Peripartum cardiomyopathy

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. Ultrasound An echocardiography (or an ultrasound of the heart) is the initial diagnostic test of choice in evaluating peripartum cardiomyopathy. The echocardiogram shows reduced systolic function and often left ventricular dilation. File:UOTW 14 - Ultrasound of the Week 2.webm|Ultrasound of peripartum cardiomyopathy File:UOTW 14 - Ultrasound of the Week 3.jpg|Ultrasound of peripartum cardiomyopathy ==Treatment==

Early detection and treatment are associated with higher rates of recovery and decreased morbidity and mortality. 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. Bromocriptine is being studied as another potential treatment option, due to its interference with the prolactin pathway which may cause PPCM for some people. 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. ==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). 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. 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. 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%. ==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. 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. ==References==