European Journal of Cardiovascular Medicine

Peripartum cardiomyopathy (PPCM) is an uncommon but life-threatening condition that occurs in the peripartum period, affecting 1 in 1,000 to 1 in 4,000 live births worldwide, with regional variation [1]. Defined by the European Society of Cardiology (ESC) as an idiopathic cardiomyopathy with reduced left ventricular ejection fraction (LVEF <45%) developing toward the end of pregnancy or in the months following delivery, PPCM remains underdiagnosed due to its nonspecific symptoms [2].

The aetiology is likely multifactorial and may include genetic predisposition, oxidative stress, autoimmunity, angiogenic imbalance, and viral myocarditis [3-5]. Risk factors include multiparity, advanced maternal age, hypertensive disorders of pregnancy, African or Asian ethnicity, and socioeconomic deprivation [6]. Left untreated, PPCM can lead to progressive heart failure, arrhythmias, thromboembolism, and death.

Prompt recognition and echocardiographic evaluation are critical for diagnosis, and management requires careful balance between maternal cardiac support and postpartum considerations, such as breastfeeding. This report discusses the clinical journey of a patient diagnosed with PPCM at term gestation highlighting diagnostic challenges due to overlapping symptoms with normal physiological changes in pregnancy and other common conditions seen in pregnancy.

A 26-year-old south Asian woman, gravida 4, para 0 and 3 previous first trimester loss, was admitted at 37 weeks gestation for induction of labour. She had background history of type 2 diabetes mellitus managed with metformin and insulin during pregnancy and late onset gestational hypertension diagnosed at 36+4 weeks and was medicated on Nifedipine 10 mg twice a day. The indication for induction was fetal growth restriction associated with oligohydramnios and altered umbilical artery doppler studies. 

On admission, it was noted that the patient's oxygen saturation were persistently hovered around 90% on room air. The patient was comfortable, afebrile, with a respiratory rate of 18/min. She was not breathlessness and had not experienced chest pain or orthopnea. Upon further questioning, she reported a recent respiratory tract infection for which she had completed a 5-day course of oral antibiotics. 

After discussing about the benefits and risks of induction of labour and caesarean section in a growth restricted fetus, the patient opted for caesarean section and a plan for category 3 cesarean section was made.

Overnight the patient's respiratory conditions deteriorated. She became breathless and tachypneic (respiratory rate of 40/min). Her oxygen saturation deteriorated further to 78% on room air requiring high flow oxygen at 10L/min. On  examination, it was noted that she had bilateral pedal edema. On auscultation, she had bilateral basal crepitations with notable tachypnea. Her blood pressure remained stable at (110/70 mmHg), and she remained afebrile. Fetal monitoring was done with CTGs were every 4 hours and these met Dawes Redman criteria suggesting low risk of fetal hypoxia. 

Our primary suspicion was pneumonia at this point given the background history of recent chest infection.

Urgent workup was initiated. Her arterial Blood Gas (ABG) confirmed type 1 respiratory failure. Her blood tests confirmed normal white cell count with a borderline CRP 33 g/L (< 35 g/L). Her NTproBNP was 650 ng/mL( normal < 400 ng/mL). 12 lead ECG confirmed sinus tachycardia.

Chest X-ray confirmed bilateral opacities in the central lung fields with blunting of left costophrenic angle suggestive of pulmonary oedema and cardiomegaly. 

CT Pulmonary Angiography (CTPA) confirmed no evidence of pulmonary embolism but reported ground glass opacities suggestive of infection. 

The presence of pulmonary oedema resulted in a working diagnosis of peripartum cardiomyopathy secondary to preeclampsia with an underlying pneumonia. The critical care outreach team opinion was sought. IV Furosemide 40 mg was given. The family were updated.

Due to concerns about maternal well-being, a category 2 caesarean section was performed after obtaining consent from the patient which itself was uncomplicated. A healthy infant weighing 2570 gms was delivered with good APGAR scores. 

Postoperatively, the patient was transferred to the high-dependency unit (HDU) for non-invasive ventilatory support. A cardiology review was requested and bedside transthoracic echocardiography confirmed severely impaired global systolic function consistent with a cardiomyopathy. 

It was not clear at this stage whether this represented a diagnosis of a peripartum cardiomyopathy or an existing dilated cardiomyopathy which has decompensated due to the chest infection and pregnancy. The patient was treated with diuretics and once offloaded, initiated on a beta-blocker (Bisoprolol), MRA (eplerenone), an ACE inhibitor (Ramipril) and, SGLT2 inhibitor (Dapagliflozin). Thromboprophylaxis with low molecular weight heparin was give and patient was closely monitored. The medications could be given as patient had decided not to breast feed. The patient drastically improved after initiating thise treatment and her oxygen requirements reduced, she self-discharged on day 4 and has been seen in the community

Heart failure remains a clinical challenge in pregnancy due to its nonspecific presentation and the overlapping symptomatology with normal pregnancy, such as dyspnoea, fatigue, and lower limb oedema. The early postpartum period is particularly high-risk, and delayed diagnosis may lead to severe morbidity or mortality [7].

In our patient, on seeing documentation retrospectively it was noted that she had reported of cough about 10 days prior to admission which was presumed to be community acquired pneumonia and treated with antibiotics. This might have been the first symptom of her heart failure. However, as other parameters were normal and the  patient symptomatically improved after taking antibiotics, arrival at the diagnosis of heart failure was delayed. This demonstrates diagnostic challenges faced by obstetricians during pregnancy the and postpartum period. Initiating this patient on Nifedipine because of her hypertension, may have resulted in further arterial vasodilatation and there is a risk she may have become hypotensive. 

The use of echocardiography was pivotal in confirming the diagnosis. The measurement of NTproBNP and cardiac enzymes are useful can be supportive. But NTproBNP is a negative predictive test. The absence of pre-existing cardiac disease and acute onset of heart failure symptoms in her third trimester aligns with a diagnosis of peripartum cardiomyopathy, but does not exclude a preexisting dilated cardiomyopathy [2].

The management of heart failure in the peripartum phase is the same as ‘normal’ heart failure (reference ESC guidelines) with critical importance needed to address maternal haemodynamics, delivery planning, medications when pregnant and with lactation, and contraception. ACE inhibitors are contraindicated during pregnancy and should only be initiated postpartum [8]. Bromocriptine, a prolactin-inhibitor, has been shown in some studies to improve outcomes in peripartum cardiomyopathy by mitigating the cardiotoxic effects of cleaved prolactin fragments, although its use remains controversial and is limited by breastfeeding preferences [9]. SGLT 2 inhibitors were originally used for treatment for diabetes are were only recently added to guideline directed therapy for patients with HFrEF[10].  

In women who do not have any other cardiac history and are diagnosed with a peripartum cardiomyopathy, the recovery  is variable. Up to 50% of patients may recover normal function within six months to a year, while others may progress to end-stage heart failure and even require mechanical support [11]. Our patient initially showed significant improvement in her symptoms however has struggled with compliance which is a concerning feature. Patients and their families often require review with the team to understand what happened during delivery and to ensure they fully understand their diagnosis. Conversations are always required about contraception and the risk of further cardiac compromise in subsequent pregnancies.

This case illustrates the diagnostic complexity and clinical significance of heart failure in the peripartum period. Early suspicion, timely echocardiography, and involvement of a multidisciplinary team were instrumental in managing this case. Clinicians should be alert to heart failure in women presenting with unexplained respiratory symptoms in the peripartum period, even in the absence of classic cardiac symptoms. Prompt diagnosis and appropriate heart failure therapy significantly improves maternal and fetal outcomes.

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