Peripartum cardiomyopathy
Encyclopedia
Peripartum cardiomyopathy (PPCM) is a form of dilated cardiomyopathy
that is defined as deterioration in cardiac function presenting typically between the last month of pregnancy
and up to five months postpartum. As with other forms of dilated cardiomyopathy, PPCM involves systolic dysfunction of the heart with a decrease of the left ventricular ejection fraction (EF) with associated congestive heart failure
and an increased risk of atrial and ventricular
arrhythmias, thromboembolism (blockage of a blood vessel by a blood clot), and even sudden cardiac death
. In essence, the heart muscle cannot contract forcefully enough to pump adequate amounts of blood for the needs of the body's vital organs.
PPCM is a diagnosis of exclusion, wherein patients have no prior history of heart disease and there are no other known possible causes of heart failure. Echocardiogram is used to both diagnose and monitor the effectiveness of treatment for PPCM.
The cause of PPCM is unknown. Currently, researchers are investigating cardiotropic viruses, autoimmunity
or immune system
dysfunction, other toxins that serve as triggers to immune system dysfunction, micronutrient
or trace mineral definciencies, and genetics as possible components that contribute to or cause the development of PPCM.
The process of PPCM begins with an unknown trigger (possibly a cardiotropic virus or other yet unidentified catalyst) that initiates an inflammatory process in the heart. Consequently, heart muscle cells are damaged; some die or become scar tissue. Scar tissue has no ability to contract; therefore, the effectiveness of the pumping action of the heart is decreased. Also, damage to the cytoskeletal framework of the heart causes the heart to enlarge, stretch or alter in shape, also decreasing the heart's systolic function or output. The initial inflammatory process appears to cause an autoimmune or immune dysfunctional process, which in turn fuels the initial inflammatory process. Progressive loss of heart muscle cells leads to eventual heart failure.
(shortness of breath on exertion), pitting edema
(swelling), cough, frequent night-time urination, excessive weight gain during the last month of pregnancy (1-2+ kg/week; two to four or more pounds per week), palpitations (sensation of racing heart-rate, skipping beats, long pauses between beats, or fluttering), and chest pain.
The shortness of breath is often described by PPCM patients as the inability to take a deep or full breath or to get enough air into the lungs. Also, patients often describe the need to prop themselves up overnight by using two or more pillows in order to breathe better. These symptoms, swelling, and/or cough may be indications of pulmonary edema
(fluid in the lungs) resulting from acute heart failure and PPCM.
Unfortunately, patients and clinicians sometimes dismiss early symptoms because they appear to be typical of normal pregnancy. Yet, early detection and treatment are critically important to the patient with PPCM. Delays in diagnosis and treatment of PPCM are associated with increased morbidity and mortality.
It is important to note that occasionally patients present with other signs or symptoms. This is demonstrated by one report of a woman with liver failure
five weeks postpartum who was being considered for liver transplant. An echocardiogram was performed and revealed PPCM and heart failure as the cause of her severe liver failure. Conventional heart failure medications were administered. She survived and completely recovered from both the liver failure and PPCM.
It is also quite common for women to present with evidence of having an embolus
(clot) passing from the heart to a vital organ, causing such complications as stroke
, loss of circulation to a limb, even coronary artery occlusion (blockage) with typical myocardial infarction
(heart attack).
For these reasons, it is paramount that clinicians hold a high suspicion of PPCM in any peri- or postpartum patient where unusual or unexplained symptoms or presentations occur.
The following is a screening tool developed by James D. Fett, MD, which may be useful to patients and medical professionals in determining the need to take further action to diagnose symptoms:
Treatment for PPCM is similar to treatment for congestive heart failure. Conventional heart failure treatment includes the use of diuretics, beta blockers (B-B), and angiotensin-converting enzyme inhibitors (ACE-I) after delivery. Diuretics, preferably furosemide
, help the body to get rid of excess water weight and also lower blood pressure. ACE-I and B-B improve blood circulation and contribute to the reversal of the immune system dysfunction associated with PPCM. If ACE-I is not well tolerated by the patient, it can be replaced by angiotensin receptor blockers (ARB). Hydralazine with nitrates may replace ACE-I in breastfeeding
mothers or before delivery; however, evidence suggests that this course of treatment may not be as effective as ACE-I but beneficial when necessary.
If EF is less than 35%, anticoagulation is indicated, as there is a greater risk of developing left ventricular thrombi (blood clots). Sometimes implantation of a left ventricular assist device (LVAD) or even heart transplant also becomes necessary.
It is important that the patient receives regular follow-up care including frequent echocardiograms to monitor improvement or the lack thereof, particularly after changes of medical treatment regimes.
Patients who do not respond to initial treatment, defined as left ventricular EF remaining below 20% at two months or below 40% at three months with conventional treatment may merit further investigation, including cardiac magnetic resonance imaging
(MRI), cardiac catheterization
, and endomyocardial biopsy for special staining and for viral polymerase chain reaction
(PCR) analysis. Antiviral therapy, immunoabsorption, intravenous gamma globulin
, or other immunomodulation therapy may then be considered accordingly, but following a controlled research-type protocol.
Since no one knows for sure exactly when to discontinue treatment, even when recovery occurs quickly, it is still recommended that both ACE-I and B-B be continued for at least one year after diagnosis.
It is a misconception that hope for recovery depends upon improvement or recovery within the first six to 12 months of diagnosis. Many women continue to improve or recover even years after diagnosis with continued medicinal treatment. Once fully recovered, if there is no subsequent pregnancy, the possibility of relapse or recurrence of heart failure is minimal.
Subsequent pregnancy should be avoided when left ventricular function has not recovered and the EF is lower than 55%. However, many women who have fully recovered from PPCM have gone on to have successful subsequent pregnancies. A significant study reports that the risk for recurrence of heart failure in recovered PPCM patients as a result of subsequent pregnancy is approximately 21% or better. The chance of relapse may be even smaller for those with normal contractile reserve as demonstrated by stress echocardiography. In any subsequent pregnancy, careful monitoring is necessary. Where relapse occurs, conventional treatment should be resumed, including hydralazine
with nitrates plus beta-blockers during pregnancy, or ACE-inhibitors plus beta-blockers following pregnancy.
Some studies assert that PPCM may be slightly more prevalent among older women who have had higher numbers of liveborn children and among women of older and younger extremes of childbearing age. However, a quarter to a third of PPCM patients are young women who have given birth for the first time.
While the use of tocolytic
agents or the development of preeclampsia (toxemia of pregnancy) and pregnancy-induced hypertension
(PIH) may contribute to the worsening of heart failure, they do not cause PPCM; the majority of women have developed PPCM who neither received tocolytics nor had preeclampsia nor PIH.
In short, PPCM can occur in any woman of any racial background, at any age during reproductive years, and in any pregnancy.
Dilated cardiomyopathy
Dilated cardiomyopathy or DCM is a condition in which the heart becomes weakened and enlarged and cannot pump blood efficiently. The decreased heart function can affect the lungs, liver, and other body systems....
that is defined as deterioration in cardiac function presenting typically between the last month of pregnancy
Pregnancy
Pregnancy refers to the fertilization and development of one or more offspring, known as a fetus or embryo, in a woman's uterus. In a pregnancy, there can be multiple gestations, as in the case of twins or triplets...
and up to five months postpartum. As with other forms of dilated cardiomyopathy, PPCM involves systolic dysfunction of the heart with a decrease of the left ventricular ejection fraction (EF) with associated congestive heart failure
Congestive heart failure
Heart failure often called congestive heart failure is generally defined as the inability of the heart to supply sufficient blood flow to meet the needs of the body. Heart failure can cause a number of symptoms including shortness of breath, leg swelling, and exercise intolerance. The condition...
and an increased risk of atrial and ventricular
Ventricle (heart)
In the heart, a ventricle is one of two large chambers that collect and expel blood received from an atrium towards the peripheral beds within the body and lungs. The Atria primes the Pump...
arrhythmias, thromboembolism (blockage of a blood vessel by a blood clot), and even sudden cardiac death
Sudden Cardiac Death
Sudden cardiac death is natural death from cardiac causes, heralded by abrupt loss of consciousness within one hour of the onset of acute symptoms. Other forms of sudden death may be noncardiac in origin...
. In essence, the heart muscle cannot contract forcefully enough to pump adequate amounts of blood for the needs of the body's vital organs.
PPCM is a diagnosis of exclusion, wherein patients have no prior history of heart disease and there are no other known possible causes of heart failure. Echocardiogram is used to both diagnose and monitor the effectiveness of treatment for PPCM.
The cause of PPCM is unknown. Currently, researchers are investigating cardiotropic viruses, autoimmunity
Autoimmunity
Autoimmunity is the failure of an organism to recognize its own constituent parts as self, which allows an immune response against its own cells and tissues. Any disease that results from such an aberrant immune response is termed an autoimmune disease...
or immune system
Immune system
An immune system is a system of biological structures and processes within an organism that protects against disease by identifying and killing pathogens and tumor cells. It detects a wide variety of agents, from viruses to parasitic worms, and needs to distinguish them from the organism's own...
dysfunction, other toxins that serve as triggers to immune system dysfunction, micronutrient
Micronutrient
Micronutrients are nutrients required by humans and other living things throughout life in small quantities to orchestrate a whole range of physiological functions, but which the organism itself cannot produce. For people, they include dietary trace minerals in amounts generally less than 100...
or trace mineral definciencies, and genetics as possible components that contribute to or cause the development of PPCM.
The process of PPCM begins with an unknown trigger (possibly a cardiotropic virus or other yet unidentified catalyst) that initiates an inflammatory process in the heart. Consequently, heart muscle cells are damaged; some die or become scar tissue. Scar tissue has no ability to contract; therefore, the effectiveness of the pumping action of the heart is decreased. Also, damage to the cytoskeletal framework of the heart causes the heart to enlarge, stretch or alter in shape, also decreasing the heart's systolic function or output. The initial inflammatory process appears to cause an autoimmune or immune dysfunctional process, which in turn fuels the initial inflammatory process. Progressive loss of heart muscle cells leads to eventual heart failure.
Signs and symptoms
Symptoms usually include one or more of the following: orthopnea (difficulty breathing while lying flat), dyspneaDyspnea
Dyspnea , shortness of breath , or air hunger, is the subjective symptom of breathlessness.It is a normal symptom of heavy exertion but becomes pathological if it occurs in unexpected situations...
(shortness of breath on exertion), pitting edema
Edema
Edema or oedema ; both words from the Greek , oídēma "swelling"), formerly known as dropsy or hydropsy, is an abnormal accumulation of fluid beneath the skin or in one or more cavities of the body that produces swelling...
(swelling), cough, frequent night-time urination, excessive weight gain during the last month of pregnancy (1-2+ kg/week; two to four or more pounds per week), palpitations (sensation of racing heart-rate, skipping beats, long pauses between beats, or fluttering), and chest pain.
The shortness of breath is often described by PPCM patients as the inability to take a deep or full breath or to get enough air into the lungs. Also, patients often describe the need to prop themselves up overnight by using two or more pillows in order to breathe better. These symptoms, swelling, and/or cough may be indications of pulmonary edema
Pulmonary edema
Pulmonary edema , or oedema , is fluid accumulation in the air spaces and parenchyma of the lungs. It leads to impaired gas exchange and may cause respiratory failure...
(fluid in the lungs) resulting from acute heart failure and PPCM.
Unfortunately, patients and clinicians sometimes dismiss early symptoms because they appear to be typical of normal pregnancy. Yet, early detection and treatment are critically important to the patient with PPCM. Delays in diagnosis and treatment of PPCM are associated with increased morbidity and mortality.
It is important to note that occasionally patients present with other signs or symptoms. This is demonstrated by one report of a woman with liver failure
Liver failure
Acute liver failure is the appearance of severe complications rapidly after the first signs of liver disease , and indicates that the liver has sustained severe damage . The complications are hepatic encephalopathy and impaired protein synthesis...
five weeks postpartum who was being considered for liver transplant. An echocardiogram was performed and revealed PPCM and heart failure as the cause of her severe liver failure. Conventional heart failure medications were administered. She survived and completely recovered from both the liver failure and PPCM.
It is also quite common for women to present with evidence of having an embolus
Embolus
An embolus is any detached, itinerant intravascular mass carried by circulation, which is capable of clogging arterial capillary beds at a site distant from its point of origin.By contrast there are non-traveling blockages that develop locally from vascular trauma or...
(clot) passing from the heart to a vital organ, causing such complications as stroke
Stroke
A stroke, previously known medically as a cerebrovascular accident , is the rapidly developing loss of brain function due to disturbance in the blood supply to the brain. This can be due to ischemia caused by blockage , or a hemorrhage...
, loss of circulation to a limb, even coronary artery occlusion (blockage) with typical myocardial infarction
Myocardial infarction
Myocardial infarction or acute myocardial infarction , commonly known as a heart attack, results from the interruption of blood supply to a part of the heart, causing heart cells to die...
(heart attack).
For these reasons, it is paramount that clinicians hold a high suspicion of PPCM in any peri- or postpartum patient where unusual or unexplained symptoms or presentations occur.
The following is a screening tool developed by James D. Fett, MD, which may be useful to patients and medical professionals in determining the need to take further action to diagnose symptoms:
| Focused medical history for PPCM screening, looking for early symptoms of heart failure, during last month of pregnancy: |
|---|
| 1. Orthopnea (difficulty breathing while lying flat): |
| a.) None = 0 points; b.) Need to elevate head = 1 point; c.) Need to elevate upper body 45º or more = 2 points |
| 2. Dyspnea (shortness of breath on exertion): |
| a.) None = 0 points; b.) Climbing 8 or more steps = 1 point; c.) Walking on level = 2 points |
| 3. Unexplained cough: |
| a.) None = 0 points; b.) Night-time = 1 point; c.) Day and night = 2 points |
| 4. Swelling (pitting edema) lower extremities: |
| a.) None = 0 points; b.) Below knee = 1 point; c.) Above and below knee and/or hands/face = 2 points. |
| 5. Excessive weight gain during last month of pregnancy: |
| a.) Under 2 pounds per week = 0 points; b.) 2 to 4 pounds per week = 1 point; c.) over 4 pounds per week = 2 points. |
| 6. Palpitations (sensation of irregular heart beats): |
| a.) None = 0 points; b.) When lying down at night = 1 point; c.) Day and night, any position = 2 points |
| Scoring and Action: |
| 0 - 2 = low risk—continue observation |
| 3 - 4 = mild risk—consider doing blood BNP and CRP; echocardiogram if BNP and CRP are elevated |
| 5 or more = high risk—do blood BNP, CRP, and echocardiogram |
Treatment
Early detection and treatment are associated with higher rates of recovery and decreased morbidity and mortality.Treatment for PPCM is similar to treatment for congestive heart failure. Conventional heart failure treatment includes the use of diuretics, beta blockers (B-B), and angiotensin-converting enzyme inhibitors (ACE-I) after delivery. Diuretics, preferably furosemide
Furosemide
Furosemide or frusemide is a loop diuretic used in the treatment of congestive heart failure and edema. It is most commonly marketed by Sanofi-Aventis under the brand name Lasix...
, help the body to get rid of excess water weight and also lower blood pressure. ACE-I and B-B improve blood circulation and contribute to the reversal of the immune system dysfunction associated with PPCM. If ACE-I is not well tolerated by the patient, it can be replaced by angiotensin receptor blockers (ARB). Hydralazine with nitrates may replace ACE-I in breastfeeding
Breastfeeding
Breastfeeding is the feeding of an infant or young child with breast milk directly from female human breasts rather than from a baby bottle or other container. Babies have a sucking reflex that enables them to suck and swallow milk. It is recommended that mothers breastfeed for six months or...
mothers or before delivery; however, evidence suggests that this course of treatment may not be as effective as ACE-I but beneficial when necessary.
If EF is less than 35%, anticoagulation is indicated, as there is a greater risk of developing left ventricular thrombi (blood clots). Sometimes implantation of a left ventricular assist device (LVAD) or even heart transplant also becomes necessary.
It is important that the patient receives regular follow-up care including frequent echocardiograms to monitor improvement or the lack thereof, particularly after changes of medical treatment regimes.
Patients who do not respond to initial treatment, defined as left ventricular EF remaining below 20% at two months or below 40% at three months with conventional treatment may merit further investigation, including cardiac magnetic resonance imaging
Magnetic resonance imaging
Magnetic resonance imaging , nuclear magnetic resonance imaging , or magnetic resonance tomography is a medical imaging technique used in radiology to visualize detailed internal structures...
(MRI), cardiac catheterization
Cardiac catheterization
Cardiac catheterization is the insertion of a catheter into a chamber or vessel of the heart. This is done for both investigational and interventional purposes...
, and endomyocardial biopsy for special staining and for viral polymerase chain reaction
Polymerase chain reaction
The polymerase chain reaction is a scientific technique in molecular biology to amplify a single or a few copies of a piece of DNA across several orders of magnitude, generating thousands to millions of copies of a particular DNA sequence....
(PCR) analysis. Antiviral therapy, immunoabsorption, intravenous gamma globulin
Gamma globulin
Gamma globulins are a class of globulins, identified by their position after serum protein electrophoresis. The most significant gamma globulins are immunoglobulins , more commonly known as antibodies, although some Igs are not gamma globulins, and some gamma globulins are not Igs.-Use as medical...
, or other immunomodulation therapy may then be considered accordingly, but following a controlled research-type protocol.
Since no one knows for sure exactly when to discontinue treatment, even when recovery occurs quickly, it is still recommended that both ACE-I and B-B be continued for at least one year after diagnosis.
Prognosis
The most recent studies indicate that with newer conventional heart failure treatment consisting of diuretics, ACE inhibitors and beta blockers, the survival rate is very high at 98% or better, and almost all PPCM patients improve with treatment. In the United States, over 50% of PPCM patients experience complete recovery of heart function (EF 55% or greater). Almost all recovered patients are eventually able to discontinue medications with no resulting relapse and have normal life expectancy.It is a misconception that hope for recovery depends upon improvement or recovery within the first six to 12 months of diagnosis. Many women continue to improve or recover even years after diagnosis with continued medicinal treatment. Once fully recovered, if there is no subsequent pregnancy, the possibility of relapse or recurrence of heart failure is minimal.
Subsequent pregnancy should be avoided when left ventricular function has not recovered and the EF is lower than 55%. However, many women who have fully recovered from PPCM have gone on to have successful subsequent pregnancies. A significant study reports that the risk for recurrence of heart failure in recovered PPCM patients as a result of subsequent pregnancy is approximately 21% or better. The chance of relapse may be even smaller for those with normal contractile reserve as demonstrated by stress echocardiography. In any subsequent pregnancy, careful monitoring is necessary. Where relapse occurs, conventional treatment should be resumed, including hydralazine
Hydralazine
Hydralazine is a direct-acting smooth muscle relaxant used to treat hypertension by acting as a vasodilator primarily in arteries and arterioles...
with nitrates plus beta-blockers during pregnancy, or ACE-inhibitors plus beta-blockers following pregnancy.
Epidemiology
It is estimated that the incidence of PPCM in the United States is between 1 in 1300 to 4000 live births. While it can affect women of all races, it is more prevalent in some countries; for example, estimates suggest that PPCM occurs at rates of one in 1000 live births in South African Bantus, and as high as one in 300 in Haiti.Some studies assert that PPCM may be slightly more prevalent among older women who have had higher numbers of liveborn children and among women of older and younger extremes of childbearing age. However, a quarter to a third of PPCM patients are young women who have given birth for the first time.
While the use of tocolytic
Tocolytic
Tocolytics are medications used to suppress premature labor . They are given when delivery would result in premature birth...
agents or the development of preeclampsia (toxemia of pregnancy) and pregnancy-induced hypertension
Pregnancy-induced hypertension
Gestational hypertension or pregnancy-induced hypertension is defined as the development of new arterial hypertension in a pregnant woman after 20 weeks gestation without the presence of protein in the urine.-Conditions:...
(PIH) may contribute to the worsening of heart failure, they do not cause PPCM; the majority of women have developed PPCM who neither received tocolytics nor had preeclampsia nor PIH.
In short, PPCM can occur in any woman of any racial background, at any age during reproductive years, and in any pregnancy.