Amniotic fluid embolism

By Genevieve Richards 5 comments

Discover the facts about the little-known killer amniotic fluid embolism Medicine's best-kept secret is a mysterious disease known as amniotic fluid embolism (AFE). It comes out of nowhere to kill mothers and their babies, usually during childbirth. According to medical literature, as many as half of the women it affects die within an hour of the onset of symptoms and many of the rest die the same day or suffer permanent neurological impairment. What is AFE? AFE is a rare obstetric emergency in which amniotic fluid, foetal cells, hair or other debris enters the mother's blood stream via the placenta and triggers an allergic reaction. This allergic reaction then results in cardiorespiratory (heart and lung) collapse. The condition is so rare that most doctors will never encounter it in their professional careers and as a result the exact process is poorly understood. However, it is believed that once the fluid and foetal cells enter the maternal pulmonary circulation a two-phase process occurs: First phase: The patient experiences acute shortness of breath and hypertension (extremely high blood pressure). This rapidly progresses to cardiopulmonary arrest as the chambers of the heart fail to dilate and there is a reduction of oxygen to the heart and lungs. Not long after this stage the patient will lapse into a coma. Second phase: Although many women do not survive beyond the first stage, about 40 per cent of the initial survivors will pass onto the second phase. This is known as the hemorrhagic phase and may be accompanied by severe shivering, coughing, vomiting and the sensation of a bad taste in the mouth. This is also accompanied by excessive bleeding as the blood loses its ability to clot. There is also foetal distress present. As hypoxia (the reduction of the oxygen supply) progresses the foetal heart rate may drop to less than 110 beats per minute (bpm). What causes AFE? It is mostly agreed that this condition results from amniotic fluid entering the uterine veins and in order for this to occur there are three prerequisites:Ruptured membranes (a term used to define the rupture of the amniotic sac)Ruptured uterine or cervical veinsA pressure gradient from uterus to vein Although exposure to foetal tissue is common and thus finding foetal tissue within the maternal circulation is not significant, in a small percentage of women this exposure leads to a complex chain of events resulting in collapse and death.

What is the survival rate? Shockingly, maternal mortality is high with figures of around 80 per cent, although the neo-natal survival rate is much better at 70 per cent. Statistics also show that 50 per cent of patients with AFE die within the first hour of the onset of symptoms and a high percentage of women who do survive have permanent neurological impairment. There is, however, no evidence to indicate that survivors of AFE are at risk for amniotic fluid embolism in future pregnancies and the condition is considered to be unpredictable and unpreventable. The cause is still unknown. There are, however, plans for a confidential register of all cases of AFE to be established for the UK. The aim is to identify any differences or common factors between survivors and fatalities with the hope that it may help to reduce the number of maternal deaths from this condition.

Amniotic fluid embolism (AFE) is a rare and incompletely understood obstetric emergency in which amniotic fluid, fetal cells, hair, or other debris enters the mother's blood stream via the placental bed of the uterus and trigger an allergic reaction. This reaction then results in cardiorespiratory (heart and lung) collapse and coagulopathy. It was first formally characterized in 1941.[2] While it is estimated to be the fifth most common cause of maternal mortality in the world, there has been discrepancy with respect to the incidence and mortality of amniotic fluid embolism. One likely explanation for this inconsistency is the lack of sensitive and specific diagnostic studies to definitively identify cases of amniotic fluid embolism, leading to both over- and underreporting.[3]

Presentation
The condition is so rare (between 1 in 8000 and 1 in 80,000 deliveries, although more recent studies show 1 in 20,464 deliveries for a more precise number)[4] that most doctors will never encounter it in their professional careers, and as a result the exact process is poorly understood. It is believed however that once the fluid and fetal cells enter the maternal pulmonary circulation in general terms there will be profound respiratory failure with deep cyanosis and cardiovascular shock followed by convulsions and profound coma, however this does occur in two phases detailed below:

First phase
The patient experiences acute shortness of breath and hypotension. This rapidly progresses to cardiac arrest leading to a reduction of perfusion to the heart and lungs. Not long after this stage the patient will lapse into a coma. While previously believed to have a maternal mortality rate of 60-80%, more recently it has been reported at 26.4%.[5]

Second phase
Although many women do not survive beyond the first stage, about 40 percent of the initial survivors will pass onto the second phase. This is known as the hemorrhagic phase and may be accompanied by severe shivering, coughing, vomiting, and the sensation of a bad taste in the mouth. This is also accompanied by excessive bleeding as the blood loses its ability to clot. Collapse of the cardiovascular system leads to fetal distress and death unless the child is delivered swiftly.

Amniotic fluid embolism (AFE) usually occurs during labor but has occurred during abortion, after abdominal trauma, and during amnioinfusion. A woman in the late stages of labor becomes acutely dyspneic with hypotension; she may experience seizures quickly followed by cardiac arrest. Massive DIC-associated hemorrhage follows and then death. Most patients die within an hour of onset. Currently no definitive diagnostic test exists. The United States and United Kingdom AFE registries recommend the following 4 criteria, all of which must be present to make the diagnosis of AFE.[7, 1, 9] 1. 2. 3. 4. Acute hypotension or cardiac arrest Acute hypoxia Coagulopathy or severe hemorrhage in the absence of other explanations All of these occurring during labor, cesarean delivery, dilation and evacuation, or within 30 minutes postpartum with no other explanation of findings