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Patients who attend the emergency department with pre-cordial pain after pregnancy present a diagnostic dilemma. While pulmonary embolism (PE) is a comparatively common cause,1 peripartum cardiomyopathy (PPCM) although rare, should always be considered in the differential diagnosis.2 PPCM is a rare form of congestive heart failure that affects women late in pregnancy or in the early peurperium.3 It is a disorder of unknown aetiology with a high mortality rate approaching 50%.3–5 We report a presentation of a patient with postnatal pre-cordial pain and the clinical findings, which permitted correct diagnosis and treatment.
A 36 year old white woman was referred to the emergency department by her general practitioner with a diagnosis of suspected PE. She had had a caesarean section seven days previously, with a three day history of exertional precordial discomfort, dyspnoea on exertion, and right calf swelling. There was no productive cough or haemoptysis. She had no personal or family history of cardiac disease. She smoked five cigarettes a day before becoming pregnant but denied smoking since. She had no history suggestive of a pre-existing heart condition as she had a previous excellent exercise tolerance.
On examination she was pale and anxious with a respiratory rate of 20 breaths per minute and a heart rate of 110 beats per minute and regular. Blood pressure was 140/80. Bilateral coarse crepitations and a gallop rhythm were heard on auscultation of her chest. On lower limb examination she had bilateral ankle oedema, which was more pronounced on the right. The right calf was also tender.
Initial impression was of congestive cardiac failure or a pulmonary embolism secondary to a right lower limb DVT.
An ECG showed left axis deviation with left bundle branch block. A chest radiograph showed pulmonary oedema and an increased cardiothoracic ratio (fig 1).
Treatment included high flow oxygen via non-rebreather mask, intravenous diuretic (frusemide 80 mg), and a therapeutic dose of a low molecular weight heparin. An echocardiogram was immediately performed that showed a dilated left ventricle with an ejection fraction of 20%. The patient was admitted to the coronary care unit and an ACE inhibitor (captopril) was added to her treatment. A left and right heart catheterisation was performed later the same day, which showed normal coronary arteries and a raised right heart and pulmonary artery pressures. V/Q scan was undertaken at day 3 and showed low probability of pulmonary embolus.
The patient made good recovery over the next five days and was discharged from hospital six days later receiving treatment with a β blocker, ACE inhibitor, and a diuretic.
While pulmonary embolism was suspected in the differential diagnosis the symptoms of exercise related chest pain, exertional dyspnoea, and orthopnea pointed to a primary cardiac pathology. The clinical signs of pulmonary oedema and gallop rhythm also pointed away from a thromboembolic cause. While the patient had raised d-dimers (commonplace in pregnancy), and positive risk factors on thromboembolic risk stratification, urgent echocardiography permitted prompt and correct diagnosis of PPCM.
PPCM is defined on the basis of four criteria.3
Development of cardiac failure in the last month of pregnancy or within five months of delivery.
Absence of an identifiable cause for the cardiac failure.
Absence of recognisable heart disease before the last month of pregnancy.
Left ventricular systolic dysfunction shown on echocardiograph.
The incidence of PPCM is unknown. An accepted incidence is 1 per 3000–4000 live births per year.6 The incidence is highest in some areas of Nigeria.7 The cause of PPCM remains unknown, however a number of potential risk factors have been proposed.
Age greater than 30 years3
Women of African descent8
Pregnancy with multiple fetuses9
A history of pre-eclampsia, eclampsia, or postpartum hypertension
Maternal cocaine misuse10
Patients with PPCM most commonly present with dyspnoea but other frequent complaints include cough, orthopnoea, paroxysmal nocturnal dyspnoea, haemoptysis, and chest discomfort.3
ECG findings may include sinus tachycardia, non-specific ST and T wave abnormalities, and voltage abnormalities. The chest radiograph typically shows enlargement of the cardiac silhouette with evidence of pulmonary venous congestion or interstitial oedema, or both. An echocardiogram needs to be performed in all patients suspected of having PPCM. It usually shows left ventricular enlargement and significant global reduction in ejection fraction. Other findings may include left atrial enlargement, mitral and tricuspid regurgitation, and a small pericardial effusion.11
Treatment of PPCM is similar to that for other types of congestive cardiac failure. However, the adverse effects of these agents on the fetus or breast feeding infant must be borne in mind. Diuretics may be used prepartum although hyponatraemia has been reported in infants born to mothers taking diuretics. Angiotensin converting enzyme inhibitors are absolutely contraindicated prepartum, when they may be associated with adverse fetal renal effects and an increase in neonatal mortality. β blockers are generally safe in pregnancy.4
Mortality estimates for patients with PPCM range from 25% to 50% with most deaths occurring within the first three months postpartum.3–5 Death is usually caused by progressive pump failure, arrhythmias, or thromboembolic events.12
The general consensus among experts is that patients with PPCM and persistent left ventricular dysfunction, are at extremely high risk of complications and death with subsequent pregnancies.13 Therefore these patients should be advised to avoid pregnancy. Even in patients where left ventricular function recovers, there seems to be some risk for recurrence.13 They need to be counselled about the potential risks before pregnancy and be carefully monitored if they choose to become pregnant again.
While rare, the diagnosis of PPCM needs a high index of suspicion, good history taking, attention to abnormal clinical signs, and rapid echocardiography. Echocardiography is a useful tool in diagnosis of both cardiac and trauma emergencies within the emergency department.14 Delay in diagnosis of PPCM or erroneous diagnosis of “PE” results in poor outcome.2
Barry Quinn did the literature review and wrote the paper. Brendan Doyle assisted with interpretation of the cardiac investigations. John McInerney made amendments to the original draft of the case report and acts as guarantor.