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Peripartum Cardiomyopathy

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Peripartum Cardiomyopathy

Basics

Description

A few cases of familial PPCM have been reported.

National Heart, Lung, and Blood Institute and Office of Rare Diseases (NIH) definition:

NHLBI criteria are somewhat arbitrary and cases presenting early in pregnancy have been reported.

Endomyocardial biopsy is not routinely recommended due to low diagnostic yield.

No clear pathognomonic findings on endomyocardial biopsy

Refer to cardiologist for help with management of LV dysfunction and monitoring with serial echocardiography

1Tidswell M. Peripartum cardiomyopathy. Crit Care Clin. 2004;20:777 " 788. [View Abstract]2Hunt SA, Abraham WT, Chin MH. 2009 focused update incorporated into the ACC/AHA 2005 Guidelines for the Diagnosis and Management of Heart Failure in Adults: A report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines. Circulation. 2009;119(14):e391. [View Abstract]3Thorley KJ, McAinsh J. Levels of the beta-blockers atenolol and propranolol in the breast milk of women treated for hypertension in pregnancy. Biopharm Drug Dispos. 1983;4:299 " 301. [View Abstract]4Cooper W, Hernandez-Diaz S, Arbogast PG. Risk of major congenital malformations among study infants according to fetal exposure to antihypertensive medication during the first trimester. N Engl J Med. 2006;354:2443 " 2451. [View Abstract]5Sliwa K, Hilfiker-Kleiner D, Petrie MC. Current state of knowledge on aetiology, diagnosis, management, and therapy of peripartum cardiomyopathy: A position statement from the Heart Failure Association of the European Society of Cardiology Working Group on peripartum cardiomyopathy. Eur J Heart Fail. 2010;12(8):767 " 778. [View Abstract]6Lampert MB, Weinert L, Hibbard J. Contractile reserve in patients with peripartum cardiomyopathy and recovered left ventricular function. Am J Obstet Gynecol. 1997;176:189 " 195. [View Abstract]

1Elkayam U, Tummala P, Kalpana R. Maternal and fetal outcomes of subsequent pregnancies in women with peripartum cardiomyopathy. New Engl J Med. 2001;24:1567 " 1571.2Lee RV, Rosene-Montella K, Barbour LA, eds. Medical care of the pregnant patient. Philadelphia, PA: American College of Physicians, 2000.3Pearson G, Veille JC, Rahimtoola S. Peripartum cardiomyopathy: National Heart, Lung and Blood institute and Office of Rare Diseases (National Institute of Health) Workshop recommendations and review. JAMA. 2000;282:1183 " 1188. [View Abstract]4Ramaraj R, Sorrell VL. Peripartum cardiomyopathy: Causes, diagnosis, and treatment. Cleve Clin J Med. 2009:289 " 296. [View Abstract]5Whitehead S, Berg C, Chang J. Pregnancy-related mortality due to cardiomyopathy: United States, 1991 " 1997. Obstet Gynecol. 2003;102:1326 " 1331. [View Abstract]

16253001 dilated cardiomyopathy secondary to peripartum heart disease (disorder)

  • Peripartum cardiomyopathy (PPCM) is a dilated cardiomyopathy that is unique to pregnancy.
  • Idiopathic cardiomyopathy that presents towards the end of pregnancy or in the months following delivery
  • Diagnosis of exclusion, requiring the absence of other identifiable causes of heart failure
  • May recur in subsequent pregnancies

Epidemiology

  • True incidence is unknown
  • Incidence varies greatly by regionOccurs 1 per 2,500 " “4,000 in the USOccurs 1 per 1,000 in South AfricaOccurs 1 per 300 in Haiti
  • Occurs 1 per 2,500 " “4,000 in the US
  • Occurs 1 per 1,000 in South Africa
  • Occurs 1 per 300 in Haiti

Risk Factors

  • Advanced maternal age
  • Multiparity
  • Multiple gestation
  • African descent
  • Hypertension
  • Preeclampsia
  • Prolonged use of tocolytics (>4 weeks)
  • Poverty

Etiology

  • The cause of PPCM is unknown but may be multifactorial.
  • Possible contributing factors include:Abnormal immune response to pregnancyMyocarditisMaladaptive response to the hemodynamic stress of pregnancyStress-activated cytokinesActivation of a cascade involving oxidative stress, a prolactin-cleaving protease cathepsin D, and the hormone prolactinGenetics
  • Abnormal immune response to pregnancy
  • Myocarditis
  • Maladaptive response to the hemodynamic stress of pregnancy
  • Stress-activated cytokines
  • Activation of a cascade involving oxidative stress, a prolactin-cleaving protease cathepsin D, and the hormone prolactin
  • Genetics

Associated Conditions

  • ThromboembolismWomen with PPCM are at increased risk of thrombosis.
  • Arrhythmia
  • Women with PPCM are at increased risk of thrombosis.

Diagnosis

  • Most patients with PPCM are young and have no comorbid cardiopulmonary conditions.
  • They may appear clinically well, even with significant left ventricular (LV) dysfunction.
  • Many of the symptoms of early heart failure, such as palpitations, dyspnea, and edema, occur in normal pregnancy.
  • High index of suspicion required for prompt diagnosis
  • Development of cardiac failure in last month of pregnancy or within 5 months of delivery
  • Absence of an identifiable cause for cardiac failure (i.e., valvular heart disease, ischemia, pericardial disease)
  • Absence of recognizable heart disease prior to pregnancy
  • LV dysfunction demonstrated by classic echocardiographic criteria

History

  • Onset of symptoms after 36 weeks gestation or in first 5 months after delivery. Symptoms include:DyspneaCoughOrthopneaParoxysmal nocturnal dyspneaFatiguePalpitationsHemoptysisChest painAbdominal pain
  • No previous history of heart disease
  • Family history of cardiomyopathy
  • Assessment of New York Heart Association (NYHA) functional class
  • Dyspnea
  • Cough
  • Orthopnea
  • Paroxysmal nocturnal dyspnea
  • Fatigue
  • Palpitations
  • Hemoptysis
  • Chest pain
  • Abdominal pain

Physical Exam

  • Height and weight
  • BP may be normal or elevated
  • Tachycardia
  • Elevated jugular venous pressure
  • Third heart sound
  • Pulmonary rales
  • Peripheral edema
  • Ascites
  • Cardiomegaly
  • Hepatomegaly
  • Arrhythmias

Tests

  • Initial lab evaluation of all patients presenting with new onset heart failure should include:CBC, urinalysis, electrolytes (including calcium and magnesium), BUN, creatinine, fasting glucose, lipid profile, liver function tests, and TSH
  • Lab testing for other causes of dilated cardiomyopathy is reasonable if there is clinical suspicion.
  • Brain natriuretic peptide (BNP)Levels increase likely due to normal volume expansion in pregnancyHas not been validated for use in pregnancy
  • EKG may reveal left ventricular hypertrophy (LVH), left bundle branch block (LBBB), left or right atrial enlargementSigns of ischemic heart disease should be assessed.
  • CBC, urinalysis, electrolytes (including calcium and magnesium), BUN, creatinine, fasting glucose, lipid profile, liver function tests, and TSH
  • Levels increase likely due to normal volume expansion in pregnancy
  • Has not been validated for use in pregnancy
  • Signs of ischemic heart disease should be assessed.
  • Chest x-ray (CXR) findings will be the same as the nonpregnant population.
  • Echocardiogram shows depressed left ventricular ejection fraction (LVEF), decreased fractional shortening, and increased LV end-diastolic dimension.There are no echocardiographic features that distinguish PPCM from other forms of dilated cardiomyopathy.
  • If available, consider cardiac MRI as complementary tool " “ more accurate assessment of ventricular function, higher sensitivity for detection of LV thrombus. However, ability to differentiate types of myocarditis through use of gadolinium is not recommended in pregnancy.
  • There are no echocardiographic features that distinguish PPCM from other forms of dilated cardiomyopathy.

Differential Diagnosis

  • Cardiogenic pulmonary edema due to previously undiagnosed cardiomyopathy or valvular heart diseaseUnlike PPCM, symptoms would most likely present at 28 " “30 weeks, coinciding with peak blood volume in pregnancy.
  • Noncardiogenic pulmonary edema occurs in pregnancy in association with:Infection (especially pyelonephritis)PreeclampsiaUse of tocolytics or excessive IV fluids
  • Pulmonary hypertension may present with progressive dyspnea and signs of right-sided heart failure.
  • Unlike PPCM, symptoms would most likely present at 28 " “30 weeks, coinciding with peak blood volume in pregnancy.
  • Infection (especially pyelonephritis)
  • Preeclampsia
  • Use of tocolytics or excessive IV fluids

Treatment

  • For acute decompensationOxygenation to keep PaO2 >95%DiuresisTreat hypertension
  • Fetal monitoring
  • Oxygenation to keep PaO2 >95%
  • Diuresis
  • Treat hypertension

Medication

  • DiureticsLoop diuretics are safe in pregnancy and breastfeeding.
  • Hydralazine and long-acting nitratesFirst-choice drugs for afterload reduction during pregnancy (1)[C]
  • Beta-blockersNo role in acute treatmentUse in the postpartum period for patients who continue to have symptoms and LV dysfunction despite >2 weeks of standard heart failure therapy (2)[A]Avoid propranolol/atenolol in women who are breastfeeding as these medications are concentrated in breast milk (2)[B]
  • Loop diuretics are safe in pregnancy and breastfeeding.
  • First-choice drugs for afterload reduction during pregnancy (1)[C]
  • No role in acute treatment
  • Use in the postpartum period for patients who continue to have symptoms and LV dysfunction despite >2 weeks of standard heart failure therapy (2)[A]
  • Avoid propranolol/atenolol in women who are breastfeeding as these medications are concentrated in breast milk (2)[B]
  • ACE inhibitorsContraindicated in pregnancy (3)[A]First drug of choice postpartumSwitch from hydralazine to ACE-I postpartumSafe in breastfeeding except use with caution if very premature infantBest data for benefit in long-term survival
  • DigoxinMay be used safely in pregnancy for symptom controlLittle benefit in the acute setting
  • Aldosterone antagonistsSpironolactone should be avoided during pregnancy because of potential anti-androgenic effects on the fetus.
  • Contraindicated in pregnancy (3)[A]
  • First drug of choice postpartum
  • Switch from hydralazine to ACE-I postpartum
  • Safe in breastfeeding except use with caution if very premature infant
  • Best data for benefit in long-term survival
  • May be used safely in pregnancy for symptom control
  • Little benefit in the acute setting
  • Spironolactone should be avoided during pregnancy because of potential anti-androgenic effects on the fetus.

Additional Treatment

  • Restriction of sodium intake
  • Diuresis
  • Afterload reduction
  • Beta-blockers

In-Patient Considerations

  • Evidence of active heart failure
  • Hypoxia " “ O2 saturation <95%
  • Arrhythmia
  • Embolism

Ongoing Care

Follow-Up Recommendations

  • Excess catecholamines associated with labor may precipitate arrhythmia.
  • Maternal cardiac monitoring recommended during labor
  • Adequate pain management important and consideration should be given to early epidural.
  • Right heart catheterMonitoring in labor and delivery controversialRisks and benefits should be considered as in the nonpregnant population.Highest risk period for worsening heart failure is postpartum period due to fluid shifts occurring in the first few postpartum days.Strict attention to fluid management required
  • Monitoring in labor and delivery controversial
  • Risks and benefits should be considered as in the nonpregnant population.
  • Highest risk period for worsening heart failure is postpartum period due to fluid shifts occurring in the first few postpartum days.
  • Strict attention to fluid management required

Diet

Patient Education

  • Activity:May be limited by symptomsBedrest should not be recommended.NYHA functional class is an important predictor of prognosis.
  • May be limited by symptoms
  • Bedrest should not be recommended.
  • NYHA functional class is an important predictor of prognosis.

Prognosis

  • MortalityIn the US, ranges from 10% to 40%Black women are 6.4 times more likely to die from PPCM than white women.Most deaths occur in first 3 months postpartum and are due to progressive heart failure, arrhythmias, and thromboembolic events.
  • LV functionSeverity of LV dysfunction at presentation is the most important determinant of prognosis.LVEF normalizes within 6 months in 50%.<10% have progressively worsening LVEF and ultimately require heart transplant.Remainder of patients may continue to have some degree of LV dysfunction.Risk in subsequent pregnancy varies according to recovery of LV function.
  • In the US, ranges from 10% to 40%
  • Black women are 6.4 times more likely to die from PPCM than white women.
  • Most deaths occur in first 3 months postpartum and are due to progressive heart failure, arrhythmias, and thromboembolic events.
  • Severity of LV dysfunction at presentation is the most important determinant of prognosis.
  • LVEF normalizes within 6 months in 50%.
  • <10% have progressively worsening LVEF and ultimately require heart transplant.
  • Remainder of patients may continue to have some degree of LV dysfunction.
  • Risk in subsequent pregnancy varies according to recovery of LV function.
  • All patients with previous PPCM should undergo echocardiography (4)[B].If results are normal should perform dobutamine stress echocardiography.Patients with normal results on echocardiogram but decreased reserve on stress testing should be warned that they may not tolerate the hemodynamic stress associated with pregnancy (5)[B].
  • Patients who have persistent LV dysfunction have highest rate of death with a subsequent pregnancy and should be strongly advised against pregnancy in the future (6)[B].
  • Patients with full recovery can be told that the mortality rate is low, but chance of worsening LV function may be as high as 35%.
  • If results are normal should perform dobutamine stress echocardiography.
  • Patients with normal results on echocardiogram but decreased reserve on stress testing should be warned that they may not tolerate the hemodynamic stress associated with pregnancy (5)[B].

Complications

  • ThrombosisIncreased risk associated with PPCMNo definitive evidence regarding when or how to initiate anticoagulation for patients with PPCM or heart failure in generalGiven hypercoagulable state of pregnancy, reasonable to consider anticoagulation in:Patients with LVEF <35%Patients with thrombus on echocardiogramAtrial fibrillationHistory of a previous embolic event
  • ArrhythmiasSupraventricular arrhythmias most commonTreatment is same as in nonpregnant women.
  • Increased risk associated with PPCM
  • No definitive evidence regarding when or how to initiate anticoagulation for patients with PPCM or heart failure in general
  • Given hypercoagulable state of pregnancy, reasonable to consider anticoagulation in:Patients with LVEF <35%Patients with thrombus on echocardiogramAtrial fibrillationHistory of a previous embolic event
  • Patients with LVEF <35%
  • Patients with thrombus on echocardiogram
  • Atrial fibrillation
  • History of a previous embolic event
  • Supraventricular arrhythmias most common
  • Treatment is same as in nonpregnant women.

References

Additional Reading

Codes

ICD9

  • 674.50 Peripartum cardiomyopathy, unspecified as to episode of care or not applicable
  • 674.51 Peripartum cardiomyopathy, delivered, with or without mention of antepartum condition
  • 674.52 Peripartum cardiomyopathy, delivered, with mention of postpartum condition
  • 674.53 Peripartum cardiomyopathy, antepartum condition or complication
  • 674.54 Peripartum cardiomyopathy, postpartum condition or complication

ICD10

SNOMED

Clinical Pearls

  • Peripartum cardiomyopathy (PPCM) occurs at the end of pregnancy or in the months following delivery.
  • Left ventricular (LV) function normalizes within 6 months in approximately 50% of patients.
  • Patients with persistent LV dysfunction have the highest rate of death with a subsequent pregnancy and should be strongly advised against pregnancy in the future.
  • Patients with PPCM are at increased risk of thrombosis.