The incidence of CHD worldwide is slightly >1% of all live births.Reference Abdulla 1 With extensive medical and surgical innovation over the past 50 years as well as high-quality care received in childhood, 85–90% of children with CHD in the United States of America can be expected to survive into adulthood.Reference Hoffman, Kaplan and Liberthson 2 – Reference Warnes, Liberthson and Danielson 4 As a result, the number of adults with CHD in the United States of America has reached an estimated 1.5 million with increasing numbers every year.Reference Hoffman, Kaplan and Liberthson 2 – Reference Balint, Siu and Mason 5 Studies have also estimated that the proportion of pregnant women with underlying CHD also continues to expand.Reference Marelli, Ionescu-Ittu, Mackie, Guo, Dendukuri and Kaouache 3
The growing number of adult CHD patients has presented unique challenges for practitioners ranging from access to healthcare, non-cardiac and cardiac surgery, ongoing psychosocial care needs, and obstetrical care. Risk factors for complications during pregnancy in women with CHD include cyanosis, management of anticoagulation in the setting of mechanical valves, paradoxical thromboembolic events, aortic dilation, pulmonary hypertension, both systemic and pulmonary ventricular dysfunction, and exacerbation of arrhythmia.Reference Bhatt and DeFaria Yeh 6 The importance of these hazards has led to the establishment of multiple guidelines to help provide a framework for risk assessment for pregnancy as well as contraceptive recommendations in both general and lesion-specific terms.Reference Warnes, Williams and Bashore 7 – Reference Regitz-Zagrosek, Blomstrom Lundqvist and Borghi 11 Challenges that remain include identifying the appropriate cardiology physician who is adequately trained to care for pregnant women with mild-, moderate-, or great-complexity CHD as well as identifying the ideal location where management of issues related to pregnancy, delivery, and any necessary procedures can be performed.
To date, there have been limited evaluations of healthcare delivery to pregnant women with CHD. Although more than 110 institutions from across the United States of America and Canada have worked to develop formal adult CHD programmes to meet the distinct needs of this population, it still remains unclear as to where and who should be providing the care for pregnant women with CHD.
Methods
In 2011–2012, we invited 199 paediatric cardiologists from across the United States of America to participate in a cross-sectional, anonymous survey. The survey was delivered via e-mail, and was re-sent up to a maximum of three times to non-respondents over a 6-month period. The survey was sent via e-mail to paediatric cardiologists from all parts of the United States of America and some parts of Canada via a central online list server. The survey consisted of items regarding basic programme demographics such as size, number of surgeries per year, and setting as well as the programme’s current model for the care of women with CHD (Appendix). Included were questions regarding knowledge of contraceptive methods, experience caring for pregnant women with CHD, and referral patterns including the utilisation of high-risk obstetric and adult CHD specialists. Each participant’s responses remained anonymous and were transmitted directly into an online, secure database.Reference Harris, Taylor and Thielke 12
Results
A total of 111 cardiologists responded to the survey. Nearly all (90%) were affiliated with an academic affiliation and 70% had ⩾10 years’ experience. More than 95% had previously reported the presence of an adult CHD clinic at their institution as part of the 2011 Adult Congenital Heart Association clinic directory. 13 Although 95% reported an understanding of available contraceptive options, 32% did not feel comfortable recommending birth control to women with CHD. Nearly all respondents (98%) discussed pregnancy with their patients and most often (32%) when they were 16 years of age. Most (96%) indicated that they would counsel women with the following medical conditions to avoid pregnancy: pulmonary hypertension (93%), aortic aneurysm with underlying connective tissue disease (77%), and Fontan palliation (71%).
Among all, 83% of the respondents encountered pregnant women with CHD in their practice. Upon learning that their patient was pregnant, 60% indicated they would refer her to see an adult CHD specialist and 73% would also refer her to a high-risk obstetrician. Nearly all respondents (98%) indicated they would not transfer the care of their pregnant patient to an adult cardiologist without dedicated adult CHD training. Although 71% of the respondents had followed-up a woman throughout her entire pregnancy, with 95% within the past 5 years, 35% reported a low level of comfort in doing so. Notably, 81% of respondents stated that they would resume their patient’s primary cardiovascular care following delivery and not transition to an adult CHD specialist.
Discussion
Our results illustrate a gap in between what physicians feel should be done and the care that they feel comfortable providing pregnant women with CHD. This discrepancy suggests that providers view pregnancy as a complicated physiological state, but not necessarily one that heralds the need for transition of care to an adult CHD specialist. Given that 35% of those surveyed reported a low level of comfort in providing care throughout pregnancy, further research is necessary among the healthcare community not only to educate the patients but also to educate healthcare providers.
Pregnant women with CHD are increasingly being seen in paediatric cardiology and obstetrical offices and, although cardiac disease complicates a small percentage of all pregnancies in developed countries (1–4% in the United States of America), it is a major cause of non-obstetric maternal morbidity and mortality.Reference Creanga, Berg and Syverson 14 Historically, rheumatic heart disease was the most common form of cardiac disease encountered in pregnant women;Reference McFaul, Dornan and Lamki 15 however, CHD has become the most common form of heart disease complicating pregnancy in the modern world.Reference Cantwell, Clutton-Brock and Cooper 16 Many women, including those with CHD, are also postponing childbearing until later in life. Indeed, the average age of the first live birth in the United States of America has recently risen to 26 years.Reference Matthews and Hamiltion 17 With advancing maternal age, other co-morbidities such as hypertension, diabetes, hypercholesterolaemia, and even coronary artery disease have become more common and have increased the incidence of acquired heart disease complicating pregnancy.Reference Marelli, Ionescu-Ittu, Mackie, Guo, Dendukuri and Kaouache 3 , Reference Harris, Taylor and Thielke 12
Pregnancy in women with CHD can lead to cardiac complications during pregnancy and their risk can be estimated by assessing a number of factors. The “CARPREG Score” is based on the single largest prospective study of 562 women with congenital or acquired cardiac disease or arrhythmias who had 617 pregnancies.Reference Siu, Sermer and Colman 10 The actual rate of primary cardiac events such as pulmonary oedema, arrhythmia requiring treatment, stroke, cardiac arrest, or death was 13% overall, with 55% occurring antepartum. Risk factors for cardiac events during pregnancy included poor functional class (NYHA class II to IV), cyanosis, previous cardiac event such as heart failure, transient ischaemic attack, stroke, or arrhythmia, left heart obstruction (mitral valve area of <2 cm2, aortic valve area of <1.5 cm2, peak left ventricular outflow gradient >30 mmHg), and left ventricular systolic dysfunction (left ventricular ejection fraction <40%).Reference Siu, Sermer and Colman 10 The ZAHARA investigators have another model for predicting pregnancy complications in women with CHD. In addition to cyanosis, need for cardiac medications before conception, and left heart obstruction, other independent risk factors included mechanical valve replacement and systemic or pulmonary atrioventricular valve regurgitation related with the underlying complex CHD.Reference Drenthen, Boersma and Balci 9 More recently, the modified World Health Organisation guidelines created a four-category schema from which risk assessment could be derived, ranging from minimal risk to pregnancy contraindicated. These guidelines promote individualised plans of care and are regarded as among the most accurate in predicting morbidity.Reference Regitz-Zagrosek, Blomstrom Lundqvist and Borghi 11
There are many challenges facing practitioners who care for adult CHD population, such as providing appropriate antenatal and peripartum management of pregnant women with CHD, the optimal location to provide care and obtain necessary procedures, and securing the expertise of and collaboration with non-cardiac subspecialties such as high-risk obstetrics and anaesthesia to assist with the management of labour, delivery, and any complications that may arise in the peripartum or postpartum period.Reference Dearani, Connolly and Martinez 18 Therefore, it must be appreciated that not all physicians or hospitals are equipped to care for pregnant women with CHD, particularly if complications arise, which may be adult-onset cardiac or non-cardiac in nature.Reference Cantwell, Clutton-Brock and Cooper 16 , Reference Giannakoulas, Dimopoulos and Engel 19 – Reference Pillutla, Shetty and Foster 23
As this study illustrates, the majority of paediatric practitioners care for women with CHD who are of childbearing age. Multiple studies have created various schemas to assess risk to the fetus and gravid mother, but none of them is perfect. In the future, larger studies hopefully can be conducted to more accurately risk stratify patients. In addition, future enquiries into clinical practice patterns might provide useful data regarding where these patients are cared for, by whom, what level of counselling they receive, and why.
Limitations
Our study has several important limitations. First, only 111 paediatric cardiology physicians responded across North America. We are also unable to provide detailed information regarding the non-responders. As a result, it is possible that the data reported in this study do not reflect the practices of all paediatric cardiologists currently in practice either in universities or private settings. Another limitation of the survey methodology is recall bias on the part of the provider. Finally, we limited our survey distribution to paediatric cardiology practitioners; therefore, our results may be falsely skewed towards patients remaining with the primary paediatric provider.
Conclusions
Even with tremendous advances in recent years, patients with CHD continue to face the prospect of both surgical and percutaneous interventions, arrhythmia, heart failure, and even premature death. This fact highlights the need of adult CHD patients to maintain lifelong cardiac care from cardiologists with appropriate training and expertise. This is particularly true during pregnancy. Best care practice for these patients requires a team approach including specialists in adult CHD cardiology, maternal–fetal medicine, and obstetrical anaesthesiology. Although the 32nd Bethesda Conference and the 2008 ACC/AHA guidelines on the care of adult CHD provide management strategies for the practicing cardiologist, we present a disparity between published guidelines and the reality of care for pregnant women with CHD. The potential implications of the findings from this survey include providing a continuation of unmet healthcare needs in pregnant women with CHD as well as insufficient attention to both adult-onset cardiac and non-cardiac diseases complicating pregnancy. Training adult CHD cardiologists with knowledge about reproductive health in women with CHD is one of the first steps to closing this care gap.
Acknowledgements
None.
Financial Support
This research received no specific grant from any funding agency, commercial, or not-for-profit sectors.
Conflicts of Interest
None.
Appendix
