Case study - A case of postpartum cardiomyopathy

Dr Louise Newson investigates extreme tiredness in a new mother.

The patient was noted to have pitting oedema in the mid-calves (Photograph: Dr P Marazzi/SPL)
The patient was noted to have pitting oedema in the mid-calves (Photograph: Dr P Marazzi/SPL)

A woman came to the surgery complaining of worsening fatigue. Aged 28, she had given birth to her third baby three weeks ago. Her previous pregnancies and deliveries had all been uncomplicated and she had no significant medical history of note.  

She is obese; her BMI was recorded as 31.2 before her pregnancy. She smoked 10 cigarettes a day; before this pregnancy she was smoking around 20 a day.

Although she had expected to be tired with a newborn baby, she said the tiredness was far worse than she could ever imagine. She had also noticed she had an occasional palpitation, but otherwise felt well.  

At that consultation, her BP was recorded as normal. The doctor who saw her thought she might be anaemic or hypothyroid, so he arranged for her to have some blood tests.

Worsening fatigue
At her review appointment the following week, her FBC, U&Es and TFTs were all within normal limits. She explained that her fatigue had worsened, despite her baby being in a good night-time routine.  

She had noticed that she had to pass urine more frequently at night. Over the preceding few days, her ankles had become slightly swollen.

She was found to have a tachycardia; her pulse was 138bpm. Auscultation of her chest revealed bibasal crackles and she was noted to have pitting oedema to her mid-calves.

The patient was admitted to hospital and a diagnosis of peripartum cardiomyopathy (PPCM) was made. She has made an uneventful recovery after receiving appropriate treatment in hospital.

Peripartum cardiomyopathy
PPCM is a rare cardiomyopathy that can affect a woman (without known cardiovascular disease) in the final month of pregnancy or within six months of delivery.

Approximately 75% of cases are diagnosed within the first month postpartum and 45% present in the first week.1
The diagnosis requires documentation of poor left ventricular function in the absence of other identifiable causes of heart failure.

A high index of suspicion is also required, because shortness of breath and ankle swelling are common symptoms in the peripartum period. If PPCM is suspected, it is important to establish the diagnosis rapidly, so treatment can be started promptly.

The most common symptoms are increasing fatigue and dyspnoea. Paroxysmal nocturnal dyspnoea can also occur, as can palpitations. There may be features of heart failure on examination, including raised JVP, cardiomegaly, third heart sound and bibasal crackles. Hepatomegaly and ascites may be present.

Investigations are the same as for patients with heart failure due to other causes. An echocardiogram is essential for diagnosis and there will be a reduction in ejection fraction (<45%) with a raised left ventricular end diastolic dimension >2.7cm/m2. Chest X-ray will often show features of heart failure, with cardiomegaly, lower lung infiltrates and sometimes pleural effusions.

Treatment
The medical management of PPCM is similar to other forms of non-ischaemic dilated cardiomyopathy. If the woman is pregnant or breastfeeding, management needs to be tailored to use drugs that are safe in pregnancy and lactation, in order
to minimise maternal and fetal morbidity.

Diuretics, vasodilators (for example, nitrates), beta-blockers (for example, bisoprolol), digoxin and ACE inhibitors (after delivery of the baby) are commonly used. Some women may need to have inotropes.  In severe cases, temporary cardiovascular support may be needed by using intra-aortic balloon pumps and left ventricular assist devices.

Very rarely, cardiac transplantation may be indicated if intractable heart failure persists despite optimal medical treatment.
There is some evidence that addition of bromocriptine can improve left ventricular ejection fraction in some women with newly diagnosed PPCM.2 Cabergoline has also been shown to be effective in improving left ventricular function.3 There is an increased risk of venous thromboembolism in patients with PPCM and anticoagulation is usually given.  

Prognosis
Prognosis is variable; mortality rate is quoted as 20-50%.4 About 30-50% of patients recover without complications, with their baseline left ventricular systolic function at rest returning to normal.  

Risk of recurrence of PPCM is high, especially if left ventricular systolic function has not fully recovered. However, the risk of severe cardiomyopathy in any subsequent pregnancies for women with normal left ventricular systolic function
after recovery is relatively low.5

An echocardiogram is usually repeated every six months to assess the recovery. Women should have regular echocardiograms performed in future pregnancies.

  • Dr Newson is a GP in the West Midlands

References
1. Sliwa K, Hilfiker-Kleiner D, Petrie MC et al. Eur J Heart Fail 2010; 12(8): 767-78.
2. Sliwa K, Blauwet L, Tibazarwa K et al. Circulation 2010; 121(13): 1465-73.  
3. Mahmod M, Chan CF, Ali A et al. Br J Cardiol 2011; 18: 243-5.
4. Ibebuogu UN, Thornton JW, Reed GL. Thromb J 2007; 5: 18.
5. Pyatt JR, Dubey G. Postgrad Med J 2011; 87: 34-9.

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