suspicious findings. We have discovered high-risk disease in our practice based on perceptive assessments of subtle changes in examination and studies found by our obstetric colleagues. In the first year of our program, while one third of patients had benign findings, two third had high-risk cardiac disease.
Symptoms may require a detailed evaluation to distinguish benign from pathologic and further review with a cardiologist may be warranted. A careful history should include prior medical, cardiac, surgical and obstetric history, medication review, family history, country of origin to assess risk of rheumatic disease, cardiac examination, review of electrocardiogram and echocardiographic imaging, or arrhythmic event monitoring. A general cardiology evaluation may then lead to subspecialty referral to heart failure, electrophysiology or adult CHD, for example.
Institutions and Departments Must Invest in Collaborative Models of Care, Which Work Locally and Nationally
Our MFM Cardiology Joint Program has hosted an evening dinner and lecture event to learn about the Pregnant Cardiac Patient for the past 3 years. This Continued Medical Education (CME) activity, free of charge, included attendees of multiple disciplines including Obstetrics, Cardiology, Anesthesia, Primary Care and different layers of skill set; nursing, physician assistant, medical doctor, and administration. The aim has been to learn and create an alertness about the Pregnant Cardiac Patient. This has created collaborations with our surrounding medical centers that may not have the resources for multidisciplinary care for the most at risk patient. We have therefore bridged the gap in communication to facilitate and expedite care for the pregnant cardiac patient.
Additional activities I have been involved in personally include directing a post graduate course with Dr Afshan B Hameed of UC Irvine at the Society of Maternal Medicine for 2 consecutive years. I have delivered Grand Rounds to local institutions on management of the Pregnant Cardiac Patient. All of these activities facilitate improved care and ultimately better outcomes. I would like to see the creation of a national consortium in the United States whereby experts have a platform to communicate and share experiences.
Research Studies Should Include Cardiovascular, Maternal, Fetal, Obstetric, Patient-centered, and Psychosocial Outcomes to Address a Multidimensional Problem
Research has helped illustrate the problem, the burden of disease and to counsel patients more effectively about risk. Risk assessment is undergoing a dynamic change as we improve in our treatment and multidisciplinary management of the pregnant cardiac patient. For example, pulmonary hypertension, often fatal in pregnancy, has improved survival with evolving treatment protocols.7,8 However, there are many gaps that remain in our knowledge, particularly in the fetal outcomes of these high-risk women. For example, there are few studies that summarize predictors of feto-neonatal complications, one by recurrent maternal arrhythmias9 and the other by maternal cardiac output,10 but additional research is needed.
Although there are several retrospective cohort studies, case series, and case reports on pregnant patients with cardiac disease, there are fewer prospective cohort studies and randomized controlled trials due to overall rarity and diversity of disease. Recent success in understanding PPCM is an example of how multicenter collaboration is vital for effective research in this regard. Research on the genetics of PPCM drew from 6 different cohorts both nationally and internationally, finding that there is a genetic predisposition.11 The Investigations of Pregnancy Associated Cardiomyopathy prospective study of women diagnosed with PPCM, included 30 centers and enrolled 100 women.12 A report on outcomes from bromocriptine treatment for PPCM has mobilized debate and is a motivation for more investigation with randomized trials.13 Multidisciplinary research drawing from various perspectives, including the patient experience as well as consideration of socioeconomic and race/ethnic disparities, would afford deeper understanding of the various contributors to genetics of disease, mortality, as well as patient motivation for future pregnancy in this high-risk birthing population. Joint MFM-Cardiology Programs naturally facilitate quantitative and qualitative research by bridging disciplines; provide a platform for continuing medical education and training opportunities for students, residents and fellows coming from pediatrics, medicine, obstetrics, and cardiology.
In conclusion, the alarming and devastating observation of rising cardiovascular mortality in the peripartum period, should be an impetus for obstetricians and cardiologists to take up the call to action in a coordinated effort. A Joint MFM-Cardiology Program, also known as the Cardio Obstetric team, is a model strategy devised to rapidly implement a potential solution.
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