Maternal Sudden Death in Pregnancy due to Cardiac Causes – Are they preventable?
Introduction Over the past decades, a steady decline in maternal mortality among women of reproductive age has been observed in the western world (1). However, despite significant improvements in the standards of care for women during the antepartum, intrapartum and postpartum period, this tragic event continues to remain a serious clinical challenge to the professionals.
In recent years cardiovascular disease has emerged as the leading cause of maternal death during pregnancy and in the postpartum period both in the developing and developed countries (2). The latest confidential enquiries into maternal deaths report in the United Kingdom and Ireland, the fourth in the annual report format, and the first to repeat the 3 yearly thermic cycle, published in December 2017 includes surveillance and confidential enquiries covering the period 2013-15 (3).
This topic specific review report into the care of women who died from haemorrhage, amniotic fluid embolism, sepsis, anaesthetic complications, neurological conditions and other surgical and medical conditions revealed that although overall there was no change in the maternal death rate in the UK between 201012 and 2013-15, which is now 8.76 per 100 000 maternities (95% confidence interval 7 .59-10.05), the cardiac disease was found to be the leading cause of indirect maternal death during or up to 6 weeks after the end of pregnancy with a rate of 2.34 per 100 000 maternities (95% CI 1.76-3.06). The previous MBRRACE UK report 2016, the 3rd of CEMD annual reports which included data on surveillance of maternal deaths between 2012-2014 did show similar picture in that the cardiovascular disease was found to be the leading causes of indirect maternal death during or up to 6 weeks after the end of pregnancy with overall no statistically significant decrease in the Maternal death rate in the UK between 2009-2001 and 2012 -2014.
A retrospective review of maternal deaths while pregnant or within 1 year of pregnancy between 2002 and 2006 in the USA also revealed that a significant proportion (25 %) of all maternal deaths was attributed to cardiovascular disease (4). Most recently Sudden Arrhythmic Deaths syndrome (SADS) has been identified as the main cause of maternal sudden death in pregnancy or in the postpartum period (5).
In this study, 50% deaths were during pregnancy and 50% were postpartum. The main cause of death was sudden cardiac death with a morphologically normal heart in 43 out of 80 cases (53.75%), followed by cardiomyopathies in 11 patients (13.80%) (table 1).
Patients’ characteristic and Risk factor for SADS Significant risk factors of maternal death due to cardiac disease are older age, high BMI and family history. These risk factors should be taken into consideration in clinical obstetric practice (5). Sudden cardiac death is more likely to happen in older female
especially women older than 35 years rather than in the younger women (5,6). High BMI (obese or overweight) is a risk factor for maternal sudden cardiac death and this should be taken into account in clinical practice.
High percentage of obese pregnant women (59.18 percent) has been found to suffer sudden cardiac death. High BMI, as a risk factor , has not been associated with the age of the woman. A history of sudden young female death in the family is very important. Women with such history would need for family screening until proven otherwise as channelopathies have been identified in up to 50% of families (7).
Circumstances leading to maternal death were interesting in that majority of death occurred mainly at rest (71.30 %) and the rest in sleep (12.5%). Women who died in sleep were under 35 years old (5). Symptoms suggestive of cardiac disease were noted in cases immediately prior to death. A significant proportion of women (37 .50%) were symptomatic with one or more cardiac symptoms which were chest pain, shortness of breath, syncope/collapse, dizziness and palpitations (5). Discussion Maternal Mortality (MM)is defined by the WHO (2004) as the death of a woman while pregnant or within 42 days of end of pregnancy, irrespective of the duration and the site of the pregnancy, from any cause related to or aggravated by the pregnancy or its management (from direct or indirect obstetric death) but not from accidental or incidental causes. Direct MMis defined as a maternal death resulting from obstetric complications of the
pregnancy state (pregnancy or labour or puerperium) from their interventions, omissions, incorrect treatment of from a chain of events resulting from any of above. Whereas, Indirect MM is defined as a pregnancy related death in a female patient resulting from with a pre-existing disease or a disease that newly developed during pregnancy and which was not due to the direct obstetric causes, but which was aggravated by the physiologic effect of pregnancy.
Maternal mortality rate (MMR)is defined as the annual number of woman’s deaths per 100,000 live births from any causes related to or aggravated by pregnancy or its management excluding accidental or incidental causes. Cardiovascular disease has now been established as an important contributor to maternal mortality worldwide. In the UK, maternal cardiac deaths are due mainly to SADS which as an important cause of maternal death has only recently been recognised.
Maternal deaths due to SADS, however, may not be so rare as successive confidential enquires into maternal death in the UK in recent years have reported cases of SADS as the cause of death since this was first reported in 2002. An electrical disturbance in the channelopathies in a morphologically normal heart has been suggested as the possibility of death in women who died of SADS (8).
It has been suggested that some of these deaths are preventable if diagnosed early. Therefore, there is now a compelling reason for the professionals to be more aware of the risks of sudden maternal deaths due to cardiac diseases and pay particular attention to the risk of cardiac conditions that may contribute to maternal deaths. Caring for women with cardiac disease in pregnancy can be challenging.
Considering the gradual rate of decline, achieving the government’s aspiration of reducing maternal death by 50% by 2030 will be a big challenge for UK health services, requiring coordinated action across multiple specialities.
It has been concluded that ‘there remain multiple opportunities to reduce women’s risk of complications in pregnancy through early and forward planning of the care of women with known risk factors. Provision of appropriate advice and referral early in pregnancy for appropriate specialist advice concerning risks, the need of cardiological screening of the family as a result of the diagnosis and planning for future pregnancies are the key improvements needed to prevent women dying needlessly or having severe complications in the future (3)’.
Lessons on cardiovascular disease from MBRRACE-UK (Maternal and Babies: Reducing Risk through Audits and Confidential Enquiries across the UK) Update The latest UK and Ireland confidential enquires into maternal death and morbidity (MBRRACE-UK 2017) report has highlighted the following lessons learnt from the investigation; Interdisciplinary Team Work and Inadequate Communication Lack of co-location of obstetric and cardiac services jeopardises interdisciplinary working and communication. Measures such as joint obstetric clinics, multidisciplinary care plans, copying letters to the woman and all clinicians involved in her care as well as staff from all specialities writing in the woman’s hand-held notes may mitigate against the inherent risk of inadequate communication between specialities.
Multidisciplinary Involvement Early involvement of senior clinicians from the obstetric and cardiology multidisciplinary team is vitally important, whether a pregnant or postpartum woman presents with suspected cardiac disease, but particularly if she presents to the emergency department.
Clinical Awareness Staff should be aware that a raised respiratory rate, chest pain, persistent tachycardia and orthopnoea are important signs and symptoms which should always be fully investigated. The emphasis should be on making a diagnosis, not simply excluding a diagnosis. A normal ECG and or a negative
Maternal Sudden Death in Pregnancy due to Cardiac causes–are they preventable?
Troponin does not exclude the diagnosis of an acute coronary syndrome. Family screening Women who die from sudden cardiac arrest and who have a morphologically normal heart should have molecular studies at post-mortem with the potential for family screening. Future sudden deaths amongst relatives may then be prevented. Counselling Pre-pregnancy counselling should be available both within the paediatric transition service and to women of childbearing age with known cardiac disease. This should include appropriate contraceptive service advice. The need for more effective pre- or postpartum counselling of women is high-lighted. Key topic specific messages for care: Health professional involved in maternity care should be aware that there may be a sign of an underlying problem in women with cardiac disease.
Obstetric community should be aware of SADS and the increased risks during pregnancy and afterwards. Women with cardiac disorders with or without risk factors should have a clear care plan for an appropriate schedule of checks with more clinic visits than those for low risk pregnant women. Women with prosthetic valves in pregnancy are at extremely high risk and should be referred to specialist centres early. They need an expert obstetrician, haematologist, cardiologist and anaesthetist input. New onset of symptoms suggestive of cardiac diseases should be considered for prompt referral to a consultant unit for urgent assessment and treatment with clear communication between health professionals.
Pregnancy may precipitate the first presentation of cardiac arrythmia and point to an underlying channelopathy or the patient may already be aware of such a diagnosis in herself or other family members.
All consultant led maternity unit should have ready access to an ECG machine and someone who can interpret ECGs. Similarly, Echocardiogram, performed by a competent practitioner should be available 7 days a week. A history of sudden young death in the family is very important. The risk of pregnancy must be considered, and obstetricians are made aware of the family history or previous diagnosis. Arrhythmias in pregnancy or post partum period, may be a recurrence of a previously diagnosed arrhythmia, but there may be no previous history of arrhythmia or a diagnosis of a channelopathies (9).
Women with risk factors need cardiac screening by a cardiologist with special interest in electrophysiology. A detailed past family history of sudden deaths or cardiac arrhythmias is essential and screening of other family members is indicated to avoid further deaths since these entities are commonly inherited. When delivery is planned, for foetal reasons, in a different unit to where women received their usual antenatal care, women’s own health issues were often overlooked. In pregnant or postpartum women with complex cardiac problems involving multiple specialities, their responsible consultant obstetrician or cardiologist must show clear leadership and be responsible for coordinating care and liaising with anaesthetists, midwives, other physicians and obstetricians, and all other professional who need to be involved in the care of these women. These women may require additional care following discharge from hospital and There is a need for senior review prior to discharge, with a clear plan for the postnatal period.
The senior review should include input from obstetricians and all relevant colleagues. After pregnancy, care should be clearly handed over to women’s GPs. Service delivery issue in particular staffing and workload imbalance has been implicated as possible contributing factor to maternal deaths. However , an impact of this on women’s deaths yet to be to be established. Conclusions The latest research evidence has revealed cardiac disease as the leading cause of Indirect maternal death during or up to 6 weeks after the end of pregnancy in the United Kingdom (3). Recently, Sudden Arrhythmic Death Syndrome (SADS) has also been recognised as the main cause of maternal sudden cardiac death in pregnancy or in the postpartum period (4).
It has been suggested that maternal deaths due to cardiac disease may be preventable if diagnosed early or may be avoided by better care (4). In this respect identification of a number of significant risk factors and a strong family history closely linked to maternal sudden cardiac deaths (SADS) can be extremely valuable in caring for pregnant women.
Accordingly, it cannot be over-emphasized that there is an urgent need for raising professional awareness of this frequently under-diagnosed clinical condition leading to sudden cardiac death in women during pregnancy and postpartum. The epidemiological evidence of a declining trend in the overall maternal death in the UK is encouraging and is a testimony to the high standard of care provided by the healthcare professionals. Although the situation has been improving, more needed to be done in order to prevent the recurrences of tragic deaths from cardiac disease which in recent years has emerged as the leading cause of maternal death in pregnancy.