Peripartum cardiomyopathy (PPCM), sometimes referred to as Post-partum cardiomyopathy, 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. 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. Echocardiography 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 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. 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. 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 six 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. 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.
Signs and symptoms 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. 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. 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. 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. 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). Peripartum cardiomyopathy is now a leading cause of maternal death in many parts of the United States and around the world. Approximately 60% of cases of cardiogenic shock during pregnancy or in the early postpartum period are caused by peripartum cardiomyopathy. 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.
Diagnosis The following screening tool may be useful to patients and medical professionals in determining the need to take further action to diagnose symptoms:
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 (which are usually elevated), EKG, echocardiogram, cardiac MRI, and cardiac catheterization. There is no specific test to get a diagnosis of PPCM. Genetic testing is indicated in mothers who have peripartum cardiomyopathy to possibly identify pathogenic genetic variants that predispose one to heart failure in pregnancy and allow other family members to be screened if such pathogenic variants exist.
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