peripartum cardiomyopathy

A rare case of peripartum cardiomyopathy complicated by renal and splenic infarctions

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Clinical record

A 41‐year‐old G1P1 woman presented with two days of dyspnoea four weeks postpartum. She had an uncomplicated caesarean delivery at 39 weeks’ gestation in the setting of uterine fibroids, and she was breastfeeding. Her medical history was otherwise unremarkable. An electrocardiogram demonstrated sinus rhythm with anterolateral T‐wave inversion. A computed tomography (CT) pulmonary angiogram demonstrated no pulmonary embolus but pleural effusions and cardiomegaly. A transthoracic echocardiogram (TTE) demonstrated a left ventricular ejection fraction (LVEF) of 19% (normal range, ≥ 50%), left ventricular end‐diastolic diameter (LVEDD) of 5.3 cm (normal range, 3.5–5.6 cm) and a 1.8 × 2.3 cm mobile apical thrombus (Box 1). In the absence of risk factors, a diagnosis of peripartum cardiomyopathy (PPCM) was made. Frusemide, spironolactone, enalapril and metoprolol were initiated within 48 hours. The thrombus was managed with warfarin and bridging enoxaparin. Digoxin was commenced for inotropy. Bromocriptine was not started given the presence of thrombus. On day 3, the patient developed significant abdominal and flank pain. A CT scan of the abdomen and pelvis demonstrated extensive left renal and splenic infarctions. Repeat TTE demonstrated thrombus resolution and the LVEF was 25%. The surgical, nephrology, and interventional radiology teams were consulted for consideration of catheter‐directed thrombolysis. Consensus was for ongoing anticoagulation given the extensive infarction. The patient was registered with the Spleen Australia registry because of functional asplenia and was discharged on day 7. She remained well on review one week later. LVEF was 52% one month after discharge. Spironolactone was ceased at one month. LVEF was preserved at three months and renal function remained stable.

Discussion

PPCM remains a major cause of maternal mortality.1 It typically occurs towards the end of pregnancy, up to five months postpartum. Complications include heart failure, arrhythmias and venous thromboembolism. Hypercoagulability in pregnancy, cardiac dysfunction and dilation, stasis and endothelial dysfunction are thought to increase risk. Left ventricular thrombus has been reported in around 17% of patients2 with subsequent thromboembolic events in around 9% of women. Arterial thromboembolism such as limb ischaemia, stroke and myocardial infarction have been described with left ventricular thrombus.

Splenic and renal infarctions are rare. Without thrombosis, anticoagulation in PPCM is a class IIb recommendation for LVEF below 30% in the American Heart Association/American College of Cardiology/Heart Failure Society of America guidelines1 and for LVEF below 35% in the European Society of Cardiology guidelines.2 However, in patients with intracardiac thrombus, systemic embolism or atrial fibrillation, anticoagulation is strongly recommended.3 Other management options for renal infarction include catheter‐directed thrombolysis, systemic thrombolysis, and surgery. Catheter‐directed thrombolysis is considered in patients with complete occlusion of a major segmental renal artery with onset under six hours or in those with significant reduction in renal function or baseline renal failure.4

PPCM management postpartum includes current standard heart failure therapy (Box 2), except for sacubitril–valsartan and sodium–glucose cotransporter 2 (SGLT2) inhibitors, drug classes for which no safety data are yet available in breastfeeding.2 Enalapril and captopril are the preferred angiotensin‐converting enzyme inhibitors due to low levels in breast milk.1 Ventricular arrhythmias have been reported to occur in up to 18.7% of patients,5 but there are no established guidelines for implantable cardioverter‐defibrillator therapy. Bromocriptine, a prolactin inhibitor, has been found to improve survival in PPCM.1 A randomised multicentre trial in Germany found expedited left ventricular recovery and minimal adverse effects with both short and long term bromocriptine use.6 Bromocriptine inhibits lactation, which also enables the institution of early intensive standard heart failure therapy. However, bromocriptine has been associated with increased cerebral and cardiovascular complications and arterial thromboembolism, thus, concurrent anticoagulation is recommended.6 Only a few studies have reported safe use of bromocriptine in patients with existing thrombus.7

Box 2

Postpartum management of peripartum cardiomyopathy: recommended medications and considerations adapted from the American Heart Association/American College of Cardiology/Heart Failure Society of Amercia1 and the European Society of Cardiology4 guidelines

Prognosis in PPCM is typically better than in other forms of heart failure. Recovery typically occurs within three to six months,2 but it can be up to two years. Deterioration after initial recovery can occur, hence the importance of follow‐up. Increased LVEDD, LVEF below 35% at diagnosis, right ventricular dysfunction, African ancestry, older age, later diagnosis and elevated inflammatory markers confer poor prognosis.8 Late gadolinium enhancement on magnetic resonance imaging has also been shown to predict persistently reduced LVEF and higher risk of recurrence in future pregnancies.9 Guideline‐directed therapy is recommended in patients with ongoing cardiac dysfunction. However, in patients with recovered LVEF, the optimal duration of treatment remains unknown. Close monitoring of clinical status and LVEF is recommended, with stepwise cessation of medications.2 Stress echocardiogram and strain imaging have been propositioned as methods to detect subclinical left ventricular dysfunction but further research is required.

In women with persistent cardiac dysfunction, future pregnancy is discouraged as risk of PPCM recurrence is high and associated with high maternal and fetal mortality. In patients with recovered LVEF, the risk of recurrence is around 20%.2 Left ventricular recovery should be stable for three months after cessation of heart failure medications before conception.2 Our case report highlights the gravity and breadth of complications in PPCM and the importance of prompt institution of medical management.

Lessons from practice

  • Peripartum cardiomyopathy (PPCM) is a major cause of mortality and morbidity in pregnancy.
  • Renal and splenic infarctions are a rare complication of PPCM, with treatment options including anticoagulation and catheter‐induced thrombolysis.
  • Bromocriptine can be considered in PPCM (class IIb recommendation) but safety in the presence of thrombus is unknown.
  • Recovery in PPCM can be prolonged up to two years and relapse is common, hence the importance of ongoing surveillance. Recovery, in our patient, was seen within one month despite recognised poor prognostic factors such as left ventricular ejection fraction below 35%, right ventricular dysfunction and older maternal age, highlighting the importance of prompt institution of medical management.

Peripartum Cardiomyopathy

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How is peripartum cardiomyopathy diagnosed and managed?

Peripartum cardiomyopathy is now a leading cause of death in pregnant persons in many parts of the United States and around the world. Read the NEJM Review Article here.

Clinical Pearls

Q: How is peripartum cardiomyopathy defined?

A: The disorder is generally defined as maternal heart failure with systolic dysfunction (left ventricular ejection fraction, <45%) that develops in the last month of pregnancy or in the first 5 months after delivery, in the absence of known preexisting cardiac dysfunction. In some cases, however, the disease occurs earlier in pregnancy or more than 5 months after delivery. Although cardiac function typically recovers in more than 50% of affected patients, morbidity and mortality are nevertheless high, with some patients requiring a left ventricular assist device or cardiac transplantation.

Q: What are some of the risk factors for peripartum cardiomyopathy?

A: Peripartum cardiomyopathy complicates approximately 1 in 2000 births worldwide, with substantial variation among regions, including rates as high as 1 in 300 births in Haiti and 1 in 100 in parts of Nigeria. In the United States, the disease is four times as likely to develop in Black women as it is in White women. One third to one half of cases occur in women with hypertensive diseases of pregnancy, including preeclampsia. Other strong risk factors for peripartum cardiomyopathy include multiple gestations, advanced maternal age, and anemia.

Morning Report Questions

Q: Do we know what causes peripartum cardiomyopathy?

A: The causes of peripartum cardiomyopathy remain poorly understood. Studies have suggested that the disorder is triggered by hormones that emanate from the pituitary and placenta during the peripartum period, synergizing, in ways still poorly understood, with intrinsic cardiac factors that render some women susceptible to these hormonal imbalances. Together, these studies of pregnancy hormones have suggested a vasculohormonal model of the pathogenesis of peripartum cardiomyopathy, whereby imbalances in peripartum hormones cause cardiovascular dysfunction and consequent heart failure in susceptible women. What intrinsic cardiac factors render some women but not others susceptible to these hormonal imbalances? This question remains largely unanswered, but recent studies have revealed a strong genetic predisposition to peripartum cardiomyopathy in some cases.

Q: How is peripartum cardiomyopathy diagnosed and managed?

A: Patients with peripartum cardiomyopathy typically present with symptoms and signs of heart failure, including dyspnea, orthopnea, elevated jugular venous pressure, pulmonary rales, and edema. The diagnosis requires a high index of suspicion and is often delayed because symptoms mirror those of pregnancy itself. Peripartum cardiomyopathy is a diagnosis of exclusion. The differential diagnosis includes preexisting structural heart disease, preeclampsia-induced pulmonary edema in the absence of systolic dysfunction, pulmonary embolism, spontaneous coronary artery dissection, and exposure to toxins, including alcohol and chemotherapeutic agents. Currently, there is no biomarker that is diagnostic for peripartum cardiomyopathy. Genetic testing is increasingly offered to patients with peripartum cardiomyopathy, and it should be considered in most cases. Few randomized trials have evaluated therapies for peripartum cardiomyopathy, and none of these studies have had conclusive results. Current management is thus largely extrapolated from guideline-directed medical treatment for nonischemic dilated cardiomyopathy and other forms of heart failure with a reduced ejection fraction.

40-Year-Old Woman with Postpartum Dyspnea and Hypoxemia .

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40-year-old woman was admitted to this hospital 10 days post partum because of dyspnea and hypoxemia associated with leg edema and blood-tinged sputum. Diagnostic procedures were performed.

Causes of dyspnea and hypoxemia in the peripartum period are predominantly of pulmonary or cardiovascular origin, the latter generally associated with pulmonary edema. The peripartum period predisposes women to a number of pathologic conditions, including pulmonary embolism, amniotic-fluid embolism, infection, aspiration, preeclampsia, and peripartum cardiomyopathy.

Clinical Pearls

• What are the clinical manifestations of an amniotic fluid embolism?

Amniotic-fluid embolism is a rare but catastrophic complication of pregnancy or labor. Patients with amniotic-fluid embolism usually present with cardiorespiratory collapse; this is typically accompanied by disseminated intravascular coagulation and systemic inflammatory responses. Delayed manifestation of amniotic-fluid embolism beyond 48 hours after delivery is extremely rare.

• What is the epidemiology and what are the clinical manifestations of peripartum cardiomyopathy?

Peripartum cardiomyopathy is characterized by congestive heart failure and left ventricular systolic dysfunction toward the end of pregnancy or in the months after delivery, in the absence of other identifiable causes of cardiac disease. The ejection fraction on echocardiography is nearly always less than 45%. More than 90% of cases present in the first weeks post partum. The incidence varies from 1 in 300 live births to 1 in 3000 live births; two “hot spots” are Haiti and Nigeria. Peripartum cardiomyopathy is most common in women of African descent but is seen throughout the world. The manifestation is similar to that of other cardiomyopathies, including such signs and symptoms of venous congestion as dyspnea, orthopnea, edema, and, in extreme cases, hypoxemia.

Morning Report Questions

Q: What are the principles of management of peripartum cardiomyopathy?

A: As with other cardiomyopathies, management of peripartum cardiomyopathy should focus on reducing preload and afterload and interrupting the maladaptive neurohormonal response to systolic heart failure. Diuretic agents and itrates are the treatments of choice for volume overload, although caution is required with the use of these agents before delivery. Angiotensin-converting-enzyme inhibitors and angiotensin II-receptor blockers could be administered but would be contraindicated if the patient were still pregnant. In the setting of a low ejection fraction and in light of the hypercoagulability of the puerperium, anticoagulation may be advised for the prevention of systemic embolism. Implantation of an automatic implantable cardioverter-defibrillator, to prevent death from arrhythmia, is relatively contraindicated, because systolic function frequently recovers. If the patient were pregnant, neither premature discontinuation of pregnancy nor delivery by cesarean section would be indicated.

Q: What is the prognosis of peripartum cardiomyopathy?

A: As many as 50% of women with peripartum cardiomyopathy eventually recover cardiac function, but 25% have progression to advanced heart failure, which often leads to cardiac transplantation or death. A lower ejection fraction at presentation predicts a worse outcome and  delayed recovery. Peripartum cardiomyopathy usually recurs in subsequent pregnancies, so decisions about repeat pregnancies need to be considered on an individual basis.