Peripartum cardiomyopathy (PPCM) is an uncommon disease of pregnancy, occurring in about 1 in 2000 live births, and is characterized by the development of heart failure, due to left ventricular systolic dysfunction. It is associated with high rates of maternal and neonatal mortality. Cardiac disease is the leading cause of maternal death in the UK: PPCM accounts for about 17% of these. Clinical findings of decompensated heart failure (HF) are often masked by the normal physiological changes seen in pregnancy making the diagnosis challenging. A high index of suspicion is essential—prompting referral for echocardiogram, which is crucial for diagnosis. Favourable prognosis is dependent on the early initiation of HF medications. Although full recovery occurs in around half of cases, left ventricular systolic dysfunction persists in a significant proportion of patients with PPCM and the risk of recurrence in subsequent pregnancies is high. The pathophysiology of PPCM is under intense research. We present four patients with PPCM and a review of the literature. Owing to the diagnostic challenge of PPCM and decompensated HF in pregnant mothers and its high mortality rate without treatment, prompt investigation and referral are key to improving maternal survival.
Cardiac disease is the leading cause of maternal death in the UK [
We present a series of four women who presented with PPCM in our centre, of which two died. Their clinical presentations were variable and, so, represented an important diagnostic challenge.
A 26-year-old woman gave birth by spontaneous vaginal delivery at 41 weeks to her third child. She presented to hospital 6 months later with dyspnoea, light-headedness, and palpitations. ECG on admission showed sinus tachycardia, left axis deviation, and T-wave changes (Figure
ECG on presentation (case one). Showing sinus tachycardia, left axis deviation, and T-wave changes.
Chest X-ray (case one). Showing implanted BiVAD.
Unfortunately, her postoperative period was complicated by acute ischaemia of the left lower limb requiring above knee amputation and a prolonged period of renal replacement therapy. Her cardiac function improved over a period of two months and the BiVAD was successfully explanted. She had, remarkably, good cardiac function after explant. She remains well to date on beta-blocker and ACE-inhibitor.
A 25-year-old primiparous woman with a history of insulin-dependent diabetes mellitus presented at 34 weeks’ gestation with preeclampsia, for which she was given steroids. Emergency Caesarean section was undertaken at 35 weeks for preeclamptic toxaemia, following which she was discharged home on day 4. She was readmitted 2 days later with dyspnoea and orthopnoea. Initial differential diagnoses were panic attack and pulmonary thromboembolism. The diagnosis of decompensated HF was only made following a chest X-ray showing pulmonary oedema.
She was intubated and transferred to the National Heart Failure Unit. She was supported by an intra-aortic balloon pump, which was successfully weaned after 5 days. Her follow up echocardiogram showed good ventricular functions. She remains well to date on beta-blocker, ACE-inhibitor, and aldosterone antagonist. She also had a trial of bromocriptine for three months.
A 31-year-old primiparous woman presented a 17 weeks’ gestation with dyspnoea, which had worsened by 22-week review. At 29 weeks, she represented with a 3-week history of palpitations, tachycardia, and worsening dyspnoea and was discharged home following a normal ECG. She subsequently deteriorated, with further worsening dyspnoea, and was treated at primary care with antibiotics, steroids, and inhalers. She was, again, readmitted at 32 + 6 weeks with dyspnoea at rest and tachycardia.
Cardiomyopathy was diagnosed, she had an emergency Caesarean section and delivered a live baby, and then she was transferred to a cardiac unit. Cardiac function further deteriorated, and she died in the postnatal period.
A 35-year-old woman, with one previous pregnancy, gave birth by elective Caesarean section at 39 weeks to a live male. She had previously collapsed at home at 28 weeks’ gestation. She died in the postnatal period, and cardiomyopathy was demonstrated at postmortem.
Peripartum cardiomyopathy (PPCM) is a rare condition, with an incidence estimated to be around 1 in 2000 live births, and is associated with high rates of maternal and neonatal morbidity and mortality [
It is characterized by the deterioration of left ventricular systolic function towards the end of pregnancy, or in the postnatal period in the absence of another cause for heart failure [
The aetiology of PPCM is currently unclear, though this is currently the subject of much promising research, which may pave the way for novel approaches to treatment, which will be discussed later. It may well be that some cases are hitherto undiagnosed cardiomyopathies that only become overt in pregnancy; it is important that it is borne in mind that cardiac disease may come to the fore during gestation and in the peripartum period.
Diagnosis is mainly based on clinical suspicion and echocardiogram. However, a problem lies in that many of the signs and symptoms of normal pregnancy are indistinguishable from heart failure, and the condition may be missed completely or misdiagnosed. Electrocardiogram (ECG) may be relatively unremarkable (Figure
The Centre for Maternal and Child Enquiries has thus suggested that “women in late pregnancy or within 6 months of delivery with symptoms of breathlessness, or oedema, or orthopnoea and the signs of tachypnoea and tachycardia may have PPCM, and investigation with a chest X-ray and echocardiogram are indicated” [
There are recognised risk factors for PPCM, which provide us with a tool to identify those who are at risk of developing the condition. Obesity, smoking, excessive alcohol consumption, and malnutrition are all potentially modifiable risk factors for the disease, and education about these risk factors should inform all patients’ antenatal period regardless of any particular suspicion of PPCM. Any previous personal or family history of heart disease should alert clinicians to a high-risk pregnancy.
Several recommendations for alterations to service provision in order to improve maternal outcomes were made by the “Saving Mothers’ Lives” report [
The exact management strategy for PPCM depends on the individual clinical case but is essentially based on the stability of the patient. Acute heart failure and stable heart failure are managed quite differently. Acute heart failure in the peripartum period is managed [
Management strategies for those with stable heart failure in PPCM [
More than 50% of women recover without complication, with left ventricular systolic function at rest returning to normal [
Recent genome-wide and family studies have shown a possible basis for the aetiology of PPCM and may present a novel screening tool for at risk populations. It has been shown to show some concordance in families, though this may simply be a first manifestation of familial dilated cardiomyopathy [
It has been shown in mice that a product of protein cleavage causes impairment of cardiac myocyte function resulting in PPCM due to its antiangiogenic and proapoptotic properties. This effect was successfully abrogated in these mice by administration of bromocriptine and by inhibiting prolactin secretion and may hold therapeutic promise in humans [
Inflammatory markers including TNF-
Antibodies directed against cardiac tissue have been found in PPCM patients [
Due to its rarity, variability in presentation, and potential for mortality, PPCM should be considered in women that present with features consistent with left ventricular failure, though it should be borne in mind that this can mimic the normal physiological changes of pregnancy. And when it does occur, careful cardiac followup is essential.
Informed consent has been obtained from the women described for publication of this case series.
This work was presented as a poster at the 15th Annual British Maternal and Fetal Medicine Society Conference 2012, Glasgow.
The authors declare that there is no conflict of interests regarding the publication of this paper.
Sean Martin has written, reviewed, prepared, and submitted the paper. Daniel Short has written and critically reviewed the paper. Chih Mun Wong has critically reviewed paper. Dina McLellan has critically reviewed the paper and was the senior consultant in charge of case.