Peripartum cardiomyopathy | |
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Other names | Post-partum cardiomyopathy [1] |
Specialty | Obstetrics & Gynecology, Cardiology |
Symptoms | orthopnea, swollen ankles or feet, cough, chest pain, palpitations, fatigue |
Complications | Thromboembolic events, Cardiogenic Shock |
Usual onset | In the last month of pregnancy or up to 5 months postpartum |
Duration | Varies |
Risk factors | Maternal age 35+; high blood pressure; multiple gestations |
Differential diagnosis | Takotsubo Cardiomyopathy, Familial Cardiomyopathy, Pre-existing Cardiomyopathy, Valvular Heart Disease, Congenital Heart Disease |
Peripartum cardiomyopathy (PPCM) is a form of dilated cardiomyopathy that is defined as a deterioration in cardiac function presenting typically between the last month of pregnancy and up to six 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. [2] [3] [4] [5] [6]
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. [2] [3] [4] [5] [6]
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 deficiencies, and genetics as possible components that contribute to or cause the development of PPCM. There is a relation with eclampsia and hypertension during pregnancy. [2] [7] [4] [8]
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. [9]
There has been increased research into the "toxic hormonal environment" that generates in late pregnancy as a contributor to the development of PPCM. Prolactin levels increase during late pregnancy and in the 6 weeks following birth. The 16 kilodalton N-terminal fragment of prolactin hormone has been implicated to have a causal role in genetically susceptible individuals. Thus, therapeutic interventions that block the prolactin pathway and prevent the generation of this fragment are being investigated as potential treatments to stop disease progression in PPCM. [10]
Special considerations should be made for delivery when PPCM diagnosis is made before birth. A multi-disciplinary team should be assembled including experts in obstetrics, cardiology, maternal fetal medicine, and anesthesiology. Stable patients can be delivered vaginally unless there are other obstetric reasons for cesarean section. Attempts to stabilize the mother to delay birth and minimize potential complications of premature birth is a reasonable strategy. Following delivery, due to the increase in venous return, patients need to be closely monitored for fluid overload and pulmonary edema. [11]
Symptoms usually include one or more of the following: orthopnea (difficulty breathing while lying flat), dyspnea (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), chest pain or tightness, fatigue and light-headedness. [2] [4] [12]
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.[ citation needed ]
Physical examination may reveal jugular venous distention, displaced apical impulse, a third heart sound, murmur consistent with mitral regurgitation, tachypnea, tachycardia, pulmonary rales, and peripheral edema. [7] [11]
Diagnosis may be delayed or dismissed as early symptoms may be interpreted as being typical of normal pregnancy. Delays in diagnosis and treatment of PPCM are associated with increased morbidity and mortality. [2] [4] [5] [6] [13] [14]
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). [15]
Peripartum cardiomyopathy is now a leading cause of maternal death in many parts of the United States and around the world [16] [17] . Approximately 60% of cases of cardiogenic shock during pregnancy or in the early postpartum period are caused by peripartum cardiomyopathy [18] .
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. [2] [4] [9] [19] [15]
The following screening tool may be useful to patients and medical professionals in determining the need to take further action to diagnose symptoms: [20]
Focused medical history for PPCM screening, looking for early symptoms of heart failure, during last month of pregnancy: |
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1. Orthopnea (difficulty breathing while lying flat): |
a.) None = 0 points; b.) Need to elevate head = 1 point; c.) Need to elevate upper body 45° 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 |
Initial evaluation should include blood work. PPCM is a diagnosis of exclusion, meaning that other conditions need to be evaluated and ruled out before this diagnosis will be used. Anemia, electrolyte abnormalities, thyroid dysfunction, and renal or liver dysfunction need to be ruled out. Further testing can also include a chest x-ray, brain natriuretic peptide levels, EKG, echocardiogram, cardiac MRI, and cardiac catheterization. There is no specific test to get a diagnosis of PPCM. [7]
Early detection and treatment are associated with higher rates of recovery and decreased morbidity and mortality. [2] [4] [5]
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. Fluid restriction is also used to help limit excess water in the body. Using diuretics during pregnancy needs to be done carefully, as diuretics can impair blood flow to the placenta which could potentially cause harm to the fetus. Hydrochlorothiazide and furosemide are safe during pregnancy and breastfeeding. [7] 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). ACE-I and ARBs are contraindicated during pregnancy and breastfeeding, but can be used during the postpartum period as long as breastfeeding is not occurring. [7] 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. [2] [4] [5] [6] [9] [14] [22]
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. [2] [5] [6] [9] [23]
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. [9]
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. [4] When considering discontinuing heart failure medications, they should be weaned gradually over time and patients should be closely monitored during this time. [11]
PPCM patients have an increased risk for sudden death and it is seen that they benefit from implantable cardioverter defibrillator (ICD) and cardiac resynchronization therapy to prevent sudden cardiac death. However, in view of reversible cardiomyopathy, sometimes Implantable cardioverter-defibrillator (ICD) or Cardiac resynchronization therapy (CRT) are not routinely used and reserved for severe LV dysfunction or high risk cases. [24]
Bromocriptine is being studied as another potential treatment option, due to its interference with the prolactin pathway which may cause PPCM for some people. [10] Several studies have shown bromocriptine in addition to standard heart failure medications produce better outcomes in terms of both overall recovery and rate of recovery. [11]
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. [22] [25] [26] In the United States, over 50% of PPCM patients experience complete recovery of heart function (EF 55% or greater). [5] [9] Almost all recovered patients are eventually able to discontinue medications with no resulting relapse and have normal life expectancy. [25]
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. [4] [27] Once fully recovered, if there is no subsequent pregnancy, the possibility of relapse or recurrence of heart failure is minimal. [22] [26]
Subsequent pregnancy should be avoided when left ventricular function has not recovered and the EF is lower than 55%. [2] [28] However, many women who have fully recovered from PPCM have gone on to have successful subsequent pregnancies. [9] 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. [28] The chance of relapse may be even smaller for those with normal contractile reserve as demonstrated by stress echocardiography. [29] [30] In any subsequent pregnancy, careful monitoring is necessary. A stress test or echocardiogram should be complete prior to a subsequent pregnancy. 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.[ citation needed ]
Some factors that are associated with a better prognosis are small left ventricle diastolic dimension, LVEF greater than 30-35% at time of diagnosis, absence of troponin elevation, absence of LV thrombus, non-African American ethnicity. Some factors that indicate a poor prognosis are a QRS greater than 120 milliseconds, a delay in diagnosis, high NYHA class, multiparity, African descent. [7]
Mortality estimates have significant differences depending on the racial group being studied, the geographical location, and the length of follow-up in the study. At 1 year follow-up in the United States, mortality rates range from 4%-11%. [11]
It is estimated that the incidence of PPCM in the United States is between 1 in 1300 to 4000 live births. [2] [27] [31] 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. [13] [27]
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. [13] [31] However, a quarter to a third of PPCM patients are young women who have given birth for the first time. [3] [4] [13] [27] [32] [33] [ excessive citations ]
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. [27] [32]
When looking at the occurrence of PPCM worldwide, the incidence of PPCM is well correlated with maternal mortality rates. This means that in areas with higher maternal mortality, there are also higher rates of PPCM. This suggests that the level of perinatal care may impact the incidence of PPCM. [34]
In short, PPCM can occur in any woman of any racial background, at any age during reproductive years, and in any pregnancy. [28]
Cardiomyopathy is a group of primary diseases of the heart muscle. Early on there may be few or no symptoms. As the disease worsens, shortness of breath, feeling tired, and swelling of the legs may occur, due to the onset of heart failure. An irregular heart beat and fainting may occur. Those affected are at an increased risk of sudden cardiac death.
Heart failure (HF), also known as congestive heart failure (CHF), is a syndrome, a group of signs and symptoms, caused by an impairment of the heart's blood pumping function. Symptoms typically include shortness of breath, excessive fatigue, and leg swelling. The shortness of breath may occur with exertion or while lying down, and may wake people up during the night. Chest pain, including angina, is not usually caused by heart failure, but may occur if the heart failure was caused by a heart attack. The severity of the heart failure is mainly decided based on ejection fraction and also measured by the severity of symptoms. Other conditions that may have symptoms similar to heart failure include obesity, kidney failure, liver disease, anemia, and thyroid disease.
Myocarditis, also known as inflammatory cardiomyopathy, is an acquired cardiomyopathy due to inflammation of the heart muscle. Symptoms can include shortness of breath, chest pain, decreased ability to exercise, and an irregular heartbeat. The duration of problems can vary from hours to months. Complications may include heart failure due to dilated cardiomyopathy or cardiac arrest.
Hypertrophic cardiomyopathy is a condition in which muscle tissues of the heart become thickened without an obvious cause. The parts of the heart most commonly affected are the interventricular septum and the ventricles. This results in the heart being less able to pump blood effectively and also may cause electrical conduction problems. Specifically, within the bundle branches that conduct impulses through the interventricular septum and into the Purkinje fibers, as these are responsible for the depolarization of contractile cells of both ventricles.
Dilated cardiomyopathy (DCM) is a condition in which the heart becomes enlarged and cannot pump blood effectively. Symptoms vary from none to feeling tired, leg swelling, and shortness of breath. It may also result in chest pain or fainting. Complications can include heart failure, heart valve disease, or an irregular heartbeat.
Cardiogenic shock is a medical emergency resulting from inadequate blood flow to the body's organs due to the dysfunction of the heart. Signs of inadequate blood flow include low urine production, cool arms and legs, and decreased level of consciousness. People may also have a severely low blood pressure and heart rate.
In cardiology, ventricular remodeling refers to changes in the size, shape, structure, and function of the heart. This can happen as a result of exercise or after injury to the heart muscle. The injury is typically due to acute myocardial infarction, but may be from a number of causes that result in increased pressure or volume, causing pressure overload or volume overload on the heart. Chronic hypertension, congenital heart disease with intracardiac shunting, and valvular heart disease may also lead to remodeling. After the insult occurs, a series of histopathological and structural changes occur in the left ventricular myocardium that lead to progressive decline in left ventricular performance. Ultimately, ventricular remodeling may result in diminished contractile (systolic) function and reduced stroke volume.
Complications of pregnancy are health problems that are related to, or arise during pregnancy. Complications that occur primarily during childbirth are termed obstetric labor complications, and problems that occur primarily after childbirth are termed puerperal disorders. While some complications improve or are fully resolved after pregnancy, some may lead to lasting effects, morbidity, or in the most severe cases, maternal or fetal mortality.
Ventricular hypertrophy (VH) is thickening of the walls of a ventricle of the heart. Although left ventricular hypertrophy (LVH) is more common, right ventricular hypertrophy (RVH), as well as concurrent hypertrophy of both ventricles can also occur.
Cardiomegaly is a medical condition in which the heart becomes enlarged. It is more commonly referred to simply as "having an enlarged heart". It is usually the result of underlying conditions that make the heart work harder, such as obesity, heart valve disease, high blood pressure (hypertension), and coronary artery disease. Cardiomyopathy is also associated with cardiomegaly.
Tachycardia-induced cardiomyopathy (TIC) is a disease where prolonged tachycardia or arrhythmia causes an impairment of the myocardium, which can result in heart failure. People with TIC may have symptoms associated with heart failure and/or symptoms related to the tachycardia or arrhythmia. Though atrial fibrillation is the most common cause of TIC, several tachycardias and arrhythmias have been associated with the disease.
Restrictive cardiomyopathy (RCM) is a form of cardiomyopathy in which the walls of the heart are rigid. Thus the heart is restricted from stretching and filling with blood properly. It is the least common of the three original subtypes of cardiomyopathy: hypertrophic, dilated, and restrictive.
Takotsubo cardiomyopathy or takotsubo syndrome (TTS), also known as stress cardiomyopathy, is a type of non-ischemic cardiomyopathy in which there is a sudden temporary weakening of the muscular portion of the heart. It usually appears after a significant stressor, either physical or emotional; when caused by the latter, the condition is sometimes called broken heart syndrome. Examples of physical stressors that can cause TTS are sepsis, shock, subarachnoid hemorrhage, and pheochromocytoma. Emotional stressors include bereavement, divorce, or the loss of a job. Reviews suggest that of patients diagnosed with the condition, about 70–80% recently experienced a major stressor, including 41–50% with a physical stressor and 26–30% with an emotional stressor. TTS can also appear in patients who have not experienced major stressors.
Tricuspid regurgitation (TR), also called tricuspid insufficiency, is a type of valvular heart disease in which the tricuspid valve of the heart, located between the right atrium and right ventricle, does not close completely when the right ventricle contracts (systole). TR allows the blood to flow backwards from the right ventricle to the right atrium, which increases the volume and pressure of the blood both in the right atrium and the right ventricle, which may increase central venous volume and pressure if the backward flow is sufficiently severe.
Acute decompensated heart failure (ADHF) is a sudden worsening of the signs and symptoms of heart failure, which typically includes difficulty breathing (dyspnea), leg or feet swelling, and fatigue. ADHF is a common and potentially serious cause of acute respiratory distress. The condition is caused by severe congestion of multiple organs by fluid that is inadequately circulated by the failing heart. An attack of decompensation can be caused by underlying medical illness, such as myocardial infarction, an abnormal heart rhythm, infection, or thyroid disease.
A postpartum disorder or puerperal disorder is a disease or condition which presents primarily during the days and weeks after childbirth called the postpartum period. The postpartum period can be divided into three distinct stages: the initial or acute phase, 6–12 hours after childbirth; subacute postpartum period, which lasts two to six weeks, and the delayed postpartum period, which can last up to six months. In the subacute postpartum period, 87% to 94% of women report at least one health problem. Long term health problems are reported by 31% of women.
Management of heart failure requires a multimodal approach. It involves a combination of lifestyle modifications, medications, and possibly the use of devices or surgery.
Familial amyloid cardiomyopathy (FAC), or transthyretin amyloid cardiomyopathy (ATTR-CM) results from the aggregation and deposition of mutant and wild-type transthyretin (TTR) protein in the heart. TTR is usually circulated as a homo-tetramer—a protein made up of four identical subunits—however, in FAC populations, TTR dissociates from this typical form and misassembles into amyloid fibrils which are insoluble and resistant to degradation. Due to this resistance to degradation, when amyloid fibrils accumulate in the heart's walls, specifically the left ventricle, rigidity prevents the heart from properly relaxing and refilling with blood: this is called diastolic dysfunction which can ultimately lead to heart failure.
Heart failure with preserved ejection fraction (HFpEF) is a form of heart failure in which the ejection fraction – the percentage of the volume of blood ejected from the left ventricle with each heartbeat divided by the volume of blood when the left ventricle is maximally filled – is normal, defined as greater than 50%; this may be measured by echocardiography or cardiac catheterization. Approximately half of people with heart failure have preserved ejection fraction, while the other half have a reduction in ejection fraction, called heart failure with reduced ejection fraction (HFrEF).
Prof. Dr. Johann Bauersachs is a German internist, cardiologist, and full professor at the Hannover Medical School. He is widely recognized for his scholarly contributions to the domains of acute coronary syndrome, left ventricular repair and remodelling following ischemia, and acute and chronic heart failure.
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