Cardiology Patient Page
Peripartum Cardiomyopathy
1. Michael M. Givertz, MD
+ Author Affiliations
1. From the Cardiovascular Division, Department of Medicine, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA.
1. Correspondence to Michael M. Givertz, MD, Cardiovascular Division, Brigham and Women’s Hospital, 75 Francis St, Boston, MA 02115. E-mail mgivertz@partners.org
Introduction
Peripartum cardiomyopathy (PPCM) is an uncommon disorder associated with pregnancy in which the heart dilates and weakens, leading to symptoms of heart failure. PPCM may be difficult to diagnose because symptoms of heart failure can mimic those of pregnancy. Affected women may recover normal heart function, stabilize on medicines, or progress to severe heart failure requiring mechanical support or heart transplantation. Even when the heart recovers, another pregnancy may be associated with a risk of recurrent heart failure. Important research is underway to understand the cause of PPCM and to develop new treatments.
Normal Cardiac Changes During Pregnancy
During normal pregnancy, several cardiovascular changes take place to increase blood flow to the placenta and developing fetus. The total volume of circulating blood increases by more than 40%. The heart rate increases from an average of 75 beats per minute before pregnancy to nearly 90 beats per minute in the third trimester. A similar increase in the amount of blood ejected with each beat also occurs. Average blood pressure increases slightly, while the blood vessels relax to accommodate increased blood flow. During the stress of labor, these physiological changes increase further and then return to normal by about 6 weeks after delivery. Several hormones released from the uterus, kidney, heart, and lungs circulate in the blood and stimulate these changes.
Definitions
Cardiomyopathy (literally heart muscle disease) is a general term used to describe an abnormality of heart muscle function that can lead to symptoms of heart failure. Patients with cardiomyopathy may also be at risk for abnormal heart rhythms (arrhythmias) and even sudden death. PPCM is a form of dilated cardiomyopathy in which the heart chambers enlarge or dilate and the muscle weakens, leading to reduced blood flow and increased heart pressures in the heart.
PPCM is diagnosed when the following 3 criteria are met:
1. Heart failure develops in the last month of pregnancy or within 5 months of delivery.
2. Heart pumping function is reduced, with an ejection fraction (EF) less than 45% (typically measured by an echocardiogram as described below).
3. No other cause for heart failure with reduced EF can be found.
PPCM is rare in the United States, Canada, and Europe, with an estimated case rate of 1 per 2500 to 4000 live births.1,2 This translates to 1000 to 1300 woman developing PPCM each year in the United States. Most patients (80%) present within 3 months of delivery, with the minority presenting in the last month of pregnancy (10%) or 4 to 5 months postpartum (10%). Some specialists believe that the definition of timing is too strict and that patients who develop symptoms of heart failure during the second or early third trimester should also be diagnosed with PPCM.3 In some countries, PPCM is much more common (eg, 1 in 1000 live births in South Africa and up to 1 in 300 live births in Haiti). This may be related to differences in diet, lifestyle, other medical conditions, or genetics.
Heart failure is a common cardiac condition in which the heart is unable to pump blood at a sufficient rate to meet the demands of the body. Most patients who develop heart failure have had a prior injury or stress on the heart that caused the heart to weaken. In the case of PPCM, the stress is presumed to be pregnancy, but the mechanisms are poorly understood. Other potential contributors include high blood pressure, heart attack, heart valve dysfunction, exposure to toxins such as alcohol or chemotherapy, or a genetic mutation that leads to heart failure in adulthood. Other medical conditions such as diabetes mellitus, obesity, and chronic kidney disease can worsen heart failure.
Risk Factors
Several risk factors are associated with PPCM. These include the following:
Older maternal age
Multiparity (1 or more prior pregnancies)
Multifetal pregnancy (eg, twins)
African descent
High blood pressure
Prior toxin exposure (eg, cocaine)
Use of certain medications to prevent premature labor
It is important to note that although PPCM is more likely to occur in a woman over the age of 30 who is pregnant with twins and has had prior pregnancies, PPCM can also occur in a young woman who is pregnant with her first child.
What Is the Cause of PPCM?
The underlying cause of PPCM has not been clearly defined. Heart biopsies performed in women with PPCM have shown inflammation in 10% to 75% of cases. This may be attributable to a prior viral illness or abnormal immune response, although there is no evidence that antiviral or immunosuppression medications improve outcomes. Other potential causes of PPCM include nutritional deficiencies, coronary artery spasm, small-vessel disease, and defective antioxidant defenses. Genetics may also play a role in the tendency to develop PPCM.
Symptoms
The major symptoms of PPCM are those of heart failure and include fatigue, shortness of breath, and fluid retention. Because there is a significant overlap between symptoms related to pregnancy, especially toward the end of the third trimester or after delivery, and heart failure (Table1), the diagnosis may be initially missed or delayed.
Fatigue is the sensation of feeling tired or weak and being unable to perform usual daily activities such as showering or dressing without stopping to rest. Fatigue can be worse in the afternoon or after engaging in a strenuous activity.
Shortness of breath can be defined as breathlessness with activities such as walking a block on flat ground or up 1 flight of stairs. As heart failure progresses, patients may become short of breath while eating, talking, or resting. Some patients will also develop difficulty breathing at night, causing them to wake up with cough or congestion or requiring them to sleep on pillows or in a recliner to breathe.
Fluid retention can manifest as swelling in the legs (also called edema); swelling in the abdomen with bloating, pain, loss of appetite, or feeling full; chest congestion leading to cough and shortness of breath (as discussed above); increased urination at night; and weight gain.
Patients with heart failure caused by PPCM may also complain of palpitations or skipped beats, racing heart, lightheadedness, or almost fainting. Rarely, patients with PPCM may present with symptoms related to a blood clot that breaks away from the heart and goes to a vital organ such as the brain, causing a stroke, or coronary artery, causing a heart attack. Blood clots to the lungs may cause shortness of breath, lightheadedness, racing heart, or coughing up blood.
In most patients, heart failure symptoms can be relieved or stabilized with medications, along with changes in lifestyle and diet (see Management below).
Evaluation
The evaluation of PPCM begins with a complete medical history and physical examination. The keys to the medical history are ruling out heart disease that may have predated pregnancy; identifying other potential causes or precipitants of heart failure, including a family history of heart disease; and defining symptom severity. Similarly, the physical examination may help to uncover other noncardiac conditions associated with cardiomyopathy while assessing for signs of reduced heart function and fluid retention. In addition, the history and physical examination may detect complications associated with PPCM such as loss of circulation to a limb caused by a blood clot.
Laboratory blood tests are a standard part of the evaluation of any patient with the diagnosis of cardiomyopathy or heart failure, including patients with PPCM. This includes tests to assess kidney, liver, and thyroid function; tests to assess electrolytes, including sodium and potassium; and a complete blood count to look for anemia or evidence of infection. In addition, markers of cardiac injury and stress such as troponin and B-type natriuretic peptide can be used to assess level of risk. Laboratory tests may also be done to rule out other causes of cardiomyopathy such lupus and human immunodeficiency virus.
Other tests that are typically performed in patients with PPCM include the following:
Chest x-ray to look for enlargement of the heart and fluid in the lungs.
Chest computed tomography (CT) scan to rule out blood clots in the lungs, which can occur during or immediately after pregnancy and cause symptoms similar to those of PPCM such as chest pain, shortness of breath, and palpitations.
Electrocardiogram (heart tracing) to assess heart rate and rhythm, to look for abnormal electric conduction, and to rule out a heart attack.
Echocardiogram (heart ultrasound) to assess the size and function of the heart and to exclude other causes of heart failure such as valve dysfunction or a congenital heart defect. Once a diagnosis of PPCM is made, follow-up echocardiograms are typically performed to assess the response to medical therapy and to monitor for heart recovery. The primary measure of heart function is the left ventricular EF. This is the percentage of blood ejected from the heart with each beat; it normally ranges from 50% to 70%. Many laboratories report an estimated EF (eg, 45%–50%); others provide a calculated EF (eg, 47%) using a formula.
Cardiac catheterization with coronary angiography, an invasive procedure, to assess the severity of heart failure and to rule out blockages or dissection of the coronary arteries. At the same time, a heart biopsy can be performed to look for an alternative cause of cardiomyopathy.
Other imaging studies to look for infla
Cardiology Patient Page
Peripartum Cardiomyopathy
1. Michael M. Givertz, MD
+ Author Affiliations
1. From the Cardiovascular Division, Department of Medicine, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA.
1. Correspondence to Michael M. Givertz, MD, Cardiovascular Division, Brigham and Women’s Hospital, 75 Francis St, Boston, MA 02115. E-mail mgivertz@partners.org
Introduction
Peripartum cardiomyopathy (PPCM) is an uncommon disorder associated with pregnancy in which the heart dilates and weakens, leading to symptoms of heart failure. PPCM may be difficult to diagnose because symptoms of heart failure can mimic those of pregnancy. Affected women may recover normal heart function, stabilize on medicines, or progress to severe heart failure requiring mechanical support or heart transplantation. Even when the heart recovers, another pregnancy may be associated with a risk of recurrent heart failure. Important research is underway to understand the cause of PPCM and to develop new treatments.
Normal Cardiac Changes During Pregnancy
During normal pregnancy, several cardiovascular changes take place to increase blood flow to the placenta and developing fetus. The total volume of circulating blood increases by more than 40%. The heart rate increases from an average of 75 beats per minute before pregnancy to nearly 90 beats per minute in the third trimester. A similar increase in the amount of blood ejected with each beat also occurs. Average blood pressure increases slightly, while the blood vessels relax to accommodate increased blood flow. During the stress of labor, these physiological changes increase further and then return to normal by about 6 weeks after delivery. Several hormones released from the uterus, kidney, heart, and lungs circulate in the blood and stimulate these changes.
Definitions
Cardiomyopathy (literally heart muscle disease) is a general term used to describe an abnormality of heart muscle function that can lead to symptoms of heart failure. Patients with cardiomyopathy may also be at risk for abnormal heart rhythms (arrhythmias) and even sudden death. PPCM is a form of dilated cardiomyopathy in which the heart chambers enlarge or dilate and the muscle weakens, leading to reduced blood flow and increased heart pressures in the heart.
PPCM is diagnosed when the following 3 criteria are met:
1. Heart failure develops in the last month of pregnancy or within 5 months of delivery.
2. Heart pumping function is reduced, with an ejection fraction (EF) less than 45% (typically measured by an echocardiogram as described below).
3. No other cause for heart failure with reduced EF can be found.
PPCM is rare in the United States, Canada, and Europe, with an estimated case rate of 1 per 2500 to 4000 live births.1,2 This translates to 1000 to 1300 woman developing PPCM each year in the United States. Most patients (80%) present within 3 months of delivery, with the minority presenting in the last month of pregnancy (10%) or 4 to 5 months postpartum (10%). Some specialists believe that the definition of timing is too strict and that patients who develop symptoms of heart failure during the second or early third trimester should also be diagnosed with PPCM.3 In some countries, PPCM is much more common (eg, 1 in 1000 live births in South Africa and up to 1 in 300 live births in Haiti). This may be related to differences in diet, lifestyle, other medical conditions, or genetics.
Heart failure is a common cardiac condition in which the heart is unable to pump blood at a sufficient rate to meet the demands of the body. Most patients who develop heart failure have had a prior injury or stress on the heart that caused the heart to weaken. In the case of PPCM, the stress is presumed to be pregnancy, but the mechanisms are poorly understood. Other potential contributors include high blood pressure, heart attack, heart valve dysfunction, exposure to toxins such as alcohol or chemotherapy, or a genetic mutation that leads to heart failure in adulthood. Other medical conditions such as diabetes mellitus, obesity, and chronic kidney disease can worsen heart failure.
Risk Factors
Several risk factors are associated with PPCM. These include the following:
Older maternal age
Multiparity (1 or more prior pregnancies)
Multifetal pregnancy (eg, twins)
African descent
High blood pressure
Prior toxin exposure (eg, cocaine)
Use of certain medications to prevent premature labor
It is important to note that although PPCM is more likely to occur in a woman over the age of 30 who is pregnant with twins and has had prior pregnancies, PPCM can also occur in a young woman who is pregnant with her first child.
What Is the Cause of PPCM?
The underlying cause of PPCM has not been clearly defined. Heart biopsies performed in women with PPCM have shown inflammation in 10% to 75% of cases. This may be attributable to a prior viral illness or abnormal immune response, although there is no evidence that antiviral or immunosuppression medications improve outcomes. Other potential causes of PPCM include nutritional deficiencies, coronary artery spasm, small-vessel disease, and defective antioxidant defenses. Genetics may also play a role in the tendency to develop PPCM.
Symptoms
The major symptoms of PPCM are those of heart failure and include fatigue, shortness of breath, and fluid retention. Because there is a significant overlap between symptoms related to pregnancy, especially toward the end of the third trimester or after delivery, and heart failure (Table1), the diagnosis may be initially missed or delayed.
Fatigue is the sensation of feeling tired or weak and being unable to perform usual daily activities such as showering or dressing without stopping to rest. Fatigue can be worse in the afternoon or after engaging in a strenuous activity.
Shortness of breath can be defined as breathlessness with activities such as walking a block on flat ground or up 1 flight of stairs. As heart failure progresses, patients may become short of breath while eating, talking, or resting. Some patients will also develop difficulty breathing at night, causing them to wake up with cough or congestion or requiring them to sleep on pillows or in a recliner to breathe.
Fluid retention can manifest as swelling in the legs (also called edema); swelling in the abdomen with bloating, pain, loss of appetite, or feeling full; chest congestion leading to cough and shortness of breath (as discussed above); increased urination at night; and weight gain.
Patients with heart failure caused by PPCM may also complain of palpitations or skipped beats, racing heart, lightheadedness, or almost fainting. Rarely, patients with PPCM may present with symptoms related to a blood clot that breaks away from the heart and goes to a vital organ such as the brain, causing a stroke, or coronary artery, causing a heart attack. Blood clots to the lungs may cause shortness of breath, lightheadedness, racing heart, or coughing up blood.
In most patients, heart failure symptoms can be relieved or stabilized with medications, along with changes in lifestyle and diet (see Management below).
Evaluation
The evaluation of PPCM begins with a complete medical history and physical examination. The keys to the medical history are ruling out heart disease that may have predated pregnancy; identifying other potential causes or precipitants of heart failure, including a family history of heart disease; and defining symptom severity. Similarly, the physical examination may help to uncover other noncardiac conditions associated with cardiomyopathy while assessing for signs of reduced heart function and fluid retention. In addition, the history and physical examination may detect complications associated with PPCM such as loss of circulation to a limb caused by a blood clot.
Laboratory blood tests are a standard part of the evaluation of any patient with the diagnosis of cardiomyopathy or heart failure, including patients with PPCM. This includes tests to assess kidney, liver, and thyroid function; tests to assess electrolytes, including sodium and potassium; and a complete blood count to look for anemia or evidence of infection. In addition, markers of cardiac injury and stress such as troponin and B-type natriuretic peptide can be used to assess level of risk. Laboratory tests may also be done to rule out other causes of cardiomyopathy such lupus and human immunodeficiency virus.
Other tests that are typically performed in patients with PPCM include the following:
Chest x-ray to look for enlargement of the heart and fluid in the lungs.
Chest computed tomography (CT) scan to rule out blood clots in the lungs, which can occur during or immediately after pregnancy and cause symptoms similar to those of PPCM such as chest pain, shortness of breath, and palpitations.
Electrocardiogram (heart tracing) to assess heart rate and rhythm, to look for abnormal electric conduction, and to rule out a heart attack.
Echocardiogram (heart ultrasound) to assess the size and function of the heart and to exclude other causes of heart failure such as valve dysfunction or a congenital heart defect. Once a diagnosis of PPCM is made, follow-up echocardiograms are typically performed to assess the response to medical therapy and to monitor for heart recovery. The primary measure of heart function is the left ventricular EF. This is the percentage of blood ejected from the heart with each beat; it normally ranges from 50% to 70%. Many laboratories report an estimated EF (eg, 45%–50%); others provide a calculated EF (eg, 47%) using a formula.
Cardiac catheterization with coronary angiography, an invasive procedure, to assess the severity of heart failure and to rule out blockages or dissection of the coronary arteries. At the same time, a heart biopsy can be performed to look for an alternative cause of cardiomyopathy.
Other imaging studies to look for infla
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Cardiology Patient Page
Peripartum Cardiomyopathy
1. Michael M. Givertz, MD
+ Author Affiliations
1. From the Cardiovascular Division, Department of Medicine, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA.
1. Correspondence to Michael M. Givertz, MD, Cardiovascular Division, Brigham and Women’s Hospital, 75 Francis St, Boston, MA 02115. E-mail mgivertz@partners.org
Introduction
Peripartum cardiomyopathy (PPCM) is an uncommon disorder associated with pregnancy in which the heart dilates and weakens, leading to symptoms of heart failure. PPCM may be difficult to diagnose because symptoms of heart failure can mimic those of pregnancy. Affected women may recover normal heart function, stabilize on medicines, or progress to severe heart failure requiring mechanical support or heart transplantation. Even when the heart recovers, another pregnancy may be associated with a risk of recurrent heart failure. Important research is underway to understand the cause of PPCM and to develop new treatments.
Normal Cardiac Changes During Pregnancy
During normal pregnancy, several cardiovascular changes take place to increase blood flow to the placenta and developing fetus. The total volume of circulating blood increases by more than 40%. The heart rate increases from an average of 75 beats per minute before pregnancy to nearly 90 beats per minute in the third trimester. A similar increase in the amount of blood ejected with each beat also occurs. Average blood pressure increases slightly, while the blood vessels relax to accommodate increased blood flow. During the stress of labor, these physiological changes increase further and then return to normal by about 6 weeks after delivery. Several hormones released from the uterus, kidney, heart, and lungs circulate in the blood and stimulate these changes.
Definitions
Cardiomyopathy (literally heart muscle disease) is a general term used to describe an abnormality of heart muscle function that can lead to symptoms of heart failure. Patients with cardiomyopathy may also be at risk for abnormal heart rhythms (arrhythmias) and even sudden death. PPCM is a form of dilated cardiomyopathy in which the heart chambers enlarge or dilate and the muscle weakens, leading to reduced blood flow and increased heart pressures in the heart.
PPCM is diagnosed when the following 3 criteria are met:
1. Heart failure develops in the last month of pregnancy or within 5 months of delivery.
2. Heart pumping function is reduced, with an ejection fraction (EF) less than 45% (typically measured by an echocardiogram as described below).
3. No other cause for heart failure with reduced EF can be found.
PPCM is rare in the United States, Canada, and Europe, with an estimated case rate of 1 per 2500 to 4000 live births.1,2 This translates to 1000 to 1300 woman developing PPCM each year in the United States. Most patients (80%) present within 3 months of delivery, with the minority presenting in the last month of pregnancy (10%) or 4 to 5 months postpartum (10%). Some specialists believe that the definition of timing is too strict and that patients who develop symptoms of heart failure during the second or early third trimester should also be diagnosed with PPCM.3 In some countries, PPCM is much more common (eg, 1 in 1000 live births in South Africa and up to 1 in 300 live births in Haiti). This may be related to differences in diet, lifestyle, other medical conditions, or genetics.
Heart failure is a common cardiac condition in which the heart is unable to pump blood at a sufficient rate to meet the demands of the body. Most patients who develop heart failure have had a prior injury or stress on the heart that caused the heart to weaken. In the case of PPCM, the stress is presumed to be pregnancy, but the mechanisms are poorly understood. Other potential contributors include high blood pressure, heart attack, heart valve dysfunction, exposure to toxins such as alcohol or chemotherapy, or a genetic mutation that leads to heart failure in adulthood. Other medical conditions such as diabetes mellitus, obesity, and chronic kidney disease can worsen heart failure.
Risk Factors
Several risk factors are associated with PPCM. These include the following:
Older maternal age
Multiparity (1 or more prior pregnancies)
Multifetal pregnancy (eg, twins)
African descent
High blood pressure
Prior toxin exposure (eg, cocaine)
Use of certain medications to prevent premature labor
It is important to note that although PPCM is more likely to occur in a woman over the age of 30 who is pregnant with twins and has had prior pregnancies, PPCM can also occur in a young woman who is pregnant with her first child.
What Is the Cause of PPCM?
The underlying cause of PPCM has not been clearly defined. Heart biopsies performed in women with PPCM have shown inflammation in 10% to 75% of cases. This may be attributable to a prior viral illness or abnormal immune response, although there is no evidence that antiviral or immunosuppression medications improve outcomes. Other potential causes of PPCM include nutritional deficiencies, coronary artery spasm, small-vessel disease, and defective antioxidant defenses. Genetics may also play a role in the tendency to develop PPCM.
Symptoms
The major symptoms of PPCM are those of heart failure and include fatigue, shortness of breath, and fluid retention. Because there is a significant overlap between symptoms related to pregnancy, especially toward the end of the third trimester or after delivery, and heart failure (Table1), the diagnosis may be initially missed or delayed.
Fatigue is the sensation of feeling tired or weak and being unable to perform usual daily activities such as showering or dressing without stopping to rest. Fatigue can be worse in the afternoon or after engaging in a strenuous activity.
Shortness of breath can be defined as breathlessness with activities such as walking a block on flat ground or up 1 flight of stairs. As heart failure progresses, patients may become short of breath while eating, talking, or resting. Some patients will also develop difficulty breathing at night, causing them to wake up with cough or congestion or requiring them to sleep on pillows or in a recliner to breathe.
Fluid retention can manifest as swelling in the legs (also called edema); swelling in the abdomen with bloating, pain, loss of appetite, or feeling full; chest congestion leading to cough and shortness of breath (as discussed above); increased urination at night; and weight gain.
Patients with heart failure caused by PPCM may also complain of palpitations or skipped beats, racing heart, lightheadedness, or almost fainting. Rarely, patients with PPCM may present with symptoms related to a blood clot that breaks away from the heart and goes to a vital organ such as the brain, causing a stroke, or coronary artery, causing a heart attack. Blood clots to the lungs may cause shortness of breath, lightheadedness, racing heart, or coughing up blood.
In most patients, heart failure symptoms can be relieved or stabilized with medications, along with changes in lifestyle and diet (see Management below).
Evaluation
The evaluation of PPCM begins with a complete medical history and physical examination. The keys to the medical history are ruling out heart disease that may have predated pregnancy; identifying other potential causes or precipitants of heart failure, including a family history of heart disease; and defining symptom severity. Similarly, the physical examination may help to uncover other noncardiac conditions associated with cardiomyopathy while assessing for signs of reduced heart function and fluid retention. In addition, the history and physical examination may detect complications associated with PPCM such as loss of circulation to a limb caused by a blood clot.
Laboratory blood tests are a standard part of the evaluation of any patient with the diagnosis of cardiomyopathy or heart failure, including patients with PPCM. This includes tests to assess kidney, liver, and thyroid function; tests to assess electrolytes, including sodium and potassium; and a complete blood count to look for anemia or evidence of infection. In addition, markers of cardiac injury and stress such as troponin and B-type natriuretic peptide can be used to assess level of risk. Laboratory tests may also be done to rule out other causes of cardiomyopathy such lupus and human immunodeficiency virus.
Other tests that are typically performed in patients with PPCM include the following:
Chest x-ray to look for enlargement of the heart and fluid in the lungs.
Chest computed tomography (CT) scan to rule out blood clots in the lungs, which can occur during or immediately after pregnancy and cause symptoms similar to those of PPCM such as chest pain, shortness of breath, and palpitations.
Electrocardiogram (heart tracing) to assess heart rate and rhythm, to look for abnormal electric conduction, and to rule out a heart attack.
Echocardiogram (heart ultrasound) to assess the size and function of the heart and to exclude other causes of heart failure such as valve dysfunction or a congenital heart defect. Once a diagnosis of PPCM is made, follow-up echocardiograms are typically performed to assess the response to medical therapy and to monitor for heart recovery. The primary measure of heart function is the left ventricular EF. This is the percentage of blood ejected from the heart with each beat; it normally ranges from 50% to 70%. Many laboratories report an estimated EF (eg, 45%–50%); others provide a calculated EF (eg, 47%) using a formula.
Cardiac catheterization with coronary angiography, an invasive procedure, to assess the severity of heart failure and to rule out blockages or dissection of the coronary arteries. At the same time, a heart biopsy can be performed to look for an alternative cause of cardiomyopathy.
Other imaging studies to look for infla
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